F-1

As filed with to the Securities and Exchange Commission on June 23, 2020

  

UNITED STATES
SECURITIES AND EXCHANGE COMMISSION
Washington, D.C. 20549

FORM F-1
REGISTRATION STATEMENT
UNDER
THE SECURITIES ACT OF 1933

AMRYT PHARMA PLC
(Exact name of Registrant as specified in its charter)

Not Applicable
(Translation of Registrant’s name into English)

England and Wales
(State or other jurisdiction of
incorporation or organization) 2834
(Primary Standard Industrial
Classification Code Number) Not Applicable
(I.R.S. Employer
Identification Number)

Dept 920a 196 High Road, Wood Green,
London, United Kingdom, N22 8HH
Tel: +44 (0)20 3026 7257
(Address, including zip code, and telephone number, including area code, of Registrant’s principal executive offices)

Puglisi & Associates
850 Library Avenue, Suite 204
Newark, Delaware 19711
Tel: (302) 738-6680

(Name, address, including zip code, and telephone number, including area code, of agent for service)

Copies to:

Boris Dolgonos
Andrew L. Fabens
Gibson, Dunn & Crutcher LLP
200 Park Avenue
New York, NY 10166
Tel: (212) 351-4000 Christopher Haynes
Gibson, Dunn & Crutcher UK LLP
Telephone House 2-4
Temple Avenue
London, EC4Y 0HB
United Kingdom
Tel: +44 (0) 20-7071 4000

Approximate date of commencement of proposed sale to the public:
As soon as practicable after the effective date of this Registration Statement.

If any of the securities being registered on this Form are to be offered on a delayed or continuous basis pursuant to Rule 415 under the Securities Act of 1933, check the following box. ☒

If this Form is filed to register additional securities for an offering pursuant to Rule 462(b) under the Securities Act, check the following box and list the Securities Act registration statement number of the earlier effective registration statement for the same offering.  ☐

If this Form is a post-effective amendment filed pursuant to Rule 462(c) under the Securities Act, check the following box and list the Securities Act registration statement number of the earlier effective registration statement for the same offering.  ☐

If this Form is a post-effective amendment filed pursuant to Rule 462(d) under the Securities Act, check the following box and list the Securities Act registration statement number of the earlier effective registration statement for the same offering.  ☐

Indicate by check mark whether the registrant is an emerging growth company as defined in Rule 405 of the Securities Act of 1933.

Emerging Growth Company ☒

If an emerging growth company that prepares its financial statements in accordance with U.S. GAAP, indicate by check mark if the registrant has elected not to use the extended transition period for complying with any new or revised financial accounting standards† provided pursuant to Section 7(a)(2)(B) of the Securities Act.  ☐

† The term “new or revised financial accounting standard” refers to any update issued by the Financial Accounting Standards Board to its Accounting Standards Codification after April 5, 2012.

CALCULATION OF REGISTRATION FEE

Title of Each Class of Securities To Be Registered	Amount to be Registered(1)	Proposed Maximum Aggregate Offering Price Per Share(2)	Proposed Maximum Aggregate Offering Price(1)	Amount of Registration Fee(2)
Ordinary shares, nominal value £0.06 per share(3)	162,876,633	$1.8765	$305,638,002	$39,672

(1)
The registrant is filing this registration statement in part in respect of its obligations under a Registration Rights Agreement, dated September 25, 2019, concerning an aggregate of 67,207,159 ordinary shares held by investors identified herein, which includes 12,966,520 shares issuable upon the exercise of zero cost warrants by such investors. The registrant is also registering hereby an aggregate of 54,113,792 ordinary shares held by other shareholders identified herein, as well as 4,229,753 shares issuable upon the exercise of zero cost warrants by investors identified herein and 37,325,929 shares issuable upon the exercise of convertible notes by investors identified herein.

(2)
Calculated pursuant to Rule 457(c) based on the average of the high and low trading prices on AIM, a market operated by the London Stock Exchange plc, of the registrant’s ordinary shares on June 19, 2020, converted into U.S. dollars at the exchange rate in effect on such date.

(3)
ADSs issuable upon deposit of the ordinary shares registered hereby will be registered pursuant to an amendment to a registration statement on Form F-6 (registration number 333-233844). Each ADS represents five ordinary shares.

The Registrant hereby amends this Registration Statement on such date or dates as may be necessary to delay its effective date until the Registrant shall file a further amendment which specifically states that this Registration Statement shall thereafter become effective in accordance with Section 8(a) of the Securities Act of 1933, as amended, or until the Registration Statement shall become effective on such date as the Commission, acting pursuant to said Section 8(a), may determine.

The information in this preliminary prospectus is not complete and may be changed. We may not sell these securities until the registration statement filed with the Securities and Exchange Commission is effective. This preliminary prospectus is not an offer to sell these securities and it is not soliciting an offer to buy these securities in any jurisdiction where the offer or sale is not permitted.

SUBJECT TO COMPLETION, DATED JUNE 23, 2020

PRELIMINARY PROSPECTUS

162,876,633 Ordinary Shares

 

Amryt Pharma plc

Represented by Approximately 32,575,327 American Depositary Shares

We have applied to list American Depositary Shares (“ADSs”), each representing five ordinary shares of Amryt Pharma plc, on the Nasdaq Global Select Market (“Nasdaq”) under the symbol “AMYT.” The ADSs are expected to begin trading on Nasdaq on    , 2020. Our ordinary shares trade on AIM, a market operated by the London Stock Exchange, under the symbol “AMYT” and on the Euronext Growth Exchange (“EGE”), a market regulated by Euronext Growth Market Dublin, under the symbol “AYP.” On June 19, 2020, the last reported sale price of our ordinary shares on AIM was £1.51 per share, which is equivalent to $9.38 per ADS, based on an exchange rate of £1.00 to $1.2427 as of June 19, 2020 and an ADS-to-share ratio of 1 to 5.

We have appointed Citibank, N.A. to act as the depositary for the ADSs representing our ordinary shares, including the Registered Shares, as defined below. Of the shares registered hereby, 34,034,130 shares are represented by 6,806,826 ADSs, and the remaining shares are held in the form of ordinary shares. Upon the effectiveness of the registration statement of which this prospectus forms a part, holders of ordinary shares that are not represented by ADSs will be able to deposit such shares with the depositary in exchange for ADSs representing such shares at the ratio of five ordinary shares per ADS. ADSs representing the shares registered hereby will be freely tradeable on the effective date of the registration statement of which this prospectus forms a part.

We are filing the registration statement of which this prospectus forms a part in respect of our obligations under a Registration Rights Agreement, dated September 25, 2019, concerning an aggregate of 67,207,159 ordinary shares held by investors identified herein, which includes 12,966,520 shares issuable upon the exercise of zero cost warrants by such investors. We are also registering hereby and an aggregate of 54,113,792 ordinary shares held by other shareholders identified herein, as well as 4,229,753 shares issuable upon the exercise of zero cost warrants by investors identified herein and 37,325,929 shares issuable upon the exercise of convertible notes by investors identified herein. Holders of all such instruments are identified in this prospectus as the “Registered Holders” and the aggregate 162,876,633 shares registered hereby as the Registered Shares. Any “Registered Shares” offered and sold in the United States by the Registered Holders will be in the form of ADSs. The Registered Holders are also permitted to sell shares not represented by ADSs in private transactions, including on AIM and EGE, which resales are not covered by this prospectus.

Unlike an initial public offering, any disposition by the Registered Holders of the Registered Shares represented by ADSs is not being underwritten by any investment bank. We expect that the opening public price of our ADSs on Nasdaq will be determined by reference to the most recent trading price of our ordinary shares on AIM, as adjusted for the currency exchange rate and an ADS-to-share ratio of 1 to 5. Thereafter, trades of our ADSs will be made through brokerage transactions on Nasdaq at prevailing market prices. The Registered Holders may, or may not, elect to dispose of Registered Shares represented by ADSs as and to the extent that they may individually determine. See the section entitled “Plan of Distribution.” We will not receive proceeds from any disposition of Registered Shares in the form of ADSs by Registered Holders.

We are an “emerging growth company” and a “foreign private issuer” as defined under the U.S. federal securities laws, and, as such, have elected to comply with certain reduced public company reporting requirements for this prospectus and future filings. See “Prospectus Summary—Implications of Being an Emerging Growth Company and a Foreign Private Issuer” for additional information.

Investing in our ADSs involves a high degree of risk. See “Risk Factors” beginning on page 14 of this prospectus.

None of the Securities and Exchange Commission, any state securities commission, nor any foreign securities commission has approved or disapproved of these securities or determined if this prospectus is truthful or complete. Any representation to the contrary is a criminal offense.

Prospectus dated       , 2020.

Table of Contents

	Page
PRESENTATION OF FINANCIAL INFORMATION	ii
NON-IFRS FINANCIAL MEASURE	ii
MARKET AND INDUSTRY DATA	ii
TRADEMARKS	iii
PROSPECTUS SUMMARY	1
RISK FACTORS	14
SPECIAL NOTE REGARDING FORWARD-LOOKING STATEMENTS	59
USE OF PROCEEDS	61
DIVIDEND POLICY	62
CAPITALIZATION	63
SELECTED CONSOLIDATED FINANCIAL DATA	64
PRO FORMA FINANCIAL INFORMATION	67
MANAGEMENT’S DISCUSSION AND ANALYSIS OF FINANCIAL CONDITION AND RESULTS OF OPERATIONS	70
BUSINESS	91
MANAGEMENT	142
RELATED PARTY TRANSACTIONS	152
PRINCIPAL AND REGISTERED HOLDERS	159
DESCRIPTION OF SHARE CAPITAL AND CERTAIN CORPORATE MATTERS	161
DESCRIPTION OF AMERICAN DEPOSITARY SHARES	179
SHARES AND AMERICAN DEPOSITARY SHARES ELIGIBLE FOR FUTURE SALE	189
INCOME TAX CONSIDERATIONS	191
PLAN OF DISTRIBUTION	199
EXPENSES OF THIS OFFERING	202
LEGAL MATTERS	203
EXPERTS	203
CHANGES IN REGISTRANT’S CERTIFYING ACCOUNTANT	203
ENFORCEMENT OF CIVIL LIABILITIES	204
WHERE YOU CAN FIND MORE INFORMATION	206
INDEX TO FINANCIAL STATEMENTS	F-1

We are responsible for the information contained in this prospectus and any free writing prospectus that we may prepare or authorize. We have not authorized anyone to provide you with different or additional information, and we do not take any responsibility for any other information that others may give you. We are not making an offer to sell ADSs representing our shares in any jurisdiction where the offer or sale thereof is not permitted. You should not assume that the information contained in this prospectus is accurate as of any date other than the date on the front cover of this prospectus, regardless of the time of delivery of this prospectus or the sale of any ADSs representing our shares.

For investors outside the United States: Neither we nor the Registered Holders have done anything that would permit this offering or possession or distribution of this prospectus in any jurisdiction other than the United States where action for that purpose is required. Persons outside the United States who come into possession of this prospectus must inform themselves about, and observe any restrictions relating to, the offering of the ADSs and the distribution of this prospectus outside the United States.

We are organized under the laws of England and Wales. Under the rules of the U.S. Securities and Exchange Commission, we are currently eligible for treatment as a “foreign private issuer.” As a foreign private issuer, we will not be required to file periodic reports and financial statements with the U.S. Securities and Exchange Commission as frequently or as promptly as domestic registrants whose securities are registered under the Securities Exchange Act of 1934, as amended, or the Exchange Act.

i

PRESENTATION OF FINANCIAL INFORMATION

On September 24, 2019, we completed the acquisition (“Acquisition”) of Aegerion Pharmaceuticals, Inc. (“Aegerion”), pursuant to the Aegerion Plan of Reorganization (“Plan of Reorganization”). Under the Plan of Reorganization, we acquired Aegerion upon its emergence from bankruptcy in an exchange for our ordinary shares.

This prospectus includes the following historical financial information:

• the audited consolidated financial statements of Amryt as of and for the years ended December 31, 2019 and December 31, 2018, prepared in accordance with International Financial Reporting Standards (“IFRS”), as issued by the International Accounting Standards Board (“IASB”);

• the audited financial statements of Aegerion as of and for the years ended December 31, 2018 and December 31, 2017 prepared in accordance with generally accepted accounting principles in the United States (“U.S. GAAP”); and

• the unaudited interim financial statements of Aegerion as of June 30, 2019, prepared in accordance with U.S. GAAP.

This prospectus also presents an unaudited pro forma consolidated statement of income for the year ended December 31, 2019, which gives effect to the Acquisition as if it had occurred on January 1, 2019. We financed the Acquisition through a combination of debt and equity financings, and the pro forma consolidated statement of income also gives effect to those financing transactions as though they had been completed on January 1, 2019. For further details regarding the pro forma consolidated statement of income, please see “Pro Forma Financial Information.”

All references in this prospectus to “€” mean Euros, all references to “£” mean pound sterling and all references to “$” mean U.S. dollars.

NON-IFRS FINANCIAL MEASURE

In this prospectus, we present Adjusted EBITDA, which is a financial measure that is not recognized by IFRS, does not have a standardized meaning prescribed by IFRS and is therefore unlikely to be comparable to similar measures presented by other companies. In addition, Adjusted EBITDA may not be permitted to appear on the face of IFRS-compliant financial statements or notes thereto. We believe Adjusted EBITDA provides investors with a supplemental measure of our operating performance and highlights trends in our core business that may not otherwise be apparent when relying solely on IFRS measures. We also believe that securities analysts, investors and other interested parties frequently use non-IFRS measures in the evaluation of issuers. Finally, our management uses Adjusted EBITDA to facilitate operating performance comparisons from period to period and to prepare annual operating budgets and forecasts. However, Adjusted EBITDA should not be considered in isolation nor as a substitute for analysis of our financial information reported under IFRS. For a discussion of Adjusted EBITDA and a reconciliation of Adjusted EBITDA to its most closely comparable IFRS measure, see “Summary Consolidated Financial Data.”

MARKET AND INDUSTRY DATA

This prospectus contains estimates, projections and other information concerning our industry, our business and the markets for our products and product candidates. Information that is based on estimates, forecasts, projections, market research or similar methodologies is inherently subject to uncertainties, and actual events or circumstances may differ materially from events and circumstances that are assumed in this information. Unless otherwise expressly stated, we obtained this industry, business, market and other data from our own internal estimates and research as well as from reports, research surveys, studies and similar data prepared by market research firms and other third parties, industry, medical and general publications, government data and similar sources. Management estimates are derived from publicly available information, our knowledge of our industry and assumptions based on such information and knowledge, which we believe to be reasonable.

In addition, assumptions and estimates of our and our industry’s future performance are necessarily subject to a high degree of uncertainty and risk due to a variety of factors, including those described in “Risk Factors.” These and other factors could cause our future performance to differ materially from our assumptions and estimates. See “Special Note Regarding Forward-Looking Statements.”

ii

TRADEMARKS

The Amryt logo, MYALEPT^®, MYALEPTA^®, JUXTAPID^®, LOJUXTA^®, EPISALVAN^®, FILSUVEZ^® and other trademarks or service marks of Amryt appearing in this prospectus are the property of Amryt. This prospectus includes trademarks, tradenames and service marks, certain of which belong to us and others that are the property of other organizations. Solely for convenience, trademarks, tradenames and service marks referred to in this prospectus appear without the ®, TM and SM symbols, but the absence of those symbols is not intended to indicate, in any way, that we will not assert our rights or that the applicable owner will not assert its rights to these trademarks, tradenames and service marks to the fullest extent under applicable law. We do not intend our use or display of other parties’ trademarks, trade names or service marks to imply, and such use or display should not be construed to imply, a relationship with, or endorsement or sponsorship of us by, these other parties.

iii

PROSPECTUS SUMMARY

This summary highlights information contained elsewhere in this prospectus and does not contain all of the information you should consider in making your investment decision. Before investing in the ADSs, you should carefully read this entire prospectus, including the information set forth under the heading “Risk Factors.” Unless the context otherwise requires, references in this prospectus to the “Company,” “Amryt,” “we,” “us” and “our” refer to Amryt Pharma plc and its subsidiaries. Unless the context otherwise requires, such references include Aegerion Pharmaceuticals, Inc., which we acquired on September 24, 2019, as well as its subsidiaries.

Overview

We are a global, commercial-stage biopharmaceutical company dedicated to commercializing and developing novel therapeutics to treat patients suffering from serious and life-threatening rare diseases. Our diversified portfolio is comprised of two commercial rare disease products, as well as a development-stage pipeline focused on rare skin diseases. Our two commercial products, lomitapide for the treatment of homozygous familial hypercholesterolemia (“HoFH”), and metreleptin for the treatment of generalized lipodystrophy (“GL”) and partial lipodystrophy (“PL”), have each been sold globally through our commercial infrastructure for over six years. We are developing our lead product candidate, AP101, for the treatment of cutaneous manifestations of severe Epidermolysis Bullosa (“EB”), a rare and devastating genetic skin disease for which there is currently no approved therapy. AP101 is currently in a pivotal Phase 3 trial for which we expect to report data in the second half of 2020. If AP101 is approved for this indication, we intend to market it under the name FILSUVEZ. Our next product candidate, AP103, is currently in preclinical development for the treatment of patients with Recessive Dystrophic EB (“RDEB”), a subset of EB. AP103 is the first gene therapy product candidate based on our novel polymer-based topical gene therapy delivery platform, which also has potential use for the treatment of other rare genetic diseases. We intend to initiate clinical development of AP103 in the second half of 2021. We have a proven track record of obtaining rare disease assets, either through acquisition or in-license, and we intend to continue building our portfolio of rare disease programs with the goal of bringing effective treatments to patients in need.

The following table summarizes key information about our product portfolio and clinical development pipeline. We have retained worldwide development and commercial rights to all of our programs, excluding Japan for lomitapide and Japan, South Korea and Taiwan for metreleptin.

 

*
Upcoming clinical milestones are subject to the impact of COVID-19 on our business.

(1)
We are conducting a Phase 3 study of HoFH in children and adolescents in Europe, Middle East and Africa (“EMEA”) as part of our European Medicines Agency (“EMA”) post-approval commitments.

1

(2)
The familial chylomicronemia syndrome Phase 2 trial is an open-label investigator-led study.

(3)
The dotted line segment indicates we have not yet commenced any clinical trials in the United States for metreleptin for the treatment of PL.

(4)
AP101 was approved in 2016 by the EMA for the treatment of partial thickness wounds in adults, but has not been commercially launched.

(5)
The dotted line segment indicates we have not yet commenced any clinical trials for radiation-induced dermatitis. This planned radiation-induced dermatitis Phase 2 trial is an investigator-led study.

Lomitapide is an oral therapy approved as an adjunct to a low-fat diet and other lipid-lowering treatments for adults with HoFH. It is marketed in the United States under the trade name JUXTAPID and in the European Union under the trade name LOJUXTA. HoFH is a rare and serious genetic condition that leads to aggressive and premature heart disease, heart attacks and strokes in patients as young as teenagers. HoFH patients are at a high risk of experiencing life-threatening cardiovascular events as a result of extremely elevated cholesterol levels in the blood and have a substantially reduced life expectancy. HoFH impairs the liver’s ability to remove low density lipoprotein (“LDL”) cholesterol, or “bad” cholesterol, from the blood, which if left untreated can cause aggressive narrowing and blocking of the blood vessels. According to a 2013 European Heart Journal article, the prevalence of HoFH is one person per million. However, according to a 2016 article published in Atherosclerosis, the number may be as high as 6.25 persons per million. Lomitapide is a small molecule microsomal triglyceride transfer protein (“MTP”) inhibitor. MTP exists in both the liver and intestines where it plays a role in the formation of cholesterol-carrying lipoproteins. As a result, inhibition of MTP is an effective cholesterol-lowering therapy in HoFH patients with limited or non-functional LDL receptors.

Metreleptin for injection is approved in the United States under the trade name MYALEPT as an adjunct to diet as replacement therapy to treat the complications of leptin deficiency in patients with congenital or acquired GL. It is approved in the European Union under the trade name MYALEPTA for the treatment of leptin deficiency in patients with congenital or acquired GL and familial or acquired PL for whom standard treatments have failed to achieve adequate metabolic control. GL and PL are rare diseases characterized by loss or lack of adipose tissues (fat cells), resulting in the deficiency of the hormone leptin. GL and PL patients experience severe metabolic abnormalities including severe insulin resistance, diabetes, hypertriglyceridemia and fatty liver disease. We estimate that the prevalence of GL is approximately one person per million and that of PL is approximately three persons per million. Metreleptin is a recombinant human leptin analog that binds to and activates the human leptin receptor. Metreleptin acts to stimulate fatty acid oxidation throughout the body and lower plasma, hepatic and myocellular triglyceride levels.

We intend to continue to evaluate additional expansion opportunities for our products. We are conducting a Phase 3 pediatric study in EMEA for the use of lomitapide in children and adolescents with HoFH, for which we expect to report data in the first half of 2022. We are also exploring the potential use of lomitapide to treat patients with familial chylomicronemia syndrome (“FCS”), which is a severe, rare genetic lipid disease characterized by extremely elevated levels of triglycerides, or hypertriglyceridemia. An investigator-led open-label Phase 2 trial studying lomitapide in patients with FCS is ongoing and we expect to report data in the second half of 2020. Upon successful completion of this Phase 2 study, we intend to discuss these results with the Food and Drug Administration (“FDA”) and the EMA in the context of agreeing on the design of a potential pivotal trial in FCS. We also intend to discuss with the FDA in the third quarter of 2020 the potential for label expansion of metreleptin in the United States to include the treatment of PL. We expect this will require a pivotal Phase 3 study in PL patients, either as a post-approval commitment or prior to potential approval.

Our lead development candidate, AP101, is being developed as a potential treatment for the cutaneous manifestations of severe EB, a rare and devastating genetic skin disease affecting young children and adults for which there is currently no approved treatment. EB is a group of diseases of the skin, mucous membranes and internal epithelial linings characterized by extreme skin fragility that blisters and tears from minor friction or trauma. Patients with severe forms of EB, including Dystrophic EB (“DEB”) and Junctional EB (“JEB”), suffer from severe and chronic blistering, ulceration, scarring, mutilating scarring of the hands and feet, joint contractures, strictures of the esophagus and mucous membranes, a high risk of developing aggressive squamous cell carcinomas, infections and risk of premature death. According to a 2013 article in the Journal of Investigative Dermatology, it is estimated that the incidence of EB is approximately one in 20,000, which implies that there are as many as 30,000 affected individuals in the United States and over 500,000 worldwide. We are conducting a pivotal Phase 3 clinical trial of AP101 in severe EB, including DEB and JEB. In January 2019, we reported that the independent Data and Safety Monitoring Board (“DSMB”) performed a pre-specified unblinded interim efficacy analysis and recommended the continuation of the study with an increase in enrollment from 182

2

to 230 evaluable patients to maintain 80% conditional statistical power. As a result of the COVID-19 pandemic which has had a material impact on recruitment into clinical trials globally, and given that the EASE trial was already close to full enrollment, we have taken advice from an independent expert and concluded that the statistical impact of further patient recruitment would most likely be negligible. We therefore decided to close the EASE trial to further enrollment in April 2020 and expect to report top line results from the EASE trial in the second half of 2020.

A representative photograph of the devastating impact of EB on a patient is shown below:

 

AP101 has been granted Pediatric Rare Disease Designation by the FDA. If approved by the FDA, we are eligible to apply for a Priority Review Voucher (“PRV”) that we can use, sell or transfer. We are also supporting an investigator-led Phase 2 study of AP101 for the treatment of severe radiation-induced dermatitis. This trial is expected to commence in the second half of 2020, with data expected in the first half of 2021.

We also have a novel polymer-based topical gene therapy delivery platform for potential use in the treatment of rare genetic diseases. Our first product candidate utilizing this platform, AP103, is currently in preclinical development for the treatment of RDEB, a subset of severe EB. We intend to initiate clinical development for AP103 in the second half of 2021.

We sell lomitapide and metreleptin in the Americas, Europe and the Middle East through our existing rare disease commercial infrastructure. Our commercial expertise includes market access, marketing, sales managers and sales representatives and is supported by our experienced medical affairs team with medical science liaisons, patient advocates and dieticians in the field. We also have a network of third-party distributors in other key markets throughout the world.

Our company was co-founded in 2015 by our Chief Executive Officer, Joseph Wiley, and our Chief Financial Officer, Rory Nealon. Our shares have been traded on AIM and EGE since April 2016. We are led by a management team and Board of Directors with deep experience at leading biotech companies, large pharmaceutical companies and academic institutions. Our company headquarters are in Dublin, Ireland, and we have nearly 170 employees throughout the world. We believe the expertise of our leadership team and the strength of our relationships within the industry and medical community are critical to our strategy as we commercialize our existing products, progress our development pipeline and explore new business development opportunities.

3

Our Strengths

We believe our key competitive strengths include the following:

• Revenue-generating commercial products. We currently generate revenue, including royalties, from global sales of lomitapide and metreleptin. This revenue stream provides us with financial flexibility to fund the continued development and potential commercialization of our existing development candidates as well as the potential acquisition or in-license of additional rare disease products and late-stage product candidates. We have retained worldwide development and commercial rights to all of our programs, excluding Japan for lomitapide, where we receive royalties, and Japan, South Korea and Taiwan for metreleptin.

• Late-stage clinical program in severe EB. We are conducting a global pivotal Phase 3 trial of AP101 for the treatment of cutaneous manifestations of severe EB and we expect to report data in the second half of 2020. This Phase 3 trial is the largest EB study conducted to date. Based on our conversations with the FDA and the EMA, we believe that positive results from this trial would allow us to apply for marketing approval for AP101 in both the United States and Europe.

• Existing, scalable global commercial and medical infrastructure. We sell lomitapide and metreleptin in the Americas, Europe and the Middle East through our existing rare disease commercial infrastructure. Our commercial expertise includes market access, marketing, sales managers and sales representatives and is supported by our experienced medical affairs team with medical science liaisons, patient advocates and dieticians in the field. We also leverage our network of third-party distributors in other key markets throughout the world. We believe we will be able to leverage our existing global infrastructure and expertise to efficiently and expeditiously commercialize additional products we may acquire or develop, including our lead product candidate, AP101, if approved.

• Proven track record of building a diversified rare disease product portfolio. We acquired AP101 through the acquisition of Birken AG in 2016, in-licensed LOJUXTA in December 2016, in-licensed our gene therapy platform, including AP103, in March 2018 and acquired metreleptin and the remaining rights to lomitapide through the Acquisition in September 2019.

• Strong patent protection and regulatory exclusivity. We believe our intellectual property portfolio as well as protection afforded by regulatory exclusivity provide us with a substantial competitive advantage in marketing our current products and also protect our development programs. Our lomitapide patent portfolio includes patents that provide protection from 2025 to 2027 in the United States and into 2025 in the European Union, with supplementary protection granted to extend patent protection in major EU countries into 2028. The metreleptin patent portfolio includes patents that provide protection from 2022 to 2027 in the United States and into 2022 in the European Union and orphan exclusivity in the European Union into 2028. The AP101 patent portfolio includes patents that provide protection in both the United States and the European Union into 2025 and 2030 and a further international patent application directed to the clinical formulation and methods of manufacturing and treatment with AP101 which, if granted, would provide worldwide protection into 2039. We have also submitted additional patent applications to further strengthen our intellectual property portfolio.

• Experienced management team comprised of industry leaders in rare diseases. Our management team has extensive expertise in the acquisition, development and commercialization of rare disease assets. We believe that the breadth of experience and successful track record of our management team and our Board, combined with our broad network of established relationships with leaders in the industry and medical community, provide us with strong drug development and commercialization capabilities.

Our Strategy

Our mission is to become a global leader in the treatment of rare diseases through commercializing, developing and acquiring novel rare disease products. To achieve this mission, we are pursuing the following strategies:

• Drive revenue growth for our existing commercial products. We intend to continue to focus on growing the sales of lomitapide and metreleptin in the markets and indications we currently sell them. We also intend to expand the market opportunity by seeking approval for the use of lomitapide to treat pediatric HoFH and for the treatment of FCS and for the use of metreleptin to treat PL in the United States.

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• Complete development and commercialize our lead product candidate, AP101, for the treatment of severe EB. AP101 is currently in a pivotal Phase 3 trial for the treatment of cutaneous manifestations of severe EB and we expect to report data in the second half of 2020. If the trial is successful, we intend to apply for approval of AP101 and commercialize it in the United States and the European Union. If approved by the FDA, we are eligible to apply for a PRV that we can use, sell or transfer.

• Leverage our global commercial and medical infrastructure. We intend to leverage our existing global infrastructure and expertise to commercialize our development-stage pipeline, including our lead product candidate, AP101, if approved, and any rare disease assets we may acquire or in-license in the future.

• Continue developing our gene therapy product candidate, AP103, for the treatment of RDEB. AP103 is currently in preclinical development for the treatment of RDEB. We intend to initiate clinical development in the second half of 2021.

• Continue evaluating opportunities to expand our rare disease product portfolio and pipeline. We believe we are well positioned to continue to opportunistically acquire or in-license rare disease assets that we believe we can efficiently sell through our existing commercial infrastructure.

Risks Associated with Our Business

Our business is subject to a number of risks of which you should be aware before making an investment decision. You should carefully consider all of the information set forth in this prospectus and, in particular, should evaluate the specific factors set forth in “Risk Factors” in deciding whether to invest in our ADSs. Among these important risks are the following:

• As a result of the Acquisition, our future results are likely to differ materially from our historical performance.

• We may not be successful in our efforts to build a pipeline of product candidates and develop additional marketable products.

• We have significant payment obligations under the terms of our long-term debt, $206.6 million of which was outstanding as of December 31, 2019.

• The terms of our debt and any requirements to incur further indebtedness or refinance our indebtedness in the future, including restrictive covenants in certain of the agreements and instruments governing our indebtedness, could have a material adverse effect on our business and results of operations.

• We may be subject to ongoing financial liabilities and other obligations that we assumed upon Aegerion’s emergence from bankruptcy.

• Adverse events involving any of our products and product candidates may lead the FDA, the EMA or other regulatory authorities to delay or deny clearance for our products or result in product recalls that could harm our reputation, business and financial results.

• The Acquisition exposes us, and any future acquisitions we make may expose us, to risks that could adversely affect our business, and we may not achieve the anticipated benefits of acquisitions of businesses or technologies.

• Our products may not gain market acceptance, in which case we may not be able to generate product revenues.

• If we are unable to complete clinical development of AP101, or experience significant delays in doing so, our business could be materially harmed.

• We rely on third parties for distribution services around the world, and a failure to manage these third parties could harm our business.

• It may be challenging or costly for us to obtain, maintain, enforce and defend our intellectual property rights.

• The outbreak of the novel strain of coronavirus disease, COVID-19, could adversely impact our business, including our preclinical studies and clinical trials.

5

Recent Developments

As we continue to actively advance all our preclinical and clinical programs, we remain in close contact with our principal investigators and clinical sites and are assessing the impact of the novel coronavirus disease (“COVID-19”) on our preclinical studies, trials, expected timelines and costs on an ongoing basis. In light of recent developments relating to the COVID-19 global pandemic, the focus of healthcare providers and hospitals on fighting the virus, and consistent with the FDA’s updated industry guidance for conducting clinical trials issued on March 18, 2020, we may experience delays in the enrollment of patients in our ongoing clinical trials. We plan to implement strategies to potentially minimize the impact of the COVID-19 pandemic on our business, preclinical studies and clinical trials, such as exploring the opening of clinical sites in regions that are currently not significantly impacted by the pandemic. We will continue to evaluate the impact of the COVID-19 pandemic on our business and expect to reevaluate the timing of our anticipated clinical milestones as we learn more and the impact of COVID-19 on our business and our industry becomes more clear.

Corporate Information

We were incorporated on July 17, 2019 as a private company limited in England and Wales under the name Amryt Pharma Holdings Limited. We re-registered as a public limited company on September 13, 2019. Our registered office is located at Dept 920a 196 High Road, Wood Green, London, N22 8HH United Kingdom, and our principal office is located at 90 Harcourt Street, Dublin 2, Ireland. Our telephone number in each office is +44 (0)20 3026 7257 and +353 (0)1 518 0200, respectively.

Our website address is https://www.amrytpharma.com. The information contained in, or that can be accessed through, our website is not incorporated by reference into this prospectus. We have included our website address as an inactive textual reference only.

Implications of Being an Emerging Growth Company and a Foreign Private Issuer

As a company with less than $1.07 billion in revenue during our last fiscal year, we are an “emerging growth company” as defined in the Jumpstart Our Business Startups Act of 2012 (“JOBS Act”). As an emerging growth company, we may take advantage of certain reduced disclosure and other requirements that are applicable to public companies that are not emerging growth companies. These provisions include:

• the option to present only two years of audited financial statements and only two years of related management’s discussion and analysis of financial condition and results of operations in this prospectus;

• not being required to have our registered independent public accounting firm attest to management’s assessment of our internal control over financial reporting;

• not being required to comply with any requirement that may be adopted by the Public Company Accounting Oversight Board (“PCAOB”) regarding mandatory audit firm rotation or a supplement to the auditor’s report providing additional information about the audit and the financial statements (i.e., an auditor discussion and analysis);

• not being required to submit certain executive compensation matters to shareholder advisory votes, such as “say-on-pay,” “say-on-frequency” and “say-on-golden parachutes;” and

• not being required to disclose certain executive compensation related items such as the correlation between executive compensation and performance and comparisons of the chief executive officer’s compensation to median employee compensation.

We may choose to take advantage of some but not all of these exemptions. For example, Section 107 of the JOBS Act provides that an emerging growth company can use the extended transition period provided in Section 7(a)(2)(B) of the Securities Act of 1933, as amended (“Securities Act”), for complying with new or revised accounting standards. Given that we currently report and expect to continue to report under IFRS as issued by the IASB, we have irrevocably elected not to avail ourselves of this extended transition period and, as a result, we will adopt new or revised accounting standards on the relevant dates on which adoption of such standards is required by the IASB. We have taken advantage of reduced reporting requirements in this prospectus. Accordingly, the information contained herein may be different from the information you receive from other public companies in which you hold equity securities.

We may take advantage of these exemptions through 2025 or until such earlier time that we are no longer an emerging growth company. We would cease to be an emerging growth company upon the earliest to occur of (1) the last day of

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the fiscal year in which our annual gross revenues exceed $1.07 billion; (2) the date we are deemed to be a “large accelerated filer” as defined in Rule 12b-2 under the Securities Exchange Act of 1934, as amended (“Exchange Act”), which would occur if the market value of our common equity held by non-affiliates exceeds $700 million as of the last business day of our most recently completed second fiscal quarter; and (3) the date on which we have issued more than $1.0 billion in non-convertible debt securities during the previous three years.

Upon the effectiveness of the registration statement of which this prospectus forms a part, we will report under the Exchange Act as a non-U.S. company with foreign private issuer status. Even after we no longer qualify as an emerging growth company, as long as we continue to qualify as a foreign private issuer under the Exchange Act, we will be exempt from certain provisions of the Exchange Act that are applicable to U.S. domestic public companies, including:

• the rules under the Exchange Act requiring domestic filers to issue financial statements prepared under U.S. GAAP;

• the sections of the Exchange Act regulating the solicitation of proxies, consents or authorizations in respect of a security registered under the Exchange Act;

• the sections of the Exchange Act requiring insiders to file public reports of their share ownership and trading activities and liability for insiders who profit from trades made in a short period of time; and

• the rules under the Exchange Act requiring the filing with the Securities and Exchange Commission (“SEC”) of quarterly reports on Form 10-Q containing unaudited financial and other specific information, or current reports on Form 8-K, upon the occurrence of specified significant events.

Notwithstanding these exemptions, we will file with the SEC, within four months after the end of each fiscal year, or such applicable time as required by the SEC, an annual report on Form 20-F containing financial statements audited by an independent registered public accounting firm.

We may take advantage of these exemptions until such time as we are no longer a foreign private issuer. We would cease to be a foreign private issuer at such time as more than 50% of our outstanding voting securities are held by U.S. residents and any of the following three circumstances applies: (a) the majority of our executive officers or directors are U.S. citizens or residents, (b) more than 50% of our assets are located in the United States or (c) our business is administered principally in the United States.

Both foreign private issuers and emerging growth companies also are exempt from certain more stringent executive compensation disclosure rules. Thus, even if we no longer qualify as an emerging growth company, but remain a foreign private issuer, we will continue to be exempt from the more stringent compensation disclosures required of companies that are neither an emerging growth company nor a foreign private issuer.

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The Registered Shares

Ordinary shares (including in the form of ADSs) issued and outstanding immediately before and after the effectiveness of the registration statement of which this prospectus forms a part

171,695,160 ordinary shares.

ADSs

Each ADS represents five ordinary shares, nominal value £0.06 per share. As an ADS holder, you will not be treated as one of our shareholders, you will not have shareholder rights and you may not be able to exercise your right to vote the shares underlying your ADSs. The depositary, the custodian or its nominee, will be the holder of the ordinary shares underlying your ADSs. You will have the contractual rights of an ADS holder, as provided in the deposit agreement among us, the depositary and holders and beneficial owners of ADSs issued thereunder.

To better understand the terms of the ADSs, see the sections of this prospectus entitled “Description of American Depositary Shares” and “Risk Factors—Risks Related to Ownership of our ADSs and Ordinary Shares and our Prospective Nasdaq Listing.” We also encourage you to read the form of amended and restated deposit agreement, which is incorporated by reference as an exhibit to the registration statement of which this prospectus forms a part.

Depositary

Citibank, N.A.

Use of proceeds

We will not receive proceeds from the disposition, if any, of Registered Shares in the form of ADSs by the Registered Holders.

Dividend policy

We do not currently pay cash dividends on our ordinary shares and have not paid any dividends within the last three financial years. See “Dividend Policy.”

Risk factors

See “Risk Factors” and other information included in this prospectus for a discussion of factors that you should consider carefully before deciding to invest in the ADSs.

Listing

We have applied to list the ADSs on the Nasdaq Global Select Market (“Nasdaq”) under the symbol “AMYT.” Our ordinary shares are admitted to trading on AIM, a market operated by London Stock Exchange plc, under the symbol “AMYT,” and on the EGE, a market regulated by Euronext Growth Market Dublin, under the symbol “AYP.”

Unless otherwise stated in this prospectus, the number of our shares set forth herein is based on 154,498,887 ordinary shares outstanding as of March 31, 2020, plus 17,196,273 zero cost warrants that may be exercised at any time, for a total outstanding share count of 171,695,160. This amount excludes:

• up to 48,343,750 ordinary shares that may be issued upon conversion of the $125 million aggregate principal amount of our 5% senior unsecured convertible notes (“Convertible Notes”);

• ordinary shares that may be issued in full satisfaction of the contingent value rights (“CVRs”) issued to holders of ordinary shares and employee option holders prior to the Acquisition, assuming all relevant milestones are achieved;

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• up to 14,481,720 ordinary shares issuable upon the exercise of share options with a weighted average exercise price of £1.16 per share;

• warrants to purchase 345,542 ordinary shares at a strike price of £1.44 per share;

• 4,864,656 ordinary shares held as treasury shares; and

• options to purchase an aggregate of 1,320,000 ordinary shares that we intend to grant to our non-executive directors immediately after the effectiveness of the registration statement of which this prospectus forms a part.

Except as otherwise noted, the information in this prospectus assumes:

• no conversion of the Convertible Notes; and

• no exercise of the warrants, CVRs or options;

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Summary Consolidated Financial Data

The tables below set forth the following summary consolidated financial data:

• Summary consolidated statements of comprehensive loss of Amryt for the years ended December 31, 2018 and 2019 and a summary consolidated statement of financial position of Amryt as of December 31, 2019, which have been derived from our audited consolidated financial statements included elsewhere in this prospectus.

• Summary condensed consolidated statements of comprehensive loss of Amryt for the three-month periods ended March 31, 2020 and 2019 and a summary condensed consolidated statement of financial position of Amryt as of March 31, 2019, which have been derived from our unaudited condensed consolidated financial statements included elsewhere in this prospectus.

• Summary consolidated statements of comprehensive loss of Aegerion for the years ended December 31, 2017 and 2018, which have been derived from Aegerion’s audited consolidated financial statements included elsewhere in this prospectus. Aegerion’s audited consolidated financial statements have been prepared in accordance with U.S. GAAP.

• Summary consolidated statements of comprehensive loss of Aegerion for the six months ended June 30, 2019 and a summary consolidated statement of financial position of Aegerion as of June 30, 2019, which have been derived from Aegerion’s unaudited interim consolidated financial statements included elsewhere in this prospectus. Aegerion’s unaudited interim consolidated financial statements have been prepared in accordance with U.S. GAAP. The accounting principles applied in Aegerion’s unaudited interim financial statements are consistent with those used in Aegerion’s annual audited financial statements. In the opinion of management, the unaudited financial data reflects all adjustments, consisting only of normal recurring adjustments, necessary for a fair presentation of the financial information in those statements.

Our consolidated financial statements have been prepared in accordance with IFRS, as issued by the IASB. Historical results for any prior period do not necessarily indicate our results to be expected for any future period. Interim financial results for the periods presented are not necessarily indicative of results for a full year or for any subsequent interim period.

You should read the following summary consolidated financial data together with the financial statements included elsewhere in this prospectus and the sections titled “Selected Consolidated Financial Data,” “Pro Forma Financial Information,” and “Management’s Discussion and Analysis of Financial Condition and Results of Operations.”

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Summary Consolidated Financial Data of Amryt

	Three Months Ended March 31		Year Ended December 31,
	2020	2019	2019	2018
	(unaudited)
	(In thousands, except per share data)
Statement of comprehensive loss data:
Revenue	$44,574	4,542	$58,124	$17,095
Cost of sales	(32,620)	(1,830)	(42,001)	(6,266)
Gross Profit	11,954	2,712	16,123	10,829
Total administrative, selling and marketing expenses	(19,151)	(3,987)	(36,339)	(18,163)
Research and development expenses	(8,934)	(1,505)	(15,827)	(10,703)
Impairment charge	—	—	(4,670)	—
Restructuring and acquisition costs	(853)	—	(13,038)	—
Operating loss before finance expense	(16,984)	(2,780)	(53,751)	(18,037)
Non-cash change in fair value of contingent consideration	(2,906)	(1,938)	(6,740)	(10,566)
Non-cash contingent value rights finance expense	(1,448)	—	(1,511)	—
Net finance expense — other	(9,416)	(661)	(4,759)	(1,841)
Loss on ordinary activities before taxation	(30,754)	(5,379)	(66,761)	(30,444)
Tax credit/(charge) on loss on ordinary activities	1,857	(6)	1,226	(43)
Loss for the period/year attributable to the equity holders of the Company	(28,897)	(5,385)	(65,535)	(30,487)
Exchange translation differences which may be reclassified through profit or loss	(13)	80	781	(77)
Total other comprehensive (loss) / income	(13)	80	781	(77)
Total comprehensive loss for the period/year attributable to the equity holders of the Company	$(28,910)	(5,305)	$(64,754)	$(30,564)
Loss per share - basic and diluted	$(0.19)	(0.12)	$(0.86)	$(0.67)
Selected Other Data (unaudited):
Adjusted EBITDA(1)	$5,358	(2,598)	$(12,180)	$(16,849)

(1)
In addition to analyzing our operating results on an IFRS basis, management also reviews our results on an “Adjusted EBITDA” basis. Adjusted EBITDA is defined as net loss before income taxes, non-cash change in fair value of contingent consideration, non-cash contingent value rights finance expense, net finance expense – other, amortization expense, depreciation expense, share-based payments, impairment charges, and restructuring and acquisition costs related to the acquisition of Aegerion. Adjusted EBITDA is not a measure of performance in accordance with IFRS and should not be considered as an alternative to net income/loss or operating cash flows determined in accordance with IFRS. We believe that the inclusion of Adjusted EBITDA in this prospectus is appropriate to provide additional information to investors because securities analysts, investors and other interested parties use this non-IFRS measure to assess our operating performance across periods on a consistent basis and to evaluate the relative risk of an investment in our securities. However, Adjusted EBITDA has limitations as an analytical tool and should not be considered in isolation or as a substitute for analysis of our results as reported under IFRS. Some of these limitations are:

•
although depreciation and amortization are non-cash charges, the assets being depreciated or amortized will often have to be replaced in the future, and Adjusted EBITDA does not reflect any cash requirements for such replacements;

•
Adjusted EBITDA does not reflect our cash expenditures, or future requirements for capital expenditures or contractual commitments;

•
Adjusted EBITDA does not reflect changes in, or cash requirements for, our working capital needs; and

•
Adjusted EBITDA does not reflect the significant interest expense, or the cash requirements necessary to service interest or principal payments, on our significant amount of indebtedness.

In addition, because not all companies use identical calculations, this presentation of Adjusted EBITDA may not be comparable to similarly titled measures of other companies, including companies in our industry. Adjusted EBITDA should not be taken as representative of our future results of operations or financial position.

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The following table reconciles Adjusted EBITDA to the most directly comparable IFRS financial performance measure, which is total comprehensive loss for the period attributable to the equity holders of the Company:

	Three Months Ended March 31		Year Ended December 31,
	2020	2019	2019	2018
	(unaudited)
	(In thousands)
Loss for the year attributable to the equity holders of the Company	$(28,897)	$(5,385)	$(65,535)	$(30,487)
Income taxes	(1,857)	6	(1,226)	43
Non-cash change in fair value of contingent consideration	2,906	1,938	6,740	10,566
Non-cash contingent value rights finance expense	1,448	3	1,511	—
Net finance expense - other	9,416	661	4,759	1,841
Amortization of inventory fair value step-up	9,503	—	10,367	—
Amortization expense - other	11,160	13	11,957	50
Depreciation expense	81	78	698	317
Share-based payments(a)	745	91	841	821
Impairment charge	—	—	4,670	—
Restructuring and acquisition costs	853	—	13,038	—
Adjusted EBITDA	$5,358	$(2,598)	$(12,180)	$(16,849)

(a)
This is a non-cash item that represents share-based compensation expense.

	As of March 31, 2020	As of December 31, 2019
	(unaudited)	(In thousands)
Statement of financial position data:
Cash and cash equivalents	$68,067	$67,229
Trade and other receivables	41,179	36,387
Inventories	33,904	43,623
Working capital(1)	31,443	47,025
Total assets	518,229	534,347
Secured Credit Facility	82,989	81,610
Convertible Notes, net of equity component	97,872	96,856
Total liabilities	417,072	405,025
Accumulated deficit	162,569	133,674
Total equity	101,157	129,322

(1)
We define working capital as current assets less current liabilities.

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Summary Consolidated Financial Data of Aegerion⁽¹⁾

	Six Months Ended June 30, 2019	Year Ended December  31,
		2018	2017
	(In thousands)
Statement of comprehensive loss data:
Net revenues	$95,857	$130,432	$138,438
Cost of product sales	35,364	59,697	77,220
Operating expenses:
Selling, general and administrative	43,424	64,437	77,793
Research and development	13,946	38,064	44,895
Restructuring charges	—	2,171	121
Related party expense (income), net	397	942	(177)
Total operating expenses	57,767	105,614	122,632
Income (loss) from operations	2,726	(34,879)	(61,414)
Reorganization items, net	(2,145)	—	—
Interest expense, net	(29,681)	(50,746)	(39,467)
Interest expense due to Novelion	(1,182)	(2,987)	(1,089)
Loss on extinguishment of debt	—	(4,333)	––
Other expense, net	(224)	(1,888)	(836)
Loss before provision for income taxes	(30,506)	(94,833)	(102,806)
Provision for income taxes	(369)	(1,705)	(594)
Net loss	$(30,875)	$(96,538)	$(103,400)

	As of June 30, 2019
	(In thousands)
Statement of financial position data:
Cash and cash equivalents	$36,080
Trade and other receivables	26,408
Inventories	51,792
Total assets	322,634
Total current liabilities	67,434
Provision for legal settlements - non-current	11,962
Other non-current liabilities	1,444
Total liabilities not subject to compromise	80,840
Liabilities subject to compromise	420,651
Total liabilities	501,491
Total equity	(178,857)

(1)
Debtor in possession as of June 30, 2019 but not as of December 31, 2017 and 2018.

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RISK FACTORS

Investing in our ADSs involves a high degree of risk. You should carefully consider the risks and uncertainties described below and the other information in this prospectus, including our consolidated financial statements and related notes, before making an investment in our ADSs. Our business, financial condition, results of operations or prospects could be materially and adversely affected if any of these risks occur, and as a result, the market price of our ADSs could decline and you could lose all or part of your investment.

Risks Related to our Business, Financial Condition and Capital Requirements

We have incurred significant operating losses since our inception and we may not achieve or maintain profitability in the future.

To date, we have financed our operations primarily through a combination of revenues from sales of our commercialized products and the sale of our equity securities and convertible bonds. We have incurred net losses in each year since our inception, including net losses of $30.6 million and $64.8 million for the years ended December 31, 2018 and 2019. Aegerion similarly incurred losses since its inception, including net losses of $96.5 million and $30.9 million for the year ended December 31, 2018 and the six-month period ended June 30, 2019. We have devoted most of our financial resources to the acquisition of attractive commercial and near-commercial rare disease assets and research and development. We anticipate that we will continue to incur significant costs associated with the continued commercialization of lomitapide and metreleptin, and in connection with ongoing clinical development efforts and post-marketing commitments for these products as well as the continued development of our product candidates. The amount of our future net losses will depend, in part, on the rate of our future expenditures, our ability to continue generating adequate revenues from sales of lomitapide and metreleptin and our ability to obtain funding through equity or debt offerings, grant funding, collaborations, strategic partnerships and/or licensing arrangements. If we do become profitable, we may not be able to sustain or increase our profitability on a quarterly or annual basis.

As a result of our September 2019 acquisition of Aegerion, our future results are likely to differ materially from our historical performance.

We completed the Acquisition in September 2019. Due to the significance of the Acquisition, our post-Acquisition financial results will differ materially from our pre-Acquisition historical performance, beginning with our audited financial results for December 31, 2019.

In addition, the Pro Forma Financial Information presented in this prospectus presents only estimates of the effects of the Acquisition and the Acquisition-related financing transactions (collectively, “Transactions”) on our and Aegerion’s historical results. The Pro Forma Financial Information is for illustrative purposes only and is not intended to, nor does it purport to, represent what our actual results or financial condition would have been if the Acquisition had been consummated in the period indicated. In addition, the Pro Forma Financial Information is based in part on certain assumptions regarding the Acquisition and post-Acquisition integration process that we believe are reasonable. The Acquisition and post-Acquisition integration process may also give rise to unexpected integration issues, including higher than expected integration liabilities and costs. Unexpected delays in connection with the post-Acquisition integration process may significantly increase the related costs and expenses we will incur. If any of these circumstances were to occur, operating expenses for the combined business may be higher than expected, reducing operating income and the expected benefits of the Acquisition. See “—Risks Related to Acquisitions, Including our Acquisition of Aegerion.”

Our future performance depends, in part, on our ability to successfully implement our strategy.

Our future success will depend on our ability to implement our strategy to develop and expand our existing portfolio of drugs to treat patients with rare diseases and to create a rare disease company with a diversified offering of multiple development stage and commercial assets that can provide us with scale to support future growth. The success of our strategy depends, in part, on our ability to drive growth in existing territories and access and drive growth in new territories and indications with existing commercial assets, build a franchise in EB through our lead product candidate, AP101, and our product candidate AP103, based on our novel polymer-based topical gene therapy delivery platform and expand and diversify our product portfolio through acquisition and in-licensing opportunities. This strategy will require us to, among other things, identify suitable targets, conduct and complete clinical trials, obtain the necessary consents and authorizations for our products and any product candidates, conduct diligence, carry out any acquisition or in-licensing transaction and obtain suitable financing.

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Implementing our strategy requires substantial time and resources from our management team. Our Board and management may not be able to successfully implement our strategy or other strategies to be developed by management, and implementing these strategies may not sustain or improve, and could even harm, our business, financial condition, results of operations and prospects. We may be unable to realize the anticipated benefits and synergies of our business strategies, which are based on assumptions about future demand for our current products and product candidates, as well as on our continuing ability to produce our products profitably. Consequently, any predictions made about our future success or viability may not be as accurate as they could be if we had a longer operating history or additional products on the market.

We are dependent primarily on two products, lomitapide and metreleptin, to generate revenue and these products may not be successful and may not generate sales at anticipated levels.

Our ability to meet expectations with respect to sales of lomitapide and metreleptin, and to generate revenues from such sales, and attain and maintain positive cash flow from operations, in the time periods anticipated, or at all, will depend on a number of factors, including, among others:

• the ability to continue to maintain and grow market acceptance for lomitapide and metreleptin among healthcare professionals and patients in the United States, European Union and other key markets for the treatment of approved indications;

• continuing market demand and medical need for these products;

• maintaining regulatory approvals without onerous restrictions or limitations in key markets and securing regulatory approvals in additional markets on a timely basis and with commercially feasible labels, and pricing and reimbursement approvals at adequate levels, where required, on a timely basis;

• side effects or other safety issues associated with the use of lomitapide and metreleptin could require us or our collaborators to modify or halt commercialization of these products or expose us to product liability lawsuits which will harm our business;

• we may be required by regulatory agencies to conduct additional studies regarding the safety and efficacy of lomitapide and metreleptin, which we have not planned or anticipated;

• generating revenues in markets that allow for sales of pharmaceutical products without regulatory approval based solely on the approvals of such products in the United States or European Union, and in which no promotion or commercialization activities are permitted; and

• adequately investing in the manufacturing, sales, marketing, market access, medical affairs and other functions that are supportive of our commercialization efforts.

If we are unable to continue to generate revenue from our current commercial products, our business, financial condition, results of operations and prospects will be adversely affected.

We may not be successful in our efforts to build a pipeline of product candidates and develop additional marketable products.

We operate in the biopharmaceutical sector and have product candidates in various stages of clinical and preclinical development. In addition, we may continue to explore other opportunities within the sector in order to expand our present development pipeline. Industry experience indicates that there may be a very high incidence of delay or failure to produce valuable scientific results in relation to our present development pipeline. In addition, disruptions caused by the COVID-19 pandemic may increase the likelihood that we encounter such difficulties or delays in initiating, enrolling, conducting or completing our planned and ongoing clinical trials. We may not be successful in developing new products based on our scientific discoveries. We will also face the risk that in developing new products we may spend substantial sums of money and the new products developed may not effectively meet the perceived need or may not be successfully commercialized. Our ability to develop new products relies on, among other things, the recruitment of sufficiently qualified research and development partners with expertise in the biopharmaceutical sector. We may not be able to develop relationships or recruit research partners of a sufficient caliber to satisfy the rate of growth and develop our future pipeline.

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We may need to raise additional funding, which may not be available on acceptable terms, or at all, and failure to obtain this capital when needed may force us to delay, limit or terminate our product development efforts or other operations or to delay payment on future obligations.

On September 24, 2019, we entered into a five-year term loan facility (“Secured Credit Facility”) and issued the Convertible Notes. As of December 31, 2019, we had borrowings of $82.5 million under our Secured Credit Facility and $126.7 million under our Convertible Notes, for total debt of $209.2 million. Furthermore, we may be required to satisfy payment obligations of up to $85 million to holders of contingent value rights (“CVRs”) issued to shareholders and option holders in connection with specified milestones, which we may elect to pay in ordinary shares or notes. If we elect to issue notes, we will settle such notes in cash 120 days after their issue.

We have significant payment obligations in connection with our Secured Credit Facility, the Convertible Notes, the CVRs, liabilities due to governmental entities that we assumed from Aegerion, and royalty obligations under certain of our license agreements.

In addition, while we are currently commercializing our approved products, we are also advancing our product candidates through preclinical and clinical development. Developing product candidates is expensive, lengthy and risky, and we expect our research and development expenses to increase in connection with our ongoing activities, particularly as we advance our product candidates toward commercialization.

As of December 31, 2019, our unrestricted cash and cash equivalents were $65.2 million. We expect that our existing cash and cash equivalents will be sufficient to fund our current operations and our payment obligations to third parties for at least the next 12 months. However, these resources might be insufficient to conduct research and development programs, the cost of product in-taking and possible acquisitions, fully commercialize products and operate our business to the full extent currently planned, and our operating plan may change as a result of many factors currently unknown to us. As a result, we may need to seek additional funds sooner than planned, through public or private equity or debt financings, government or other third-party funding, marketing and distribution arrangements and other collaborations, strategic alliances and licensing arrangements or a combination of these approaches. Even if we believe we have sufficient funds for our current or future operating plans as well as to satisfy our payment obligations to third parties, we may seek additional capital if market conditions are favorable or if we have specific strategic considerations. Any additional fundraising efforts may divert our management from their day-to-day activities, which may adversely affect our ability to develop and commercialize our product candidates.

If we cannot obtain adequate funds, we may be required to significantly curtail our commercial plans or one or more of our research and development programs or obtain funds through additional arrangements with corporate collaborators or others that may require us to relinquish rights to some of our technologies or product candidates. In addition, we cannot guarantee that future financing will be available in sufficient amounts or on terms acceptable to us, if at all. Moreover, the terms of any financing may adversely affect the holdings or the rights of our shareholders and the issuance of additional securities, whether equity or debt, by us, or the possibility of such issuance, may cause the market price of our ADSs or ordinary shares to decline. The sale of additional equity or convertible securities may be dilutive to our shareholders. We may also enter into additional credit facilities from time to time, which may be secured, to fund certain of our operations. If we raise additional capital through debt financing, we would be subject to payment obligations and may be subject to security interests in our assets and covenants restricting our ability to take specific actions, such as incurring additional debt, making capital expenditures or declaring dividends. If we raise additional capital through marketing and distribution arrangements, sales of assets or other collaborations, or licensing arrangements with third parties, we may have to relinquish certain valuable rights to our product candidates, technologies, future revenue streams or research programs. We also could be required to seek collaborators for one or more of our current or future product candidates at an earlier stage than otherwise would be desirable or relinquish our rights to product candidates or intellectual property that we otherwise would seek to develop or commercialize ourselves. If we are unable to raise additional capital in sufficient amounts, at the right time, on favorable terms, or at all, we may have to significantly delay, scale back or discontinue the development or commercialization of one or more of our products or product candidates, or one or more of our other research and development initiatives. Any of the above events could significantly harm our business, prospects, financial condition and results of operations, cause the price of our ADSs and ordinary shares to decline, and negatively impact our ability to fund operations.

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The terms of our debt and any requirements to incur further indebtedness or refinance our indebtedness in the future could have a material adverse effect on our business and results of operations.

Our level of indebtedness, together with any additional indebtedness we may incur in the future, could adversely affect our business, financial condition, results of operations and prospects. For example, our anticipated level of indebtedness or any additional financing may:

• make it more difficult for us to pay or refinance debts as they become due;

• require us to use a larger portion of cash flow for debt service, reducing funds available for other purposes;

• limit our ability to pursue business opportunities, such as potential acquisitions, and to react to changes in market or industry conditions;

• reduce the funds available for other purposes, such as implementing our strategy, funding capital expenditures and making distributions to shareholders;

• increase our vulnerability to adverse economic, industry or competitive developments;

• affect our ability to obtain additional financing, particularly as substantially all of our assets (including our intellectual property) are subject to liens securing indebtedness under our Secured Credit Facility;

• decrease our profitability, if we become profitable, or cash flow, or require us to dispose of significant assets in order to satisfy debts and other obligations if we are not able to satisfy these obligations using cash from operations or other sources; and

• disadvantage us compared to competitors.

Any of the foregoing, alone or in combination, could have a material adverse effect on our business, financial condition, results of operations and prospects. A breach of, or the inability to comply with, the covenants in the Secured Credit Facility and the Convertible Notes could result in an event of default, in which case the lenders will have the right to declare all borrowings to be immediately due and payable, which would have a material adverse effect on our business, financial condition, results of operations and prospects and could lead to foreclosure on our assets.

In the future, we may need to refinance our indebtedness. However, additional financing may not be available on favorable commercial terms to us, or at all. If, at such time, market conditions are materially different or our credit profile has deteriorated, the cost of refinancing such debt may be significantly higher than our indebtedness existing at that time. Furthermore, we may not be able to procure refinancing at all. Any failure to meet any future debt service obligations through use of cash flow, refinancing or otherwise, could have a material adverse effect on our business, financial condition, results of operations and prospects.

Restrictive covenants in certain of the agreements and instruments governing our indebtedness may adversely affect our financial and operational flexibility.

The terms of our indebtedness as of the date of this prospectus include covenants that, among other things, restrict our ability to: incur liens; dispose of our assets (both material assets and exclusive licensing transactions in material territories); consolidate and merge with other entities; make loans and investments; incur indebtedness; engage in transactions with affiliates; make specified payments; and engage in other customary business activities.

If we breach a restrictive covenant under the Secured Credit Facility or the Convertible Notes, or an event of default occurs with respect to such indebtedness, all amounts owing in respect of such indebtedness may be able to be delcared due and payable immediately, which could have a material adverse effect on our business, prospects, liquidity, financial condition and results of operations. In addition, both our Secured Credit Facility and the Convertible Notes include cross-default provisions, which generally provide that a default under one facility also results in a default under the other. See “Related Party Transactions—Agreements with Principal Shareholders— Secured Credit Facility” and “Related Party Transactions—Agreements with Principal Shareholders—Convertible Notes.”

If we enter into future debt agreements, they may include similar or more restrictive provisions. These restrictions may also make more difficult or discourage a takeover of our company, whether favored or opposed by management or the Board. Our ability to comply with these covenants may be affected by events beyond our

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control, and any material deviations from our forecasts could require us to seek waivers or amendments of covenants or alternative sources of financing, or to reduce expenditures. We cannot guarantee that such waivers, amendments or alternative financing could be obtained or, if obtained, would be on terms acceptable to us. A breach of any of the covenants or restrictions in our debt agreements or instruments could result in an event of default. Such a default could allow our debt holders to accelerate repayment of the related debt, as well as any other debt to which a cross-acceleration or cross-default provision applies, or to declare all borrowings outstanding under these agreements to be due and payable. If our debt is accelerated, our assets may not be sufficient to repay such debt.

Holders of the Convertible Notes have the right to require the repurchase of their notes for cash upon the occurrence of a fundamental change, such as a change of control of our company, at a repurchase price equal to 100% of the respective principal amount, plus accrued and unpaid interest, if any. Subject to certain exceptions as provided in the indenture governing the Convertible Notes, a fundamental change includes (a) the acquisition of 50% or more of the voting interests in our company, (b) an event in which we merge or consolidate with another entity, (c) an event in which we convey, sell, transfer or lease all or substantially all of our assets to another entity, (d) our liquidation or (e) delisting of our ordinary shares. Among the exceptions provided in the indenture are for transactions described in (a), (b) or (c) in which (i) our stockholders immediately prior to the transaction have the right to exercise, directly or indirectly, 50% or more of the total voting power of the capital stock of the continuing or surviving entity or transferee or parent thereof following the transaction or (ii) 90% of the consideration paid for our ordinary shares in a transaction consists of stock that is or will be quoted on AIM, the EGE, the New York Stock Exchange or the Nasdaq. Further, unless we elect to deliver our ordinary shares to settle any conversions of Convertible Notes, we would be required to settle a portion or all of the conversion obligation through the payment of cash. We may not have enough available cash or be able to obtain financing at the time it is required to make repurchases of Convertible Notes surrendered therefor or Convertible Notes being converted. The failure to repurchase Convertible Notes at a time when the repurchase is required by the indenture or to pay any cash payable on future conversions of the Convertible Notes as required by the indenture would constitute a default under the indenture. A default under the indenture or a fundamental change itself could also lead to a default under agreements governing our current and future indebtedness. If the repayment of such indebtedness were to be accelerated after any applicable notice or grace periods, we are unlikely to have sufficient funds to repay such indebtedness and repurchase the Convertible Notes or make cash payments upon conversions thereof or other required payments on its other indebtedness, which would materially and adversely affect our business, financial condition and results of operations on a consolidated basis.

We may be subject to ongoing financial liabilities and other obligations that we assumed upon Aegerion’s emergence from bankruptcy.

On September 24, 2019, we completed the Acquisition upon Aegerion’s emergence from Chapter 11 bankruptcy pursuant to a Plan of Reorganization approved by the Bankruptcy Court for the Southern District of New York. Prior to filing bankruptcy, Aegerion, its parent, Novelion Therapeutics Inc. (“Novelion”), and Aegerion’s primary creditors entered into, among other things, a Restructuring Support Agreement. We purchased Aegerion pursuant to certain transactions effectuated in accordance with Aegerion’s Plan of Reorganization, as contemplated by the Restructuring Support Agreement. Pursuant to the Plan of Reorganization, Aegerion was discharged from all claims, interests, rights and liabilities against Aegerion that arose prior to the effective date of the Plan of Reorganization (“Effective Date”), except as expressly set forth in the Plan of Reorganization.

The Plan of Reorganization contemplated that certain prepetition liabilities of Aegerion would continue to be obligations of Aegerion, notwithstanding the general discharge of all prepetition claims against Aegerion. Specifically, the Plan of Reorganization contemplated the following post-Effective Date liabilities: (i) in satisfaction of certain of its prepetition secured debt obligations, Aegerion entered into a new secured term loan debt facility in the amount of $81,020,618.73 with interest accruing at (a) 11% per annum paid in cash on a quarterly basis; or (b) 6.5% per annum paid in cash plus 6.5% per annum paid in kind on a quarterly basis; (ii) in satisfaction of certain prepetition liabilities, Aegerion issued $125 million in Convertible Notes; (iii) Aegerion assumed certain prepetition contracts (including all pre- and post-petition associated liabilities) with trade creditors providing goods and services to Aegerion; and (iv) Aegerion excepted from discharge its obligations to indemnify, defend, reimburse, exculpate, advance fees and expenses to, or limit the liability of directors, officers or employees of Aegerion, or any of Aegerion’s subsidiaries, who served in such capacity after Aegerion’s bankruptcy petition. In addition, prior to the bankruptcy proceedings, Aegerion entered into 38 settlement agreements with governmental entities (including the Department of Justice (“DOJ”) and the FDA)

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in connection with the JUXTAPID investigations. These settlement agreements require Aegerion to pay specified fines and engage in extensive regulatory compliance efforts. The Plan of Reorganization provided that each of these settlement agreements were assumed by Aegerion pursuant to the Plan of Reorganization and would therefore continue to constitute obligations of Aegerion after the Effective Date. Following the Acquisition, we are subject to the obligations levied by these settlement agreements as well as the other liabilities that survived the bankruptcy cases. This may result in unanticipated costs and may harm our financial condition, results of operations and prospects.

We face potential product liability exposure, and if claims are brought against us, we may incur substantial liability.

The use of any product in clinical trials or early access programs, and the sale and use of any product for which we have obtained marketing approval exposes us to the risk of product liability claims. Product liability claims might be brought against us by consumers, healthcare providers or others selling or otherwise coming into contact with our products. If we cannot successfully defend against product liability claims, we could incur substantial liabilities. In addition, regardless of merit or eventual outcome, product liability claims may result in:

• decreased demand or coverage for our products;

• impairment of our business reputation and exposure to adverse publicity;

• warnings on product labels;

• withdrawal of clinical trial participants;

• substantial monetary awards to trial participants or patients;

• significant time and costs to defend the related litigation;

• distraction of management’s attention from our primary business;

• substantial monetary awards to patients or other claimants;

• loss of revenues; and

• the inability to successfully commercialize our products.

Although we have obtained product liability insurance coverage for both our clinical trials and our commercial exposure, this insurance coverage may not be sufficient to reimburse us for expenses or losses that we may suffer. Moreover, insurance coverage is becoming increasingly expensive, and in the future, we may not be able to maintain insurance coverage at a reasonable cost or in sufficient amounts to protect us against potential liability. If a claim is brought against us, we might be required to pay legal and other expenses to defend the claim, as well as pay uncovered damages awards resulting from a claim brought successfully against us and these damages could be significant and have a material adverse effect on our financial condition. On occasion, large judgments have been awarded in class action lawsuits relating to drugs that had unanticipated side effects or warnings found to be inadequate. The cost of any product liability litigation or other proceedings, even if resolved in our favor, could be substantial. A product liability claim or series of claims brought against us could harm our reputation and cause the price of our securities to decline and, if the claim is successful and judgments exceed our insurance coverage, it could have a material adverse impact on our business, financial condition, results of operations and prospects.

Adverse events involving any of our products and product candidates may lead the FDA, the EMA or other regulatory authorities to delay or deny clearance for our products or result in product recalls that could harm our reputation, business and financial results.

The FDA and the EMA, as well as similar governmental authorities in other jurisdictions, have the authority to require the recall of certain commercialized products in the event of adverse side effects, material deficiencies or defects in design or manufacture. Manufacturers may, under their own initiative, recall a product if any material deficiency in a product is found. A government-mandated recall or voluntary recall by us or one of our distributors could occur as a result of adverse side effects, impurities or other product contamination, manufacturing errors, design or labeling defects or other deficiencies and issues. Recalls of any of our products or product candidates would divert managerial and financial resources and have an adverse effect on our financial condition and results of operations. A recall announcement could harm our reputation with customers and negatively affect our sales, if any.

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Our future success depends on our ability to hire and retain key executives and to attract, retain and motivate qualified personnel.

Our future success depends on our ability to attract and retain key management personnel, scientific and technical personnel, particularly in the biopharmaceutical industry. Our ability to continue our operations and implement our strategy depends upon retaining, recruiting and motivating employees, especially with respect to our management team and research personnel. Experienced employees in the biopharmaceutical and biotechnology industries are in high demand and competition for their talents can be intense, especially in Germany, Ireland, and Boston, Massachusetts, where we maintain our principal operations. We have entered into employment agreements with executive officers and other key employees, but any employee may terminate his or her employment at any time or may be unable to continue in his or her role. The loss of any executive or key employee, or an inability to recruit desirable candidates or find adequate third parties to perform such services on reasonable terms and on a timely basis, could have a material adverse effect on our business, financial condition, results of operations and prospects. If we are not able to attract, retain and motivate necessary personnel to accomplish our business objectives, we may experience constraints that could significantly impede our ability to achieve our development and commercial objectives, our ability to raise additional capital and our ability to implement our business strategy.

We expect that certain U.S. federal income tax rules regarding “inversion transactions” will apply to us, which could result in adverse U.S. federal income tax consequences.

We believe that we are a “surrogate foreign corporation” and that Aegerion is an “expatriated entity,” within the meaning of section 7874 of the U.S. Internal Revenue Code of 1986, as amended (“Code”), as a result of the Acquisition. We are a surrogate foreign corporation with respect to Aegerion if (1) pursuant to a plan, we complete the direct or indirect acquisition of substantially all of the properties held, directly or indirectly, by Aegerion, (2) after the acquisition at least 60% of our stock (by vote or value) is held by former shareholders and certain creditors of Aegerion by reason of their holding Aegerion stock or debt obligations (such percentage held by such persons being the “Section 7874 Percentage”), and (3) after the Acquisition, the expanded affiliated group that includes Amryt, Aegerion and their respective more-than-50% controlled subsidiaries (“Enlarged Amryt Group”) does not have substantial business activities in the United Kingdom relative to the group’s worldwide business activities.

If the Section 7874 Percentage is at least 60% but less than 80% and we are a surrogate foreign corporation with respect to Aegerion, several limitations apply to Aegerion, including, but not limited to, the prohibition, for a period of ten years, of the use of net operating losses, foreign tax credits and other tax attributes to offset the income or gain recognized by reason of transfer of any property to a foreign related person or to offset any income received or accrued during such period by reason of our license of any property to a foreign related person and an additional minimum tax under Section 59A of the Code on certain “base eroding” payments to members of the Enlarged Amryt Group that are foreign corporations. In addition, under section 4985 of the Code and the rules related thereto, an excise tax at a rate of currently 20% is imposed on the value of certain share compensation held directly or indirectly by certain “disqualified individuals” (including certain of our officers and directors). Further, shareholders that are United States persons for U.S. federal income tax purposes may not be eligible for reduced rates on dividends paid by us.

If the Section 7874 Percentage is at least 80% and we are a surrogate foreign corporation with respect to Aegerion, we will be treated as a U.S. domestic corporation, regardless of the fact that we are also incorporated in England and Wales, and managed and controlled in the United Kingdom, and therefore generally classified as a UK corporation for UK tax purposes. If we were treated as a U.S. domestic corporation, our entire net income would be subject to U.S. federal income tax on a net income basis and would be determined under U.S. federal income tax principles.

While we expect to be treated as a surrogate foreign corporation for U.S. federal income tax purposes, we believe that the Section 7874 Percentage with respect to Aegerion is less than 80%. We therefore do not expect to be treated as a U.S. domestic corporation for U.S. federal income tax purposes. Determining the Section 7874 Percentage, however, is complex and subject to factual and legal uncertainties. As a result, there can be no assurance that the Internal Revenue Service (“IRS”) will agree with our conclusions regarding the Section 7874 Percentage. Holders are urged to consult their own tax advisors regarding the potential application of section 7874 of the Code and its potential tax consequences. A determination by the IRS that we are a U.S. domestic corporation for the purposes of section 7874 of the Code may have material adverse effects on the business, financial condition, results of operations and prospects of the Enlarged Amryt Group.

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Our global operations subject us to significant tax risks.

We are subject to tax rules in the jurisdictions in which we operate. Changes in tax rates, tax relief and tax laws, changes in practice or interpretation of the law by the relevant tax authorities, increasing challenges by relevant tax authorities or any failure to manage tax risks adequately could result in increased charges, financial loss, penalties and reputational damage. Tax authorities may actively pursue additional taxes based on retroactive changes to tax laws which could result in a material restatement to its tax position. Any of these factors could have a negative impact on our business, financial condition, results of operations and prospects.

Legal, political and economic uncertainty surrounding the exit of the United Kingdom from the European Union may be a continued source of instability in international markets and currency exchange rate volatility, and could materially and adversely affect our business, financial condition, results of operations and prospects.

In June 2016, the United Kingdom voted in a referendum to leave the European Union (commonly referred to as “Brexit”). On March 29, 2017, the United Kingdom formally notified the Council of the European Union of its intention to leave the European Union. Following negotiations on the terms of the United Kingdom’s exit from the European Union, a withdrawal agreement (“Withdrawal Agreement”) setting out the terms of the exit was entered into on January 24, 2020. The Withdrawal Agreement became effective, and the United Kingdom formally left the European Union, on January 31, 2020. Although the United Kingdom has officially exited the European Union, the pre-January 31, 2020 legal status quo is continuing during a so-called “transition period” during which the United Kingdom and the European Union are continuing to negotiate the terms of their future relationship including any future trade agreement. The transition period may be extended beyond 2020 if both the United Kingdom and the European Union agree to an extension before July 1, 2020.

These developments may have a significant adverse effect on global economic conditions and continue to be a source of instability in the global financial markets, and could significantly reduce global market liquidity and limit the ability of key market participants to operate in certain financial markets. In particular, it could also lead to a period of considerable uncertainty in relation to the United Kingdom financial and banking markets, as well as on the regulatory process in the United Kingdom. Asset valuations and currency exchange rates may also be subject to continued market volatility as a result of Brexit and other factors, including those relating to the COVID-19 pandemic.

Ongoing uncertainty on whether and when the member states of the European Union (the “EU Member States”) and the United Kingdom will come to an agreement on the terms of the future relationship including any trade agreement sustains the possibility that the United Kingdom will leave the European Union without an agreement in place. The long-term effects of Brexit will depend on such agreement(s) (or lack thereof) between the United Kingdom and the European Union and, in particular, any arrangements for the United Kingdom to retain access to European Union markets following the transition period. In addition, depending on the terms of any such agreement(s), barrier-free access between the United Kingdom and EU Member States, or among the European Economic Area overall, could be diminished or eliminated.

The ultimate impact of Brexit on our business operations could vary depending on the details of such agreement(s) and, while negotiations are still underway, Brexit could significantly affect the financial, trade, regulatory and legal landscape in the United Kingdom, and could have a material impact on its economy and the future growth of its various industries, including the pharmaceutical and biotechnology industries. Further, Brexit could lead to legal uncertainty and regulatory divergence between the United Kingdom and the European Union. Given the lack of comparable precedent, it is unclear what financial, trade, regulatory and legal implications the withdrawal of the United Kingdom from the European Union will have and how such withdrawal will affect us. Any of the foregoing could have a material adverse effect on our business, financial condition, results of operations and prospects.

Fluctuations in currency exchange rates and increased inflation could materially adversely affect our financial condition and results of operations.

We have operations in Ireland, the United Kingdom, the United States, Germany, Switzerland, Brazil, France, Italy, Spain and other select markets throughout the world. As a result of the international scope of our operations, fluctuations in exchange rates, particularly between the U.S. dollar, our reporting currency, and the Euro, may adversely affect us. In the year ended December 31, 2019, 3.9% of our sales were denominated in

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pound sterling (£), 57.2% of our sales were denominated in U.S. dollars, 35.5% were denominated in Euros and the balance was denominated in other currencies. As a result, strengthening of the Euro or pound sterling relative to the U.S. dollar presents the most significant risk to us. Any significant fluctuations in currency exchange rates may have a material impact on our business.

In addition, economies in Central European and Latin American countries have periodically experienced high rates of inflation. Periods of higher inflation may slow economic growth in those countries. As a substantial portion of our expenses (excluding currency losses and changes in deferred tax) is denominated in U.S. dollars or Euros, the relative movement of inflation significantly affects our results of operations. Inflation also is likely to increase some of our costs and expenses, including wages, rents, leases and employee benefit payments, which we may not be able to pass on to our customers and, as a result, may reduce our profitability. To the extent inflation causes these costs to increase, such inflation may materially adversely affect our business. Inflationary pressures could also affect our ability to access financial markets and lead to counter-inflationary measures that may harm our financial condition, or results of operations or materially adversely affect the market price of our securities.

The occurrence of cyber incidents, or a deficiency in cybersecurity, could negatively impact our business by causing a disruption to its operations, a compromise or corruption of confidential information, exposure to legal and regulatory action, or damage to our patient, partner or employee relationships, any of which could subject us to loss and reputational harm.

A cyber incident is considered to be any event that threatens the confidentiality, integrity or availability of information resources. More specifically, a cyber incident is an intentional attack or an unintentional event that can include gaining unauthorized access to systems to disrupt operations, corrupt data or steal confidential information about patients, suppliers, partners or employees. A number of companies have recently experienced serious cyber incidents and breaches of their information technology systems. Cyber incidents pose risks both to our internal systems and to those we have outsourced operations to, including the risk of operational interruption, damage to our reputation and relationships with patients, partners and employees, and private data exposure. We have implemented processes, procedures and controls to help mitigate these risks. However, these measures, as well as an increased awareness of the risk of a cyber incident, do not guarantee that our reputation, operations and financial results will not be adversely affected by such an incident.

In the ordinary course of business, we collect and store sensitive data, including intellectual property, proprietary business information and patient data. This data includes, where required or permitted by applicable laws, personally identifiable information. Certain third parties with whom we contract also collect and store data related to clinical trial subjects and patients. The secure maintenance of this information is critical to our operations and business strategy. Despite our current security measures, our information technology and infrastructure may be vulnerable to attacks by hackers or breaches due to employee error, malfeasance or other disruptions. Any such breach could compromise information stored on networks or those of our partners. Any access, disclosure or other loss of information could result in legal claims or proceedings, liability under laws that protect the privacy of personal information, recovery costs, disruption of operations, including delays in our regulatory approval efforts, and damage our reputation, which could adversely affect our business.

The outbreak of COVID-19 could adversely impact our business, including our preclinical studies and clinical trials.

Since a novel strain of coronavirus, SARS-CoV-2, causing a disease referred to as COVID-19, was first reported in December 2019, the disease has spread across the world, including countries in which we have planned or active clinical trial sites. The outbreak and government measures taken in response have also had a significant impact, both direct and indirect, on businesses and commerce, as worker shortages have occurred; supply chains have been disrupted; facilities and production have been suspended; and demand for certain goods and services, such as medical services and supplies, has spiked, while demand for other goods and services, such as travel, has fallen. In response to the spread of COVID-19, we have closed our executive offices with our administrative employees continuing their work outside of our offices and limited the number of staff in any given manufacturing facility. As COVID-19 continues to spread around the globe, we may experience disruptions that could affect our business, preclinical studies and clinical trials, including:

• unsuccessful and/or untimely completion of preclinical and clinical development of our product candidates and any other future candidates, as well as the associated costs, including any unforeseen costs we may incur as a result of preclinical study or clinical trial delays;

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• delays or difficulties in initiating, enrolling, conducting or completing our planned and ongoing clinical trials;

• risk that participants enrolled in our clinical trials will acquire COVID-19 while the clinical trial is ongoing, which could impact the results of the clinical trial, including by increasing the number of observed adverse events;

• existing patients with serious diseases included in our clinical trials may die as a result of contracting COVID-19 or suffer other adverse medical events for reasons that may not be related to our products or candidates;

• delays or difficulties in clinical site initiation, including difficulties in recruiting clinical site investigators and clinical site staff;

• healthcare budgets may be adversely affected and as a result, funding may not be available to pay for our products;

• diversion of healthcare resources away from the conduct of clinical trials, including the diversion of hospitals serving as our clinical trial sites and hospital staff supporting the conduct of our clinical trials;

• interruption of key clinical trial activities, such as clinical trial site monitoring, due to limitations on travel imposed or recommended by federal, state or local governments, employers and others or interruption of clinical trial subject visits and study procedures (such as pre-planned clinical trial assessments), which may impact the integrity of subject data and clinical study endpoints;

• limitations in employee resources that would otherwise be focused on the conduct of our preclinical studies and clinical trials, including because of sickness of employees or their families or the desire of employees to avoid contact with large groups of people;

• interruption or delays in the operations of the FDA or other regulatory authorities, which may impact review and approval timelines;

• delays in receiving approval from local regulatory authorities to initiate our planned clinical trials;

• delays in clinical sites receiving the supplies and materials needed to conduct our clinical trials due to staffing shortages, production slowdowns or stoppages and disruptions in delivery systems;

• suspension or termination of a clinical trial by us, by the Institutional Review Boards (“IRBs”) of the institutions in which such trial is being conducted, by a DSMB for such trial or by the FDA, the EMA or comparable foreign regulatory authorities due to a number of factors, including failure to conduct the clinical trial in accordance with regulatory requirements or our clinical protocols, inspection of the clinical trial operations or trial site by the FDA, the EMA or comparable foreign regulatory authorities resulting in the imposition of a clinical hold, unforeseen safety issues or adverse side effects;

• refusal of the FDA to accept data from clinical trials in affected geographies outside the United States;

• changes in local regulations as part of a response to the COVID-19 pandemic which may require us to change the ways in which our clinical trials are conducted, which may result in unexpected costs, or to discontinue the clinical trials altogether;

• delays in necessary interactions with local regulators, ethics committees and other important agencies and contractors due to limitations in employee resources or forced furlough of government employees;

• impairment of our operations, including among others, employee mobility and productivity, availability of facilities, conduct of clinical trials, manufacturing and supply capacity, disruption of our supply chain, availability of shipping and distribution channels, restrictions on import and export regulations and the availability and productivity of third party service suppliers;

• incurrence of delays in the delivery of our products or our inability to deliver products to our patients;

• interruption in global shipping that may affect the transport of clinical trial materials, such as investigational drug product used in our clinical trials; and

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• disruption and volatility in the global capital markets, which increases the cost of capital and adversely impacts access to capital should we have specific strategic considerations which require it.

The global pandemic of COVID-19 continues to evolve rapidly. The ultimate impact of the COVID-19 pandemic or a similar health epidemic is highly uncertain and subject to change. We do not yet know the full extent of potential delays or impacts on our business, preclinical studies, clinical trials, healthcare systems or the global economy as a whole. However, these effects could have a material impact on our operations, and we will continue to monitor the COVID-19 situation closely.

Risks Related to Acquisitions, Including our Acquisition of Aegerion

We have faced, and we may continue to face, challenges integrating the businesses and operations of our company and Aegerion and, as a result, may not realize the expected benefits of the Acquisition.

Integrating the businesses of our company and the significantly larger business of Aegerion is a complex process. At June 30, 2019, shortly before completion of the Acquisition, Aegerion had total assets of $322.6 million compared to our total assets of $75.4 million. We cannot guarantee that the integration of our business with Aegerion’s will be completed successfully or that we will be able to achieve any of the anticipated benefits. In order for the integration to be successful, we must maintain our and Aegerion’s prior regulatory relationships, clinical trials, product initiatives, license, service and DOJ agreements and their related obligations. We must also undertake certain actions required to maintain manufacturing, supply and distribution processes. While we have made significant progress, we may not be able to successfully integrate the businesses of our company and Aegerion, and even if successful, the integration may be costly and time consuming, and the anticipated synergies may not be realized, either of which may have a material and adverse effect on our business, financial condition, results of operations and prospects.

Any future acquisitions we make may expose us, to risks that could adversely affect our business, and we may not achieve the anticipated benefits of acquisitions of businesses or technologies.

As a part of our growth strategy, we may make additional acquisitions of complementary businesses. Any future acquisition will involve numerous risks and operational, financial and managerial challenges, including the following, any of which could adversely affect our business, financial condition or results of operations:

• limited support and user knowledge for legacy systems of acquired companies;

• problems maintaining uniform procedures, controls and policies with respect to our financial accounting systems;

• difficulties in managing geographically dispersed operations, including risks associated with entering foreign markets in which we have no or limited prior experience;

• underperformance of any acquired technology, product or business relative to our expectations and the price we paid;

• negative near-term impacts on financial results after an acquisition, including acquisition-related earnings charges;

• the potential loss of key employees, customers and strategic partners of acquired companies;

• claims by terminated employees and shareholders of acquired companies or other third parties related to the transaction;

• the assumption or incurrence of additional debt obligations or expenses, or use of substantial portions of our cash;

• the issuance of equity securities to finance or as consideration for any acquisitions that dilute the ownership of our shareholders;

• any collaboration, strategic alliance and licensing arrangement may require us to relinquish valuable rights to our technologies or product candidates, or grant licenses on terms that are not favorable to us;

• risks and uncertainties associated with the other party to such a transaction, including the prospects of that party and their existing products or product candidates and regulatory approvals;

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• diversion of management’s attention and company resources from existing operations of the business;

• inconsistencies in standards, controls, procedures and policies;

• the impairment of intangible assets as a result of technological advancements, or worse-than-expected performance of acquired companies;

• assumption of, or exposure to, historical liabilities of the acquired business, including unknown contingent or similar liabilities that are difficult to identify or accurately quantify;

• our inability to generate revenue from acquired technology or products sufficient to meet our objectives in undertaking the acquisition or even to offset the associated acquisition and maintenance costs; and

• risks associated with acquiring intellectual property, including potential disputes regarding acquired companies’ intellectual property.

There can be no assurance that any of the acquisitions we may make will be successful or will be, or will remain, profitable. Our failure to successfully address the foregoing risks may prevent us from achieving the anticipated benefits from any acquisition in a reasonable time frame, or at all.

If intangible assets and goodwill that we record in connection with our acquisitions become impaired, we may have to take significant charges against earnings.

In connection with the accounting for our acquisitions, a significant value may be recognized in respect of intangible assets, including developed technology and customer relationships relating to the acquired product lines, and goodwill. Under IFRS, we must assess, at least annually and potentially more frequently, whether the value of intangible assets and goodwill has been impaired. Intangible assets and goodwill will be assessed for impairment in the event of an impairment indicator. Any reduction or impairment of the value of intangible assets and goodwill will result in a charge against earnings, which could materially adversely affect our results of operations and shareholders’ equity in future periods.

Risks Related to the Commercialization of our Products

Our assumptions and estimates regarding prevalence and the addressable markets for our products and product candidates may be inaccurate. If there are fewer actual patients than estimated, or if any product approval is based on narrower definitions of patient populations, our revenues and cash position could be materially and adversely affected.

The patient population for the diseases that our products treat is very small, and networking, data gathering and support channels are not as established as those for more prevalent and researched disease indications. There is no patient registry or other method of establishing with precision the actual number of HoFH, GL and PL, or EB patients in any geography. Estimating the prevalence of a rare disease is difficult and we therefore must rely on assumptions, beliefs and an amalgam of information from multiple sources, resulting in potential under or over-reporting. There is no guarantee that our assumptions and beliefs are correct, or that the methodologies used and data collected have generated or will continue to generate accurate estimates. There is therefore uncertainty around the estimated total potential addressable patient population for treatment with lomitapide and metreleptin worldwide.

In addition, the potential market opportunity for our product candidates that we may develop is difficult to estimate precisely, particularly given that the orphan drug markets which are targeted are, by their nature, relatively unknown. Our estimates of the potential market opportunity for each of these product candidates are predicated on several key assumptions, such as industry knowledge and publications, third-party research reports and other surveys. If any of our assumptions prove to be inaccurate, then the actual market for lomitapide, metreleptin, AP101 or AP103, or our other or future product candidates, could be smaller than our estimates of the potential market opportunity. If that turns out to be the case, our product revenue may be limited, and we may be unable to achieve or maintain profitability, which could have a material adverse effect on our business, financial condition, results of operations and prospects.

Our products may not gain market acceptance, in which case we may not be able to generate product revenues.

Physicians, healthcare providers, patients, payers or the medical community may not accept or use our approved products. Efforts to educate the medical community and third-party payers on the benefits of the products may require significant resources and may not be successful. Notwithstanding the level of revenues historically

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generated from the sale of lomitapide and metreleptin, if any of our existing marketed products or product candidates do not achieve an adequate level of acceptance, we may struggle to continue to generate significant product revenues and may not in the future generate any profits from operations. The degree of market acceptance will depend on a variety of factors, including, but not limited to:

• whether clinicians and potential patients perceive product candidates to have better efficacy, safety, tolerability profile and ease of use, when compared with the products marketed by our competitors and the prevailing standard of care (“SOC”);

• the timing and location of market introduction of any approved products;

• our ability to provide acceptable evidence of safety and efficacy;

• the frequency and severity and causal relationships of any side effects and a continued acceptable safety profile following approval;

• relative convenience and ease of administration;

• cost effectiveness;

• patient diagnostics and screening infrastructure in each market;

• marketing and distribution support;

• the availability of healthcare coverage, reimbursement and adequate payment from health maintenance organizations and other third-party payers, both public and private; and

• competition from other therapies.

We face significant competition from other biotechnology and pharmaceutical companies.

The specific markets in which we operate are highly competitive and this competition could harm our results of operations, cash flows and financial condition. Our competitors include major international pharmaceutical companies as well as smaller or regional specialty pharmaceutical and biotechnology companies. We may be forced to either lower the selling prices of our products in response to competitor pricing or lose patients who choose lower-priced products. Many of our competitors are larger, have greater financial resources and a lower cost structure. As a result, our competitors may be better equipped to withstand changes in economic and industry conditions. These competitors currently engage in, have engaged in or may in the future engage in the development, manufacturing, marketing and commercialization of new pharmaceuticals, some of which may compete with our products. Competition may also arise from, among other things, other drug development technologies, methods of preventing or reducing the incidence of disease, including vaccines and new small molecule or other classes of therapeutic agents. Smaller or early stage companies may also be significant competitors, particularly through collaborative arrangements with large, established companies. Key competitive factors affecting the commercial success of our products and any other products that we develop or acquire are likely to be safety, efficacy, tolerability profile, reliability, convenience of dosing, price and reimbursement. We may also face future competition from companies selling generic alternatives to our products in countries where we do not have patent coverage, Orphan Drug status or another form of data or marketing exclusivity or where patent coverage or data or marketing exclusivity has expired, is not enforced, or may, in the future, be challenged.

A significant competitor to our lomitapide product is a class of drugs known as PCSK9 inhibitors. One PCSK9 inhibitor is currently approved and commercialized in the European Union and the United States for the same treatment indication as lomitapide. Sales of this PCSK9 inhibitor compete with sales of lomitapide and we expect that this product will continue to compete with lomitapide. In addition, one of our competitors, Regeneron Pharmaceuticals Inc., is developing Evinacumab for the treatment of HoFH and also for hypertriglyceridemia. Recent Phase 3 clinical data in HoFH have demonstrated a promising efficacy and safety profile. Although the drug is administered by intravenous infusion, if it is approved, physicians may consider use of this product in HoFH patients before initiation of lomitapide treatment. Other competitors may succeed in developing, acquiring or licensing additional pharmaceutical products that are introduced into the market and that are more effective, have a more favorable safety profile, or are less costly than our products.

Although MYALEPT is the first and only product approved in the United States for the treatment of complications of leptin deficiency in patients with GL, there are a number of therapies approved to treat these complications independently that are not specific to GL. MYALEPTA also faces competition in the European

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Union, both for the treatment of GL and PL. Our competitors are also developing products, which, if approved, and depending on the labelled indication, could potentially compete with metreleptin.

Although there are no approved products in the United States and the European Union for the treatment of EB, our competitors are developing products, which, if approved, and depending on the labelled indication, could potentially compete with AP101.

Other competitors may succeed in developing, acquiring or licensing additional pharmaceutical products that are introduced into the market and that are more effective, have a more favorable safety profile, or are less costly than our products. If we do not compete successfully, our operating margins, financial condition and cash flows could be adversely affected.

We commercialize our products through both a direct sales force and through strategic relationships with third parties for commercialization, distribution, sales and marketing in certain jurisdictions. If we are unable to adequately develop and maintain our sales, marketing and distribution capabilities or enter into business arrangements, we may not be successful in commercializing our product candidates.

We sell lomitapide and metreleptin directly in the United States using our own marketing and sales resources and also market and sell, or plan to market and sell, our products directly in certain key countries outside the United States where such products are, or may be, approved using country managers or local distributors to the extent rights to commercialize such products are not out-licensed. We sell metreleptin in other countries including: Italy, Greece, France, Germany, United Kingdom, Spain, Portugal, Saudi Arabia, Israel, Turkey, Oman, Qatar, Bahrain/UAE, Colombia and Argentina. Metreleptin was out-licensed to Shionogi & Co. Ltd. (“Shionogi”) in Japan. We sell lomitapide in the Netherlands, Germany, Spain, Greece, Italy, United Kingdom, Sweden, Norway, Denmark, Lithuania, France, Hungary, Qatar, Kuwait, Saudi Arabia, Brazil, Colombia and Argentina. We also out-licensed lomitapide for sale by Recordati Rare Diseases Inc. (“Recordati”) in Japan. Lomitapide is also sold, on a named patient sales basis, in Brazil and in a limited number of other countries outside the United States where such sales are permitted before local regulatory approval is obtained as a result of the approval of lomitapide in the United States or the European Union. We use third parties to provide sales, warehousing, shipping, third-party logistics, invoicing, collections and other distribution services on our behalf in connection with the sale of its products globally. For example, there is currently a contract with a single specialty pharmacy distributor in the United States for the distribution of lomitapide and metreleptin, a single distributor in Brazil for both products, and single distributors, third-party logistics providers, importers and/or specialty pharmacies in certain other countries, including the European Union. We have entered into or may selectively seek to establish sales, distribution and similar forms of arrangements to grow revenue in existing territories and gain access to new territories and to reach patients in certain geographies that we do not believe can be cost-effectively addressed with our own sales and marketing capabilities. If we are unable to establish, maintain and finance the capabilities to sell, market and distribute our products, either through our own capabilities or through arrangements with third parties, and to effectively manage those third parties, or if we are unable to enter into distribution agreements in countries that we do not believe can be cost-effectively addressed with our own sales and marketing capabilities, we may not be able to successfully sell our products. We cannot guarantee that we will be able to establish, maintain and finance our own capabilities or to enter into and maintain favorable distribution agreements with third parties on acceptable terms, if at all.

To the extent that we enter into arrangements with third parties to perform sales, marketing or distribution services, the product revenues or the profitability of these product revenues may be lower than if we were to commercialize the products on our own. We will have limited control over such third parties, and any of them may fail to devote the necessary resources and attention to sell and market the products effectively, and may also, despite our compliance diligence reviews, audits and training, engage in non-compliant activities that, directly or indirectly, impact the use or sales of the products or damage our relationships with relevant stakeholders. Any performance failure, inability or refusal of our specialty pharmacy distributors, or third-party service providers to perform, or any failure to renew existing agreements on favorable terms, or at all, could cause serious disruption and impair our commercial or named patient sales of the products, which may have a material adverse effect on our business, financial condition and results of operations. Furthermore, the expenses associated with maintaining sales force and distribution capabilities may continue to be substantial compared to the revenues we generate. If we are unable to establish and effectively maintain adequate sales, marketing and

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distribution capabilities, whether independently or with third parties, we may not be able to generate product revenues, which may have a material adverse effect on our business, financial condition, results of operations and prospects. See “—Risks Related to our Reliance on Third Parties.”

The successful commercialization of our product candidates will depend in part on the extent to which governmental authorities and health insurers establish adequate coverage, reimbursement levels and pricing policies. Failure to obtain or maintain coverage and adequate reimbursement for our product candidates, if approved, could limit our ability to market those products and decrease revenue generating ability.

The availability and adequacy of coverage and reimbursement by governmental healthcare programs such as Medicare and Medicaid, private health insurers and other third-party payers is essential for many patients to be able to afford prescription medications such as our products and potential product candidates, assuming regulatory approval is obtained. Our ability to achieve acceptable levels of coverage and reimbursement for products by governmental authorities, private health insurers and other organizations will affect the success of our approved products and product candidates. Assuming we obtain coverage for our product candidates by third-party payers, the resulting reimbursement payment rates may not be adequate or may require co-payments that patients find unacceptably high. We cannot be sure that coverage and reimbursement in the United States, the EU Member States, or elsewhere will be available for the product candidates or any product that we may develop, and any reimbursement that may become available may be decreased or eliminated in the future.

Further, it is possible that a third-party payer may consider our product candidates as substitutes and only offer to reimburse patients for a less expensive product. Even if we show improved efficiency or convenience of administration with our product candidates compared to products marketed by our competitors and the prevailing SOC, the pricing of existing therapies may still limit the amount we could charge. Third-party payers may deny or revoke the reimbursement status of any given product or establish new prices for existing marketed products that inhibit us from realizing an appropriate return on our investment in the product candidates. If reimbursement is not available or is available only at limited levels, we may not be able to successfully commercialize our product candidates, and may not be able to obtain a satisfactory financial return on them.

Outside the United States, the success of our products and operations is subject to extensive governmental price controls and other market regulations which may materially and adversely affect our ability to generate commercially reasonable revenue and profits.

Our operations are subject to extensive governmental price controls and other market regulations in the United Kingdom and other countries outside of the United States. The increasing emphasis on cost-containment initiatives in the various EU Member States and other countries can put pressure on the pricing and usage of currently marketed products and product candidates in the future. In many countries, the prices of medical products are subject to varying price control mechanisms as part of national health systems. Other countries allow companies to fix their own prices for medical products, but monitor and control company profits. Additional foreign price controls or other changes in pricing regulation could restrict the amount that we are able to charge for our product candidates. Accordingly, in markets outside the United States, the reimbursement for our currently marketed products and our product candidates in the future may be reduced and may be insufficient to generate sufficient revenues and profits. Moreover, increasing efforts by governmental and third-party payers in the United States and abroad to control healthcare costs may cause such organizations to limit both coverage and the level of reimbursement for newly approved products and, as a result, they may not cover or provide adequate payment for our products, or any other product candidates we may develop in the future.

A number of adverse effects have been reported in clinical trials for metreleptin and lomitapide, and the prescribing information for each of lomitapide and metreleptin contains significant limitations on use and other important warnings and precautions, any of which could negatively affect the market acceptance, dropout rates and marketing approval for these products, and post-marketing commitments could identify additional adverse events and safety or efficacy risks, which could further harm our business.

The prescribing information for lomitapide in the United States and the European Union and in other countries in which lomitapide is approved contains significant limitations on use and other important warnings and precautions, including, but not limited to, a boxed warning in the JUXTAPID U.S. labeling, additional monitoring which is identified by a black inverted triangle in the product information for LOJUXTA in the European Union, warnings in the prescribing information for LOJUXTA citing concerns over liver toxicity

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associated with use of lomitapide and a U.S. Risk Evaluation Mitigation Strategy (“REMS”) program. The prescribing information for metreleptin in the United States and European Union contains important warnings and precautions, including but not limited to, a boxed warning on the MYALEPT label in the United States, citing the risk of anti-metreleptin antibodies with neutralizing activity, risk of lymphoma and a U.S. REMS program. As with the U.S. label, the MYALEPTA Summary of Product Characteristics in the European Union notes that the consequences of neutralizing antibodies with respect to the loss of efficacy or serious or severe infections is not well characterized but could reduce how well the leptin found naturally in the body works or how well MYALEPTA works.

Patients reported various adverse reactions in the Phase 3 study of lomitapide, including reports of gastrointestinal events by 93% of patients, and in the HoFH clinical trial, including diarrhea, nausea, vomiting, dyspepsia, abdominal pain, weight loss, abdominal discomfort, abdominal distension, constipation, flatulence, increased alanine aminotransferase, chest pain, influenza, nasopharyngitis, and fatigue. Elevations in liver enzymes and hepatic (liver) fat were also observed.

GL patients in the Phase 3 study of metreleptin reported various adverse drug reactions, including weight loss, hypoglycemia, decreased appetite, fatigue, neutralizing antibodies and alopecia. Additionally, although none were assessed as drug related, there were four reported treatment-emergent deaths over the course of the 14-year study duration. Upon further investigation, these reports were consistent with the underlying morbidity of lipodystrophy and included renal failure, cardiac arrest (with pancreatitis and septic shock), progressive end-stage liver disease (chronic hepatic failure), and hypoxic-ischemic encephalopathy. In the open-label, long-term, investigator-sponsored study of metreleptin for the treatment of metabolic disorders associated with lipodystrophy syndromes (initiated in 2000 and conducted at the National Institutes of Health (“NIH”)), there were two cases of peripheral T-cell lymphoma and one case of a localized anaplastic lymphoma (kinase-positive anaplastic large cell lymphoma, which is a type of T-cell lymphoma). Both of the cases of peripheral T-cell lymphoma were reported in patients with acquired GL, and both had evidence of pre-existing lymphoma and/or bone marrow/hematologic abnormalities before metreleptin therapy. A third case of anaplastic large cell lymphoma occurred in the context of a specific chromosomal translocation.

These adverse events, coupled with the boxed warnings and other label restrictions, could cause healthcare providers, regulators and patients or potential patients to view the risks associated with our products as outweighing the benefits. This could cause patients to discontinue use and limit the number of new patients, thereby negatively affecting our business, financial condition, results of operations and prospects. In addition, as part of the post-marketing commitment to the FDA for both lomitapide and metreleptin, we are conducting post-marketing registries to better understand their long-term safety and effectiveness. For lomitapide, we are conducting an observational cohort study to better understand the long-term safety, patterns of use, compliance and long-term effectiveness of controlling LDL levels. For metreleptin, we are conducting a long-term, prospective, observational study (product exposure registry) to identify and better understand any serious risks related to the use of the product. Finally, we are conducting sequential programs to expand the understanding of metreleptin immunogenicity and manufacturing. The final program regarding the immunogenicity of metreleptin was initiated in 2018, and the two final post-marketing studies related to the manufacturing of metreleptin are expected to be completed by the deadlines set by the FDA. In addition, we are working to implement post-marking commitments in the European Union for metreleptin, including a pediatric study in GL patients, an immunogenicity program, a post-approval study in PL and a European product exposure registry. A failure to meet post-marketing commitments to the FDA, the EMA or other regulatory authorities could impact the ability to continue to market lomitapide or metreleptin, respectively, in countries where we are unable to meet such commitments.

In the course of conducting observational cohort studies, additional clinical studies (such as pursuant to a pediatric investigation plan (“PIP”) in the European Union), post-marketing surveillance, or re-evaluation of any completed clinical study data could identify, additional safety information on known or unknown side effects or new undesirable side effects caused by our products or product candidates, or the data may raise other issues with respect to the products. In such instances, a number of potentially significant negative consequences could result, including:

• we may experience a negative impact on market acceptance and increased dropout rates;

• regulatory authorities may suspend, withdraw or alter their approval of the relevant product;

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• regulatory authorities may require the addition of labeling statements, such as warnings or contraindications or distribution and use restrictions such as, for example, the modifications to the JUXTAPID label to include language instructing patients to cease therapy upon the occurrence of severe diarrhea;

• regulatory authorities may issue, or require us to issue additional specific communications such as safety alerts, field alerts, or “Dear Doctor” letters to healthcare professionals;

• regulatory authorities may require us to recall, withdraw, or stop selling a product or take other enforcement action;

• we may receive negative publicity;

• we may be required to change the way the relevant product is administered, conduct additional preclinical studies or clinical trials or restrict the distribution or use of the relevant product;

• patients could suffer harm, and we could be sued and held liable for harm caused to patients;

• the regulatory authorities may require us to amend the relevant REMS program, Risk Management Plan or comparable equivalent; and

• our reputation may suffer.

Any known safety concerns for our products or product candidates, or any unknown safety issues that may develop or be discovered, including drug interaction problems or an increase in the severity or frequency of known adverse events or the discovery of previously unknown adverse events, or the evaluation or reevaluation of study data, could prevent us from achieving or maintaining market acceptance of the respective product, affect our ability to obtain or retain regulatory marketing approval of the respective product in one or more countries, result in onerous restrictions on such approval or the implementation or modification of the REMS programs (if applicable) or risk management plans for our products, or any other enforcement actions, result in claims, lawsuits and increased regulatory scrutiny, and affect our ability to achieve our financial goals.

Enacted and future legislation and related regulations may increase the difficulty and cost for us to commercialize metreleptin or lomitapide or development candidates and may affect the prices we are able to obtain for our products, if and where approved.

In the United States, there have been a number of legislative and regulatory changes and proposed changes regarding the healthcare system that restrict or regulate post-approval activities, which may affect our ability to profitably sell our products. Legislative and regulatory proposals have been made to expand post-approval requirements and restrict sales and promotional activities for pharmaceutical products. We cannot predict whether additional legislative changes will be enacted, or whether the FDA regulations, guidance or interpretations will be changed, or what the impact of such changes for the products may be. In addition, increased scrutiny by Congress of the FDA’s approval process may subject us to more stringent product labeling, post-marketing testing and other requirements. In May 2019, the Centers for Medicare and Medicaid Services adopted a final rule permitting Medicaid Part D plans to apply certain utilization controls on new starts of protected class drugs.

In the United States, most outpatient prescription drugs, including MYALEPT and JUXTAPID, may be covered under Medicare Part D. Medicare Part D prescription drug plans are authorized to use formularies where they can limit the number of drugs that will be covered in any therapeutic class and impose differential cost sharing or other utilization management techniques. The possibility that our products and product candidates will be subject to such formularies places pressure on them to contain and reduce costs, which could negatively impact our commercialization efforts. Changes to Medicare Part D, which give plans more freedom to limit coverage or manage utilization, and other cost reduction initiatives in the program could decrease the coverage and price that we receive for any approved products, and could be detrimental to our business.

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The Patient Protection and Affordable Care Act (“PPACA”) (as amended by the Health Care and Education Reconciliation Act of 2010) substantially changed the way healthcare is financed by both governmental and private insurers. The law contains a number of provisions that affect coverage and reimbursement of drug products and that could potentially reduce the demand for our products, such as:

• increasing drug rebates under state Medicaid programs for brand name prescription drugs and extending those rebates to Medicaid managed care; and

• requiring drug manufacturers to provide a 50% discount on Medicare Part D brand name prescription drugs sold to Medicare beneficiaries whose prescription drug costs cause the beneficiaries to be subject to the Medicare Part D coverage cap (i.e., the so-called donut hole).

Modifications to or repeal of all or certain provisions of the PPACA has been a considerable focus of President Trump’s administration, and certain changes have already been approved and other changes may be pursued. We cannot predict the ultimate content, timing or effect of any changes to the PPACA or other federal and state reform efforts. There is no assurance that federal or state healthcare reform will not adversely affect our business and financial results, and we cannot predict how future federal or state legislative, judicial or administrative changes relating to healthcare reform will affect our business.

Any significant spending reductions affecting Medicare, Medicaid or other publicly funded or subsidized health programs that may be implemented and/or any significant taxes or fees that may be imposed, as part of any broader deficit reduction effort or legislative replacement to current laws or regulations, could have an adverse impact on our results of operations. In addition, countries outside the United States may make changes to their healthcare systems, which may in the future affect the revenue generated from sales of lomitapide and metreleptin or any of our future commercial products.

Recent legislation and proposed federal regulations and guidance may permit reimportation of drugs from foreign countries into the United States where the drugs are sold at lower prices and this may adversely affect our operating results and overall financial condition.

The U.S. Medicare Prescription Drug, Improvement, and Modernization Act of 2003 (“MMA”) contains provisions that may change importation laws and expand pharmacists’ and wholesalers’ abilities to import lower-priced versions of an approved drug and competing products from Canada, where there are government price controls. These changes to U.S. importation laws will only take effect if the Secretary of Health and Human Services certifies that the changes will pose no additional risk to the public’s health and safety. We do not know the timing and likelihood of this certification.

In December 2019, the U.S. Department of Health and Human Services and the FDA issued a proposed rule and draft guidance concerning two new pathways for importing lower-cost drugs into the United States. The proposed rule, if finalized, would allow certain prescription drugs to be imported from Canada, but would not permit the import of biologics. The draft guidance describes procedures for drug manufacturers to facilitate the importation of FDA approved drugs and biologics manufactured abroad and originally intended for sale in a foreign country in the United States.

If distributors or other purchasers of MYALEPT or JUXTAPID in the United States are able to import lower-priced products from countries outside the United States that place price controls on pharmaceutical products, this may result in a negative impact on the revenues of our products. In addition, some state governments have implemented importation schemes for their citizens and, in the absence of federal action to curtail such activities, other state governments may launch importation efforts.

The reimportation of metreleptin or lomitapide into the U.S. market from a foreign market may negatively impact our revenues and anticipated financial results.

Although the European Union does not permit the re-importation of medicinal products from outside the European Union, parallel trade between EU Member States is possible and can result in third party imports from EU Member States offering lower prices for a product into those reimbursing products at higher costs.

We rely on named patient sales of our products in certain territories, but there are no assurances that named patient sales of our products will continue at current levels, or at all.

In Brazil, Turkey and a limited number of other countries where permitted based on U.S. or EU approval, metreleptin and lomitapide are available on a named patient sales or similar basis. Named patient basis means

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physician-requested treatment for patients in territories where marketing authorization has not yet occurred. There is no assurance that named patient sales will continue to be authorized in any particular country. Even if they are authorized, we will likely not be permitted to promote, market or otherwise engage in proactive selling activities for products sold on a named patient basis, which makes named patient sales much less predictable, and susceptible to unexpected decreases. If violations of any laws or governmental regulations are found to have occurred in connection with our products significant criminal or civil lawsuits may be filed, or investigations may be commenced. For example, in Brazil, under certain circumstances, we could be barred from further named patient sales of their products to federal or state governments in Brazil due to penalties imposed by Brazilian regulatory authorities or through civil actions initiated by federal or state public prosecutors, or we could face administrative penalties imposed by Brazilian regulatory authorities and additional damages and fines. It is believed that the investigations in respect of Aegerion in Brazil have contributed to a slower turnaround between price quotation and orders, including reorders, from the federal government, and, in some cases, delays in orders and reorders from the government of the State of São Paulo after a patient has obtained access to JUXTAPID through the judicial process. These delays may continue, and we may experience other delays or suspensions of the ordering process. Similarly, there has been, and may continue to be, some reluctance by physicians to prescribe JUXTAPID, and some patients to take or stay on JUXTAPID, while the investigations are ongoing, particularly given that some of the investigators in Brazil made formal inquiries of certain prescribers of JUXTAPID, and there has been significant local media coverage of such inquiries and Aegerion’s past activities in Brazil. Further, in October 2017, a new set of regulatory requirements governing use of product candidates was published in Brazil which has added complexity to the process for the purchase, on a named patient basis, of drugs which have not received regulatory and/or pricing and reimbursement approval in Brazil, such as metreleptin and lomitapide, which has, along with the ongoing court proceeding, resulted in delays in the receipt of orders from Brazil for existing metreleptin and lomitapide patients. We believe that this has led certain patients to discontinue therapy with metreleptin and lomitapide. Aegerion filed in Brazil for regulatory approval for JUXTAPID in August 2018. However, the approval process can be lengthy, even with the new regulation that aims at expediting the review process of new drugs for the treatment of rare diseases, and there is no guarantee that we will be able to obtain such approval. As a result, we may have to rely on our ability to generate named patient sales for a considerable amount of time, or indefinitely. These factors could significantly negatively affect product revenues from named patient sales of products in Brazil.

We do not know the full extent of the impact that the approval of PCSK9 inhibitor products in the United States, or the approval of a PCSK9 inhibitor product, will have on the named patient sales of lomitapide in Brazil or other countries. We also do not know whether we will be permitted to sell metreleptin or lomitapide on a named patient basis in any additional countries. In certain countries, we may decide not to pursue named patient sales even if permitted. Even if named patient sales (or equivalent sales) are permitted in a certain country, and we elect to make metreleptin or lomitapide available on such basis, there is no guarantee that physicians in such country will prescribe the product, which they can only do if they proactively reach out to us or our distributors and also undertake the effort, time and cost of following the stringent local requirements to get their patient on therapy on a named patient basis, and that patients will be willing to start and adhere to therapy, or that the country will pay for the product at all, or at a level that is acceptable to us, without delay or imposing other hurdles on payment. These risks may be heightened in Brazil for the reasons outlined above and also in light of the 2016 approval of a PCSK9 inhibitor product in Brazil.

Further, there are countries where we choose to make our products available under an expanded access program at no cost prior to approval in such countries. There is no assurance that we will be able to obtain marketing approval or reimbursement at all or at acceptable levels or to maintain reimbursement for our products in any country where we have expanded access programs or that patients on such programs will convert to commercial product even if we do obtain requisite approvals. In certain countries where we seek reimbursement for the product during the pre-approval phase, we are able to establish the price for the product, while in other countries we need to negotiate the price. Such negotiations may not result in a price acceptable to us, in which case we may elect not to distribute the products in such country prior to approval or it may curtail distribution. Our expanded access program may result in significant expenses and may not result in expected future sales at desired levels or at all, and could negatively impact our financial results.

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Risks Related to Clinical Development

If we are unable to complete clinical development of AP101, or experience significant delays in doing so, our business could be materially harmed.

Our lead product candidate, AP101, is currently in a pivotal Phase 3 trial (EASE) to assess its efficacy and safety in treating patients with dystrophic and junctional EB. In January 2019, the DSMB performed an unblinded interim efficacy analysis and recommended continuing the EASE trial with an increase in enrollment from 182 patients to 230 evaluable patients to maintain 80% conditional statistical power. As a result of the COVID-19 pandemic which has had a material impact on recruitment into clinical trials globally, and given that the EASE trial was already close to full enrollment, we have taken advice from an independent expert and concluded that the statistical impact of further patient recruitment would most likely be negligible. We therefore decided to close the EASE trial to further enrollment in April 2020 and expect to report top line results from the EASE trial in the second half of 2020.

The EASE trial requires the investment of substantial expense and time, and it may be subject to significant delays relating to various causes, including difficulties in identifying and enrolling additional patients who meet trial eligibility criteria, failure of patients to complete the clinical trial, unexpected adverse events and failure to achieve specified endpoints. If we are unable to complete the EASE trial and any required additional testing of AP101 in a timely manner or at all, it will be difficult or impossible for us to receive regulatory approval and we will be unable to commercialize AP101. Moreover, the continuation of the EASE trial does not guarantee that we will successfully further develop, commercialize or receive regulatory approval for AP101. Our inability to obtain approval for and commercialize AP101 would materially adversely affect our business, results of operations and prospects.

Clinical trials are expensive, time consuming and difficult to design and implement and involve uncertain outcomes and, furthermore, results of earlier preclinical studies and clinical trials may not be predictive of results of future preclinical studies or clinical trials.

To obtain the requisite regulatory approvals to market and sell any of our product candidates, or to obtain regulatory approvals to market and sell any of our commercial products for new indications, we must demonstrate, through extensive preclinical studies and clinical trials, that our product candidates are safe and effective in humans. Clinical testing is expensive and can take many years to complete and has inherently uncertain outcomes. Failure can occur at any time during the clinical trial process and in addition regulatory authorities may require further studies at additional cost. Furthermore, regulatory authorities may not agree on the same trial design for pivotal studies. The results of preclinical studies and earlier clinical trials, or the results from earlier stages of preclinical studies or clinical trials, may not be predictive of the results of later-stage clinical trials. For example, the results generated to date in preclinical studies or Phase 1 or Phase 2 clinical trials for product candidates do not ensure that later clinical trials will demonstrate similar results. Product candidates in later stages of clinical trials may fail to show the desired safety and efficacy outcomes despite having progressed through preclinical studies and initial clinical trials. We may suffer setbacks in advanced clinical trials due to lack of efficacy or adverse safety profiles, notwithstanding any promising results in earlier clinical trials. As product candidates are developed from preclinical through early to late stage clinical trials towards approval and commercialization, it is customary that various aspects of the development program, such as manufacturing and methods of administration, are altered along the way in an effort to optimize processes and results. While these types of changes are common and are intended to optimize the product candidates for late stage clinical trials, approval and commercialization, such changes carry the risk that they will not achieve these intended objectives. In addition, we may experience delays in ongoing or future preclinical studies or clinical trials and we have no certainty as to whether future preclinical studies or clinical trials will begin on time, will need to be redesigned, will enroll an adequate number of subjects or patients on time, if at all, or will be completed on schedule, if at all. Such factors may have a material adverse effect on our business, financial condition, results of operations and prospects.

Our product candidates may not work as intended, may cause undesirable side effects or may have other properties that could delay or prevent their regulatory approval, limit the commercial profile of an approved label, or result in significant negative consequences following marketing approval, if any.

Use of our product candidates could be associated with side effects or adverse events which can vary in severity from minor reactions to serious and/or severe adverse events, and in frequency from infrequent to prevalent. Undesirable side effects or unacceptable toxicities caused by our product candidates could cause us or regulatory

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authorities to interrupt, delay or halt clinical trials and could result in a more restrictive label or the delay or denial of regulatory approval by the FDA, the EMA or comparable regulatory authorities. Results of our trials could reveal a high and unacceptable severity and prevalence of side effects.

If unacceptable side effects arise in the development of our product candidates, we, the FDA, competent authorities of EU Member States, ethics committees, the institutional review boards, at the institutions in which our studies are conducted, or the DSMB, could suspend or terminate our clinical trials. The FDA or comparable regulatory authorities could also order us to cease clinical trials or deny approval of our product candidates for any or all targeted indications. Treatment-related side effects could also affect patient recruitment or the ability of enrolled patients to complete any of our clinical trials or result in potential product liability claims. In addition, these side effects may not be appropriately recognized or managed by the treating medical staff. We expect to have to train medical personnel using our product candidates to understand the side effect profiles of our product candidates in our clinical trials and upon any commercialization of any of our product candidates. Inadequate training in recognizing or managing the potential side effects of our product candidates could result in patient injury or death. Any of these occurrences may harm our business, financial condition, results of operations and prospects significantly.

The regulatory approval processes of the EMA, the FDA and other comparable regulatory agencies may be lengthy and time-consuming, and the outcome is unpredictable.

Our future success is partly dependent upon our ability to successfully develop, obtain regulatory approval for, and commercialize one or more of our product candidates. There can be no assurance that any development product candidates will be successful in clinical trials or receive regulatory approval. We cannot predict with certainty if or when we might submit for regulatory approval of any of our product candidates currently under development. Any approvals we may obtain may not cover all of the clinical indications for which we are seeking approval. Also, an approval might contain significant limitations in the form of narrow indications, warnings, precautions, or contra-indications with respect to conditions of use. Applications for any of our product candidates could fail to receive regulatory approval for many reasons, including, but not limited to, the following:

• the EMA, the FDA or any other comparable regulatory agency may disagree with the design or implementation of clinical trials or interpretation of data from non-clinical trials or clinical trials;

• the population studied in the clinical program may not be sufficiently broad or representative to ensure that the clinical data can be relied on safely in the full population for which we are seeking approval;

• the data collected from clinical trials of our product candidates may not be sufficient to support a finding that has statistically significant clinical meaningfulness or support the submission of a new drug application or other submission, or to obtain regulatory approval in relevant jurisdictions, such as the European Union and the United States;

• we may be unable to demonstrate to the EMA, the FDA or any other comparable regulatory agency that a product candidate’s risk-benefit ratio for its proposed indication is acceptable;

• the EMA, the FDA or any other comparable regulatory agency may fail to approve the manufacturing processes, test procedures and specifications or facilities of third-party manufacturers with which we contract for clinical and commercial supplies; and

• the approval policies or regulations of the EMA, the FDA or any other comparable regulatory agency may significantly change in a manner rendering clinical data insufficient for approval.

Disruptions at the FDA, the EMA and other regulatory agencies may also slow the time necessary for new drugs to be reviewed and/or approved by necessary government agencies, which would adversely affect our business. For example, in recent years, including in 2018 and 2019, the U.S. government shut down several times and certain regulatory agencies, such as the FDA, had to furlough critical employees and stop critical activities. Separately, in response to the COVID-19 pandemic, on March 10, 2020 the FDA announced its intention to postpone most inspections of foreign manufacturing facilities and products through April 2020. On March 18, 2020, the FDA announced its intention to temporarily postpone routine surveillance inspections of domestic manufacturing facilities and provided guidance regarding the conduct of clinical trials. Regulatory authorities outside the United States may adopt similar restrictions or other policy measures in response to the COVID-19 pandemic. If a prolonged government shutdown occurs, or if global health concerns continue to prevent the FDA

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or other regulatory authorities from conducting their regular inspections, reviews, or other regulatory activities, it could significantly impact the ability of such regulatory authorities to timely review and process our regulatory submissions, which could have a material adverse effect on our business. Further, in our operations as a public company, future government shutdowns or delays could impact our ability to access the public markets and obtain necessary capital in order to properly capitalize and continue our operations.

Any of our current or future product candidates could take a significantly longer time to gain regulatory approval than expected or may never gain regulatory approval. This could delay or eliminate any potential product revenue by delaying or terminating the potential commercialization of product candidates. For example, any centralized marketing authorization application made to the EMA involving ‘Advanced Therapy Medicinal Products’ (such as AP103) will be subject to scientific evaluation by the Committee for Advanced Therapies, in addition to the Committee for Medicinal Products for Human Use (“CHMP”).

We intend to seek regulatory approvals to commercialize the product candidates in the United States and the European Union. To obtain regulatory approval in other countries, we must comply with numerous and varying regulatory requirements of such other jurisdictions, which may include (without limitation) safety, efficacy, chemistry, manufacturing and controls, clinical trials, commercial sales, pricing and distribution of product candidates. Even if we are successful in obtaining approval in one jurisdiction, there can be no guarantee that it will obtain approval in other jurisdictions. Failure to obtain any marketing authorizations for the product candidates will result in us being unable to market and sell such products. If we fail to obtain approval in any jurisdiction, the geographic market for the product candidates could be limited. Similarly, regulatory agencies may not approve the labelling claims that are necessary or desirable for the successful commercialization of the product candidates.

We may fail to obtain or maintain Orphan Drug marketing exclusivity for our products and product candidates, and may face significant competitive threats to the commercialization of these compounds from other manufacturers.

Obtaining Orphan Drug Designation does not guarantee that we will be the first to obtain marketing approval for any particular orphan indication due to the uncertainties associated with developing pharmaceutical products, which could prevent us from marketing the product candidates if another company is able to obtain Orphan Drug exclusivity before we do. Exclusive marketing rights in the United States may be unavailable or lost if: (i) an approval is sought for an indication broader than the orphan-designated indication; (ii) the FDA later determines that the request for designation was materially defective; or (iii) the applicant is unable to secure sufficient quantities of the drug to meet the needs of patients with the rare disease or condition following approval. In the European Union, a marketing authorization granted for an Orphan Drug must only cover the therapeutic indications that meet the Orphan Drug Designation criteria. Further, even if Orphan Drug exclusivity has been obtained, and maintained that exclusivity may not effectively protect the product candidates from competition because different drugs with different active moieties may be approved for the same condition. In addition, the FDA and the EMA may subsequently approve products with the same active moiety for the same condition if the FDA or the EMA concludes that the later drug is safer, more effective, or makes a major contribution to patient care. Orphan Drug Designation neither shortens the development time or regulatory review time of a drug nor gives the drug any advantage in the regulatory review or approval process.

We intend to seek Orphan Drug Designation for existing and future product candidates and we may never receive such designations. Despite exclusivity protections, another company nevertheless could market another version of the product if such company submits a full New Drug Application (“NDA”) in the United States or a full application for marketing authorization in the European Union with a complete human clinical trial program and obtains marketing approval of its product. Failure by us to obtain or maintain Orphan Drug marketing exclusivity for our products and product candidates may have a material and adverse effect on our business, financial condition, results of operations and prospects.

The FDA’s Orphan Drug exclusivity regulations may face legal challenges that could lead to changes which might adversely affect our business.

There have been legal challenges to aspects of the FDA’s regulations and policies concerning the exclusivity provisions of the U.S. Orphan Drug Act of 1983, and future challenges could lead to changes that affect the protections afforded to our product candidates in ways that are difficult to predict and which may have a material

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adverse effect on their business, financial condition, results of operations and prospects. In 2014, a U.S. district court invalidated the FDA’s denial of orphan exclusivity to an orphan designated drug, which the FDA had based on its determination that the drug was not proven to be clinically superior to a previously approved “same drug.” In response to the decision, the FDA released a policy statement stating that the court’s decision is limited to the facts of that particular case and that the FDA will continue to deny Orphan Drug exclusivity to a designated drug upon approval if the drug is the “same” as a previously approved drug, unless the drug is demonstrated to be clinically superior to that previously approved drug. Since then, similar legal challenges have been initiated against the FDA for its denial of Orphan Drug exclusivity to other designated drugs, and in 2017, Congress amended the U.S. Orphan Drug Act of 1983 to require a demonstration of clinical superiority upon approval as a condition to receiving Orphan Drug exclusivity when another “same drug” has already been approved for the same indication. In the future, there is the potential for additional legal challenges to the FDA’s Orphan Drug regulations and policies, and it is uncertain how ongoing and future challenges might affect our business.

We may seek and fail to obtain Fast Track or breakthrough therapy designations for our current or future product candidates. If we are successful, these programs may not lead to a faster development or regulatory review process, and they do not guarantee we will receive approval for any product candidate.

If a product is intended for the treatment of a serious or life-threatening condition and preclinical or clinical data demonstrate the potential to address an unmet medical need for this condition, the product sponsor may apply for Fast Track Designation. The FDA has broad discretion whether or not to grant this designation, so even if we believe a particular product candidate is eligible for this designation, we cannot assure you that the FDA would decide to grant it. We have received Fast Track Designation for AP101. However, Fast Track Designation does not mean we will necessarily experience a faster development process, review or approval compared to conventional FDA procedures. The FDA may rescind the Fast Track Designation if it believes that the designation is no longer supported by data from our clinical development program.

Risks Related to Government Regulation and Compliance

The laws and regulations in the areas of sales and marketing of pharmaceutical products, and interacting with healthcare professionals and patients, are very complex and onerous, and require a robust compliance program. Failure to comply with these laws and regulations could have a material adverse effect on our business, financial condition and results of operations.

Failure to comply with certain laws and regulations could lead to governmental investigations and result in financial penalties and remedial and compliance measures. For example, compliance failures by Aegerion led to a DOJ investigation and ultimately resulted in three separate settlements (Corporate Integrity Agreement, Consent Decree and Deferred Prosecution Agreement) with multiple government agencies (Office of Inspector General (“OIG”), the FDA and DOJ) and aggregate penalties of approximately $40.1 million, which include $7.2 million in restitution payable over three years beginning January 2018, a civil penalty of $4.1 million to be paid to the SEC pursuant to the SEC judgment, and $28.8 million to be paid pursuant to the settlement agreement with the DOJ. Aegerion had been making the required payments and following the Acquisition we have assumed responsibility for payment. Pursuant to the settlement, we are also required to maintain various remedial and compliance measures, which were implemented as required by the settlement. We may be unsuccessful in implementing and complying with all of the elements of the settlement in a timely or satisfactory manner, or at all. Failure to comply with any provisions of these settlements could result in the imposition of additional fines, penalties and obligations by the applicable government agency, and could subject us to prosecution.

Furthermore, the investigation by the Brazilian authorities of Aegerion’s activities could result in the commencement of formal proceedings, and if the investigation finds any violation of any laws or governmental regulations, then our Brazilian subsidiary may be subject to civil lawsuits and administrative penalties and other potential damages and fines. Under certain circumstances, the Brazilian subsidiary and our company could be barred from further sales to federal or state governments in Brazil, including sales of JUXTAPID or MYALEPTA, due to penalties imposed by Brazilian regulatory authorities or through civil actions initiated by federal or state public prosecutors.

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We are subject to extensive legal and compliance obligations as a pharmaceutical company that commercializes products, as well as under Aegerion’s settlements with the DOJ, OIG, FDA, SEC and other federal and state government agencies.

As a pharmaceutical company that develops and commercializes pharmaceutical products, we are subject to an extensive array of broad and complex laws and regulations. These include, without limitation, regulations and laws in the United States and outside the United States related to manufacturing, clinical, quality, drug safety, commercialization, payments to and interactions with healthcare professionals and healthcare organizations, anti-kickbacks, fraud and abuse, the requirement to report payments and other transfers of value to healthcare professionals and healthcare organizations, data protection and privacy, pricing, reimbursement, price reporting, anti-corruption and anti-bribery, and a myriad of other areas and levels of regulation. Any failure by us or our key vendors, contractors, distributors, licensors or other key third-party vendors or service providers to comply with such laws and regulations could have a material adverse effect on our results of operations and financial condition, could result in product approvals being suspended, withdrawn, delayed or denied, could result in litigation or investigations which could be costly and be a significant distraction to executive management and other employees, and could result in damages or prosecution.

In September 2017, Aegerion entered into various settlements with the DOJ, OIG, FDA and SEC (“Aegerion Settlements”) of investigations regarding Aegerion’s U.S. commercial activities and disclosures related to JUXTAPID, as described in more detail in Note 15 to the Consolidated Financial Statements of Aegerion included in this prospectus. Under the terms of the Aegerion Settlements, Aegerion was required to pay approximately $40.1 million in penalties, which include $7.2 million in restitution payable over three years beginning January 2018, a civil penalty of $4.1 million to be paid to the SEC pursuant to the SEC judgment, and $28.8 million to be paid pursuant to the settlement agreement with the DOJ. Such payments are subject to acceleration in the event of certain change of control transactions or the sale of JUXTAPID or MYALEPT or if we elect to transfer the licenses to another group entity. We assumed these obligations in connection with the Acquisition and have been making these penalty payments in accordance with the terms of the Aegerion Settlements. If we fail to satisfy obligations under the Aegerion Settlements, any outstanding amount will accrue interest at a rate of 1.75% per annum, and we may be subject to further penalties or litigation. The Aegerion Settlements also mandates extensive remedial and compliance measures. The failure to comply with any provisions of the Aegerion Settlements, including the financial, remedial and compliance measures, could result in the imposition of additional fines, penalties and obligations, and could subject us to prosecution or exclusion from federal healthcare programs in the United States.

The FDA, the EU Member States and other regulatory agencies outside the United States and the European Union enforce laws and regulations governing drug marketing and promotional activities, including prohibiting the promotion of off-label uses. Violations of these laws and regulations, and the resulting enforcement actions by these agencies, can result in significant liability.

The FDA, the regulatory authorities of the EU Member States and other comparable regulatory agencies outside the United States and the European Union strictly regulate the promotional claims that may be made about prescription drug products. In particular, a drug product may not be promoted in a jurisdiction prior to obtaining regulatory marketing approval or for uses that are not approved by the FDA, the European Commission (“EC”), the regulatory authorities of the EU Member States or such other regulatory agencies, as applicable, as reflected in the product’s approved prescribing information or summary of product characteristics. Since the United Kingdom has left the European Union and the negotiations regarding a long-term trade agreement are still ongoing, it is not entirely clear how the regulatory landscape with regard to the United Kingdom will evolve and which risks this will entail. During the current transition period, which lasts until the end of 2020 (but which can be extended by agreement), the general applicability of the EU pharmaceutical laws to the United Kingdom remains unchanged and all EU agencies continue to hold the powers conferred upon them by EU law (including among other things EU regulations on drug marketing). In the United States, marketing and promotion of drug products is regulated by the FDA and the Federal Trade Comission (“FTC”) to ensure that any claims about such products are consistent with regulatory approvals, not misleading or false in any particular way, and adequately substantiated by clinical data. In the United States and the European Union, the promotions of a drug product in a manner that is false, misleading, unsubstantiated, or for unapproved (or off-label) uses may result in enforcement letters or notices, inquiries and investigations, and civil and criminal sanctions by the FDA, FTC and regulatory authorities of the EU Member States (as applicable). Promotion of products for off-label uses in the United States can also result in false claims litigation under federal and state statutes, which can lead to

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consent decrees, civil monetary penalties, restitution, criminal fines and imprisonment, and exclusion from participation in Medicare, Medicaid and other federal and state healthcare programs. Aegerion was the subject of such investigations and enforcement in connection with the investigations into JUXTAPID and the related Aegerion Settlements. Cooperation with such investigations and negotiation of, and compliance with, the settlement has been and will continue to be costly and burdensome on our management and other resources.

Any future investigations and litigation involving us could have a material adverse effect on our business, reputation, financial condition, results of operations and prospects. It could also distract our management from operating the business and may be disruptive to employees, leading to further employee attrition. In addition, the Aegerion Settlements have impacted, and may continue to impact, our reputation and the willingness of some physicians to prescribe our licensed products.

If we fail to comply with UK, EU or U.S. privacy and data security laws and regulations, we may be subject to civil and criminal penalties and other liability.

We are also subject to laws and regulations covering data privacy and the protection of health-related and other personal information. The legislative and regulatory landscape for privacy and data protection continues to evolve, and there has been an increasing focus on privacy and data protection issues which may affect our business, including recently enacted laws in many jurisdictions where we operate. The collection and use of personal health data in the European Union is governed by the provisions of the General Data Protection Regulation (EU) 2016/679 (“GDPR”). This directive, which is wide-ranging in scope and includes extraterritoriality provisions that apply to certain entities located outside of the European Union, imposes several requirements relating to the consent of the individuals to whom the personal data relates, the information provided to the individuals, notification of data processing obligations to the competent national data protection authorities and the security and confidentiality of the personal data, and substantial fines for breaches of the data protection rules. The GDPR also imposes strict rules on the transfer of personal data out of the European Union to other countries (including the United States). Failure to comply with the requirements of the GDPR and the related national data protection laws of the EU Member States and the United Kingdom may result in large fines and other administrative penalties: a failure to comply could result in fines up to the greater of 4% of annual worldwide turnover for the preceding financial year or €20 million, with infringements being grouped into tiers which attract different maximum fine levels. Turnover in this context may include not only the entity in breach but also other group entities. Recent enforcement actions against multinational companies have resulted in significant fines. Following the United Kingdom’s formal departure from the European Union on January 31, 2020, the United Kingdom entered a transition period until December 31, 2020, during which time the GDPR will remain applicable to the United Kingdom, alongside the United Kingdom’s Data Protection Act 2018 (“DPA 2018”). At the end of the transition period, there is uncertainty regarding the future relationship between the United Kingdom and the European Union in relation to data protection. Once the United Kingdom leaves the European Union and the transition period has expired, the United Kingdom will become a “third country” for the purposes of data protection law. A “third country” is a country other than the EU Member States and the three additional European Economic Area countries (Norway, Iceland and Liechtenstein) that have adopted a national law implementing the GDPR. Under the GDPR, personal data can only be transferred to third countries in compliance with specific conditions for cross-border data transfers. Appropriate safeguards are required to enable transfers of personal data from the European Union and EEA Member States. This status has a number of significant practical consequences, in particular for international data transfers, competent supervisory authorities and enforcement of the GDPR. In the United Kingdom, the DPA 2018 supplements the GDPR, and in particular sets out specific requirements related to the processing of “special categories of personal data,” including personal data related to health, genetic information and personal data related to criminal offenses or convictions. The DPA also creates a number of criminal offenses (punishable by uncapped fines) for organizations and in certain cases their directors and officers. While we have taken steps to comply with the GDPR and the DPA 2018, we cannot assure you that our efforts to achieve and remain in compliance have been or will continue to be fully successful. The GDPR regulations and the DPA 2018 may impose additional responsibility and liability in relation to personal data that we process and we may be required to put in place additional mechanisms ensuring compliance with these or new data protection rules. This may be onerous and adversely affect our business, financial condition, results of operations and prospects.

In addition, we obtain patient health information from most healthcare providers that prescribe our products and research institutions with which we collaborate, and they are subject to privacy and security requirements under the Health Insurance Portability and Accountability Act of 1996 (“HIPAA”) in the United States. Although we

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are not directly subject to HIPAA other than with respect to providing certain employee benefits, we could potentially be subject to criminal penalties if we knowingly obtain or disclose individually identifiable health information maintained by a HIPAA-covered entity in a manner that is not authorized or permitted by HIPAA.

Failure to comply with healthcare laws and laws and regulations covering data privacy and the protection of health-related and other personal information could result in government enforcement actions, which could include civil or criminal penalties, private litigation and adverse publicity and could negatively affect our business, financial condition, results of operations and prospects.

Our relationships with customers and payers in the United States are subject to applicable anti-kickback, fraud and abuse and other healthcare laws and regulations, any breaches of which could expose us to criminal sanctions, civil penalties, contractual damages and reputational harm, could diminish future earnings and could prevent us from achieving our expected financial results.

Our arrangements with third-party payers and customers in the United States expose us to broadly applicable fraud and abuse and other healthcare laws and regulations, including the federal healthcare Anti-Kickback Statute, the False Claims Act, HIPAA and the Physician Payment Sunshine Act, and similar state and foreign laws and regulations that may regulate the business or financial arrangements and relationships through which we market, sell and distribute our products. The number and complexity of both federal and state laws continue to increase, and additional governmental resources are being used to enforce these laws and to prosecute companies and individuals who are believed to be violating them.

In March 2010, the PPACA was passed, which substantially changes the way healthcare is financed by both the government and private insurers, and significantly impacts the U.S. pharmaceutical industry. The PPACA, among other things: (i) addresses a new methodology by which rebates owed by manufacturers under the Medicaid Drug Rebate Program are calculated for drugs that are inhaled, infused, instilled, implanted or injected; (ii) increases the minimum Medicaid rebates owed by manufacturers under the Medicaid Drug Rebate Program and extends the rebate program to individuals enrolled in Medicaid managed care organizations; (iii) establishes annual fees and taxes on manufacturers of certain branded prescription drugs; (iv) expands the availability of lower pricing under the 340B drug pricing program by adding new entities to the program; and (v) establishes a new Medicare Part D coverage gap discount program effective 2019, in which manufacturers must agree to offer 70% point-of-sale discounts off negotiated prices of applicable brand drugs to eligible beneficiaries during their coverage gap period, as a condition for the manufacturer’s outpatient drugs to be covered under Medicare Part D. The PPACA also includes a number of provisions aimed at strengthening the government’s ability to pursue anti-kickback and false claims cases against pharmaceutical manufacturers and other healthcare entities, including substantially increased funding for healthcare fraud enforcement activities, enhanced investigative powers, and amendments to the False Claims Act that make it easier for the government and whistleblowers to pursue alleged violations of the Anti-Kickback Statute, the Federal Food, Drug, and Cosmetic Act (“FDCA”), the False Claims Act and other relevant laws. While the evolving nature of this regulatory framework makes it difficult to predict what effect the framework and any recent or future changes will have on our business, we anticipate that government scrutiny of pharmaceutical sales and marketing practices will continue for the foreseeable future, and the risk of government investigations and enforcement actions will continue. For example, federal enforcement agencies recently have shown interest in, and engaged in enforcement actions against, pharmaceutical companies’ product and patient assistance programs, including manufacturer reimbursement support services and relationships with specialty pharmacies, as well as contributions by companies to third-party 501(c)(3) charitable organizations that assist patients in accessing treatment for certain diseases and conditions. This was also a part of the JUXTAPID investigations, including certain contributions that were not resolved in the Aegerion Settlements. Some of these investigations have resulted in significant civil and criminal settlements. Responding to a government investigation or enforcement action would be expensive and time-consuming, and could have a material adverse effect on our reputation, business, financial condition, results of operations and prospects.

Anti-bribery rules in many jurisdictions also prohibit the offer of kick-backs and other inappropriate inducements to prescribe.

We are subject to the UK Bribery Act, the U.S. Foreign Corrupt Practices Act, and other anti-corruption laws, export control laws, import and customs laws, trade and economic sanctions laws and other laws which govern our operations.

Our operations are subject to anti-corruption laws, including the UK Bribery Act, the U.S. Foreign Corrupt Practices Act of 1977 (“FCPA”), the U.S. domestic bribery statute, the U.S. Travel Act, and other anti-corruption

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laws that apply in countries where we conduct business. The UK Bribery Act, the FCPA and other anti-corruption laws generally prohibit us and our employees and intermediaries from authorizing, promising, offering or providing, directly or indirectly, improper or prohibited payments, or anything else of value, to government officials or other persons to obtain or retain business or gain some other business advantage. Under the UK Bribery Act, we may also be liable for failing to prevent a person associated with us from committing a bribery offense. We and our commercial partners operate in a number of jurisdictions that pose a high risk of potential UK Bribery Act or FCPA violations, and we also participate in collaborations and relationships with third parties whose corrupt or illegal activities could potentially subject us to liability under the UK Bribery Act, FCPA or local anti-corruption laws, even if we did not explicitly authorize or have actual knowledge of such activities. In addition, we cannot predict the nature, scope or effect of future regulatory requirements on our international operations or the manner in which existing laws might be administered or interpreted.

We are also subject to other laws and regulations governing our international operations, including regulations administered by the governments of the United Kingdom and the United States, and authorities in the European Union, including applicable export control regulations, economic sanctions and embargoes on certain countries and persons, anti-money laundering laws, import and customs requirements and currency exchange regulations (collectively, “Trade Control Laws”).

There is no assurance that we will be completely effective in ensuring compliance with all applicable anti-corruption laws, including the UK Bribery Act and the FCPA, or other legal requirements, including Trade Control Laws. If we are not in compliance with the UK Bribery Act, the FCPA and other anti-corruption laws or Trade Control Laws, we may be subject to criminal and civil penalties, disgorgement and other sanctions and remedial measures, and legal expenses, which could have an adverse effect on our business, financial condition, results of operations and liquidity. Likewise, any investigation of any potential violations of the UK Bribery Act, the FCPA, other anti-corruption laws or Trade Control Laws by the United Kingdom, United States, or other authorities could also have an adverse impact on our reputation, business, financial condition, results of operations and prospects.

If we fail to comply with our reporting and payment obligations under the Medicaid Drug Rebate Program or other governmental pricing programs in the United States, we could be subject to additional reimbursement requirements, penalties, sanctions and fines which could materially and adversely affect our business, financial condition, results of operations and prospects.

We participate in various government programs and contracts that require us to calculate and report certain prices for our products to government agencies or provide rebates or discounted pricing on products purchased to certain purchasers or government payers. The requirements for calculating prices and rebates are complex and subject to change. Changes to such requirements may affect our business and operations. We may also have reimbursement obligations or be subject to penalties if we fail to provide timely and accurate information to the government, pay the correct rebates or offer the correct discounted pricing.

To maintain coverage of products under the Medicaid Drug Rebate Program and Medicare Part D, we will be required to extend significant discounts to certain “covered entities” (defined by statute to include certain types of hospitals and other healthcare providers that receive federal grants) that purchase products under the 340B Program. The 340B Program requires participating manufacturers to agree to charge such covered entities no more than the 340B “ceiling price” for the manufacturers’ covered outpatient drugs. The 340B ceiling price is calculated using a statutory formula, which is based on the average manufacturer price and rebate amount for the covered outpatient drug as calculated under the Medicaid Drug Rebate Program. Orphan drugs, such as MYALEPT and JUXTAPID, are exempt from the ceiling price requirements for drugs purchased by certain covered entities (i.e., rural referral centers, sole community hospitals, critical access hospitals and freestanding cancer hospitals). Because of our participation in the Medicaid Drug Rebate Program, we are required to pay a rebate for each unit of metreleptin or lomitapide reimbursed by a state Medicaid program as a condition to avail us of federal funds to the states for our drugs under Medicaid and Medicare Part D.

Pricing and rebate calculations vary among products and programs. The calculations are complex and often subject to interpretation by governmental or regulatory agencies and the courts. For example, the Medicaid rebate amount is computed each quarter based on our submission to the Centers for Medicare & Medicaid Services (“CMS”) of our average manufacturer price and best price for the quarter. If we become aware that Aegerion’s reporting for prior quarters was incorrect, or has changed as a result of recalculation of pricing data, we must resubmit the corrected data for a period not exceeding 12 quarters from the quarter in which the data was

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originally due. Such restatements and recalculations would serve to increase our costs for complying with the laws and regulations governing the Medicaid Drug Rebate Program. Any corrections to our rebate calculations could result in an overage or underage in our rebate liability for past quarters, depending on the nature of the correction. Price recalculations also may affect the price that we will be required to charge certain safety net providers under the Public Health Service 340B drug discount program.

If we fail to comply with our reporting and payment obligations under the Federal Supply Schedule pricing program, we could be subject to penalties.

Federal Supply Schedule (“FSS”) contracts are federal procurement contracts that include standard government terms and conditions, separate pricing for each product, and extensive disclosure and certification requirements. All federal agencies and some non-federal entities are authorized to access FSS contracts. FSS contractors are permitted to charge FSS purchasers other than the four federal agencies “negotiated pricing” for covered drugs that is not capped by the statutory Federal Ceiling Price (“FCP”); instead, such pricing is negotiated based on a mandatory disclosure of the contractor’s commercial “most favored customer” pricing. Moreover, all items on FSS contracts are subject to a standard FSS contract clause that requires FSS contract price reductions under certain circumstances where pricing to an agreed “tracking” customer is reduced.

In July 2016, Aegerion concluded negotiations with the Department of Veterans Affairs (“VA”), and on August 15, 2016, Aegerion secured an FSS contract for both MYALEPT and JUXTAPID. Under this program, we are obligated to make MYALEPT and JUXTAPID available for procurement on an FSS contract at a negotiated price and also charge a price to four federal agencies (the VA, Department of Defense, Public Health Service and Coast Guard) that is no higher than the statutory FCP, which we calculate and report to the VA on a quarterly and annual basis. If we fail to comply with our reporting and payment obligations under the FSS pricing program, we could be subject to penalties in the future, which may have a material and adverse effect on our business, financial condition and results of operations.

If we overcharge U.S. government payers for our products, we may be found liable and could be subject to penalties for errors associated with the submission of pricing data.

To maintain coverage of products under the Medicaid Drug Rebate Program and Medicare Part D and when purchased by the Department of Veterans Affairs, Department of Defense, Public Health Service and Coast Guard, typically a pharmaceutical company must participate in the FSS.

In addition to the FSS contract with the VA, we also participate in the Tricare Retail Pharmacy program, under which we pay quarterly rebates on utilization of MYALEPT and JUXTAPID when the products are dispensed through the Tricare Retail Pharmacy network to Tricare beneficiaries.

If we are found to have knowingly submitted false average manufacturer price or best price information to the government, we may be liable for civil monetary penalties in the amount of $100,000 per item of false information. Failure by us to submit monthly or quarterly average manufacturer price and best price data on a timely basis could result in a civil monetary penalty of $10,000 per day for each day the submission is late beyond the due date. In the event that CMS were to terminate the rebate agreement, no federal payments would be available under Medicaid or Medicare Part D for our products. In addition, if we overcharge the government in connection with the FSS contract or under any other government program, we will be required to refund the difference to the government. Failure to make necessary disclosures or to identify contract overcharges could result in allegations against us under the federal civil False Claims Act and other laws and regulations.

Risks Related to our Reliance on Third Parties

We rely on third parties to conduct clinical trials and registry studies and perform related services, and those third parties may not perform satisfactorily, including by failing to meet established deadlines for the completion of such clinical trials and compliance with post-marketing requirements.

We do not have the resources to independently conduct clinical trials or registry studies, or perform pharmacovigilance and REMS program and other risk management plan monitoring and reporting, and we rely on third parties, such as contract research organizations, medical institutions, academic institutions, clinical investigators, specialty pharmacies and other third-party service providers, to perform these functions. Reliance on third parties for these functions reduces our control over such functions. However, if we sponsor clinical

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trials, we are responsible for ensuring that each of the sponsored clinical trials is conducted in accordance with the general investigational plan and protocols for the trial. Our reliance on third parties does not relieve us of these responsibilities and requirements. Furthermore, these third parties may have relationships with other entities, some of which may be their competitors.

If the third parties we rely upon fail to successfully carry out their contractual duties or meet expected deadlines, if they need to be replaced or if the quality or accuracy of the data they provide is compromised or delayed due to the failure to adhere to regulatory requirements or clinical trial protocols, or for other reasons, our current marketing authorizations may be revoked, suspended, or revised to be more stringent. Further, our development programs, including any potential clinical studies, may be extended, delayed or terminated. If we were to experience an unexpected loss of supply of any of our product candidates or any of our future product candidates for any reason, whether as a result of manufacturing, supply or storage issues or otherwise, we could experience delays, disruptions, suspensions or terminations of, or be required to restart or repeat, any pending or ongoing clinical trials. For example, the extent to which the COVID-19 pandemic impacts our ability to procure sufficient supplies for the development of our products and product candidates will depend on the severity and duration of the spread of the virus, and the actions undertaken to contain COVID-19 or treat its effects. Additional marketing approvals for metreleptin or lomitapide may be delayed or denied in the targeted indication or jurisdiction, and efforts to successfully commercialize AP101, metreleptin, lomitapide, or any other product for targeted indications or in the targeted jurisdiction may be delayed or unsuccessful. Should this occur, any existing approvals could be negatively impacted, which could materially and adversely affect our commercialization efforts.

We depend on third-party manufacturers to produce the drug substance and the drug product for lomitapide and metreleptin sold globally, as well as the drug product for commercial supply and clinical trials. Even though we have reserve stock, interruption in supply could materially and adversely affect sales.

We have limited internal manufacturing facilities for the production of the active pharmaceutical ingredient in AP101. We employ a small number of personnel with manufacturing experience but we are currently dependent upon contract manufacturers to produce the drug substance for metreleptin and lomitapide and the drug product for commercial supplies and clinical trials, including for AP101, if it is approved.

Aegerion entered into long-term supply agreements with a manufacturer of metreleptin drug substance and a manufacturer of metreleptin drug product. In February 2017, the original contract manufacturer for metreleptin drug product received a warning letter from the FDA citing significant violations of current Good Manufacturing Practice (“cGMP”) regulations at the manufacturing facility where metreleptin drug product was manufactured. In addition, in December 2017, this contract manufacturer informed Aegerion that it would terminate its supply contract in December 2019. As a result, to mitigate metreleptin supply risks, Aegerion maintained a supply of safety stock and entered into a long-term supply agreement with another drug product manufacturer. This new manufacturer has finished validation for all metreleptin drug product sizes and commercial supply is now on stream for all sizes of the drug product. Given the inventory levels of the drug product and the fact that metreleptin is now being commercially supplied from this new manufacturer, we do not expect that there should be any material effect on supply if there are any delays in market approvals for ongoing changes in the supply chain. Aegerion also entered into long-term supply agreements with a manufacturer of the lomitapide drug substance and a manufacturer of lomitapide. We have sufficient inventory of metreleptin and lomitapide drug substances to maintain supply for more than one year. We do not have any other agreements in place for redundant supply or a second source for drug substance or drug product for either product. Any interruption in supply of drug product or drug substance for our products could have a material and adverse impact on our sales, depending upon the length of interruption, which in turn may have a material adverse effect on our business, financial condition, results of operations and prospects.

If we are unable to maintain arrangements for third-party manufacturing, are unable to do so on commercially reasonable terms, or are unable to obtain timely regulatory approvals in connection with contract manufacturers, we may not be able to complete development of our product candidates or successfully commercialize our products. We may incur significant added costs and substantial delays in identifying and qualifying any replacement manufacturers, and in obtaining regulatory approval to use such replacement manufacturer in the manufacture of the products. Any such delays could result in significant delay in the supply of drug product for an ongoing clinical trial due to the need to replace a third-party manufacturer and could delay completion of the trial. If for any reason we are unable to obtain adequate supplies of lomitapide or metreleptin, or the drug

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substances used to manufacture them, it will be more difficult or impossible for us to compete effectively, generate revenues, meet expectations for financial performance and further develop our products. In addition, if we are unable to assure a sufficient quantity of the drug for patients with rare diseases or conditions, we may lose any Orphan Drug exclusivity to which our product otherwise would be entitled.

We are subject to minimum production levels with some of these manufacturing companies. If we cannot sell sufficient levels of metreleptin or lomitapide, this will result in excess levels of inventory being manufactured and resulting obsolescence.

If our third-party manufacturers are unable to comply with applicable regulatory requirements, unable to source sufficient raw materials, experience manufacturing or distribution difficulties, or are otherwise unable to manufacture and distribute sufficient quantities to meet demand, our commercialization efforts may be materially harmed.

While we manufacture the drug substance for AP101 on our own, we do not own or operate manufacturing facilities for the production of clinical or commercial supplies of our products. We have limited personnel with experience in drug manufacturing and lack the resources and the capabilities to manufacture any of our product candidates on a clinical or commercial scale. We currently rely on third parties for supply of drug substances and drug products, and our strategy is to outsource all manufacturing of our product candidates and products to third parties.

We rely on contract manufacturers to consistently produce drug substances and drug products to required specifications, including those imposed by the FDA, the EMA and other regulatory authorities. There can be no assurance that our contractors will consistently be able to produce commercial supplies of drug substance or drug product meeting the approved specifications. A number of factors could cause production interruptions at the facilities of the contract manufacturers, including equipment malfunctions, facility contamination, labor problems, raw material shortages or contamination, the impact of the COVID-19 pandemic, natural disasters, disruption in utility services, terrorist activities, regulatory actions resulting from failure to comply with cGMP, human error or disruptions in the operations of the suppliers. Aegerion experienced failures by third-party manufacturers to produce products that meet specifications in the past, and any future failure by third-party manufacturers to produce products that meet specifications could lead to a shortage of lomitapide or metreleptin.

The FDA, the EMA and other regulatory authorities require that drug products be manufactured according to cGMPs relating to methods, facilities and controls used in the manufacturing, processing and packaging of the product, which are intended to ensure that drug products are safe and that they consistently meet applicable requirements and specifications. Our contract manufacturers are subject to periodic announced and unannounced inspections by the FDA to assess compliance with cGMP requirements. If an FDA inspection on a manufacturer’s facilities reveals conditions that the FDA determines do not comply with applicable regulatory requirements, the FDA may issue observations through a Notice of Inspectional Observations, commonly referred to as a “Form FDA 483” report. If observations in the Form FDA 483 Report are not addressed in a timely manner and to the FDA’s satisfaction, the FDA may issue a Warning Letter or proceed directly to other forms of enforcement action. Any failure by third-party manufacturers to comply with cGMP or to provide adequate and timely corrective actions in response to deficiencies indentified in a regulatory inspection could result in further enforcement action that could lead to a shortage of products and harm our business, including to withdrawal of approvals previously granted, seizure, injunction or other civil or criminal penalties. The failure of any third-party manufacturer to address any concerns raised by the FDA or foreign regulators could also lead to plant shutdown or the delay or withholding of product approval by the FDA in additional indications, or by foreign regulators in any indication. Certain countries may impose additional requirements on the manufacturing of drug products or drug substances, and on third-party manufacturers, as part of the regulatory approval process for products in such countries. The failure by us or our third-party manufacturers to satisfy such requirements could impact our ability to obtain or maintain approval of our products in such countries.

The manufacture of biologic pharmaceuticals, such as metreleptin, is more difficult and riskier than the manufacture of small molecule pharmaceuticals, such as lomitapide. The process of manufacturing biologics is highly susceptible to product loss due to contamination, oxidation, equipment failure or improper installation or operation of equipment, or vendor or operator error. Even minor deviations from normal manufacturing processes could result in reduced production yields, product defects and other supply disruptions. If microbial, viral or other contaminations are discovered in metreleptin or the facilities of the contract manufacturer, we may need to cease the manufacture of metreleptin for an extended period of time to investigate and remediate the

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contaminant. A contamination, recall, raw material shortage, or other supply disruption could adversely impact or disrupt commercial manufacturing of metreleptin or could result in a withdrawal of metreleptin from the market. Any such disruption, in turn, could adversely affect our ability to satisfy demand for metreleptin, which could materially and adversely affect our business, financial condition, results of operations and prospects.

We may be unable to scale up our manufacturing capability or successfully outsource manufacturing to third-party contractors.

We may underestimate the cost, time or complexity of installing or operating the manufacturing equipment required for our business. There also is a risk that any new equipment may not function as expected once installed. We may decide to outsource future manufacturing needs to third parties but may be unable to find sufficient supply for our manufacturing needs. Any inability to successfully scale up manufacturing capability or meet manufacturing need through third-party contractors may materially and adversely affect our business, financial condition, results of operations and prospects.

We rely on third parties for distribution services around the world, and a failure to manage these third parties could harm our business.

We use, and plan to use, third parties to provide warehousing, shipping, third-party logistics, invoicing, collections and other distribution services on our behalf in the United States and in other countries throughout the world. Our failure to establish, maintain and finance the capabilities to sell, market or distribute our products, either through our own commercial infrastructure or through arrangements with third parties, and to effectively manage such third parties, could result in us not being able to successfully sell our products and could, as a result, have a material adverse effect on business, financial condition, results of operations and prospects.

If our licensees do not successfully commercialize the licensed products, we will not receive anticipated royalties and other payments and our results of operations will suffer.

The product sales, milestone payments and royalties that we may be entitled to receive under our license arrangements with Shionogi and Recordati are dependent on such licensees’ ability to successfully develop and commercialize the licensed products for their respective indications. Our licensees may not succeed in their product development efforts. It is possible that our licensees may be unable to obtain regulatory approval of product candidates using our technologies or successfully market and commercialize any such products for which regulatory approval is obtained. We have no control over these licensees and the contractual provisions may not provide adequate remedies in the event that a licensee fails to satisfy its obligations and commitments under the licensing arrangements. In addition, we have limited experience in maintaining multiple licensing arrangements and may be unable to adequately administer or monitor, or comply with, these arrangements, especially in light of limited personnel and cash resources, and these or future license arrangements may require us to provide services, supply or other efforts to the licensee. For example, under the license agreement with Recordati, we are required to provide supply product to Recordati. Moreover, the licensees have the ability to terminate or elect not to renew their respective license agreement in certain circumstances, including if we materially breach the agreement. A decision by a licensee to terminate its relationship, or a failure by a licensee to successfully develop or commercialize the licensed products or indications, could materially and adversely affect our business, financial condition, results of operations and prospects.

Risks Related to our Intellectual Property

It may be challenging or costly for us to obtain, maintain, enforce and defend our intellectual property rights. Failure to obtain or protect these rights could adversely affect our business and our ability to compete.

Our success and ability to compete effectively is in large part dependent upon exploitation of proprietary technologies and product candidates that have been developed internally or have been acquired or in-licensed, our ability to protect and enforce our intellectual property rights so as to preserve our exclusive rights in respect of our technologies and product candidates, and our ability to preserve the confidentiality of our know-how. We rely primarily on exclusivity granted by a combination of Orphan Drug approval, data exclusivity, patent laws and trade secrets/confidentiality to protect our intellectual property rights. There can be no assurance that patents pending or future patent applications will be issued, nor that the lack of any such patents will not have a material adverse effect on our ability to develop and market its proposed candidates, or that, if issued, we would have the resources to protect or enforce any such issued patent from infringement. Also, no assurance can be given that

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we will develop technologies or candidates which are patentable or that patents will be sufficient in their scope to provide protection for our products or intellectual property rights against third parties. Nor can there be any assurance as to the ownership, validity, patentability, enforceability or scope of any patents which have been, or may in the future be, issued to us or that claims with respect thereto would not be asserted by third parties. Furthermore, there are some areas of technology that are important for our businesses which cannot be patented due to the existence of prior disclosures or rights. AP103 currently has no granted patent protection in the European Union. We intend to rely on patent protection once it is approved and also on exclusivity from a possible future Orphan Drug approval. LOJUXTA did not receive Orphan Drug approval in Europe and relies on data exclusivity and patent protection. This may make our reimbursement discussions more difficult. In addition, there can be no assurance that we will be able to obtain and/or maintain Orphan Drug Designation or Orphan Drug approval for our product candidates. For example, the EMA’s Committee for Orphan Medicinal products reviews an orphan design of a product if it is approved for a marketing authorization; for a product to benefit from market exclusivity, it must maintain its orphan designation at the time of marketing authorization review by the EMA and approval by the EC.

The pharmaceutical industry is characterized by extensive litigation regarding patents and other intellectual property rights. For example, on August 28, 2015, the Coalition for Affordable Drugs VIII L.L.C. filed two separate inter partes review (“IPR”) petitions with the Patent Trial and Appeal Board (“PTAB”) of the U.S. Patent and Trademark Office, challenging the validity of U.S. Patent Nos. 7,932,268 and 8,618,135, which are directed to methods-of-use for lomitapide. Although the PTAB ruled in our favor, third parties may obtain patents in the future and allege that our products or the use of our technologies infringe their patent claims or that we are employing their proprietary technology and other intellectual property without authorization. Likewise, third parties may infringe upon our existing or future patents.

We also rely on copyright, trademark and trade secret laws, as well as confidentiality procedures and agreements, non-compete and work for hire invention assignment agreements and licensing arrangements with our employees, consultants, contractors, customers and vendors, to establish and protect our and their rights to their respective technology and other developments and, to the best extent possible, control the access to and distribution of our technology, software, documentation and proprietary information.

Despite software, documentation and proprietary information and other precautions, it may be possible for a third party to copy or otherwise obtain and use our technology without authorization. Once granted, a patent can be challenged both in the patent office and in the courts by third parties. Third parties can bring material and arguments, for example, which the patent office granting the patent may not have been aware of. Therefore, issued patents may be found by a court of law or by the patent office to be invalid or unenforceable or in need of further restriction.

In particular, our commercial success depends on our ability to obtain and maintain the following intellectual property protections:

• product, composition of matter, formulation and method of use patents in the European Union, the United States and other key global markets for existing and future products;

• Orphan Drug exclusivity granted to our products because they aim to treat rare diseases and conditions, which entitle us to exclusivity protections for a period of up to seven years after approval in the United States (although metreleptin should also qualify for a 12-year period of exclusivity from biosimilar or interchangeable products) and up to ten years in the European Union and Japan as well as certain financial incentives. The ten-year Orphan Drug exclusivity period in the European Union can be extended a further two years upon successful completion of a PIP. Conversely, the ten-year exclusivity period may be reduced to six years, if at the end of the fifth year, it is established that a product no longer fulfills the criteria for Orphan Drug Designation;

• medicinal products granted a marketing authorization in the European Union entitles us to eight years’ data exclusivity after approval, and up to ten years’ market exclusivity protection which can be extended for a further year if a new indication is granted; and

• available extensions to the terms of our Orphan Drug exclusivity, product and methods of use patents in the European Union and United States.

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If we lose the competitive advantage provided by these intellectual property and other protections, we will not be able to generate sustainable revenues or profits from our product portfolio. If we do not adequately protect and enforce our intellectual property, competitors may erode or negate any competitive advantage we may have, which could materially harm our business and ability to achieve expected financial results.

We may infringe or be alleged to infringe the intellectual property rights of others, which may prevent or delay product development and commercialization efforts, requiring us to expend resources on litigation or other resolutions, which may materially and adversely affect our business.

Our success will depend in part on our ability to operate without infringing the intellectual property and other proprietary rights of third parties. Identification of third-party patent rights that may be relevant to our products and proprietary technology is difficult because patent searching is imperfect due to differences in terminology among patents, incomplete databases and the difficulty and uncertainty in assessing the meaning of patent claims. There could be issued patents of which we are or were not aware that our products infringe. There also could be patents that we believe do not infringe, but that may ultimately be found to infringe. Moreover, patent applications are in some cases maintained in secrecy until patents are issued. The publication of discoveries in the scientific or patent literature frequently occurs substantially later than the date on which the underlying discoveries were made and patent applications were filed. Because patents can take many years to issue, there may be currently pending applications of which we are unaware that may later result in issued patents that our products infringe. For example, pending applications may exist that provide support or can be amended to provide support for a claim that results in an issued patent that our product infringes.

Proceedings involving our patents or patent applications or those of others could:

• put one or more of our patents at risk of being invalidated, rendered unenforceable or interpreted narrowly;

• adversely impact the patentability of our inventions relating to our products;

• result in monetary damages, injunctive relief or otherwise harm our competitive position, including by limiting marketing and selling activities, increasing the risk for generic competition, limiting development and commercialization activities or requiring us to obtain licenses to use the relevant technology (which licenses may not be available on commercially reasonable terms, if at all); and

• otherwise negatively impact the enforceability, validity or scope of protection offered by the patents relating to the products.

We may not have the resources to adequately defend such claims, and even if successful in any such proceedings, we would incur substantial costs and divert management’s time and attention in pursuing these proceedings, putting further strain on our resources, which could have a material adverse effect on our business, financial condition, results of operations and prospects. If we are unable to avoid infringing the patent rights of others, we may be required to seek a license, defend an infringement action or challenge the validity of the patents in court or other venue. Patent litigation is costly and time consuming. We may not have sufficient resources to bring these actions to a successful conclusion.

In addition, if we do not obtain a license, develop or obtain non-infringing technology, fail to defend an infringement action successfully or have infringed patents declared invalid, we may:

• incur substantial monetary damages;

• encounter significant delays in expanding the market of our products; and

• be precluded from manufacturing or selling any products;

which, in each case, could have a material adverse effect on our business, financial condition, results of operations and prospects.

Our existing patent protections will expire and protection for these rights may not be extended.

We depend on patent protection to provide exclusive marketing rights for our products. Loss of patent protection for a product typically leads to a significant and rapid loss of sales for that product as lower-priced generic versions of that drug become available. The expiration of patent protection for any product that contributes significantly to our sales can have a material and adverse effect on our business, cash flow, results of operations, financial position and prospects.

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The AP101 patent portfolio includes one U.S. patent covering oleogel compositions and two U.S. patents covering treatment of EB that provide exclusivity in the United States into 2025 (compositions) and into 2030 (treatment of EB). There is currently no other intellectual property protection for the AP101 product in the United States beyond 2025 or AP101 indications in the United States beyond 2030. The existing Orphan Drug designation for AP101 would extend Orphan Drug exclusivity for AP101 seven years from the approval date in the United States. The non-U.S. patents for AP101 include European patents covering the AP101 composition and methods of healing wounds with AP101. Supplementary protection certificates have been obtained in various EU countries, extending the expiration of the composition patents in the European Union from 2025 to 2030. The method of use patents are also due to expire in the European Union in 2030. There is currently a non-provisional application filed for AP101 covering future AP101 indications which, if granted, would provide worldwide coverage until 2039.

The AP103 patent portfolio includes one granted European patent (validated in various European, countries including the United Kingdom, Germany, France, Italy and Spain) claiming highly branched poly β-amino ester (“HPAE”) polymers and polyplexes comprising HPAE polymers, and transfection methods using such polymers, as well as pending U.S. and European divisional applications. These patents and applications have been in-licensed from the University College Dublin, and are expected to provide exclusivity into 2035. We have also filed an international application covering a proprietary genus of HPAEs and novel HPAE polyplexes, as well as an international application claiming a proprietary and scalable HPAE polyplex manufacturing methods, which if granted would expire in 2039.

The lomitapide patent portfolio includes a method-of-use patent that provides protection in the United States into 2027. There is currently no intellectual property protection or any Orphan Drug, data, or marketing exclusivity for lomitapide in the United States beyond 2027. The non-U.S. patents for lomitapide, including an EU method of use patent, is due to expire in 2025. The EU method of use patent is eligible for up to approximately three years of supplemental protection in certain EU countries. Aegerion applied for such protection in the countries in the European Union in which LOJUXTA is approved, on a country-by-country basis, and in some countries, supplemental protection was granted to extend patent protection to July or August of 2028, while in other countries, the applications are still pending.

The metreleptin patent portfolio includes a method-of-use patent that provides protection in the United States into 2027. There is currently no intellectual property protection or any Orphan Drug, data, or marketing exclusivity for metreleptin in the United States beyond 2027. In the United States there is seven years of Orphan Drug exclusivity for MYALEPT in the treatment of GL which is scheduled to expire in February 2021. In July 2018, Aegerion received approval for marketing authorization by the EC for metreleptin in the European Union, under the brand name MYALEPTA, as replacement therapy to treat complications of leptin deficiency in patients with GL and PL. The patent issued in the European Union directed to metreleptin methods-of-use expires in 2022. Metreleptin has orphan exclusivity in the European Union until July 2028. The ten-year Orphan Drug exclusivity period in the European Union can be extended a further two years upon successful completion of a PIP.

The loss of patent protection could have a material adverse effect on our business, financial condition, results of operations and prospects.

Previously granted regulatory exclusivity will expire and we may not be entitled to such exclusivity in the future.

Orphan Drug Designation can confer various benefits, depending on the jurisdiction, including market exclusivity and regulatory and fiscal incentives. Because our products and product candidates treat rare diseases and conditions that affect relatively small patient populations, we depend on the financial incentives and market exclusivity provided by Orphan Drug Designation to generate revenue from our product portfolio and protect our products from competition from lower-priced generic versions of such drugs. The Orphan Drug Designation in the United States for JUXTAPID for the treatment of HoFH expired on December 21, 2019, and Orphan Drug Designation for MYALEPT for treatment of GL will expire in 2021. Aegerion also obtained Orphan Drug Designation for metreleptin for the treatment of PL in the United States, which provides seven years exclusivity after approval in the United States. In 2012, metreleptin was granted Orphan Drug Designation by the EC for the treatment of Barraquer-Simons syndrome, Berardinelli-Seip syndrome, Lawrence syndrome and familial PL, which is expected to expire ten years from approval of any marketing authorization(s) for such indication(s) by the EMA. The EC provided that at the end of the fifth year into the Orphan Drug exclusivity period it is established that metreleptin continues to fulfill the criteria for Orphan Drug Designation. If not, such exclusivity

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period is reduced to six years. Following approval by the EMA in July 2018, metreleptin was entitled to ten years of market and Orphan Drug exclusivity in the European Union. Upon the expiration of the Orphan Drug exclusivity periods for our products, we may face substantial competition from generic drugs which could materially and adversely affect the price we are able to receive for the products and its business. Furthermore, the Orphan Drug Designation may not be available in the future by virtue of a number of factors, such as changes in law or regulation, increased competition in the Orphan Drug market, clinical and technological developments, among others. The designation could also result in significant payments to meet ongoing compliance obligations. The expiration or loss of Orphan Drug Designation in respect of one of our products or future products means that financial incentives by governments and non-government entities may not be available, which may have a material adverse effect on our business, financial condition, results of operations and prospects.

We also rely on the data and market exclusivity granted to innovative medicinal products in the European Union to protect our products from competition from lower-priced generic versions of the drugs. Innovative medicinal products authorized in the European Union on the basis of a full marketing authorization application (as opposed to an application for marketing authorization that relies on data in the marketing authorization application for another, previously approved medicinal product) are entitled to eight years’ data exclusivity from the date of notification of the marketing authorization. During this period, applicants for approval of generics of these innovative products cannot rely on data contained in the marketing authorization application submitted for the innovative medicinal product. Innovative medicinal products are also entitled to ten years’ market exclusivity. During this ten-year period, no generic medicinal product can be placed on the EU market. The ten-year period of market exclusivity can be extended to a maximum of 11 years if, during the first eight of those ten years, the marketing authorization holder for the innovative product obtains an authorization for one or more new therapeutic indications which, during the scientific evaluation prior to their authorization, are held to bring a significant clinical benefit in comparison with existing therapies.

We currently benefit from marketing and data exclusivity periods in the European Union. Metreleptin is entitled to up to ten years of market exclusivity in the European Union from its approval in July 2018, subject to possible reductions to six years when the marketing authorization is renewed if the product no longer meets the criteria for orphan designation. The product is also entitled to eight years' data exclusivity and ten years’ marketing exclusivity in the European Union from its date of authorization. Lomitapide is entitled to eight years’ data exclusivity and ten years’ marketing exclusivity in the European Union from July 31, 2013, the date of the EC’s approval of lomitapide. Upon the expiration of these data and market exclusivity periods, in relation to existing and new products we may face substantial competition from generic drugs which could have a material adverse effect on the price we are able to receive for the products and business, which may have a material and adverse effect on our business, financial condition, results of operations and prospects.

Our patents may be challenged, deemed unenforceable, invalidated or circumvented, and if we do not obtain or maintain patent protection for the products, our business may be materially harmed.

The patent positions of biotechnology and pharmaceutical companies involve complex legal and factual questions and, therefore, validity and enforceability cannot be predicted with certainty. U.S. patents and patent applications also may be subject to interference proceedings, ex parte re-examination, IPR and post-grant review proceedings, derivation proceedings and supplemental examination and may be challenged in district courts. Patents granted in certain other countries may be subjected to opposition or comparable proceedings lodged in various national and regional patent offices. These proceedings could result in either loss of the patent or denial of the patent application or loss or reduction in the scope of one or more of the claims of the patent or patent application. In addition, such interference, re-examination, opposition, post-grant review, IPR, derivation proceedings, supplemental examination or revocation proceedings may be costly. We will be able to protect our proprietary rights from unauthorized use by third parties only to the extent that our proprietary technologies are covered by valid and enforceable patents or are effectively maintained as trade secrets.

The degree of future protection for our products and proprietary rights is uncertain, and it cannot be guaranteed that:

• we will be able to successfully develop or commercialize our product before some or all of the relevant patents or regulatory exclusivity expire, or in countries where we do not have patent protection or exclusivity;

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• we or our licensors were the first to make the inventions covered by each of the pending patent applications and patents;

• we or our licensors were the first to file patent applications for these inventions;

• others will not independently develop similar or alternative technologies or duplicate any of our technologies;

• any of our pending patent applications or those that we have licensed will result in issued patents;

• any of our patents or those we have licensed will be valid or enforceable;

• we will be able to license the patents or pending patent applications necessary or desirable to enforce or protect our patent rights on commercially reasonable terms or at all;

• any patents issued to us or our licensors or collaborators will provide a basis for any additional commercially viable products, will provide us with any competitive advantages or will not be challenged by third parties;

• we will be able to develop additional proprietary technologies that are patentable;

• Orphan Drug exclusivity marketing rights for our products in the United States will be maintained, if, for example, the FDA determines in the future that the request for Orphan Drug Designation was materially defective or if the manufacturer is unable to assure sufficient quantity of the drug to meet the needs of patients with the rare disease or condition. In addition, the FDA, and the EMA for the European Union, may subsequently approve products with the same active moiety for the same condition if the FDA or the EMA concludes that the later drug is safer, more effective, or makes a major contribution to patient care. Orphan Drug Designation neither shortens the development time nor regulatory review time of a drug nor gives the drug any advantage in the regulatory review or approval process; or

• the patents of others will not have an adverse effect on our business.

We enjoy only limited geographical protection with respect to certain patents.

Filing and prosecuting patent applications and defending patents covering product candidates in all countries throughout the world would be prohibitively expensive. Competitors may use our and our licensors’ technologies in jurisdictions where patent protection has not yet been obtained to develop their own products or may export infringing products to territories where enforcement rights are not as strong as in the United States or European Union. These products may compete with our product candidates, and our intellectual property rights may not be effective or sufficient to prevent such products from competing. Patent applications may be issued in some non-U.S. jurisdictions with different scope or they may be refused in certain jurisdictions, such as in China, which has different requirements for patentability.

Proceedings to enforce our patent rights in other jurisdictions, whether or not successful, could result in substantial costs and divert efforts and attention from other aspects of the business. They could also put our patents and patent applications at risk of being invalidated, denied or interpreted narrowly, and could provoke third parties to assert claims against us. We may not prevail in any lawsuits or damages or other remedies awarded to us, if any, and they may not be commercially meaningful. Accordingly, our intellectual property rights as enforced may be inadequate to obtain a significant commercial advantage and our efforts to protect our intellectual property rights may be unsuccessful or inadequate, which may adversely affect our ability to successfully commercialize our product candidates, which may have a material adverse effect on our business, financial condition, results of operations and prospects. Furthermore, while we intend to protect our intellectual property rights in our expected significant markets, we cannot ensure that we will be able to initiate or maintain similar efforts in all jurisdictions in which we may wish to market our product candidates. Accordingly, our efforts to protect our intellectual property rights in such countries may be inadequate, which may have an adverse effect on our ability to successfully commercialize our drug candidates in all of our expected, significant international markets.

Some countries also have compulsory licensing laws under which a patent owner may be compelled to grant licenses to third parties or laws limiting the enforceability of patents against government agencies or government contractors. In those countries, a patent owner may have limited recourse, which could materially diminish the

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value of such patents. If we or any of our licensors are forced to grant a license to third parties with respect to any patents relevant to our business, our competitive position may be adversely affected.

If we do not obtain protection under the Hatch-Waxman Amendments and similar non-U.S. legislation for extending the term of patents covering our product candidates, our ability to compete effectively could be impaired.

Depending upon the timing, duration and conditions of FDA marketing approval of our product candidates, one or more of our U.S. patents may be eligible for patent term extension under the Drug Price Competition and Patent Term Restoration Act of 1984 (“Hatch-Waxman Amendments”). The Hatch-Waxman Amendments permit a patent term extension of up to five years for a patent covering an approved product or method of use as compensation for patent term lost during product development and the FDA regulatory review process. A maximum of one patent may be extended per FDA approved product as compensation for the patent term lost during the FDA regulatory review process. A patent term extension cannot extend the remaining term of a patent beyond a total of 14 years from the date of product approval and only those claims covering such approved drug product, a method for using it or a method for manufacturing it may be extended. Similar patent term extensions may be available in other jurisdictions. In the European Union, a Supplementary Protection Certificate may be applied for approval to recover some of the time lost between the patent application filing date and the date of first marketing authorization. However, we may not receive an extension if we fail to apply within applicable deadlines, fail to apply prior to expiration of relevant patents, or otherwise fail to satisfy applicable requirements. Moreover, the length of the extension could be less than requested. If we are unable to obtain patent term extensions or the term of any such extension is less than requested, the period during which we can enforce our patent rights for that product will be shortened and competitors may obtain approval to market competing products sooner. As a result, our revenue from applicable products could be reduced materially.

We applied for supplemental protection in the countries in which LOJUXTA is approved, on a country-by-country basis, and in some countries, supplemental protection has been granted to extend patent protection to 2028, while in other countries, the applications are still pending.

If we are not able to adequately prevent disclosure of trade secrets and other proprietary information, the value of our technology and product portfolio could be significantly diminished.

We rely on trade secrets to protect our proprietary technologies, especially where we do not believe patent protection is adequate or obtainable. However, trade secrets are difficult to protect. We rely in part on confidentiality agreements with our employees, consultants, outside scientific collaborators, sponsored researchers and other advisors to protect trade secrets and other proprietary information. These agreements may not effectively prevent disclosure of confidential information, and may not provide an adequate remedy in the event of unauthorized disclosure of confidential information. In addition, others may independently discover our trade secrets and proprietary information. For example, the FDA is currently considering whether to make additional information publicly available on a routine basis, and the EMA is planning to amplify its disclosure rules. These changes could mean that information that we may consider to be trade secrets or other proprietary information may be disclosed, and it is currently unclear how the FDA’s and the EMA’s disclosure policies may change in the future, if at all. Costly and time-consuming litigation could be necessary to enforce and determine the scope of proprietary rights and we may not have adequate resources at the time to enforce our rights. Failure to obtain or maintain trade secret protection could adversely affect our competitive business position and have a material adverse effect on our business, financial condition, results of operations and prospects.

If we fail to comply with our obligations in the license agreements for our products or are alleged to have breached such agreements, we could lose license rights that are important to our business, have to make additional payments to our licensors or become involved in costly litigation, which would further reduce cash resources.

Our existing license agreements impose various diligence, milestone payment, royalty, insurance, reporting, audit and other obligations on us. If we fail to comply with such obligations or encounters disagreements with its license partners, we could lose license rights that are important to the business. Further, if the license partners allege that a breach of any such license agreements has occurred, defense of such allegations, even if untrue, can be costly to pursue and could lead to litigation or costly settlements, which would be burdensome on our resources. Even if we were able to successfully settle any disagreements or disputes under the license

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agreements, the settlement arrangements are unlikely to foreclose additional claims from the licensing party or other third parties, and the terms of such settlement could have a material adverse effect on our business, financial condition, results of operations and prospects.

If we fail to comply with the obligations and restrictions under the license agreements, the applicable licensor may have the right to seek financial payments or damages and terminate the license, in which case we may not be able to market the products that are covered by the license, or the licensor might require amendments to the license agreement that are overly burdensome. Further, the terms of such license agreements are intensely negotiated, and therefore tend to be complex, which could lead to differing interpretations by the parties. Any breach or alleged breach (whether intentional or unintentional), or termination, of the license agreements applicable to our products, or any disagreements as to the application of the terms of the agreements with licensing partners, could expose us to considerable costs and expenses as such allegations, terminations or disputes can be costly to remedy or resolve (regardless of the merits), and may not be remedied or resolved in a manner favorable to us, or at all. Any breach or alleged breach of the quantitative or qualitative diligence provisions of these agreements could result in termination of the co-development agreement and loss of the program and also expose us to considerable costs and expenses and any disputes may not be remedied or resolved in a manner favorable to us, or at all. Any such difficulties with the license agreements could have a significant adverse effect on our business.

Risks Related to Ownership of our ADSs and Ordinary Shares and our Prospective Nasdaq Listing

An active and liquid market for our securities may fail to develop, which could harm the market price of our ADSs.

There is currently no public market on a U.S. national securities exchange for our ADSs or our ordinary shares. Although we anticipate our ADSs will be approved for listing on the Nasdaq, an active trading market for our ADSs may never develop or be sustained. In the absence of an active trading market for our ADSs, investors may not be able to sell their ADSs at the time that they would like to sell.

The price and trading volume of our ADSs and ordinary shares may be volatile, and purchasers of our ADSs or ordinary shares could incur substantial losses.

The market price of our ADSs is likely to be volatile, and could decline significantly. The stock market in general, and the market for biotechnology and emerging pharmaceutical companies in particular, have experienced extreme volatility that has often been unrelated to the operating performance of particular companies. In addition, our securities have a relatively small public float and may be less liquid and more volatile than securities of companies with broader public ownership. The market price for our ADSs and ordinary shares may be influenced by a variety of factors, including:

• actual or anticipated variations in our financial condition and operating results;

• actual or anticipated changes in our growth rate relative to our competitors;

• announcements of technological partnerships, innovations or new products by us or our competitors;

• the success of competitive products or technologies;

• changes in management and members of our Board;

• changes in financial estimates or recommendations by securities analysts;

• changes in the trading volume of our ADSs on the Nasdaq and of our ordinary shares on AIM and EGE;

• sales of our ADSs or ordinary shares by executive officers or future holders of our equity securities;

• announcements or expectations of additional debt or equity financing efforts;

• unanticipated losses or gains due to unexpected events, including events related to the success of our clinical trials or regulatory approvals;

• significant acquisitions or business combinations, strategic partnerships, joint ventures or capital commitments by or involving us or our competitors;

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• changes in our accounting policies or practices;

• disputes or other developments related to proprietary rights, including patents, litigation matters and our ability to obtain patent protection for our technologies;

• failure to integrate successfully the Aegerion business with ours or to realize anticipated benefits from the integration;

• changes in government regulations, including any changes that may affect pricing or reimbursement; and

• conditions in the financial markets or changes in general economic conditions.

These and other market and industry factors may cause the market price and demand for our ADSs and ordinary shares to fluctuate substantially.

In addition, the trading prices for common stock of other biopharmaceutical companies have been highly volatile as a result of the COVID-19 pandemic. The COVID-19 outbreak continues to rapidly evolve. The extent to which the outbreak may impact our business, and the prices of our ADSs and ordinary shares will depend on future developments, which are highly uncertain and cannot be predicted with confidence.

The trading of our ordinary shares on AIM and EGE and of our ADSs on the Nasdaq may adversely affect the liquidity and value of our ADSs.

Our ordinary shares are traded on AIM and EGE, and we intend to list our ADSs on the Nasdaq. We cannot predict the effect of this listing on the value of our ordinary shares and ADSs. However, these arrangements may dilute the liquidity of these securities in one or more markets and may adversely affect the development of an active trading market for the ADSs in the United States or the ordinary shares on AIM and/or EGE. The price of our ADSs could also be adversely affected by trading in our ordinary shares on AIM and/or EGE and the price of our ordinary shares traded on AIM and/or EGE could be adversely affected by trading in ADSs on the Nasdaq.

Future sales of our ordinary shares or ADSs, or the perception that such sales may occur, could depress the prices of such ordinary shares or ADSs.

Sales of a substantial number of our ADSs in the public market, or the perception that these sales might occur, could depress the market price of the ADSs and could impair our ability to raise capital through the sale of additional equity securities. We are unable to predict the effect that such sales may have on the prevailing market price of our ADSs.

The Athyrium Funds hold a significant interest in our company and may be able to influence matters relating to our business.

The Athyrium Funds hold approximately 28.0% of our share capital. The Athyrium Funds are in a position to exert influence over matters requiring approval of the holders of our equity securities and will have the ability to block certain actions requiring shareholder approval, including (but not limited to) disapplication of preemption rights. We are not required to enter and have not entered into a relationship agreement with Athyrium or the Athyrium Funds to manage the relationship between them.

The interests of the Athyrium Funds may be different from the interests of other shareholders and, as a result, the Athyrium Funds’ interests in our ordinary shares, if of sufficient individual or aggregate size, or if aggregated in any circumstances, or should the Athyrium Funds convert their warrants into ordinary shares, may permit them to effect certain transactions without the support of other holders of our equity securities, or delay or prevent certain transactions that are in the interests of other holders of our equity securities. Decisions made by Athyrium (on behalf of the Athyrium Funds) may therefore influence our business, financial condition, results of operations and prospects. These decisions may also conflict with the interest of other holders of our equity securities and could prevent other holders of our equity securities from receiving a premium on their securities. The market price of our ADSs and ordinary shares may decline if Athyrium uses its influence in ways that are or may be adverse to the interests of other holders of our equity securities.

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We will incur significant increased costs as a result of operating as a U.S. listed public company, and our management will be required to devote substantial time to new compliance initiatives.

After we become a U.S. publicly traded company, and particularly after we cease to be an “emerging growth company” as defined in the JOBS Act, we will incur significant legal, accounting and other expenses that we did not incur prior to the listing of our ADSs in the United States as a result of reporting requirements under the Exchange Act. In addition, the federal securities laws, including the Sarbanes-Oxley Act of 2002 (“Sarbanes-Oxley Act”), and rules subsequently implemented by the SEC and the Nasdaq Stock Market LLC have imposed various requirements on public companies, including requirements to file annual and event-driven reports with respect to our business and financial condition, and to establish and maintain effective disclosure and financial controls and corporate governance practices. Our management and other personnel will need to devote a substantial amount of time to these compliance initiatives. Moreover, these rules and regulations will increase our legal and financial compliance costs and will make some activities more time-consuming and costly. For example, these regulations may make it more difficult to attract and retain qualified members of the Board and various corporate committees, and obtaining director and officer liability insurance will be more expensive. We may not be able to produce reliable financial statements or file these financial statements as part of a periodic report in a timely manner with the SEC or comply with the Nasdaq listing requirements. In addition, we could make errors in our financial statements that could require us to restate our financial statements.

Shareholder activism, the current political environment, and the current high level of government intervention and regulatory reform may lead to substantial new regulations and disclosure obligations, which may lead to additional compliance costs and impact the manner in which we operate our business in ways we cannot currently anticipate. Our management and other personnel will need to devote a substantial amount of time to these compliance initiatives.

We must maintain effective internal control over financial reporting, and if we are unable to do so, the accuracy and timeliness of our financial reporting may be adversely affected, which could have a material adverse effect on our business, investor confidence and market price.

As a U.S. listed public company, we will be required to maintain effective internal control over financial reporting in order to accurately and timely report our results of operations and financial condition. As required by Section 404 of the Sarbanes-Oxley Act, we will engage in a process to document and evaluate our internal control over financial reporting, which is both costly and challenging. In this regard, we will need to continue to dedicate internal resources, potentially engage outside consultants, and adopt a detailed work plan to assess and document the adequacy of internal control over financial reporting, continue steps to improve control processes as appropriate, validate through testing that controls are functioning as documented, and implement a continuous reporting and improvement process for internal control over financial reporting. Despite our efforts, there is a risk that neither we nor our independent registered public accounting firm will be able to conclude within the prescribed timeframe that our internal control over financial reporting is effective as required by Section 404. This could result in an adverse reaction in the financial markets due to a loss of confidence in the reliability of our financial statements.

We will be required to disclose changes made in our internal controls and procedures on a quarterly basis and our management will be required to assess the effectiveness of these controls annually. However, for as long as we are an emerging growth company, our independent registered public accounting firm will not be required to attest to the effectiveness of our internal controls over financial reporting pursuant to Section 404. We could be an emerging growth company for up to five years. An independent assessment of the effectiveness of our internal controls over financial reporting could detect problems that our management's assessment might not. Undetected material weaknesses in our internal controls over financial reporting could lead to restatements of our financial statements and require us to incur the expense of remediation.

If we fail to staff our accounting and finance function adequately or maintain internal control over financial reporting adequate to meet the requirements of the Sarbanes-Oxley Act, our business and reputation may be harmed. Moreover, if we are not able to comply with the applicable requirements of Section 404 in a timely manner, we may be subject to sanctions or investigations by regulatory authorities, including the SEC and the Nasdaq. Furthermore, if we are unable to conclude that our internal control over financial reporting is effective or if our independent registered public accounting firm identifies deficiencies in our internal control over financial reporting that are deemed to be material weaknesses, we could lose investor confidence in the accuracy and completeness of our financial reports, the market price of our ADSs and ordinary shares could decline, and

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we could be subject to sanctions or investigations by the SEC, the Nasdaq or other regulatory authorities. Failure to implement or maintain effective internal control systems as required of public companies could also restrict our access to the capital markets. The occurrence of any of the foregoing would also require additional financial and management resources.

A material weakness was identified in Aegerion’s internal control over financial reporting, and we may not be able to remedy this material weakness effectively.

During 2016, a material weakness in Aegerion’s internal control over financial reporting process was identified as management did not design and maintain sufficiently precise or effective review and approval controls over the forecasts used to develop management estimates, including those related to balances acquired in a business combination that occurred during fiscal year 2016. Prior to the Acquisition, Aegerion’s remediation efforts included, but were not limited to, enhancing its procedures and controls over the assessment, determination and documentation of its management’s assumptions and estimates used in forecasts; improving its management’s review controls over management’s judgments process; and engaging a third-party firm to assist it in updating its forecast, budget and long-range plan. While Aegerion implemented a number of remediation efforts over the course of 2017, 2018 and 2019 with respect to the material weakness, it did not completely remediate the material weakness.

Following the Acquisition, our management is responsible for implementing changes and improvements to our internal control over financial reporting and for remediating the control deficiencies that gave rise to Aegerion’s material weakness. Although we are committed to remediating the control deficiencies that constituted the material weakness, we may be unable to remediate the material weakness in internal control over financial reporting, and even if we are successful in our remediation efforts, we may in the future identify additional material weaknesses.

We are an “emerging growth company,” and the reduced disclosure requirements applicable to emerging growth companies may make our ADSs less attractive to investors and, as a result, adversely affect the price of the ADSs and result in a less active trading market for the ADSs.

We are an emerging growth company as defined in the JOBS Act, and we may take advantage of certain exemptions from various reporting requirements that are applicable to other public companies that are not emerging growth companies. These exemptions include:

• only being required to present two years of audited financials and related discussion in Management’s Discussion & Analysis;

• not being required to comply with the auditor attestation requirements of Section 404 of the Sarbanes-Oxley Act;

• not being required to comply with any requirement that may be adopted by the PCAOB regarding mandatory audit firm rotation or a supplement to the auditor’s report providing additional information about the audit and the financial statements;

• reduced disclosure obligations regarding executive compensation; and

• exemptions from the requirements of holding a nonbinding advisory vote on executive compensation and shareholder approval of any golden parachute payments not previously approved.

We may choose to take advantage of some, but not all, of the available exemptions. For example, in this prospectus, we have elected to rely on the exemption from the auditor attestation requirement and the exemption that permits only two years of financial statements to be presented. We may avail ourselves of these disclosure exemptions until we are no longer an emerging growth company. We cannot predict whether investors will find our ADSs less attractive because of our reliance on some or all of these exemptions. If investors find our ADSs less attractive, it may adversely affect the price of the ADSs and there may be a less active trading market for the ADSs.

We will cease to be an emerging growth company upon the earliest of:

• the last day of the fiscal year during which we have total annual gross revenues of $1.07 billion (as such amount is indexed for inflation every five years by the SEC) or more;

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• the date on which we have, during the previous three-year period, issued more than $1.0 billion in non-convertible debt;

• the date on which we are deemed to be a “large accelerated filer,” as defined in Rule 12b-2 of the Exchange Act, which would occur if the market value of our ordinary shares and ADSs that are held by non-affiliates exceeds $700 million as of the last day of our most recently completed second fiscal quarter; and

• the last day of the fiscal year following the fifth anniversary of the completion of our first sale of common equity securities pursuant to this registration statement under the Securities Act, or December 31, 2025.

In addition, under the JOBS Act, emerging growth companies can delay adopting new or revised accounting standards issued subsequent to the enactment of the JOBS Act until such time as those standards apply to private companies. We have irrevocably elected not to avail ourselves of this exemption from new or revised accounting standards and, therefore, will be subject to the same new or revised accounting standards as other public companies that are not emerging growth companies. If we are unable to comply timely with these accounting standards, we may be delayed in providing the disclosures required by the Exchange Act.

As a “foreign private issuer,” we are exempt from a number of rules under the U.S. securities laws and the Nasdaq rules, and we are permitted to file less information with the SEC than are U.S. companies. In addition, we are permitted and expect to follow certain home country corporate governance practices in lieu of certain Nasdaq requirements applicable to domestic issuers. This may make our ADSs and ordinary shares less attractive to investors.

We are a “foreign private issuer,” as defined in the rules and regulations of the SEC, and, consequently, we are not subject to all of the disclosure requirements applicable to companies organized within the United States For example, we are exempt from certain rules under the Exchange Act that regulate disclosure obligations and procedural requirements related to the solicitation of proxies, consents or authorizations applicable to a security registered under the Exchange Act. In addition, our officers and directors are exempt from the reporting and “short-swing” profit recovery provisions of Section 16 of the Exchange Act and related rules with respect to their purchases and sales of our securities. Moreover, we are not required to file periodic reports and financial statements with the SEC as frequently or as promptly as U.S. public companies. Accordingly, there may be less publicly available information concerning our company than there is for U.S. public companies.

As a foreign private issuer traded on AIM and EGE, we are permitted to follow certain home country corporate governance practices in lieu of certain Nasdaq requirements. The rights of holders of ordinary shares and, therefore, certain of the rights of holders of the ADSs, are governed by English law, including the provisions of the Companies Act, and by our Articles of Association, which may provide less protection than is afforded to investors under Nasdaq rules applicable to domestic issuers.

In particular, we expect to follow English law instead of Nasdaq practice in the following ways:

• We do not intend to follow Nasdaq Rule 5620(c) regarding quorum requirements applicable to meetings of shareholders. Such quorum requirements are not required under English law. In accordance with generally accepted business practice, our Articles of Association provide alternative quorum requirements that are generally applicable to meetings of shareholders.

• We do not intend to follow Nasdaq Rule 5605(b)(2), which requires that independent directors regularly meet in an executive session, where only independent directors are present. The independent directors may choose to meet in an executive session at their discretion.

Although we may rely on certain home country corporate governance practices, we must comply with Nasdaq Rule 5625, Notification of Noncompliance and Rule 5640, Voting Rights. Further, we must have an audit committee that satisfies Rule 5605(c)(3), which addresses audit committee responsibilities and authority, and that consists of committee members who meet the independence requirements of Rule 5605(c)(2)(A)(ii).

We intend to take all actions necessary to maintain compliance as a foreign private issuer under the applicable corporate governance requirements of the Sarbanes-Oxley Act, the rules adopted by the SEC and the Nasdaq corporate governance rules and listing standards.

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It is possible that we will be a passive foreign investment company for U.S. federal income tax purposes.

A non-U.S. corporation will be classified as a passive foreign investment company (a “PFIC”) for U.S. federal income tax purposes for any taxable year if either:

• at least 75% of its gross income is “passive income,” or

• at least 50% of the value, determined on the basis of a quarterly average, of its gross assets is attributable to assets that produce or are held for the production of “passive income.”

Passive income generally includes dividends, interest, royalties, rents (other than certain rents and royalties derived in the active conduct of a trade or business), annuities and gains from assets that produce passive income. Cash is a passive asset for PFIC purposes, even if held as working capital.

Based on the composition of our income and assets, we do not expect to be a PFIC for U.S. federal income tax purposes for any taxable year, but this conclusion is a factual determination that is made annually and thus may be subject to change. Because PFIC status is determined based on the composition of our income and assets annually and generally cannot be determined until the end of the taxable year, there can be no assurance that we will not be a PFIC for the current taxable year or any future taxable year.

If we are a PFIC for any taxable year during which you own ADSs and ordinary shares, and you are a U.S. Holder (as defined in this prospectus in the section titled “Income Tax Considerations”), you will generally be subject to additional taxes and interest charges on the sale of ADSs and ordinary shares or upon receipt of an “excess distribution” with respect to the ADSs and ordinary shares. In general, a U.S. Holder would receive an “excess distribution” if the amount of any distribution for U.S. federal income tax purposes in respect of the ADSs and ordinary shares is more than 125% of the average distributions made with respect to the ADSs and ordinary shares within the three preceding taxable years (or shorter period in which such U.S. Holder held the ADSs and ordinary shares). A U.S. Holder of a PFIC generally may mitigate these adverse U.S. federal income tax consequences by making a “qualified electing fund” election or a “mark-to-market” election. However, there is no assurance that we will provide such information that would enable a U.S. Holder to make a qualified electing fund election. In addition, as a PFIC, dividends on the ADSs and ordinary shares would not be eligible for the special tax rate available to non-corporate U.S. Holders applicable to “qualified dividend income.” U.S. owners of ADSs and ordinary shares should consult their own U.S. tax advisors regarding the potential application of the PFIC rules.

Future sales, or the possibility of future sales, of a substantial number of ADSs representing our shares or our shares could adversely affect the price of such securities.

Future sales of a substantial number of ADSs representing our shares or our shares, or the perception that such sales will occur, could cause a decline in the market price of ADSs representing our shares and our shares. As of March 31, 2020, we had 154,498,887 shares issued and outstanding (not including 17,186,273 zero cost warrants that may be exercised at any time or 4,864,656 ordinary shares held as treasury shares), including 45,715,070 shares represented by ADSs. All of our outstanding shares are freely tradeable on AIM and EGE. Upon the effectiveness of the registration statement of which this prospectus forms a part, holders of our shares registered will be able to sell such shares in the form of ADSs, and such ADSs will be freely tradeable. If holders sell substantial amounts of ADSs or shares in the respective public markets therefor, or if the market perceives that such sales may occur, the market price of ADSs representing our shares and our shares and our ability to raise capital through an issue of equity securities in the future could be adversely affected.

Holders of our ADSs have fewer rights than our shareholders and are subject to certain additional risks.

ADS holders do not hold ordinary shares directly and, as such, are subject to, among others, the following additional risks:

• As an ADS holder, we will not treat you as one of our shareholders and you will not be able to exercise shareholder rights, except through the depositary as permitted by the deposit agreement.

• Distributions on the ordinary shares represented by your ADSs will be paid to the depositary, and before the depositary makes a distribution to you on behalf of your ADSs, any withholding taxes that must be paid will be deducted. Additionally, if the exchange rate fluctuates during a time when the depositary cannot convert the foreign currency, you may lose some or all of the value of the distribution.

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• We and the depositary may amend or terminate the deposit agreement without the ADS holders’ consent in a manner that could prejudice ADS holders.

ADS holders must act through the depositary to exercise their voting rights and, as a result, may be unable to exercise their voting rights on a timely basis.

We will not treat holders of our ADSs (rather than the ordinary shares underlying the ADSs) as shareholders, and they will not be able to exercise shareholder rights. The depositary will be the holder of the ordinary shares underlying the ADSs, and ADS holders will be able to exercise voting rights with respect to the ordinary shares represented by the ADSs only in accordance with the deposit agreement relating to the ADSs. There are practical limitations on the ability of ADS holders to exercise their voting rights due to the additional procedural steps involved in communicating with these holders. For example, holders of our ordinary shares will receive notice of shareholders’ meetings by mail and will be able to exercise their voting rights by either attending the shareholders meeting in person or voting by proxy. ADS holders, by comparison, will not receive notice directly from us. Instead, in accordance with the deposit agreement, we will use commercially reasonable endeavors to provide at least 30 days’ notice to the depositary of any such shareholders’ meeting and details concerning the matters to be voted on in advance of the meeting date. If we so instruct, the depositary will mail to holders of ADSs the notice of the meeting and a statement as to the manner in which voting instructions may be given by holders as soon as practicable after receiving notice from us of any such meeting. To exercise their voting rights, ADS holders must then instruct the depositary as to voting the ordinary shares represented by their ADSs. Due to these procedural steps involving the depositary, the process for exercising voting rights may take longer for ADS holders than for holders of ordinary shares. The ordinary shares represented by ADSs for which the depositary fails to receive timely voting instructions will not be voted.

ADS holders may have difficulty in effecting service of process on our company and certain directors or officers in the United States in enforcing U.S. judgements in the United Kingdom or in enforcing U.S. securities laws in UK courts.

We are incorporated and located outside the United States and certain of our directors and officers are located outside of the United States. As a result, it may not be possible for ADS holders to effect service of process within the United States upon all such persons or our company, or to obtain discovery of relevant documents and/or the testimony of witnesses. ADS holders based in the United States may also have difficulty enforcing in courts outside the United States judgments obtained in U.S. courts against our company or our directors (including actions under the civil liability provisions of the U.S. securities laws). ADS holders may also have difficulty enforcing liabilities under the U.S. securities laws in legal actions originally brought in jurisdictions located outside the United States.

Currency fluctuations may adversely affect the price of our ADSs.

Our ordinary shares are quoted in Sterling pence on AIM, and our ADSs will be quoted in U.S. dollars on the Nasdaq. Movements in the Sterling/U.S. dollar exchange rate may adversely affect the U.S. dollar price of the ADSs on the Nasdaq or the Sterling price on AIM. For example, if the Sterling weakens against the U.S. dollar, the U.S. dollar price of our ADSs could decline, even if the price of our ordinary shares in Sterling increases or remains unchanged.

We have never declared or paid dividends on our ordinary shares, and we do not anticipate paying dividends in the foreseeable future.

We have never declared or paid cash dividends on our ordinary shares. For the foreseeable future, we intend to retain all available funds and any future earnings to support our operations and to finance the growth and development of our business. Any future determination to declare cash dividends will be made at the discretion of the Board, subject to compliance with applicable laws and covenants under current or future debt facilities, which may restrict or limit our ability to pay dividends, and will depend on our financial condition, operating results, capital requirements, general business conditions and other factors that the Board may deem relevant. We do not anticipate paying any cash dividends on our ordinary shares in the foreseeable future. As a result, a return on any investment will only occur if the price of our ordinary shares increases.

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ADS holders may not receive distributions on the ordinary shares represented by our ADSs or any value for such distribution if it is illegal or impractical to make them available to holders of ADSs.

While we do not anticipate paying any dividends on its ordinary shares in the foreseeable future, if such a dividend is declared, the depositary for the ADSs has agreed to pay to you the cash dividends or other distributions it or the custodian receives on our ordinary shares or other deposited securities after deducting its fees and expenses. You will receive these distributions in proportion to the number of ordinary shares your ADSs represent. However, in accordance with the limitations set forth in the deposit agreement, it may be unlawful or impractical to make a distribution available to holders of ADSs. We have no obligation to take any other action to permit the distribution of the ADSs, ordinary shares, rights or anything else to holders of our ADSs. This means that you may not receive the distributions we make on our ordinary shares or any value from them if it is unlawful or impractical to make them available to you. These restrictions may have a material adverse effect on the value of your ADSs.

ADS holders may not be able to participate in rights offerings and may experience dilution of their holdings as a result.

We may from time to time distribute rights to our shareholders, including rights to acquire our securities. However, we may not, and under the deposit agreement for the ADSs, the depositary will not, offer those rights to ADS holders unless both the rights and the underlying securities to be distributed to ADS holders are registered under the Securities Act or the distribution of them to ADS holders is exempt from registration under the Securities Act with respect to all holders of ADSs. We are under no obligation to file a registration statement with respect to any such rights or underlying securities or to endeavor to cause such a registration statement to be declared effective. In addition, we may not be able to rely on an exemption from registration under the Securities Act to distribute such rights and securities. Accordingly, holders of the ADSs may be unable to participate in our rights offerings and may experience dilution in their holdings as a result.

ADS holders may be subject to limitations on transfer of the ADSs.

The ADSs are only transferable on the books of the depositary. However, the depositary may close its transfer books at any time or from time to time when it deems expedient in connection with the performance of its duties. In addition, the depositary may refuse to deliver, transfer or register transfers of ADSs generally when our books or the books of the depositary are closed, or at any time if we or the depositary deem it advisable to do so because of any requirement of law or of any government or governmental body, or under any provision of the deposit agreement, or for any other reason.

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SPECIAL NOTE REGARDING FORWARD-LOOKING STATEMENTS

This prospectus contains forward-looking statements concerning our business, operations and financial performance and condition, as well as our plans, objectives and expectations for our business operations and financial performance and condition. Any statements contained herein that are not statements of historical facts may be deemed to be forward-looking statements. In some cases, you can identify forward-looking statements by terminology such as “aim,” “anticipate,” “assume,” “believe,” “contemplate,” “continue,” “could,” “due,” “estimate,” “expect,” “goal,” “intend,” “may,” “might,” “objective,” “plan,” “predict,” “potential,” “positioned,” “seek,” “should,” “target,” “will,” “would,” and other similar expressions that are predictions of or indicate future events and future trends, or the negative of these terms or other comparable terminology, although not all forward-looking statements contain these identifying words.

By their nature, forward-looking statements involve risk and uncertainty because they relate to future events and circumstances. Forward-looking statements are not guarantees of future performance and have not been reviewed by our auditors. Actual results and developments could differ materially from those expressed or implied by the forward-looking statements due to a variety of factors, including, but not limited to, those identified under the section titled “Risk Factors” in this prospectus.

These risks and uncertainties include factors related to:

• our significant operating losses since our inception and ability to obtain and maintain profitability in the future;

• our commercial products, including statements regarding the expected strategies and profitability thereof;

• our product candidates, including statements regarding the expected initiation, timing, progress and availability of data from clinical trials, all of which may be adversely impacted by the rapidly evolving COVID-19 global pandemic;

• our ability to successfully commercialize, or enter into strategic relationships with third parties to commercialize, our products or product candidates, if approved;

• our ability to acquire or in-license new product candidates;

• our competition, most of whom have far greater resources than we have, which may make it more difficult for us to achieve significant market penetration;

• the size of our addressable markets and market trends;

• potential strategic relationships;

• our ability to obtain and maintain intellectual property rights;

• the impact of potential fluctuations in foreign currency exchange rates;

• estimates regarding expenses, future revenues, capital requirements and the need for additional financing; and

• risks associated with the COVID-19 pandemic, which may adversely impact our business, preclinical studies and clinical trials.

The preceding list is not intended to be an exhaustive list of all of our risks and uncertainties. As a result of these factors, we cannot assure you that the forward-looking statements in this prospectus will prove to be accurate. Furthermore, if our forward-looking statements prove to be inaccurate, the inaccuracy may be material. In light of the significant uncertainties in these forward-looking statements, you should not regard these statements as a representation or warranty by us or any other person that we will achieve our objectives and plans in any specified time frame, or at all.

In addition, statements that “we believe” and similar statements reflect our beliefs and opinions on the relevant subject. These statements are based upon information available to us as of the date of this prospectus, and while we believe such information forms a reasonable basis for such statements, such information may be limited or incomplete, and our statements should not be read to indicate that we have conducted an exhaustive inquiry into, or review of, all relevant information. These statements are inherently uncertain and investors are cautioned not to unduly rely upon these statements.

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The forward-looking statements and opinions contained in this prospectus are based upon information available to us as of the date of this prospectus and, while we believe such information forms a reasonable basis for such statements, such information may be limited or incomplete, and our statements should not be read to indicate that we have conducted an exhaustive inquiry into, or review of, all potentially available relevant information. The forward-looking statements contained in this prospectus speak only as of the date of this prospectus, and we do not undertake any obligation to update them in light of new information or future developments or to release publicly any revisions to these statements in order to reflect later events or circumstances or to reflect the occurrence of unanticipated events.

You should read this prospectus and the documents referenced herein and have filed as exhibits to the registration statement, of which this prospectus forms a part, completely and with the understanding that our actual future results may be materially different from expectations. We qualify all forward-looking statements in this prospectus by these cautionary statements.

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USE OF PROCEEDS

We will not receive proceeds from the disposition, if any, of Registered Shares in the form of ADSs by Registered Holders.

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DIVIDEND POLICY

We have never paid or declared any cash dividends on our ordinary shares, and we do not anticipate paying any cash dividends on our ordinary shares in the foreseeable future. We intend to retain all available funds and any future earnings to fund the development and expansion of our business. See “Risk Factors—Risks Related to Ownership of our ADSs and Ordinary Shares and Our Prospective Nasdaq Listing—We have never declared or paid dividends on our ordinary shares, and we do not anticipate paying dividends in the foreseeable future.”

All of the ordinary shares represented by the ADSs offered by this prospectus will generally have the same dividend rights as all of our other outstanding ordinary shares. However, the depositary may limit distributions based on practical considerations and legal limitations. See “Description of American Depositary Shares—Dividends and Other Distributions.” Cash dividends on our ordinary shares, if any, will be paid in Euros or U.S. dollars with respect to the ADSs as provided in the deposit agreement.

Under English law, among other things, we may pay dividends only if we have sufficient distributable reserves (on a non-consolidated basis), which are our accumulated realized profits that have not been previously distributed or capitalized less our accumulated realized losses, so far as such losses have not been previously written off in a reduction or reorganization of capital.

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CAPITALIZATION

The following table sets forth our cash and cash equivalents and our capitalization as of March 31, 2020.

The data below is derived from our unaudited condensed consolidated financial statements. You should read this information together with our audited and unaudited condensed consolidated financial statements appearing elsewhere in this prospectus and the information set forth under the headings “Selected Consolidated Financial Data,” “Use of Proceeds” and “Management’s Discussion and Analysis of Financial Condition and Results of Operations.”

	As of March 31, 2020
	(in thousands)
Cash and cash equivalents	$68,067
Long-term debt
Secured Credit Facility	$82,989
Convertible Notes, principal amount	125,000
Total long-term debt	207,989
Equity:
Share capital	11,918
Share premium	2,422
Other reserves	249,386
Accumulated deficit	(162,569)
Total equity	101,157
Total capitalization	$309,146

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SELECTED CONSOLIDATED FINANCIAL DATA

The tables below set forth the following selected consolidated financial data:

• Selected consolidated statements of comprehensive loss of Amryt for the years ended December 31, 2018 and 2019 and a selected consolidated statement of financial position of Amryt as of December 31, 2019, which have been derived from our audited consolidated financial statements included elsewhere in this prospectus.

• Selected condensed consolidated statements of comprehensive loss of Amryt for the three-month periods ended March 31, 2020 and 2019 and a selected condensed consolidated statement of financial position of Amryt as of March 31, 2019, which have been derived from our unaudited condensed consolidated financial statements included elsewhere in this prospectus.

• Selected consolidated statements of comprehensive loss of Aegerion for the years ended December 31, 2017 and 2018, which have been derived from Aegerion’s audited consolidated financial statements included elsewhere in this prospectus. Aegerion’s audited consolidated financial statements have been prepared in accordance with U.S. GAAP.

• Selected consolidated statements of comprehensive loss of Aegerion for the six months ended June 30, 2019 and a selected consolidated statement of financial position of Aegerion as of June 30, 2019, which have been derived from Aegerion’s unaudited interim consolidated financial statements included elsewhere in this prospectus. Aegerion’s unaudited interim consolidated financial statements have been prepared in accordance with U.S. GAAP. The accounting principles applied in Aegerion’s unaudited interim financial statements are consistent with those used in Aegerion’s annual audited financial statements. In the opinion of management, the unaudited data reflects all adjustments, consisting only of normal recurring adjustments, necessary for a fair presentation of the financial information in those statements.

Our consolidated financial statements have been prepared in accordance with IFRS, as issued by the IASB. Historical results for any prior period do not necessarily indicate our results to be expected for any future period. Interim financial results for the periods presented are not necessarily indicative of results for a full year or for any subsequent interim period.

You should read the following selected consolidated financial data together with the financial statements included elsewhere in this prospectus and the sections titled “Selected Consolidated Financial Data,” “Pro Forma Financial Information,” and “Management’s Discussion and Analysis of Financial Condition and Results of Operations.”

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Selected Consolidated Financial Data of Amryt

	Three Months Ended March 31		Year Ended December 31,
	2020	2019	2019	2018
	(unaudited)
	(In thousands, except per share data)
Statement of comprehensive income/(loss) data:
Revenue	$44,574	4,542	$58,124	$17,095
Cost of sales	(32,620)	(1,830)	(42,001)	(6,266)
Gross profit	11,954	2,712	16,123	10,829
Total administrative, selling and marketing expenses	(19,151)	(3,987)	(36,339)	(18,163)
Research and development expenses	(8,934)	(1,505)	(15,827)	(10,703)
Impairment charge	—	—	(4,670)	—
Restructuring and acquisition costs	(853)	—	(13,038)	—
Operating loss before finance expense	(16,984)	(2,780)	$(53,751)	$(18,037)
Non-cash change in fair value of contingent consideration	(2,906)	(1,938)	(6,740)	(10,566)
Non-cash contingent value rights finance expense	(1,448)	—	(1,511)	—
Net finance expense — other	(9,416)	(661)	(4,759)	(1,841)
Loss on ordinary activities before taxation	(30,754)	(5,379)	$(66,761)	$(30,444)
Tax credit/(charge) on loss on ordinary activities	1,857	(6)	1,226	(43)
Loss for the period/year attributable to the equity holders	(28,897)	(5,385)	(65,535)	(30,487)
Total other comprehensive (loss)/income	(13)	80	781	(77)
Total comprehensive loss for the period/year attributable to the equity holders	$(28,910)	(5,305)	$(64,754)	$(30,564)
Loss per share — basic and diluted	$(0.19)	(0.12)	$(0.86)	$(0.67)

	As of March 31, 2020	As of December 31, 2019
	(unaudited)
	(In thousands)
Statement of financial position data:
Cash and cash equivalents	$68,067	$67,229
Trade and other receivables	41,179	36,387
Inventories	33,904	43,623
Working capital(1)	31,443	47,025
Total assets	518,229	534,347
Secured Credit Facility	82,989	81,610
Convertible Notes, net of equity component	97,872	96,856
Total liabilities	417,072	405,025
Accumulated deficit	162,569	133,674
Total equity	101,157	129,322

(1)
We define working capital as current assets less current liabilities.

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Selected Consolidated Financial Data of Aegerion⁽¹⁾

	Six Months Ended June 30, 2019	Year Ended December 31,
		2018	2017
	(In thousands)
Statement of comprehensive loss data:
Net revenues	$95,857	$130,432	$138,438
Cost of product sales	35,364	59,697	77,220
Operating expenses:
Selling, general and administrative	43,424	64,437	77,793
Research and development	13,946	38,064	44,895
Restructuring charges	—	2,171	121
Related party expense (income), net	397	942	(177)
Total operating expenses	57,767	105,614	122,632
Loss from operations	2,726	(34,879)	(61,414)
Reorganization items, net	(2,145)	—	—
Interest expense, net	(29,681)	(50,746)	(39,467)
Interest expense due to Novelion	(1,182)	(2,987)	(1,089)
Loss on extinguishment of debt	—	(4,333)	—
Other expense, net	(224)	(1,888)	(836)
Loss before provision for income taxes	(30,506)	(94,833)	(102,806)
Provision for income taxes	(369)	(1,705)	(594)
Net loss	$(30,875)	$(96,538)	$(103,400)

	As of June 30, 2019
	(In thousands)
Statement of financial position data:
Cash and cash equivalents	$36,080
Trade and other receivables	26,408
Inventories	51,792
Total assets	322,634
Total current liabilities	67,434
Provision for legal settlements - non-current	11,962
Other non-current liabilities	1,444
Total liabilities not subject to compromise	80,840
Liabilities subject to compromise	420,651
Total liabilities	501,491
Total equity	(178,857)

(1)
Debtor in possession as of June 30, 2019 but not as of December 31, 2017 and 2018.

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PRO FORMA FINANCIAL INFORMATION

On September 24, 2019, we completed the acquisition (the “Acquisition”) of Aegerion Pharmaceuticals, Inc. (“Aegerion”) pursuant to the Aegerion Plan of Reorganization. As a result of the Acquisition, Aegerion became an indirect wholly owned subsidiary of Amryt Pharma plc. The following transactions were completed in connection with the Acquisition and Aegerion’s emergence from bankruptcy:

• Prior to consummation of the Acquisition, we conducted a private placement of ordinary shares to certain accredited investors, which generated gross proceeds of approximately $8 million. Proceeds from this issuance were used primarily to pay for transaction costs incurred in connection with the Acquisition.

• Prior to consummation of the Acquisition, we also issued the CVRs to holders of ordinary shares and to employee option holders entitling them to proceeds of up to $85 million upon the occurrence of specified milestones related to the regulatory approval and commercialization of AP101.

• On September 24, 2019, we completed a $60 million fundraising by way of the issue of (i) new ordinary shares at a price of $1.79 per ordinary share, and (ii) zero cost warrants to new and existing investors, and certain creditors of Aegerion. Proceeds from this issuance were used to fund development of our product pipeline and potential new indications for our late stage product candidates and for general corporate purposes.

• On September 24, 2019, Aegerion issued $125 million in aggregate principal amount of the Convertible Notes, to certain of its pre-bankruptcy creditors. The Convertible Notes were issued in satisfaction of the creditors’ claims against Aegerion pursuant to Section 1145 of the Bankruptcy Code.

• On September 24, 2019, we entered into a new five-year $81 million first-lien Secured Credit Facility guaranteed by certain of our subsidiaries and bearing an interest rate of (x) 11% per annum paid in cash, or (y) 6.5% per annum paid in cash plus 6.5% per annum paid in kind, in each case, on a quarterly basis.

For purposes of the following pro forma financial information and the notes hereto, we refer to the foregoing series of transactions, including the Acquisition, as the “Transactions.” The pro forma financial information was prepared (i) based on our historical financial statements and the assumptions and adjustments as described in the notes to the unaudited pro forma financial information, and (ii) consistent with the requirements of Article 11 of Regulation S-X.

The unaudited pro forma financial statement of loss for the year ended December 31, 2019 gives effect to the Transactions as if they had occurred on January 1, 2019. The pro forma adjustments are based upon available information and methodologies that are factually supportable and directly attributable to the Transactions. In addition, the unaudited pro forma financial information reflects only those adjustments that are expected to have a continuing impact on our results of operations. The pro forma adjustments are based on currently available information and certain estimates and assumptions. Therefore, the actual adjustments may differ from the pro forma adjustments. Because the Acquisition was completed on September 24, 2019, no unaudited pro forma condensed combined balance sheet as at December 31, 2019 is presented herein.

The unaudited pro forma financial information is presented for illustrative purposes only and does not purport to represent our consolidated results of operations that would actually have occurred had the Transactions been consummated on January 1, 2019 or to project our consolidated results of operations for any future date or period.

The unaudited pro forma financial information should be read together with the sections titled “Capitalization,” “Management’s Discussion and Analysis of Financial Condition and Results of Operations,” and the historical financial statements of Amryt and Aegerion (together with the related notes thereto) included elsewhere in this prospectus.

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UNAUDITED PRO FORMA STATEMENT OF LOSS

	Amryt consolidated loss for the year ended December 31, 2019	Aegerion consolidated loss for the period to June 30, 2019	Aegerion consolidated profit for the period from July 1 to September 24, 2019	Adjustments	Notes	Pro forma consolidated loss for the year ended December 31, 2019
	IFRS	U.S. GAAP	U.S. GAAP			IFRS
	(In thousands, except per share data)
Net revenues	$58,124	$67,362	$33,742	$(2,463)	3a	$156,765
Upfront license fee	—	28,495	—	—		28,495
Total Revenues	58,124	95,857	33,742	(2,463)		185,260
Cost of product sales	(19,803)	(22,209)	(11,127)	2,463	3a	(50,676)
Amortization of acquired intangibles	(11,831)	(12,548)	(5,856)	(9,772)	3b	(40,007)
Amortization of inventory fair value step-up	(10,367)	(607)	(1,359)	—		(12,333)
Total cost of sales	(42,001)	(35,364)	(18,342)	(7,309)		(103,016)
Gross profit	16,123	60,493	15,400	(9,772)		82,244
Selling, general and administrative expenses	(35,498)	(45,607)	(29,244)	50,172	3c	(60,177)
Research and development expenses	(15,827)	(13,676)	(8,628)	5		(38,126)
Restructuring and acquisition costs	(13,038)	—	—	13,038	3d	—
Shared based payment expense	(841)	(703)	20	(704)	3e	(2,228)
Impairment of intangible assets	(4,670)	—	—	—		(4,670)
Operating (loss)/profit before finance expense	(53,751)	507	(22,452)	52,739		(22,957)
Net finance (expense)/income	(4,759)	(31,012)	77,281	(60,382)	3f	(18,872)
Non-cash change in fair value of contingent consideration	(6,740)	—	—	—		(6,740)
Non-cash contingent value rights finance expense	(1,511)	—	—	(3,860)	3g	(5,371)
(Loss)/profit on ordinary activities before taxation	(66,761)	(30,505)	54,829	(11,503)		(53,940)
Tax credit/(charge) on ordinary activities	1,226	(369)	26	—		883
(Loss)/profit for the year attributable to the equity holder of the company	$(65,535)	$ (30,874)	$54,855	$(11,503)		$(53,057)
Loss per share – basic and diluted, attributable to ordinary equity holders of the parent (US$)	$(0.86)	—	—	—		$(0.34)
Weighted average number of ordinary shares in issue	75,871,562	—	—	—		154,498,887

Notes to Unaudited Pro Forma Statement of Loss

1.
Basis of preparation

The historical consolidated financial statements of Amryt and Aegerion have been adjusted in the pro forma combined financial information to give effect to pro forma events that are (1) directly attributable to the Acquisition, (2) factually supportable and (3) expected to have a continuing impact on our combined results. The Acquisition was accounted for as a business combination under the acquisition method of accounting in accordance with IFRS 3, Business Combinations.

2.
Accounting policy conformity change

The historical financial information of Aegerion was prepared in accordance with U.S. GAAP. The adjustments convert Aegerion’s financial information from U.S. GAAP to IFRS and align Aegerion’s accounting policies to those applied by Amryt. These adjustments are detailed in Note 3.

3.
Pro forma adjustments

The pro forma adjustments are based on Amryt’s preliminary estimates and assumptions that are subject to change. The following adjustments have been reflected in the unaudited pro forma combined financial information:

a)
Adjustment to eliminate inter-company transactions between Amryt and Aegerion. Revenues have been reduced to reflect the royalty income of $2.4 million recognized by Aegerion prior to the Acquisition, and cost of product sales has been reduced by the same amount to reflect the cost in Amryt’s historical financial statements.

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b)
Adjustment to amortization of acquired intangibles of $9.8 million consists of:

i)
amortization expense in the historical financial statements of Aegerion converted to IFRS, resulting in a reduction in amortization expense of $0.6 million;

ii)
elimination of the historical amortization expense on the Aegerion intangible assets acquired by Amryt, including the IFRS adjustment above, of $18.4 million; and

iii)
recognition of a revised amortization expense of $28.2 million, based on the fair value of intangible assets acquired. Amortization is calculated on a straight-line basis.

c)
Adjustment to selling, general and administrative expenses of $50.2 million consists of:

i)
the elimination of Aegerion non-recurring restructuring, acquisition, severance and bankruptcy expenses, totalling $50.4 million. These costs were incurred by Aegerion prior to the Acquisition and are excluded on the basis that they are not expected to have a continuing impact on our combined results following the Acquisition; and

ii)
additional selling, general and administrative expenses of $0.2 million arising from reclassification of $0.7 million of expenses and $0.5 million of income from finance expenses to selling, general and administrative expenses.

d)
Adjustment to restructuring and acquisition expenses to eliminate Amryt’s non-recurring restructuring and acquisition expenses. These expenses are excluded on the basis that they are not expected to have a continuing impact on our combined results following the Acquisition.

e)
Increase in share based payment expenses of $0.7 million to reflect the increase in Aegerion’s share based payment expenses under IFRS 2.

f)
Adjustment made for the following:

i)
elimination of finance charges, unamortized debt discounts and debt issuance costs totalling $39.4 million and gains on the extinguishment of debt of $86.2 million, recognized by Aegerion in its historical financial statements;

ii)
elimination of finance charges of $2.8 million relating to the EIB loan facility held by Amryt which was fully repaid in connection with the Acquisition;

iii)
inclusion of interest expenses of $8.1 million relating to the new Secured Credit Facility and $7.6 million relating to the Convertible Notes, assuming that these liabilities were incurred on January 1, 2019;

iv)
reclassification of $0.5 million included in interest income in the historical financial statements of Aegerion to selling, general and administrative expenses; and

v)
additional net finance charges of $0.1 million to reflect $0.8 million booked as an IFRS adjustment for amortization of Aegerion’s debt issuance costs, partially offset by a reclassification of $0.7 million to selling, general and administrative expenses relating to interest expense on prepetition claims and interest on liability settlements in Aegerion’s historical financial statements.

g)
Adjustment for non-cash financing expenses, assuming that the CVRs were issued on January 1, 2019.

4.
Exclusion from Pro Forma Adjustments

In early 2019, Aegerion out-licensed the rights to JUXTAPID for distribution in Japan to Recordati. This has not been eliminated in the pro forma statement of loss on the grounds that it is not directly attributable to the Acquisition.

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MANAGEMENT’S DISCUSSION AND ANALYSIS OF FINANCIAL CONDITION AND
RESULTS OF OPERATIONS

You should read the following discussion and analysis of our financial condition and results of operations together with the information in “Selected Consolidated Financial Data,” “Pro Forma Financial Information” and the Consolidated Financial Statements of each of Amryt and Aegerion, including the notes thereto. The following discussion is based on financial information prepared, in the case of Amryt, in accordance with IFRS as issued by the IASB, and, in the case of Aegerion, in accordance with U.S. GAAP. The following discussion includes forward-looking statements that involve risks, uncertainties and assumptions. Actual results may differ materially from those anticipated in these forward-looking statements as a result of many factors, including but not limited to those described under “Risk Factors” and elsewhere in this prospectus.

Overview

We are a global, commercial-stage biopharmaceutical company dedicated to commercializing and developing novel therapeutics to treat patients suffering from serious and life-threatening rare diseases. We have built a diverse portfolio of commercial and development stage assets including:

• lomitapide, an approved treatment in the United States and the European Union for adult patients with HoFH;

• metreleptin, an approved treatment in the United States for GL and in the European Union for GL and PL;

• AP101, our lead development asset, which is currently in a pivotal Phase 3 trial as a potential treatment for severe EB (if AP101 is approved for this indication, we intend to market it under the name FILSUVEZ);

• AP103, our first product candidate utilizing our novel polymer-based topical gene therapy delivery platform, which is in preclinical development as a potential treatment for patients with EB and other topical indications; and

• Imlan, a range of derma-cosmetic products marketed solely in Germany as a treatment for sensitive, allergy-prone and dry skin.

On September 24, 2019, we completed the Acquisition of Aegerion, a wholly-owned operating subsidiary of Novelion Therapeutics Inc. We believe that this Acquisition will advance our ambition to create a global leader in rare and orphan diseases. Prior to the Acquisition, we licensed the rights from Aegerion to sell lomitapide in Europe and the Middle East, while Aegerion retained the rights to this product in the United States and elsewhere. Following the Acquisition, we sell lomitapide and metreleptin globally, excluding Japan for lomitapide and Japan, South Korea and Taiwan for metreleptin.

Due to the significance of the Acquisition, our post-acquisition financial results differ materially from our pre-Acquisition historical performance, beginning with our audited financial results for the year ended December 31, 2019. In addition, due to how recently we completed the Acquisition, we may not be able to accurately predict our near-term combined financial performance.

Acquisition of Aegerion

The following transactions were completed in connection with the Acquisition and Aegerion’s emergence from bankruptcy:

• Prior to consummation of the Acquisition, we conducted a private placement of ordinary shares to certain accredited investors, which generated gross proceeds of approximately $8 million. Proceeds from this issuance were used primarily to pay for transaction costs incurred in connection with the Acquisition.

• Prior to consummation of the Acquisition, we also issued the CVRs to holders of ordinary shares and to employee option holders entitling them to proceeds of up to $85 million upon the occurrence of specified milestones related to the regulatory approval and commercialization of AP101.

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• On September 24, 2019, we completed a $60 million fundraising by way of the issue of (i) new ordinary shares at a price of $1.79 per ordinary share, and (ii) zero cost warrants to new and existing investors, and certain creditors of Aegerion. Proceeds from this issuance are being used to fund development of our product pipeline and potential new indications for our late stage product candidates and for general corporate purposes.

• On September 24, 2019, we issued $125 million in aggregate principal amount of the Convertible Notes, to certain of Aegerion’s pre-bankruptcy creditors. The Convertible Notes were issued in satisfaction of the creditors’ claims against Aegerion pursuant to Section 1145 of the Bankruptcy Code.

• On September 24, 2019, we entered into a five-year $81 million Secured Credit Facility, guaranteed by certain of our subsidiaries and bearing an interest rate of (x) 11% per annum paid in cash, or (y) 6.5% per annum paid in cash plus 6.5% per annum paid in kind, in each case, on a quarterly basis.

Aegerion Investigations and Legal Proceedings

Aegerion had been the subject of certain investigations and other legal proceedings (some of which remain ongoing), including investigations of Aegerion’s marketing and sales activities of JUXTAPID by the DOJ and the SEC, and an investigation by federal prosecutors in Brazil to determine whether there have been violations of Brazilian laws related to sales of JUXTAPID. In September 2017, Aegerion entered into a settlement with the DOJ, the SEC and other federal and state government agencies (“Aegerion Settlements”) that required Aegerion, in addition to paying certain penalties and Aegerion Settlement amounts, to plead guilty to two misdemeanors involving misbranding violations of the Food, Drug and Cosmetics Act and to enter into a three-year deferred prosecution agreement, which is scheduled to end in September 2020, with regard to a charge that it engaged in a conspiracy to violate HIPAA), and to enter into a Consent Decree of Permanent Injunction with the FDA. Aegerion was sentenced by the U.S. District Court on January 30, 2018. Under the terms of the Aegerion Settlements and the sentence, Aegerion was required to pay approximately $40.1 million in aggregate penalties, which includes $7.2 million in restitution payable over three years beginning January 2018, a civil penalty of $4.1 million to be paid to the SEC pursuant to the SEC judgment, and $28.8 million to be paid pursuant to the settlement agreement with the DOJ. We assumed these obligations in connection with the Acquisition and have been making these penalty payments in accordance with the terms of the Aegerion Settlements. If we fail to satisfy obligations under the Aegerion Settlements, any outstanding amount will accrue interest at a rate of 1.75% per annum, and we may be subject to further penalties or litigation. The Aegerion Settlements also mandate extensive remedial and compliance measures. The failure to comply with any provisions of the Aegerion Settlements, including the financial, remedial and compliance measures, could result in the imposition of additional fines, penalties and obligations, and could subject us to prosecution or exclusion from federal healthcare programs in the United States. As of December 31, 2019, the total remaining outstanding payment due under the Aegerion Settlements, inclusive of interest, was $19.7 million.

Key Factors Affecting Our Business

Our ability to expand approved indications and markets for lomitapide and metreleptin

We intend to take steps necessary to seek approval for the use of lomitapide to treat pediatric HoFH and for the treatment of FCS and for the use of metreleptin to treat PL in the United States. We also intend to seek marketing approval for lomitapide in Brazil and for metreleptin in certain South American countries. Our ability to generate revenue from these potential new indications and markets depends on our success in completing development of and commercializing such products, which will require a significant investment of time and resources and may not ultimately prove successful.

Our ability to commercialize AP101, if approved

We expect to report data from our pivotal Phase 3 trial of AP101 for the treatment of severe EB in the second half of 2020. If the trial is successful, we intend to apply for approval of AP101 and, if approved, commercialize it under the name FILSUVEZ in the United States and Europe through our existing commercial infrastructure. If the commercialization effort requires more than the minimal additions we are anticipating, our financial results could suffer.

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Our ability to maintain and grow our revenues from our existing commercial products, metreleptin and lomitapide, given the impact of the current COVID-19 pandemic

Since a novel strain of coronavirus (SARS-CoV-2) causing a disease referred to as COVID-19 was first reported in December 2019, the disease has spread across the world, including countries in which we have patients and in which we have planned or active clinical trial sites. The outbreak and government measures taken in response thereto have had a significant impact, both direct and indirect, on businesses and commerce as supply chains have been disrupted, facilities and production have been suspended and demand for certain goods and services has spiked while demand for other goods and services has fallen. As COVID-19 continues to spread around the globe, we may experience disruptions that could affect our business, preclinical studies and clinical trials. See “Risk Factors” for additional information.

International operations and foreign currency exchange

We operate on a global basis with offices, sales and activities throughout the world. Our worldwide operations and sales have increased as a result of the Acquisition and the continued expansion of our commercial organization. Our global operations subject our financial results to fluctuations in foreign currency exchange rates, changes in general economic and political conditions in countries where we operate, particularly as a result of ongoing economic instability within foreign jurisdictions, sensitivity to governmental actions relating to tariffs or trade agreements, complex and restrictive employment and labor laws and regulations, as well as union and works council restrictions, sensitivity to changes in tax laws or rulings in jurisdictions across the world, longer payment cycles from customers in certain geographies, and legal compliance costs and risks.

Our ability to add rare disease assets to our portfolio

Identifying, acquiring and developing new products and product candidates to build shareholder value is key to our goal of becoming a global leader in the treatment of rare diseases. Our future profitability and growth will depend on our ability to acquire and in-license product candidates on favorable terms.

Key Components of Our Results of Operations

Revenues

Prior to the Acquisition, we generated revenues from the sale of LOJUXTA (the marketing name for lomitapide in the European Union) and Imlan, either directly to customers or through third parties that distribute these products to customers. For the remainder of 2019 following the Acquisition, we also generated revenues from (a) the sale of JUXTAPID (the non-EU marketing name for lomitapide), (b) the sale of metreleptin globally (marketed as MYALEPT in the United States and as MYALEPTA in the European Union), and (c) royalties paid to us from our JUXTAPID licensee in Japan.

Cost of Sales

Cost of sales is comprised of the cost of producing products, royalties on sales and the cost of delivery of goods sold to customers, including the costs associated with the services provided by the distributors to import and deliver the goods. Cost of sales also includes amortization of intangible assets that we acquired as part of the Acquisition, as well as amortization of the step-up in the fair value of the inventory acquired in the Acquisition.

Selling, General and Administrative (“SG&A”) expenses

SG&A expenses includes payroll and payroll-related costs, real estate and lease costs, insurance costs, fees payable to auditors, pension costs, marketing expenses and general corporate expenses. Following the Acquisition, our SG&A expenses have increased significantly with the expansion of the selling, regulatory, quality and compliance infrastructure in place for a company with a global commercial footprint.

We expect SG&A expenses in 2020 to increase compared to 2019, primarily due to the full-year effect of the Acquisition, including an increase in compensation-related expenses driven by higher headcount and other expenses related to the expansion and support of our business. We also expect an increase in expenses related to the preparation for the potential commercial launch of AP101 and the continuation of the commercial launch of metreleptin in the European Union following its approval in July 2018. In addition, as a result of becoming a U.S. public company listed on the Nasdaq, we will incur legal, accounting and other expenses that we did not previously incur, resulting in further increases in our SG&A expenses in future periods.

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Research and Development (“R&D”) expenses

R&D expenses include employee-related expenses, such as salaries and other benefits, for our R&D personnel; costs for production of drug product used in our clinical trials and development of our manufacturing processes; fees and other costs paid to third parties in connection with R&D, consultants and other suppliers to conduct our clinical trials, preclinical and non-clinical studies, and post approval commitment studies; and costs of facilities, materials and equipment related to our clinical trials and preclinical and non-clinical studies. We expense R&D costs as incurred, because they do not qualify to be capitalized as intangible assets under IFRS since the technical feasibility of the materials is not proven and no alternative use for them exists in the absence of marketing approval.

For 2020 and beyond, we expect that our R&D expenses will continue to increase from previous levels, particularly when a full year’s R&D costs associated with the acquired assets from Aegerion are reflected in the Consolidated Statement of Comprehensive Loss. We also anticipate that costs will increase as we prepare for anticipated regulatory submissions and data read-outs from clinical trials, initiate and undertake additional clinical trials and related development work, and potentially acquire rights to additional product candidates. Specific anticipated drivers of increases in R&D expenses are our efforts to complete clinical development and seek marketing approval for AP101, initiate clinical development of AP103, and continue with life cycle development opportunities and post approval commitments for lomitapide and metreleptin. R&D expenses may vary substantially from period to period based on the timing of our research and development activities, including timing due to regulatory approval and moving from preclinical to the clinic and enrolling patients into clinical trials.

If we are successful in our AP101 clinical development efforts we expect to incur significant milestone payment obligations as AP101 progresses toward potential commercialization as well as significant financial obligations under our CVRs.

Finance Items

Finance income (expense) includes interest on our marketable securities and realized and unrealized exchange rate gains (losses), as well as interest and fees paid on our outstanding indebtedness.

As part of the Acquisition, we entered into a new U.S. dollar denominated $81,021,000 Secured Credit Facility with various lenders. The Secured Credit Facility matures on September 24, 2024. Interest will be payable on a quarterly basis at the rate of (i) 11% per annum paid in cash on a quarterly basis or, our option, (ii) at the rate of 6.5% paid in cash plus 6.5% paid in kind. The Secured Credit Facility may be prepaid, in whole or in part, at any time subject to payment of an exit fee, which depending on the stage of the loan term, ranges from 5.0% to 0% of the principal then outstanding.

As part of the Acquisition, we also issued Convertible Notes with an aggregate principal amount of $125,000,000 to certain of Aegerion’s pre-bankruptcy creditors. The Convertible Notes are senior unsecured obligations and bear interest at a rate of 5.0% per year, payable semi-annually in arrears on April 1 and October 15 of each year. The Convertible Notes will mature on April 1, 2025, unless earlier repurchased or converted. As a result of the conversion feature in the Convertible Notes, the notes were assessed to have both a debt and an equity component. The two components were assessed separately and classified as a financial liability and equity instrument, respectively. The financial liability component was measured at fair value based on the discounted cash flows expected over the estimated term of the notes using a discount rate based on a market interest rate that a similar debt instrument without a conversion feature would be subject to. The liability component is recorded on our Consolidated Statement of Financial Position at amortized cost, and interest is calculated by applying the estimated prevailing market interest rate at the time of issue. The equity component is recognized in equity and is not subsequently remeasured.

All interest-bearing loans and borrowings are recognized initially at fair value less attributable transaction costs which are subsequently carried at amortized cost using the effective interest method. Interest is charged to the Consolidated Statement of Comprehensive Loss.

Exchange rate adjustments recognized in the financial income or expenses reflect adjustments to the value of assets and liabilities denominated in foreign currencies, other than our functional currency, which is U.S. dollars for the year ended December 31, 2019.

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We include the non-cash discount component of contingent consideration which is being unwound to the Consolidated Statement of Comprehensive Loss in Finance expenses. The contingent consideration arose as part of the acquisition of Birken AG (now Amryt GmbH) in 2016 through which we acquired AP101. The fair value of contingent consideration was initially determined by discounting the contingent amounts payable to their present value at the date of acquisition. The discount component is being unwound as a non-cash financing charge in the Consolidated Statement of Comprehensive Loss over the life of the obligation. We review the fair value of the contingent consideration at each reporting date.

We include the non-cash CVR finance expenses which are being unwound to the Consolidated Statement of Comprehensive Loss in Finance expenses. The CVR finance expense arises from the issuance of CVRs to the shareholders and option holders of Amryt as at September 20, 2019, prior to the Acquisition of Aegerion. The value of the potential payout of the CVRs was calculated using the probability-weighted expected returns method. Using this method, the potential payment amounts were multiplied by the probability of achievement and discounted to present value. The discount component is being unwound as a non-cash financing charge in the Consolidated Statement of Comprehensive Loss over the life of the obligation. We review the estimates and where necessary, will adjust the carrying amount of CVR to reflect the actual and revised estimated contractual cash fows. At each reporting date, we recalculate the amortized cost of the CVR as the present value of the re-estimated future contractual cash flows at original effective interest rate.

Tax on ordinary activities

Our corporate tax is comprised of current tax and the adjustment of deferred taxes during the period. In any given period, the adjustment to our deferred tax position, including the reversal of valuation allowances, may partially or wholly offset current tax expense.

Results of Operations of Amryt

Financial results of Amryt for the three months ended March 31, 2020 compared to the three months ended March 31, 2019

The following table sets forth certain unaudited consolidated statement of operations data for Amryt (in thousands, except per share data):

	Three months ended March 31,
Statement of comprehensive loss data:	2020	2019
	(Unaudited)
	(In thousands, except per share data)
Revenue	$44,574	$4,542
Cost of sales	(32,620)	(1,830)
Gross profit	11,954	2,712
Research and development expenses	(8,934)	(1,505)
Selling, general and administrative expenses	(18,406)	(3,896)
Acquisition and severance related costs	(853)	—
Share based payment expenses	(745)	(91)
Operating loss before finance expense	(16,984)	(2,780)
Non-cash change in fair value of contingent consideration	(2,906)	(1,938)
Non-cash contingent value rights finance expense	(1,448)	—
Net finance expense — other	(9,416)	(661)
Loss on ordinary activities before taxation	(30,754)	(5,379)
Tax credit / (charge) on loss on ordinary activities	1,857	(6)
Loss for the period attributable to the equity holders of the Company	(28,897)	(5,385)
Exchange translation differences which may be reclassified through profit or loss	(13)	80
Total other comprehensive (loss)/income	(13)	80
Total comprehensive loss for the period attributable to the equity holders of the Company	$(28,910)	$(5,305)
Loss per share – basic and diluted, attributable to ordinary equity holders of the parent	$(0.19)	$(0.12)

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Revenues

The revenues for each of our significant products were as follows:

	Three months March 31,		Increase / (Decrease)
Revenues:	2020	2019
	Unaudited	Unaudited
	$000		$000	%
Metreleptin	$26,927	$—	$26,927	100%
Lomitapide	17,421	4,419	13,002	294.2%
Other	26	123	103	83.7%
Total revenues	$44,574	$4,542	$40,032	881.4%

Total revenues were $44.6 million for the three months ended March 31, 2020, compared to $4.5 million for the three months ended March 31, 2019. The increase in revenues was due to our acquisition of Aegerion in September 2019. Sales of metreleptin and lomitapide comprise product sales to third parties and royalties on sales made by our licensees.

Metreleptin

We generated revenues from product sales of metreleptin of $26.9 million for the three months to March 31, 2020. 55% of sales for metreleptin were in the United States, with the remaining 45% in the European Union and other international markets.

Future net revenues of metreleptin are highly dependent on our ability to:

• maintain existing patients on therapy;

• continue to build market acceptance for metreleptin in the United States;

• build market acceptance in the European Union following the approval by the EMA in July 2018, and continue to obtain pricing and reimbursement approvals in key markets in the European Union;

• secure regulatory approval from the FDA for the treatment of PL in the United States; and

• obtain regulatory approvals for metreleptin in new markets for the treatment of GL and PL based on the data package which secured approval in Europe.

In addition, we expect to continue to pay significant Medicaid rebates for metreleptin in the United States. An increase in the relative mix of patients that have Medicaid as their primary insurance coverage would have an adverse impact on our ability to increase revenues.

Lomitapide

We generated revenues from product sales of lomitapide of $16.8 million and royalties of $0.6 million from Recordati for the three months ended March 31, 2020. This includes revenues from product sales of LOJUXTA in the EMEA region and product sales and royalties of JUXTAPID in other jurisdictions.

This compares to $4.4 million of LOJUXTA product sales (all in the EMEA region) for the three months ended March 31, 2019.

Future net revenues of lomitapide are highly dependent on our ability to:

• maintain existing patients on therapy;

• continue to build market acceptance for lomitapide in existing markets;

• continue to support patient access programs in all territories;

• obtain pricing and reimbursement approvals in new markets in the European Union; and

• secure regulatory approval for lomitapide in Brazil and other key markets.

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In addition, we expect to continue to pay significant Medicaid rebates for lomitapide in the United States. An increase in the relative mix of patients that have Medicaid as their primary insurance coverage would have an adverse impact on our ability to increase revenues.

Other

Other revenues relate to sales from our in-house derma-cosmetic range of products, Imlan, and our early access program for AP101. Imlan is marketed solely in Germany as a treatment for sensitive, allergy-prone skin. The increase in revenues in the three months ended March 31, 2020 was due to higher sales from our early access program product, AP101. We intend to market AP101 under the brand name of FILSUVEZ if it is approved for the treatment of EB.

Cost of Sales

	Three months ended March 31,		Increase / (Decrease)
Cost of Sales:	2020	2019
	Unaudited
	$000		$000	%
Cost of product sales	$6,247	$1,053	$5,194	493.3%
Amortization of acquired intangibles	11,092	—	11,092	100%
Amortization of inventory fair value step-up	9,503	—	9,503	100%
Royalty expenses	5,778	777	5,001	643.6%
Total cost of sales	$32,620	$1,830	$30,790	1682.5%

Total cost of sales was $32.6 million for the three months ended March 31, 2020, representing the cost, including royalties, of selling metreleptin and lomitapide, non-cash intangible amortization and non-cash inventory fair value step-up expenses. Total cost of sales was $1.8 million for the three months ended March 31, 2019, which represented the cost, including royalties, from sales of LOJUXTA, Imlan and our Early Access Program for AP101.

The cost of product sales in the three months ended March 31, 2020 increased by $5.2 million, and royalty expenses increased by $5.0 million in 2020 compared to the three months ended March 31, 2019. The acquisition of lomitapide for markets outside the EMEA and metreleptin for all markets largely drove this increase in costs. Following the Acquisition, we are now selling two commercial products on a global basis, which results in a higher cost of producing our commercial products, higher royalties on sales, and higher costs of delivery of goods sold to customers, including the costs associated with the services provided by our distributors to import and deliver the goods.

Amortization of acquired intangible assets was $11.1 million for the three months ended March 31, 2020 and relates to the amortization charge on the two commercial assets purchased as part of the Acquisition.

The non-cash inventory step-up was $9.5 million for the three months ended March 31, 2020. This relates to the difference between the estimated fair value and the book value of inventory acquired from Aegerion which is being amortized over the estimated period that we expect to sell this inventory.

Research and Development Expenses

Research and development expenses consist primarily of costs related to clinical studies and outside services, post-approval commitment studies, personnel expenses and other research and development costs. Study costs and outside services costs relate primarily to services performed by clinical research organizations, materials and supplies and other third-party fees. Research and development expenses for the three months ended March 31, 2020 were $8.9 million, representing 32% of our total operating expenses, compared to $1.5 million, or 28% of total operating expenses, for the three months ended March 31, 2019. Research and development expenses in both periods were primarily driven by the clinical advancement of AP101 as we continued our global clinical trial sites. Research expenses for the three months ended March 31, 2020 comprised $3.1 million in employee compensation, $4.0 million of amounts paid to clinical research organizations and $1.8 million of other outsourced services. Research expenses in the three months ended March 31, 2019 comprised $0.6 million in employee compensation, $0.4 million of amounts paid to clinical research organizations and $0.5 million of other outsourced services.

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Selling, General and Administrative Expenses

Selling, general and administrative expenses were $18.4 million for the three months ended March 31, 2020, representing 65% of our total operating expenses, compared to $3.9 million for the three months ended March 31, 2019, representing 72% of our total operating expenses. The increase in selling, general and administrative expenses was primarily due to an increase in compensation-related expenses, primarily driven by higher headcount following the Acquisition, and an increase in other expenses related to the expansion and support of our business.

Restructuring and Acquisition Costs

Restructuring and acquisition costs arising from the Acquisition were $0.9 million for the three months ended March 31, 2020. These costs primarily relate to severance costs associated with the relocation of a number of roles from the Boston, UK and EMEA offices of Aegerion to our head office in Dublin, Ireland following the completion of the Acquisition.

Share-Based Payment Expenses

Non-cash share-based payment expenses for the three months ended March 31, 2020 were $0.7 million, compared to $0.1 million for the three months ended March 31, 2019. The increase is due to options granted to directors, officers and other key employees in connection with the Aegerion acquisition. We issue share options as an incentive to executive directors, senior management and employees. The fair value is measured at the grant date using the Black-Scholes model and amortized over the period during which the awards vest.

Non-Cash Change in Fair Value of Contingent Consideration

For each reporting period, we compute the fair value of the contingent consideration arising from the acquisition of Birken AG (now Amryt GmbH). The Amryt GmbH consideration relates to milestone payments of up to $35 million and royalty payments that are payable to the previous owners of Amryt GmbH, which are triggered by future regulatory approvals of AP101 for the treatment of EB from both the FDA and EMA, as well as future sales-driven milestones.

The $2.9 million and $1.9 million non-cash changes in fair value of contingent consideration for the three months ended March 31, 2020 and March 31, 2019, respectively, represent re-measurement of the contingent consideration fair value for each reporting period.

Non-Cash Contingent Value Rights Finance Expense

The $1.5 million non-cash CVR finance expense for the three months ended March 31, 2020 represents the effective interest rate unwinding on amortized cost between the carrying value of the CVRs from January 1, 2020 to the reporting date of March 31, 2020.

We issued CVRs pursuant to which up to $85 million may become payable to Amryt shareholders and option holders who were shareholders prior to completion of the Acquisition, if certain regulatory approval and revenue milestones are met in relation to AP101.

Net Finance Expense - Other

Other net finance expense was $9.4 million for the three months ended March 31, 2020. Other net finance expense relates to interest on loans of $5.3 million and foreign exchange losses of $4.1 million. The foreign exchange loss primarily relates to the revaluation of Euro, Swiss Franc and Sterling denominated intercompany balances held by subsidiaries.

Net finance expense was $0.7 million for the three months ended March 31, 2019, which primarily related to interest on our credit facility (“EIB Facility”) with the European Investment Bank (“EIB”). This loan facility was repaid in 2019.

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Financial results of Amryt for the Year Ended December 31, 2019 compared to the Year Ended December 31, 2018

The following table sets forth certain consolidated statement of operations data for Amryt (in thousands, except per share data):

	Year ended December 31,
Statement of comprehensive loss data:	2019	2018
	(In thousands, except per share data)
Revenue	$58,124	$17,095
Cost of sales	(42,001)	(6,266)
Gross profit	16,123	10,829
Research and development expenses	(15,827)	(10,703)
Selling, general and administrative expenses	(35,498)	(17,342)
Restructuring and acquisition costs	(13,038)	—
Share based payment expenses	(841)	(821)
Impairment charge	(4,670)	—
Operating loss before finance expense	(53,751)	(18,037)
Non-cash change in fair value of contingent consideration	(6,740)	(10,566)
Non-cash contingent value rights finance expense	(1,511)	—
Net finance expense — other	(4,759)	(1,841)
Loss on ordinary activities before taxation	(66,761)	(30,444)
Tax credit/(charge) on ordinary activities	1,226	(43)
Loss for the year attributable to the equity holders of the Company	(65,535)	(30,487)
Total other comprehensive profit/(loss)	781	(77)
Total comprehensive loss for the year attributable to the equity holders of the Company	$(64,754)	$(30,564)
Loss per share – basic and diluted, attributable to ordinary equity holders of the parent	$(0.86)	$(0.67)

Revenues

The revenues for each of our significant products were as follows:

	Year ended December 31,		Increase / (Decrease)
Revenues:	2019	2018
	$000		$000	%
Metreleptin	$25,088	$—	$25,088	100%
Lomitapide	32,260	16,110	16,150	100.2%
Other	776	985	(209)	(21.2%)
Total revenues	$58,124	$17,095	$41,029	240.0%

Total product sales were $58.1 million for the year ended December 31, 2019, compared to $17.1 million for the year ended December 31, 2018. Sales of metreleptin and lomitapide comprise product sales and royalties on sales, respectively, made by our licensees.

Metreleptin

We generated revenues from product sales of metreleptin of $25.1 million for the period from the date of Acquisition on September 24, 2019 to December 31, 2019. 59.6% of product sales for metreleptin were in the United States, with the remaining 40.4% in the European Union and other international markets.

Future net revenues of metreleptin are highly dependent on our ability to:

• maintain existing patients on therapy;

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• continue to build market acceptance for metreleptin in the United States;

• build market acceptance in the European Union following the recent approval by the EMA in July 2018, and continue to obtain pricing and reimbursement approvals in key markets in the European Union;

• secure regulatory approval from the FDA for the treatment of PL in the United States; and

• obtain regulatory approvals for metreleptin in new markets for the treatment of GL and PL based on the data package which secured approval in Europe.

In addition, we expect to continue to pay significant Medicaid rebates for metreleptin in the United States. An increase in the relative mix of patients that have Medicaid as their primary insurance coverage would have an adverse impact on our ability to increase revenues.

Lomitapide

We generated revenues from product sales of lomitapide of $31.6 million and royalties of $0.7 million from Recordati for the year ended December 31, 2019. This includes revenues from product sales of LOJUXTA in the EMEA region for the full year together with revenues from product sales and royalties of JUXTAPID in other jurisdictions from the date of Acquisition on September 24, 2019.

This compares to $16.1 million of LOJUXTA product sales (all in the EMEA region) for the year ended December 31, 2018.

Future net revenues of lomitapide are highly dependent on our ability to:

• maintain existing patients on therapy;

• continue to build market acceptance for lomitapide in existing markets;

• continue to support patient access programs in all territories;

• obtain pricing and reimbursement approvals in new markets in the European Union; and

• secure regulatory approval for lomitapide in Brazil and other key markets.

In addition, we expect to continue to pay significant Medicaid rebates for lomitapide in the United States. An increase in the relative mix of patients that have Medicaid as their primary insurance coverage would have an adverse impact on our ability to increase revenues.

Other

Other revenues relate to sales from our in-house derma-cosmetic range of products, Imlan, and our early access program for AP101. Imlan is marketed solely in Germany as a treatment for sensitive, allergy-prone skin. The decrease in revenues in 2019 was due to a decrease in customers following a reduction in product offerings in 2019.

Cost of Sales

	Year ended December 31,		Increase / (Decrease)
Cost of Sales:	2019	2018
	$000		$000	%
Cost of product sales	$11,384	$3,588	$7,796	217.3%
Amortization of acquired intangibles	11,831	—	11,831	100.0%
Amortization of inventory fair value step-up	10,367	—	10,367	100.0%
Royalty expenses	8,419	2,678	5,741	214.3%
Total cost of sales	$42,001	$6,266	$35,735	570.3%

Total cost of sales was $42.0 million for the year ended December 31, 2019, representing the cost, including royalties, of selling metreleptin and lomitapide, non-cash intangible amortization and non-cash inventory fair value step-up expenses. Total cost of sales was $6.3 million for the year ended December 31, 2018, which represented the cost, including royalties, from sales of LOJUXTA, Imlan and our Early Access Program for AP101.

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The cost of product sales in the year ended December 31, 2019 increased by $7.8 million, and royalty expenses increased by $5.7 million in 2019 compared to the year ended December 31, 2018. The acquisition of lomitapide for markets outside the EMEA and metreleptin for all markets largely drove this increase in costs. Following the Acquisition, we are now selling two commercial products on a global basis, which results in a higher cost of producing our commercial products, higher royalties on sales, and higher costs of delivery of goods sold to customers, including the costs associated with the services provided by our distributors to import and deliver the goods.

Amortization of acquired intangible assets was $11.8 million in 2019 and relates to the amortization charge, for the post Acquisition period, on the two commercial assets purchased as part of the Acquisition.

The non-cash inventory step-up was $10.4 million in 2019. This relates to the difference between the estimated fair value and the book value of inventory acquired from Aegerion which is being amortized over the estimated period that we expect to sell this inventory.

Research and Development Expenses

Research and development expenses consist primarily of costs related to clinical studies and outside services, post-approval commitment studies, personnel expenses and other research and development costs. Study costs and outside services costs relate primarily to services performed by clinical research organizations, materials and supplies, and other third-party fees. Research and development expenses for the year ended December 31, 2019 were $15.8 million, representing 23% of our total operating expenses, compared to $10.7 million, or 37% of total operating expenses, for the year ended December 31, 2018. Research and development expenses in both years were primarily driven by the clinical advancement of AP101 as we continued our global clinical trial sites. Research expenses in 2019 comprised $4.8 million in employee compensation, $7.7 million of amounts paid to clinical research organizations, and $3.3 million of other outsourced services. Research expenses in 2018 comprised $2.6 million in employee compensation, $4.4 million of amounts paid to clinical research organizations, and $3.7 million of other outsourced services.

Selling, General and Administrative Expenses

Selling, general and administrative expenses were $35.5 million for the year ended December 31, 2019, representing 51% of our total operating expenses, compared to $17.3 million for the year ended December 31, 2018, representing 60% of our total operating expenses. The increase in selling, general and administrative expenses was primarily due to an increase in compensation-related expenses, primarily driven by higher headcount following the Acquisition, and an increase in other expenses related to the expansion and support of our business.

Restructuring and Acquisition Costs

Restructuring and acquisition costs arising from the Acquisition were $13.0 million for the year ended December 31, 2019. These costs primarily relate to professional fees associated with the Acquisition. The expenses also include severance costs associated with the relocation of a number of roles from the Boston office of Aegerion to our head office in Dublin, Ireland following the completion of the Acquisition.

Share-Based Payment Expenses

Non-cash share-based payment expenses for the year ended December 31, 2019 were $0.8 million, unchanged from the same amount in the year ended December 31, 2018. We issue share options as an incentive to senior management and employees. The fair value is measured at the grant date using the Black-Scholes model and amortized over the period during which the awards vest.

Impairment charge

In 2019, an impairment charge of $4.7 million was recorded to write off the remaining carrying value of an in process intangible asset, AP102, an early stage drug asset which represents a novel, next generation somatostatin analogue (“SSA”) peptide medicine for patients with rare neuroendocrine diseases, where there is a high unmet medical need, including acromegaly. Acromegaly is a rare endocrine disorder in which the body produces excessive growth hormone, leading to abnormal growth throughout the body over time. Following the

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Acquisition, we made the decision to concentrate resources on those development pipeline activities that will better complement our existing commercial assets, lomitapide and metreleptin. We may look to partner AP102 in the long-term future but in the short to medium term, we will continue to concentrate our efforts on AP101, AP103 and expansion opportunities for the existing commercial assets.

Non-Cash Change in Fair Value of Contingent Consideration

We compute the fair value of the contingent consideration arising from the acquisition of Birken AG (now Amryt GmbH).

The Amryt GmbH consideration relates to milestone payments of up to $35 million and royalty payments that are payable to the previous owners of Amryt GmbH, which are triggered by future regulatory approvals of AP101 for the treatment of EB from both the FDA and EMA, as well as future sales-driven milestones.

Non-Cash Contingent Value Rights Finance Expense

The $1.5 million non-cash CVR finance expense for the year ended December 31, 2019 represents the effective interest rate unwind on amortized cost between the carrying value of the CVRs from the initial recognition date to the reporting date of December 31, 2019.

We issued CVRs pursuant to which up to $85 million may become payable to Amryt shareholders and option holders who were shareholders prior to completion of the Acquisition, if certain regulatory approval and revenue milestones are met in relation to AP101.

Net Finance Expense - Other

Other net finance expense was $4.8 million for the year ended December 31, 2019. Other net finance expense relates to interest on loans of $8.5 million, partially offset by foreign exchange gains of $3.8 million. The foreign exchange gain primarily relates to the translation of euro- and sterling-denominated net monetary amounts held by subsidiaries with a non U.S. dollar functional currency.

Other net finance expense was $1.8 million for the year ended December 31, 2018, which primarily related to interest on our EIB Facility with the EIB. This loan facility was repaid in 2019.

Results of Operations of Aegerion

The following discussion of Aegerion’s historical results of operations is based on Aegerion’s audited and unaudited consolidated financial statements, included elsewhere in this prospectus. Aegerion’s financial statements were prepared in accordance with U.S. GAAP, which may differ in material respects from generally accepted accounting principles in other jurisdictions, including IFRS, which is the basis under which Amryt’s historical results of operations have been prepared.

In addition, as of the issuance of Aegerion’s financial statements there was substantial doubt regarding Aegerion’s ability to continue as a going concern. See Note 1, Description of Business, in the Notes to Aegerion’s Consolidated Financial Statements, included elsewhere in this prospectus, for more information regarding the conditions that raised substantial doubt about its ability to continue as a going concern.

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Financial results of Aegerion for the Six Months Ended June 30, 2019 compared to the Six Months Ended June 30, 2018

The following table summarizes the results of Aegerion’s operations for the six-month periods ended June 30, 2019 and 2018, together with changes in those items in thousands of dollars:

	Six Months Ended June 30,		$ Increase / (Decrease)
	2019	2018
	(in thousands)
Net revenues	$95,857	$59,388	$36,469
Cost of product sales	35,364	29,208	6,156
Operating expenses:
Selling, general and administrative	43,424	38,568	4,856
Research and development	13,946	21,113	(7,167)
Related party expense, net	397	617	(220)
Total operating expenses	57,767	60,298	(2,531)
Income (loss) from operations	2,726	(30,118)	(32,844)
Reorganization items, net	(2,145)	—	2,145
Interest expense, net	(29,681)	(22,628)	7,053
Interest expense due to Novelion	(1,182)	(1,406)	(224)
Other expense, net	(224)	(1,054)	(830)
Loss before provision for income taxes	(30,506)	(55,206)	(24,700)
Provision for income taxes	(369)	(1,205)	(836)
Net loss	$(30,875)	$(56,411)	$(25,536)

Net Revenues

The revenues for each of Aegerion’s commercial products were as follows:

	Six Months Ended June 30,		Increase / (Decrease)
Revenues:	2019	2018
	(in thousands)
Metreleptin	$38,915	$29,751	$9,164
Lomitapide	56,942	29,637	27,305
Total net revenues	$95,857	$59,388	$36,469

Total net revenues were $95.9 million for the six months ended June 30, 2019, compared to $59.4 million for the six months ended June 30, 2018. During the second quarter of 2019, Aegerion recognized $30.0 million of licensing revenues, net of 60% of net sales benefit to Recordati for the period of the transition per the agreement, or approximately $1.5 million, under the license agreement with Recordati.

Metreleptin

Metreleptin sales were $38.9 million for the six months ended June 30, 2019, compared to $29.8 million for the six months ended June 30, 2018. The increase of $9.1 million for the six months ended June 30, 2019 compared to the six months ended June 30, 2018 was primarily attributable to higher sales in the United States, arising from increased sales volumes and a price increase effective from January 1, 2019, as well as increased sales in EMEA following the EMA approval in July 2018, which resulted in an increase in the average number of patients in existing markets and our first commercial sales in the United Kingdom, Spain and Portugal.

Lomitapide

Lomitapide revenues were $29.6 million for the six months ended June 30, 2018, compared to $56.9 million for the six months ended June 30, 2019. The increase of $27.3 million for the six months ended June 30, 2019 compared to the six months ended June 30, 2018 was primarily attributable to the license agreement signed with Recordati in February 2019 for the commercialization of JUXTAPID in Japan. During the second quarter of

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2019, Aegerion recognized $30.0 million of licensing revenues, net of 60% of net sales benefit to Recordati for the period of the transition per the agreement, or approximately $1.5 million, under the license agreement with Recordati. The marketing authorization was transferred to Recordati on May 10, 2019 and subsequent to this date, Aegerion began recognizing royalty revenues on product sales from Recordati rather than product revenues.

While 2019 net revenues from Japan were significantly higher than 2018 due to the recognition of revenue of $30.0 million of upfront and milestone payments, net of 60% of net sales benefit to Recordati for the period of the transition per the agreement, subsequent net revenues of lomitapide from Japan would decrease as a result of the license agreement with Recordati as Aegerion (and now Amryt) would recognize royalty revenues on product sales rather than product revenues.

Cost of Product Sales

Aegerion’s cost of product sales, which primarily consisted of the cost of producing products, royalties on sales, non-cash amortization of intangible assets and non-cash inventory fair value step-up expenses, were $29.2 million during the six months ended June 30, 2018, compared to $35.4 million for the six months ended June 30, 2019. The increase of $6.2 million was primarily attributed to the increase in royalty expenses incurred in the period for both metreleptin and lomitapide. Increased revenues generated from metreleptin in the United States in the six months ended June 30, 2019 resulted in higher royalty expenses incurred by Aegerion during such period. For lomitapide, pursuant to Aegerion’s license agreement with the University of Pennsylvania (“UPenn”), UPenn received 15% of the licensing payments from Recordati, resulting a $4.5 million royalty payable to UPenn during the second quarter of 2019.

Selling, General and Administrative Expenses

During the six months ended June 30, 2019, selling, general and administrative expenses were $43.4 million, compared to $38.6 million during the six months ended June 30, 2018. The $4.8 million increase was driven by non-recurring legal and consulting fees incurred in connection with Aegerion’s strategic alternatives review and bankruptcy proceedings, as well as the $1.5 million in fees incurred to extend the maturity date of Aegerion’s debt arrangements. The increase in selling, general and administrative expenses as a result of these non-recurring expenses was partially offset by lower compensation-related expenses as the result of cost saving efforts implemented in late 2018.

Research and Development Expenses

During the six months ended June 30, 2019, research and development expenses were $13.9 million, compared to $21.1 million during the six months ended June 30, 2018. The $7.2 million decrease was primarily driven by the lower compensation-related expenses, lower clinical research organizations and outsourced services expenses as a result of cost saving efforts and the delay of certain projects and initiatives.

Related Party Expense, Net

The related party expense, net, was incurred with or earned from Aegerion’s former parent company, Novelion, and such income and expense was related to the shared service agreement with Novelion. As part of the agreement, Novelion provided executive, business development and administrative service to Aegerion. Aegerion provided back-office type services such as finance and human resources to Novelion. Each party was compensated for its services rendered with a reimbursement of costs plus a margin on a quarterly basis intended to approximate an arm’s length transaction. The related party expense was $0.4 million during the six months ended June 30, 2019, compared to $0.6 million during the six months ended June 30, 2018. The decrease of $0.2 million was primarily related to reduced efforts required by Novelion management to work on Aegerion’s corporate matters during the six months ended June 30, 2019.

Reorganization Items, Net

Reorganization items, net represent the professional fees incurred after May 20, 2019, the date that Aegerion filed the bankruptcy proceedings with the U.S. Bankruptcy Court.

Interest Expense, net

Net interest expense was $29.7 million for the six months ended June 30, 2019, compared to $22.6 million for the six months ended June 30, 2018. The increase of $7.1 million was primarily attributable to the accrued interest and the amortization of debt discount related to the Bridge Loan Credit Agreement that Aegerion entered

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into in November 2018, the amortization of debt discount on the Convertible Notes and, to a lesser degree, the interest due on amounts owed to the DOJ and SEC during the period. Interest expense included non-cash components of $24.2 million and $19.1 million in the six months ended June 30, 2019 and June 30, 2018, respectively.

Interest Expense due to Novelion

Interest expense due to Novelion was $1.2 million during the six months ended June 30, 2019, compared to Novelion amounted to $1.4 million during the six months ended June 30, 2018. The interest expense due to Novelion decreased by $0.2 million during the six months ended June 30, 2019 compared to the six months ended June 30, 2018. The interest charge in 2019 covers the period from January 1, 2019 to May 20, 2019, which is the date that Aegerion and its U.S. subsidiary, Aegerion Pharmaceuticals Holdings, Inc. filed voluntary petitions under Chapter 11 in the United States Bankruptcy Court for the Southern District of New York.

Other Expense, net

Other expense, net, was $0.2 million for the six months ended June 30, 2019, compared to $1.1 million for the six months ended June 30, 2018. The decrease of $0.9 million was primarily due to a lower level of foreign exchange losses arising from foreign currency transactions.

Provision for Income Taxes

During the six months ended June 30, 2019, Aegerion had a provision for income taxes of $0.4 million, compared to $1.2 million during the six months ended June 30, 2018. The decrease of $0.8 million was primarily driven by the establishment of a valuation on Aegerion’s foreign deferred tax assets during the second quarter of 2018.

Financial results of Aegerion for the Year Ended December 31, 2018 compared to the Year Ended December 31, 2017

The following table summarizes the results of Aegerion’s operations for the years ended December 31, 2018 and 2017, together with changes in those items in thousands of dollars:

	Year ended December 31,		$ Increase / (Decrease)
	2018	2017
	(in thousands)
Net revenues	$130,432	$138,438	$(8,006)
Cost of sale	59,697	77,220	(17,523)
Operating expenses:
Selling, general and administrative expenses	64,437	77,793	(13,356)
Research and development expenses	38,064	44,895	(6,831)
Restructuring charges	2,171	121	2,050
Related party expenses (income), net	942	(177)	1,119
Total operating expenses	105,614	122,632	(17,018)
Loss from operations	(34,879)	(61,414)	(26,535)
Interest expense, net	(50,746)	(39,467)	11,279
Interest expense due to Novelion	(2,987)	(1,089)	1,898
Loss on extinguishment of debt	(4,333)	—	4,333
Other expense, net	(1,888)	(836)	1,052
Loss before provision for income taxes	(94,833)	(102,806)	(7,973)
Provision for income taxes	(1,705)	(594)	1,111
Net loss	$(96,538)	$(103,400)	$(6,862)

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Net Revenues

	Year ended December 31,		$ Increase / (Decrease)
	2018	2017
	(in thousands)
Metreleptin	$71,360	$66,308	$5,052
Lomitapide	59,072	72,130	(13,058)
Total net revenues	$130,432	$138,438	$(8,006)

Net revenues for 2018 were primarily generated from net product sales of metreleptin and lomitapide. Approximately 2% of net revenues were derived from royalties on sales of metreleptin and lomitapide made by Aegerion’s licensees in the European Union and other territories.

Metreleptin

Aegerion generated revenues from net sales of metreleptin of approximately $71.4 million for the year ended December 31, 2018, compared to $66.3 million for the year ended December 31, 2017. The increase of $5.1 million was primarily attributable to a $9.6 million increase in revenues from France, Germany and Turkey, partially offset by a $3.1 million decrease in the United States and a $1.6 million decrease in Brazil. Increased revenues in Turkey were primarily due to an increasing number of patients on treatment compared to the year ended December 31, 2017. Revenues in France and Germany increased during the year ended December 31, 2018 due to a higher number of orders in 2018, specifically, in Germany, attributable to the launch of MYALEPTA in November 2018. The lower 2018 revenues in the United States were primarily due to the decline in shipments as a result of the number of patients who discontinued metreleptin therapy. In addition, the decrease in U.S. revenues was partially attributable to a one-time revenue recognition of $2.3 million in the second quarter of 2017, resulting from changing the method of revenue recognition on sales of MYALEPT within the United States from the sell-through to the sell-in method. See Note 2 - Revenue Recognition, in the Notes to Aegerion’s Consolidated Financial Statements for more information. In addition, the decrease in Brazil revenues was attributable to additional local medical and regulatory requirements which have increased complexity in the process for named patient sales in Brazil and resulted in lower orders during the year ended December 31, 2018.

Lomitapide

Aegerion generated revenues from net sales of lomitapide of approximately $59.1 million for the year ended December 31, 2018, compared to $72.1 million for the year ended December 31, 2017. The decrease of $13.0 million was primarily attributable to an $11.0 million decrease in the United States and a $6.3 million decrease in Brazil, partially offset by a $5.0 million increase in Japan. The decrease in the United States was primarily attributable to the availability of PCSK9 inhibitor products, restrictions on reimbursement for our product, patients not adhering to lomitapide therapy for a variety of reasons that are unique to those taking JUXTAPID, and other reasons common to chronic therapies that treat very rare diseases such as HoFH. There was one order from Brazil totaling $0.4 million during the year ended December 31, 2018, compared to sales of $6.7 million during the year ended December 31, 2017. The decrease in revenues from Brazil was for similar reasons described above for metreleptin, as well as the ongoing investigations in Brazil. The increased revenues in Japan during the year ended December 31, 2018 were primarily due to an increasing number of patients on treatment compared to the year ended December 31, 2017, as the product was launched in Japan in late 2016.

Cost of Product Sales

During the year ended December 31, 2018, cost of product sales, which primarily consist of cost of producing products, royalties on sales, non-cash amortization of intangible assets and non-cash inventory fair value step-up expenses, decreased by $17.5 million to $59.7 million, compared to $77.2 million for the year ended December 31, 2017. During the year ended December 31, 2018, Aegerion recorded $2.1 million of charges for excess or obsolete inventory compared to $18.8 million during the year ended December 31, 2017, primarily driven by the change in forecasted sales activities. The charges for excess or obsolete inventory were determined based on an analysis of forecasted sales activities, product shelf-life and the estimated fair value of the inventory. Royalties payable on U.S. sales of metreleptin increased in 2018 compared to 2017 in accordance with the licensing agreement for this product, adding approximately $2.4 million in cost of product sales in 2018.

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Selling, General and Administrative Expenses

During the year ended December 31, 2018, Aegerion’s selling, general and administrative expenses were $64.4 million, compared to $77.8 million during the year ended December 31, 2017. The decrease of $13.4 million was primarily driven by lower employee-related expenses, outsourced services spending and lower spending in information technology infrastructure as a result of the cost saving efforts in 2018.

Research and Development Expenses

During the year ended December 31, 2018, Aegerion’s research and development expenses were $38.1 million, compared to $44.9 million for 2017. The decrease of $6.8 million was primarily driven by lower employee-related expenses, consulting fees, and spending in clinical research as a result of the cost saving efforts in 2018.

Restructuring Charges

As a result of Aegerion’s August 2018 cost reduction plan, which included a workforce reduction, Aegerion incurred restructuring charges of $2.2 million during the year ended December 31, 2018, which include consulting fees related to the restructuring event and termination benefits principally comprised of severance payments. The charges were fully paid by the end of the third quarter in 2019.

Related Party Expenses (Income), Net

The related party expense or income was incurred or earned from Aegerion’s former parent company, Novelion, and such income and expense were related to the shared service agreement with Novelion. The related party expense was $0.9 million during the year ended December 31, 2018, compared to related party income of $0.2 million during the year ended December 31, 2017. The increase of $1.1 million in related party expense is primarily related to additional efforts required from Novelion management to work on Aegerion’s corporate matters including the debt restructuring and strategic alternatives review.

Interest Expense, net

Interest expense, net, was $50.7 million during the year ended December 31, 2018, compared to $39.5 million for the year ended December 31, 2017. The increase of $11.2 million was primarily attributable to the amortization of debt discount on Aegerion’s finance arrangements, as well as interest due on amounts owed to the DOJ and SEC during the year ended December 31, 2018. Interest expense included non-cash components totaling $39.7 million and $33.0 million during the year ended December 31, 2018 and 2017, respectively.

Interest Expense due to Novelion

Interest expense due to Novelion was $3.0 million during the year ended December 31, 2018, compared to $1.1 million for the year ended December 31, 2017. The increase of $1.9 million is attributed to the accrued unpaid interest on the higher principal amount, which resulted from the additional $15.0 million of principal that Aegerion borrowed from Novelion in February 2018.

Loss on Extinguishment of Debt

Loss on extinguishment of debt was $4.3 million during the year ended December 31, 2018, which resulted from the extinguishment of certain finance arrangements, upon repayment in November 2018.

Other Expense, Net

Other expense, net was $1.9 million during the year ended December 31, 2018, compared to $0.8 million for the year ended December 31, 2017. The increase of $1.1 million was primarily attributed to a higher loss from foreign currency transactions.

Provision for Income Taxes

Aegerion’s provision for income taxes was $1.7 million for the year ended December 31, 2018, an increase of $1.1 million compared to the year ended December 31, 2017. The provision for income taxes consisted primarily of current tax expense related to profitable operations in foreign tax jurisdictions.

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Liquidity and Capital Resources

We had unrestricted cash and cash equivalents of $67.0 million, $65.2 million and $9.9 million as at March 31, 2020, December 31, 2019, and December 31, 2018, respectively. We have financed our operations primarily through sales of our commercial products, sales of our ordinary shares and debt financing. We expect to incur significant expenses for the foreseeable future as we continue commercializing our approved products and advancing the clinical development of our product candidates. We expect that our R&D and SG&A costs will increase in connection with conducting clinical trials for our product candidates and any new product candidates we acquire or develop and due to the costs of seeking marketing approval for our product candidates in Europe, the United States and other jurisdictions.

Cash Flows

The table below provides selected cash flow information for the periods indicated (in thousands):

	Three months ended March 31,		Year ended December 31,
	2020	2019	2019	2018
	Unaudited	Unaudited
	(in thousands)
Net cash flow from / (used in) operating activities	$6,187	$(5,441)	$(37,497)	$(15,454)
Net cash used in investing activities	(13)	(4)	24,425	(229)
Net cash (used in) / flow from financing activities	(1,506)	5,675	65,942	3,265
Exchange and other movements	(3,830)	(74)	3,133	(767)
Net change in cash and cash equivalents	$838	$156	$56,003	$(13,185)

Net Cash Flow From / (Used in) Operating Activities

Net cash from operating activities was $6.2 million for the three months ended March 31, 2020, compared to net cash used in operating activities of $5.4 million for the three months ended March 31, 2019. The increase of $11.6 million was primarily driven by the increased scale of our business and working capital fluctuations.

Net cash used in operating activities was $37.5 million for the year ended December 31, 2019, compared to $15.5 million for the year ended December 31, 2018. The increase of $22.0 million was primarily related to restructuring and acquisition costs of $13.0 million and working capital fluctuations.

Net Cash Flow From / (Used in) Investing Activities

Net cash from investing activities was $24.4 million for the year ended December 31, 2019 and primarily related to the Aegerion cash balance of $25.0 million, which we acquired in the Acquisition. A significant proportion of this cash balance was restricted and held in escrow to meet costs associated with the Aegerion bankruptcy process.

Net cash used in investing activities was $0.2 million for the year ended December 31, 2018 and primarily related to the fees paid for the extension of our license agreement with Aegerion to cover the addition of certain territories, and payments for property, plant and equipment.

Net Cash (Used in) / Flow From Financing Activities

Net cash flow used in financing activities was $1.5 million for the three months ended March 31, 2020 and related to interest payments on our term loan facility. Net cash flow from financing activities was $5.7 million for the three months ended March 31, 2019, primarily due to the drawdown of the second tranche of our EIB Facility of $5.7 million.

Net cash flow from financing activities was $65.9 million for the year ended December 31, 2019 and primarily related to net proceeds from the issuance of shares of $63.0 million and the issuance of new debt of $31.2 million. These cash inflows were partially offset by the repayment of our EIB Facility of $22.0 million and interest paid to EIB and on our Secured Credit Facility of $6.3 million.

Net cash flow from financing activities was $3.3 million for the year ended December 31, 2018, primarily due to the drawdown of the final tranche of our EIB Facility of $5.9 million, partially offset by a milestone payment of $2.4 million relating to our acquisition of Amryt GmbH in 2016.

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Debt Financing

In December 2016, we entered into the EIB Facility, a €20 million credit facility split into three tranches: €10 million available immediately, and two further tranches of €5 million available upon the achievement of certain milestones. In April 2017, we drew down the first tranche of €10 million. In September 2018, we drew down the second tranche of €5 million. In December 2018 the terms of the third tranche were amended to give us the option to draw down this final tranche on the condition that the EASE Phase 3 trial interim efficacy results were positive. In February 2019, after we reported the outcome of an unblinded interim efficacy analysis of the EASE trial, we drew down the final tranche of €5 million. The EIB Facility was secured by our intellectual property assets. It also contained a negative covenant restricting our ability to grant security interests over any of our assets over the course of the loan period.

The EIB Facility was repaid in full on September 24, 2019 in connection with the closing of the Acquisition. In connection with the Acquisition we entered into the $81 million Secured Credit Facility and issued $125 million of Convertible Notes. For more information on this indebtedness, see “Related Party Transactions.”

Contractual Obligations

The following summarizes our contractual obligations as of December 31, 2019 (in thousands):

	Payments due by period
(in thousands)	Less than 1 year	1 to 3 years	3 to 5 years	More than 5 years	Total
Principal debt obligations	$11,957	$24,796	$136,927	$128,125	$301,805
Operating leases obligations	969	916	143	20	2,048
Contingent consideration and contingent value rights	—	99,559	27,998	—	127,557
Other liabilities	15,722	3,928	—	—	19,650
Total	$28,648	$129,199	$165,068	$128,145	$451,060

The principal debt obligations relate to our $81 million Secured Credit Facility and our Convertible Notes with an aggregate principal amount of $125 million and the interest associated with these facilities. The Secured Credit Facility has a five-year term from date of draw down and matures in 2024. Interest will be payable at our option at the rate of 11% per annum paid in cash on a quarterly basis or at a rate of 6.5% paid in cash plus 6.5% paid in kind that will be paid when the principal is repaid, which rolls up and is included in the principal balance outstanding, on a quarterly basis. For the purposes of the contractual obligations table above, we assume that we choose to pay interest at a rate of 6.5% paid in cash plus 6.5% paid in kind that will be paid when the principal is repaid. The Convertible Notes bear interest at a rate of 5.0% per year, payable semi-annually in arrears on April 1 and October 1 of each year, beginning on April 1, 2020. The Convertible Notes will mature on April 1, 2025, unless earlier repurchased or converted. For the purposes of the contractual obligations table above, we assume that there is no conversion and that the Convertible Notes are repaid in full on April 1, 2025. For further detail on our principal debt, see Note 19 and Note 20 of our Consolidated Financial Statements.

We have operating leases commitments for offices in the United States, European Union and Latin America, a production facility in Germany and office equipment leases.

Contingent consideration and contingent value rights arose as part of (i) the acquisition of Amryt GmbH in 2016, through which we acquired AP101, and (ii) the issuance of CVRs to Amryt shareholders and option holders prior to the Acquisition of Aegerion. The contingent consideration and contingent value rights arising on these transactions are payable on achieving various milestones. For further detail, see Note 6 of our Consolidated Financial Statements.

Other liabilities relate to our obligations, inclusive of interest, under Aegerion’s settlement agreements with the SEC and DOJ. For further detail, see Note 25 of our Consolidated Financial Statements.

Off-Balance Sheet Arrangements

At December 31, 2019, we did not have any off-balance sheet arrangements that have or are reasonably likely to have a current or future effect on our financial condition, changes in financial condition, revenues or expenses, results of operations, liquidity, capital expenditures or capital resources that is material to investors.

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Quantitative and Qualitative Disclosures about Market Risk

Our operations expose us to some financial risks arising from our use of financial instruments, the most significant ones being liquidity, market risk and credit risk. The Board is responsible for our risk management policies and while retaining responsibility for them it has delegated the authority for designing and operating processes that ensure the effective implementation of the objectives and policies to our finance function. See Note 24 of our Consolidated Financial Statements for the year ended December 31, 2019.

Critical Accounting Judgments and Estimates

Our financial statements have been prepared in accordance with IFRS as issued by the IASB. In the application of accounting policies, certain judgments, estimates and assumptions about the value of assets and liabilities for which there is no definitive third-party reference were required. The estimates and associated assumptions are based on historical experience and other factors that were considered to be relevant. Actual results may differ from these estimates. These estimates and assumptions are reviewed on an ongoing basis. Revisions to accounting estimates are recognized in the period in which the estimate is revised if the revision affects only that period or in the period of the revisions and future periods if the revision affects both current and future periods. For a discussion of the estimates and assumptions used by us in the preparation of our financial statements, see Note 2 of our Consolidated Financial Statements for the year ended December 31, 2019.

Implications of Being an Emerging Growth Company and a Foreign Private Issuer

We qualify as an “emerging growth company,” as defined in the JOBS Act. An emerging growth company may take advantage of specified reduced reporting and other burdens that are otherwise applicable generally to public companies. These provisions include:

• a requirement to include only two years of audited financial statements and only two years of related Management’s Discussion and Analysis of Financial Condition and Results of Operations disclosure in the registration statement of which this prospectus forms a part; and

• an exemption from the auditor attestation requirement in the assessment of our internal control over financial reporting pursuant to the Sarbanes-Oxley Act.

We may choose to take advantage of some but not all of these reduced burdens, and therefore the information that we provide holders of shares and ADSs may be different from the information you might receive from other public companies in which you hold equity. In addition, Section 107 of the JOBS Act provides that an emerging growth company can take advantage of an extended transition period for complying with new or revised accounting standards applicable to public companies. We currently prepare our consolidated financial statements in accordance with IFRS as issued by the IASB and as a result we are unable to make use of the extended transition period. However, in the event that we convert to accounting principles generally accepted in the United States (which we do not currently intend to do) while we remain an emerging growth company, we have irrevocably elected to opt out of such extended transition period.

We may take advantage of these provisions for up to five years or such earlier time that we are no longer an emerging growth company. We will cease to be an emerging growth company upon the earliest of the following:

• the last day of the first fiscal year in which our annual revenues were at least $1.07 billion;

• the last day of the fiscal year following the fifth anniversary of the effectiveness of the registration statement of which this prospectus forms a part;

• the date on which we have issued more than $1 billion of non-convertible debt securities over a three-year period; and

• the last day of the fiscal year during which we meet the following conditions: (i) the worldwide market value of our common equity securities held by non-affiliates as of our most recently completed second fiscal quarter is at least $700 million, (ii) we have been subject to U.S. public company reporting requirements for at least 12 months and (iii) we have filed at least one annual report as a U.S. public company.

Upon the effectiveness of the registration statement of which this prospectus forms a part, we will report under the Exchange Act as a non-U.S. company with foreign private issuer status. Even after we no longer qualify as

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an emerging growth company, as long as we continue to qualify as a foreign private issuer under the Exchange Act, we will be exempt from certain provisions of the Exchange Act that are applicable to U.S. domestic public companies, including:

• the sections of the Exchange Act regulating the solicitation of proxies, consents or authorizations in respect of a security registered under the Exchange Act;

• the sections of the Exchange Act requiring insiders to file public reports of their stock ownership and trading activities and liability for insiders who profit from trades made in a short period of time; and

• the rules under the Exchange Act requiring the filing with the SEC of quarterly reports on Form 10-Q containing unaudited financial and other specified information, or current reports on Form 8-K, upon the occurrence of specified significant events.

In addition, we will not be required to file annual reports and financial statements with the SEC as promptly as U.S. domestic companies whose securities are registered under the Exchange Act, and are not required to comply with Regulation FD, which restricts the selective disclosure of material information.

Both foreign private issuers and emerging growth companies are also exempt from certain more stringent executive compensation disclosure rules for U.S. public companies under the Dodd-Frank Wall Street Reform and Consumer Protection Act of 2010. Even if we no longer qualify as an emerging growth company, so long as we remain a foreign private issuer, we will continue to be exempt from such compensation disclosures.

Internal Control Over Financial Reporting

As a public company listed on the Nasdaq, we will be required under the Sarbanes-Oxley Act, among other things, to assess the effectiveness of our internal control over financial reporting at the end of each fiscal year. We anticipate being required to issue management’s assessment of internal control over financial reporting pursuant to Section 404(a) of the Sarbanes-Oxley Act for the first time in connection with issuing our consolidated financial statements as of and for the year ending December 31, 2021.

We may discover future deficiencies in our internal controls over financial reporting, including those identified through testing conducted by us pursuant to Section 404(a) of the Sarbanes-Oxley Act or subsequent testing by our independent registered public accounting firm. Such deficiencies may be deemed to be significant deficiencies or material weaknesses and may require changes to our consolidated financial statements or identify other areas for further attention or improvement.

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BUSINESS

Overview

We are a global, commercial-stage biopharmaceutical company dedicated to commercializing and developing novel therapeutics to treat patients suffering from serious and life-threatening rare diseases. Our diversified portfolio is comprised of two commercial rare disease products, as well as a development-stage pipeline focused on rare skin diseases. Our two commercial products, lomitapide for the treatment of homozygous familial hypercholesterolemia (“HoFH”), and metreleptin for the treatment of generalized lipodystrophy (“GL”) and partial lipodystrophy (“PL”), have each been sold globally through our commercial infrastructure for over six years. We are developing our lead product candidate, AP101, for the treatment of cutaneous manifestations of severe Epidermolysis Bullosa (“EB”), a rare and devastating genetic skin disease for which there is currently no approved therapy. AP101 is currently in a pivotal Phase 3 trial for which we expect to report data in the second half of 2020. If AP101 is approved for this indication, we intend to market it under the name FILSUVEZ. Our next product candidate, AP103, is currently in preclinical development for the treatment of patients with Recessive Dystrophic EB (“RDEB”), a subset of EB. AP103 is the first gene therapy product candidate based on our novel polymer-based topical gene therapy delivery platform, which also has potential use for the treatment of other rare genetic diseases. We intend to initiate clinical development of AP103 in the second half of 2021. We have a proven track record of obtaining rare disease assets, either through acquisition or in-license, and we intend to continue building our portfolio of rare disease programs with the goal of bringing effective treatments to patients in need.

The following table summarizes key information about our product portfolio and clinical development pipeline. We have retained worldwide development and commercial rights to all of our programs, excluding Japan for lomitapide and Japan, South Korea and Taiwan for metreleptin.

 

*
Upcoming clinical milestones are subject to the impact of COVID-19 on our business.

(1)
We are conducting a Phase 3 study of HoFH in children and adolescents in Europe, Middle East and Africa (“EMEA”) as part of our European Medicines Agency (“EMA”) post-approval commitments.

(2)
The familial chylomicronemia syndrome Phase 2 trial is an open-label investigator-led study.

(3)
The dotted line segment indicates we have not yet commenced any clinical trials in the United States for metreleptin for the treatment of PL.

(4)
AP101 was approved in 2016 by the EMA for the treatment of partial thickness wounds in adults, but has not been commercially launched.

(5)
The dotted line segment indicates we have not yet commenced any clinical trials for radiation-induced dermatitis. This planned radiation-induced dermatitis Phase 2 trial is an investigator-led study.

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Lomitapide is an oral therapy approved as an adjunct to a low-fat diet and other lipid-lowering treatments for adults with HoFH. It is marketed in the United States under the trade name JUXTAPID and in the European Union under the trade name LOJUXTA. HoFH is a rare and serious genetic condition that leads to aggressive and premature heart disease, heart attacks and strokes in patients as young as teenagers. HoFH patients are at a high risk of experiencing life-threatening cardiovascular events as a result of extremely elevated cholesterol levels in the blood and have a substantially reduced life expectancy. HoFH impairs the liver’s ability to remove low density lipoprotein (“LDL”) cholesterol, or “bad” cholesterol, from the blood, which if left untreated can cause aggressive narrowing and blocking of the blood vessels. According to a 2013 European Heart Journal article, the prevalence of HoFH is one person per million. However, according to a 2016 article published in Atherosclerosis, the number may be as high as 6.25 persons per million. Lomitapide is a small molecule microsomal triglyceride transfer protein (“MTP”) inhibitor. MTP exists in both the liver and intestines where it plays a role in the formation of cholesterol-carrying lipoproteins. As a result, inhibition of MTP is an effective cholesterol-lowering therapy in HoFH patients with limited or non-functional LDL receptors.

Metreleptin for injection is approved in the United States under the trade name MYALEPT as an adjunct to diet as replacement therapy to treat the complications of leptin deficiency in patients with congenital or acquired GL. It is approved in the European Union under the trade name MYALEPTA for the treatment of leptin deficiency in patients with congenital or acquired GL and familial or acquired PL for whom standard treatments have failed to achieve adequate metabolic control. GL and PL are rare diseases characterized by loss or lack of adipose tissues (fat cells), resulting in the deficiency of the hormone leptin. GL and PL patients experience severe metabolic abnormalities including severe insulin resistance, diabetes, hypertriglyceridemia and fatty liver disease. We estimate that the prevalence of GL is approximately one person per million and that of PL is approximately three persons per million. Metreleptin is a recombinant human leptin analog that binds to and activates the human leptin receptor. Metreleptin acts to stimulate fatty acid oxidation throughout the body and lower plasma, hepatic and myocellular triglyceride levels.

We intend to continue to evaluate additional expansion opportunities for our products. We are conducting a Phase 3 pediatric study in the EMEA for the use of lomitapide in children and adolescents with HoFH, for which we expect to report data in the first half of 2022. We are also exploring the potential use of lomitapide to treat patients with familial chylomicronemia syndrome (“FCS”), which is a severe, rare genetic lipid disease characterized by extremely elevated levels of triglycerides, or hypertriglyceridemia. An investigator-led open-label Phase 2 trial studying lomitapide in patients with FCS is ongoing and we expect to report data in the second half of 2020. Upon successful completion of this Phase 2 study, we intend to discuss these results with the Food and Drug Administration (“FDA”) and the EMA in the context of agreeing on the design of a potential pivotal trial in FCS. We also intend to discuss with the FDA in the third quarter of 2020 the potential for label expansion of metreleptin in the United States to include the treatment of PL. We expect this will require a pivotal Phase 3 study in PL patients, either as a post-approval commitment or prior to potential approval.

Our lead development candidate, AP101, is being developed as a potential treatment for the cutaneous manifestations of severe EB, a rare and devastating genetic skin disease affecting young children and adults for which there is currently no approved treatment. EB is a group of diseases of the skin, mucous membranes and internal epithelial linings characterized by extreme skin fragility that blisters and tears from minor friction or trauma. Patients with severe forms of EB, including Dystrophic EB (“DEB”) and Junctional EB (“JEB”), suffer from severe and chronic blistering, ulceration, scarring, mutilating scarring of the hands and feet, joint contractures, strictures of the esophagus and mucous membranes, a high risk of developing aggressive squamous cell carcinomas, infections and risk of premature death. According to a 2013 article in the Journal of Investigative Dermatology, it is estimated that the incidence of EB is approximately one in 20,000, which implies that there are as many as 30,000 affected individuals in the United States and over 500,000 worldwide. We are conducting a pivotal Phase 3 clinical trial of AP101 in severe EB, including DEB and JEB. In January 2019, we reported that the independent Data and Safety Monitoring Board (“DSMB”) performed a pre-specified unblinded interim efficacy analysis and recommended the continuation of the study with an increase in enrollment from 182 to 230 evaluable patients to maintain 80% conditional statistical power. As a result of the COVID-19 pandemic which has had a material impact on recruitment into clinical trials globally, and given that the EASE trial was already close to full enrollment, we have taken advice from an independent expert and concluded that the statistical impact of further patient recruitment would most likely be negligible. We therefore decided to close the EASE trial to further enrollment in April 2020 and expect to report top line results from the EASE trial in the second half of 2020.

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A representative photograph of the devastating impact of EB on a patient is shown below:

 

AP101 has been granted Pediatric Rare Disease Designation by the FDA. If approved by the FDA, we are eligible to apply for a Priority Review Voucher (“PRV”) that we can use, sell or transfer. We are also supporting an investigator-led Phase 2 study of AP101 for the treatment of severe radiation-induced dermatitis. This trial is expected to commence in the second half of 2020, with data expected in the first half of 2021.

We also have a novel polymer-based topical gene therapy delivery platform for potential use in the treatment of rare genetic diseases. Our first product candidate utilizing this platform, AP103, is currently in preclinical development for the treatment of RDEB, a subset of severe EB. We intend to initiate clinical development for AP103 in the second half of 2021.

We sell lomitapide and metreleptin in the Americas, Europe and the Middle East through our existing rare disease commercial infrastructure. Our commercial expertise includes market access, marketing, sales managers and sales representatives and is supported by our experienced medical affairs team with medical science liaisons, patient advocates and dieticians in the field. We also have a network of third-party distributors in other key markets throughout the world.

Our company was co-founded in 2015 by our Chief Executive Officer, Joseph Wiley, and our Chief Financial Officer, Rory Nealon. Our shares have been traded on AIM and EGE since April 2016. We are led by a management team and board of directors (“Board”) with deep experience at leading biotech companies, large pharmaceutical companies and academic institutions. Our company headquarters are in Dublin, Ireland, and we have nearly 170 employees throughout the world. We believe the expertise of our leadership team and the strength of our relationships within the industry and medical community are critical to our strategy as we commercialize our existing products, progress our development pipeline and explore new business development opportunities.

Our Strengths

We believe our key competitive strengths include the following:

• Revenue-generating commercial products. We currently generate revenue, including royalties, from global sales of lomitapide and metreleptin. This revenue stream provides us with financial flexibility to fund the continued development and potential commercialization of our existing development candidates as well as the potential acquisition or in-license of additional rare disease products and late-stage product candidates. We have retained worldwide development and commercial rights to all of our programs, excluding Japan for lomitapide, where we receive royalties, and Japan, South Korea and Taiwan for metreleptin.

• Late-stage clinical program in severe EB. We are conducting a global pivotal Phase 3 trial of AP101 for the treatment of cutaneous manifestations of severe EB and we expect to report data in the second

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half of 2020. This Phase 3 trial is the largest EB study conducted to date. Based on our conversations with the FDA and the EMA, we believe that positive results from this trial would allow us to apply for marketing approval for AP101 in both the United States and Europe.

• Existing, scalable global commercial and medical infrastructure. We sell lomitapide and metreleptin in the Americas, Europe and the Middle East through our existing rare disease commercial infrastructure. Our commercial expertise includes market access, marketing, sales managers and sales representatives and is supported by our experienced medical affairs team with medical science liaisons, patient advocates and dieticians in the field. We also leverage our network of third-party distributors in other key markets throughout the world. We believe we will be able to leverage our existing global infrastructure and expertise to efficiently and expeditiously commercialize additional products we may acquire or develop, including our lead product candidate, AP101, if approved.

• Proven track record of building a diversified rare disease product portfolio. We acquired AP101 through the acquisition of Birken AG in 2016, in-licensed LOJUXTA in December 2016, in-licensed our gene therapy platform, including AP103, in March 2018 and acquired metreleptin and the remaining rights to lomitapide through the Acquisition in September 2019.

• Strong patent protection and regulatory exclusivity. We believe our intellectual property portfolio as well as protection afforded by regulatory exclusivity provide us with a substantial competitive advantage in marketing our current products and also protect our development programs. Our lomitapide patent portfolio includes patents that provide protection from 2025 to 2027 in the United States and into 2025 in the European Union, with supplementary protection granted to extend patent protection in major EU countries into 2028. The metreleptin patent portfolio includes patents that provide protection from 2022 to 2027 in the United States and into 2022 in the European Union and orphan exclusivity in the European Union into 2028. The AP101 patent portfolio includes patents that provide protection in both the United States and the European Union into 2025 and 2030 and a further international patent application directed to the clinical formulation and methods of manufacturing and treatment with AP101 which, if granted, would provide worldwide protection into 2039. We have also submitted additional patent applications to further strengthen our intellectual property portfolio.

• Experienced management team comprised of industry leaders in rare diseases. Our management team has extensive expertise in the acquisition, development and commercialization of rare disease assets. We believe that the breadth of experience and successful track record of our management team and our Board, combined with our broad network of established relationships with leaders in the industry and medical community, provide us with strong drug development and commercialization capabilities.

Our Strategy

Our mission is to become a global leader in the treatment of rare diseases through commercializing, developing and acquiring novel rare disease products. To achieve this mission, we are pursuing the following strategies:

• Drive revenue growth for our existing commercial products. We intend to continue to focus on growing the sales of lomitapide and metreleptin in the markets and indications we currently sell them. We also intend to expand the market opportunity by seeking approval for the use of lomitapide to treat pediatric HoFH and for the treatment of FCS and for the use of metreleptin to treat PL in the United States.

• Complete development and commercialize our lead product candidate, AP101, for the treatment of severe EB. AP101 is currently in a pivotal Phase 3 trial for the treatment of cutaneous manifestations of severe EB and we expect to report data in the second half of 2020. If the trial is successful, we intend to apply for approval of AP101 and commercialize it in the United States and the European Union. If approved by the FDA, we are eligible to apply for a PRV that we can use, sell or transfer.

• Leverage our global commercial and medical infrastructure. We intend to leverage our existing global infrastructure and expertise to commercialize our development-stage pipeline, including our lead product candidate, AP101, if approved, and any rare disease assets we may acquire or in-license in the future.

• Continue developing our gene therapy product candidate, AP103, for the treatment of RDEB. AP103 is currently in preclinical development for the treatment of RDEB. We intend to initiate clinical development in the second half of 2021.

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• Continue evaluating opportunities to expand our rare disease product portfolio and pipeline. We believe we are well positioned to continue to opportunistically acquire or in-license rare disease assets that we believe we can efficiently sell through our existing commercial infrastructure. Our Commercial Products

Our Commercial Products

Lomitapide for the Treatment of HoFH

Overview

HoFH is a rare genetic disease, which impairs the body’s ability to remove LDL cholesterol, or “bad” cholesterol, typically leading to abnormally high LDL cholesterol levels in the blood. HoFH patients are at a high risk of experiencing life-threatening cardiovascular events at an early age as a result of extremely elevated cholesterol levels in the blood and have a substantially reduced life expectancy relative to unaffected individuals. According to a 2013 European Health Journal article, the prevalence of HoFH is one person per million. However, according to a 2016 article published in Atherosclerosis, the number may be as high as 6.25 persons per million. Aggressive treatment, including dietary modifications plus combination therapy with currently approved lipid lowering drugs at maximum tolerated doses, often fails to reduce LDL cholesterol levels to their recommended targets in these patients. Lomitapide is a small molecule MTP inhibitor with the potential to provide significant reductions in LDL cholesterol levels in this high-risk patient population. Lomitapide, which is marketed as JUXTAPID in the United States and as LOJUXTA in EMEA, is an oral, once-a-day treatment for adult patients with HoFH, as an adjunct to a low-fat diet and other lipid-lowering medicinal products, with or without LDL apheresis.

In October 2007, the FDA granted lomitapide Orphan Drug Designation for the treatment of HoFH. The FDA approved JUXTAPID in December 2012 and the EMEA granted marketing authorization for LOJUXTA in July 2013. In addition to the United States and the European Union, lomitapide has received regulatory approval in Japan, Canada and certain Latin American countries. Lomitapide has also been made available upon physician request on a named patient sales basis in certain other jurisdictions. In 2019, we out-licensed to Recordati the rights to sell lomitapide in Japan.

HoFH – Background and Current Treatments

HoFH is a serious, rare genetic disease usually caused by defects in both alleles of the low-density lipoprotein receptor (“LDL-R”) gene, resulting in the impairment of the function of the LDL-R. The LDL-R is a protein on the surface of cells that is responsible for binding and removing LDL from the blood. Malfunction of the LDL-R results in significant elevation of blood cholesterol levels. Cholesterol naturally occurs in the body, synthesized in the liver, and also enters the bloodstream through absorption from food. Cholesterol is transported in the blood for use as a source of energy and cell structure. Excess levels of cholesterol in the blood, also known as hypercholesterolemia, can cause significant complications. HoFH is most commonly caused by genetic mutations in both alleles of the primary gene responsible for LDL-R production, the LDL-R gene, but can also be caused by mutations in other genes. To date, more than 1,600 mutations have been identified that can impair the function of the LDL-R gene, with some mutations leading to a significant reduction or a total lack of LDL-R activity. As a result of elevated levels of LDL, HoFH patients often develop premature and progressive atherosclerosis, a narrowing or blocking of the arteries, usually in combination with arterial thrombosis, and are at high risk of experiencing premature cardiovascular events, such as heart attack or stroke, often experiencing their first cardiovascular event in early adulthood, and have a significantly shortened life expectancy.

Physicians in the United States and in many other countries often use clinical findings and family history to make a clinical diagnosis of HoFH. Clinical diagnosis is typically made using the following criteria: significantly elevated LDL cholesterol levels; physical signs, which may include the presence of cutaneous xanthomas, Achilles tendon thickening, xanthelasma and/or corneal arcus; limited response to statins that is not attributed to statin intolerance or to another identifiable cause (usually dependent on functional LDL receptors); evidence of premature cardiovascular disease (often in the second and third decade of life); and a positive history of high cholesterol and/or premature cardiovascular disease, consistent with having familial hypercholesterolemia on both sides of the family.

The clinical approach taken with HoFH patients typically involves an aggressive treatment plan to reduce lipid levels as much as possible through dietary modifications and a combination of lipid-lowering drug therapies.

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Current drug therapies for reducing LDL levels in HoFH patients include statins, cholesterol absorption inhibitors, PCSK9 inhibitors and lomitapide. Patients are also managed through LDL apheresis (lipid dialysis). Since the introduction of PCSK9 inhibitors in the United States in 2015, healthcare professionals have started most new adult HoFH patients on a PCSK9 inhibitor product before trying lomitapide and have switched some existing lomitapide patients to a PCSK9 inhibitor product because such products may have fewer side effects, are significantly less expensive and do not require that patients follow a special low-fat diet. However, because many therapies, including statins and PCSK9 inhibitor products, act by increasing the activity of LDL-R, HoFH patients often have an inadequate response due to their impaired LDL-R function. For example, high dose statin therapies that typically produce 35% to 47% reductions in LDL levels in the broad hypercholesterolemic patient population, on average, produce only a 10% to 25% reduction in HoFH patients. Also, a study published in Lancet in 2014 showed that although PCSK9 inhibitor evolocumab produced a mean LDL reduction of 23% in HoFH patients at 12 weeks, a subset of HoFH patients with very low or no LDL-R function did not respond to treatment. HoFH patients who are unable to reach their recommended target LDL levels on drug therapy are sometimes treated using LDL apheresis. However, apheresis provides only temporary reductions in LDL levels, so it must be repeated frequently to avoid rapid rebound that typically occurs within approximately four days. In addition, except in certain countries in the European Union, apheresis is often not readily available, due to the limited number of treatment centers that perform this procedure.

Our Solution: Lomitapide for the Treatment of HoFH

We believe lomitapide has the potential to address and mitigate the root cause of HoHF. Lomitapide is a small molecule MTP inhibitor. MTP exists in both the liver and intestines where it plays a role in the formation of cholesterol-carrying lipoproteins. As a result, the inhibition of MTP is an effective cholesterol-lowering therapy in HoFH patients with limited or non-functional LDL receptors. We believe lomitapide has the potential to become the standard of care (“SOC”) for the reduction of LDL levels in HoFH patients who have not responded to other therapies.

Treatment and Adverse Reaction Rates

The recommended starting dosage of lomitapide is 5 mg once daily (up to a maximum of 60 mg, depending on patient response to treatment and goal of therapy), and the dose should be escalated gradually based on acceptable safety and tolerability. Lomitapide should be taken once daily with a glass of water, without food, at least two hours after the evening meal. Lomitapide is contraindicated in the following conditions: pregnancy, concomitant administration of lomitapide with moderate or strong CYP3A4 inhibitors, patients with moderate or severe hepatic impairment and patients with active liver disease.

One single-arm, open-label, 78-week trial has been conducted treating 29 patients with lomitapide for HoFH, 23 of whom completed at least one year of treatment. The initial dosage of lomitapide in such trial was 5 mg daily, with titration up to 60 mg daily during an 18-week period based on safety and tolerability. In this trial, the mean age was 30.7 years (range, 18 to 55 years), 16 (55%) patients were men, 25 (86%) patients were Caucasian, two (7%) were Asian, one (3%) was African American, and one (3%) was multi-racial. The most common adverse reactions were gastrointestinal, reported by 27 (93%) of 29 patients. Adverse reactions reported by ≥ 8 (28%) patients in the HoFH clinical trial included diarrhea, nausea, vomiting, dyspepsia and abdominal pain. Other common adverse reactions, reported by five to seven (17-24%) patients, included weight loss, abdominal discomfort, abdominal distension, constipation, flatulence, increased alanine aminotransferase (“ALT”), chest pain, influenza, nasopharyngitis and fatigue. Adverse reactions of severe intensity were reported by eight (28%) of 29 patients, with the most common being diarrhea, vomiting, increased ALT or hepatotoxicity and abdominal pain, distension, and/or discomfort.

Expansion Opportunities in Pediatric HoFH and FCS and Post-Approval Obligations

We are conducting a Phase 3 pediatric study in the European Union for the use of lomitapide in children and adolescents with HoFH. We expect to report data from this trial in the first half of 2022. We are also exploring the potential use of lomitapide to treat patients with FCS, a severe, rare genetic lipid disease characterized by extremely elevated levels of triglycerides, or hypertriglyceridemia. These patients are at an increased risk of developing acute pancreatitis, a significant and sometimes life-threatening inflammation of the pancreas. Published prevalence estimates suggest FCS affects approximately one to two persons per million. In 2010 the EMA, and in 2011 the FDA, granted orphan designation for lomitapide for the treatment of FCS. An

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investigator-led open-label Phase 2 trial in FCS is ongoing and we expect to report data in the second half of 2020. Upon successful completion of this Phase 2 study, we intend to discuss these results with the FDA and the EMA in the context of agreeing on the design of a potential pivotal trial in FCS.

Clinical Development and Post-Approval Obligations

In 2014, we initiated an observational cohort study in the United States and European Union to generate additional data on the long-term safety profile of lomitapide, the patterns of use and compliance and the long-term effectiveness of lomitapide in controlling LDL levels. The FDA has required target enrollment of 300 HoFH patients worldwide, and has mandated study of enrolled patients for a period of ten years. The EMA has required that all patients taking lomitapide in the European Union be encouraged to participate in the study, and that the study period be open-ended. In the study, investigators will follow each patient to track malignancies, tumors, teratogenicity, hepatic effects and gastrointestinal adverse reactions, events associated with coagulopathy, major adverse cardiovascular events and death. The EMA also required that a vascular imaging study be conducted to determine the impact of lomitapide on vascular endpoints. In addition, certain drug-drug interaction studies have been completed with results submitted to the EMA.

Metreleptin for the Treatment of GL and PL

Overview

Metreleptin is a recombinant analog of human leptin. It is marketed as MYALEPT in the United States as an adjunct to diet as a replacement therapy to treat the complications of leptin deficiency in patients with congenital or acquired GL. It is marketed as MYALEPTA in the European Union as an adjunct to diet as a replacement therapy to treat the complications of leptin deficiency in adults and children two years of age and above with congenital or acquired GL. MYALEPTA is also approved in the European Union for adults and children 12 years of age and above with familial or acquired PL for whom standard treatments have failed to achieve adequate metabolic control with congenital or acquired GL and also congenital or acquired PL. Leptin, which is deficient in patients with GL, is the key hormone responsible for regulating appetite and also has an important regulatory effect on energy expenditure. Leptin is a naturally occurring hormone derived from fat cells and an important regulator of energy, fat and glucose metabolism, reproductive capacity and other physiological functions. The predominant cause of metabolic complications in GL is excess triglyceride accumulation in the liver and skeletal muscle due to the inability to store triglycerides in fat cells. As a result of the deficiency of leptin associated with GL, patients experience significant fatigue as well as hyperphagia, or unregulated appetite. The loss of fat tissue caused by this disease often leads to severe metabolic abnormalities that contribute to increased morbidity and mortality.

Lipodystrophy – GL, PL and Current Treatments

Lipodystrophy is a heterogeneous group of rare syndromes characterized by selective but variable loss of fat tissue. Both the total amount and the appropriate distribution of fat deposits contribute to patients’ metabolic state. Because of the loss of fat tissue and fat cells, secreted hormone leptin is very low. Leptin circulates in blood and acts on the brain to regulate food intake and energy expenditure. When fat mass falls, plasma leptin levels fall, stimulating appetite and suppressing energy expenditure until fat mass is restored. When fat mass increases, leptin levels increase, suppressing appetite until weight is lost. This system maintains homeostatic control of fat cell mass.

Due to the lack of fat cells in individuals with lipodystrophy, energy can no longer be stored as fat in these cells and fat accumulates in the muscles and organs such as the heart, liver and pancreas, causing lipotoxicity and organ damage. In addition, deposition of fat in these unusual locations leads to extreme insulin resistance and its associated complications such as diabetes mellitus, hypertriglyceridemia, hepatic steatosis, polycystic ovary syndrome and high blood pressure. These severe metabolic abnormalities are typically resistant to conventional therapies. As a result of the deficiency of leptin associated with lipodystrophy, patients experience significant fatigue as well as unregulated appetite. The voracious appetite itself significantly aggravates the metabolic abnormalities and further reduces the ability to successfully treat these metabolic abnormalities with conventional therapies.

GL is characterized by a near complete lack of fat cells and, consequently, leads to early and significant morbidity and mortality. Classification of GL (versus PL) is made based on the anatomical distribution of fat loss, which is widespread in GL patients, as well as the younger age and greater rapidity of onset and severity of the metabolic abnormalities. The severe metabolic abnormalities associated with GL may result in premature diabetic nephropathy,

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retinopathy, cardiomyopathy, recurrent attacks of acute pancreatitis, enlarged liver and organ failure. These complications themselves increase morbidity and mortality due to their negative long-term impacts. The key diagnostic indicators of GL are most commonly a combination of physical appearance and severe metabolic abnormalities.

PL is characterized by a less uniform loss of fat cells and with a later age of onset. There can be considerable heterogeneity in the extent of fat cell loss, levels of leptin and degree of metabolic abnormalities. In PL patients with relative or near complete leptin deficiency, the metabolic abnormalities and longer impact on disease progression can closely mirror that of patients with GL.

Clinical diagnosis of lipodystrophy is largely based on history and physical exam but may be informed by genetic markers and blood tests, including adiponectin and leptin levels. Patients may be diagnosed well into adulthood even after seeing multiple doctors. Treatment is usually initiated by specialists, including adult and pediatric endocrinologists and lipidologists. A multi-disciplinary approach is required for ongoing care, which can also include hepatologists, cardiologists, nephrologists and rheumatologists.

Just under one person per million suffers from GL and approximately three persons per million for PL. We believe that the prevalence rate of GL in countries outside the United States is likely to be consistent with the prevalence rate in the United States. However, we anticipate that physicians will use metreleptin to treat the approximately one in three PL patients who suffer from severe PL.

Our Solution: Metreleptin for the Treatment of GL and PL

Metreleptin is a recombinant human leptin analog that binds to and activates the human leptin receptor. Clinical studies show that metreleptin stimulates fatty acid oxidation throughout the body and lowers plasma, hepatic and myocellular lipid levels (especially triglycerides), resulting in increased insulin sensitivity; improves insulin suppression of glucose production by the liver and increases insulin-stimulated peripheral glucose uptake into muscle; and corrects hyperphagia secondary to leptin deficiency with concomitant reduction in caloric and fat intake. Clinical studies have demonstrated the potential for metreleptin to effectively and consistently target leptin deficiencies and related health complications and improve patient outcomes.

Treatment and Adverse Reaction Rates

Metreleptin should be injected subcutaneously once daily at the same time every day, and may be administered any time of day without regard to the timing of meals. Metreleptin should be administered to patients as prescribed by their physician. Recommended dosage is as follows: Persons with body weight 40 kg or less: starting dose 0.06 mg/kg/day, increase or decrease by 0.02 mg/kg to a maximum daily dose of 0.13 mg/kg; Men greater than 40 kg body weight: starting dose 2.5 mg/day, increase or decrease by 1.25 mg to 2.5 mg/day to a maximum dose of 10 mg/day; and women greater than 40 kg body weight: starting dose 5 mg/day, increase or decrease by 1.25 mg to 2.5 mg/day to a maximum dose of 10 mg/day. Metreleptin is contraindicated in patients with general obesity not associated with congenital leptin deficiency. The safety of metreleptin was evaluated in 48 patients with GL in a single-arm, open-label study. The median duration of exposure in this trial was 2.7 years with a range of 3.6 months to 10.9 years. Five to six patients (10-13%) experienced the most common adverse reactions: headache, hypoglycemia, decreased weight and abdominal pain.

Expansion Opportunities and Post-Approval Obligations

In the third quarter of 2020, we intend to discuss with the FDA the potential for label expansion of metreleptin in the United States to include the treatment of PL. We expect this will require a pivotal Phase 3 study in PL patients, either as a post-approval commitment or prior to potential approval.

Clinical Development and Post-Marketing Commitments

A long-term, prospective, observational study (product exposure registry) in patients to evaluate serious risks related to the use of the product is under way. The registry will attempt to enroll at least 100 new patients treated with metreleptin. Enrollment will close after five years or after 100 new patients have been enrolled, whichever occurs first. The registry will continue for ten years from the date of last patient’s enrollment.

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We set forth below the post-approval obligations required by the FDA for metreleptin:

• the development, validation and implementation of a ligand binding assay to supplement the neutralizing bioassay that tests for the presence of neutralizing antibodies in serum samples from patients with GL;

• testing all banked clinical samples from the GL clinical program for the presence of neutralizing antibodies against leptin using the ligand binding assay and to correlate neutralizing antibodies with clinical events; and

• a prospective study to assess the immunogenicity of metreleptin in patients receiving metreleptin.

The presence of neutralizing antibodies will be assessed using both a validated cell-based assay and a validated ligand-binding assay in samples that are confirmed positive for binding antibodies to leptin. In addition, we are required to conduct certain studies related to the manufacture of metreleptin, including in order to validate new test methods, implement a risk-based reference standard program approach, and reassess product acceptance criteria with a larger data set from more manufactured batches. Three post-approval obligations related to manufacturing metreleptin are completed or are on track for completion by their respective deadlines. Finally, we have ongoing obligations to assess spontaneous reports of serious risks related to the use of metreleptin, including the risk to exposed pregnancies and pregnancy outcomes, regardless of indication, for ten years from the date of approval of metreleptin in the United States.

In connection with specific obligations to the EMA to complete post-marketing measures, it has been our intention to conduct the following studies:

• a non-interventional, prospective, observational study (product exposure registry) of GL and PL patients initiating treatment with metreleptin. This study will be open to all patients treated with metreleptin and will continue to the life-span of the product;

• a post-approval efficacy study in PL patients; and

• an integrated analysis of immunogenicity that includes testing, using validated assays, on samples from historical studies, as well as the registry, the pediatric investigation plan (“PIP”) and the post-approval efficacy study in PL patients.

We have also committed to the EMA, as part of our PIP, to conduct a study in GL patients under six years old to further evaluate the pharmacokinetics, activity and safety of metreleptin in this pediatric sub-population as well as an in vitro study to assess the binding of metreleptin to proteins in serum, and a non-clinical tissue distribution study.

Imlan: Therapeutic Topical Cream

We acquired Imlan, a derma-cosmetic product, as part of our acquisition of Birken AG in 2016. Imlan is a topical skin cream marketed solely in Germany for the daily care of highly sensitive skin.

Our Product Candidates

AP101 for the Treatment of Severe EB

Overview

AP101 is an oil-based gel that we are developing as a potential treatment for severe EB, a rare and devastating genetic skin disease that causes the skin layers and internal body linings to separate, affecting infants, children and adults, for which there is currently no approved treatment. Patients with EB suffer from a genetic disease resulting in the inability to produce a certain type of collagen, which plays an important role in anchoring the dermal and epidermal layers of the skin. Patients with severe forms of EB, including DEB and JEB, suffer from severe and chronic blistering, ulceration, scarring, mutilating scarring of the hands and feet, joint contractures, strictures of the esophagus and mucous membranes, a high risk of developing aggressive squamous cell carcinomas, infections and risk of premature death.

We commenced our Phase III Efficacy And Safety Study of Oleogel-S10 in EB (“EASE”), a pivotal study of AP101 in patients with severe EB, in March 2017 and enrolled the first patient in April 2017. This study is the largest Phase 3 study conducted in patients with EB. In January 2019, we reported that the DSMB performed a

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pre-specified unblinded interim efficacy analysis and recommended the continuation of the study with an increase in enrollment from 182 to 230 evaluable patients to maintain 80% conditional statistical power. As a result of the COVID-19 pandemic which has had a material impact on recruitment into clinical trials globally, and given that the EASE trial was already close to full enrollment, we have taken advice from an independent expert and concluded that the statistical impact of further patient recruitment would most likely be negligible. We therefore decided to close the EASE trial to further enrollment in April 2020 and expect to report top line results from the EASE trial in the second half of 2020.

AP101 has been granted Orphan Drug Designation for the treatment of EB by both the FDA and the EMA. AP101 was granted Fast Track Designation by the FDA in September 2019. AP101 was also granted Rare Pediatric Disease Designation by the FDA. Therefore, if AP101 is approved by the FDA, we expect to be eligible to apply for a Rare Pediatric Disease PRV that can be used, sold or transferred.

About EB

EB is a heterogeneous group of diseases of the skin, mucous membranes and internal epithelial linings characterized by extreme skin fragility that blisters and tears from minor friction or trauma. The severity and symptoms of EB can range from blistering of the hands, feet, knees and elbows in mild cases to widespread blistering that can lead to vision loss, disfigurement and serious medical problems in severe cases. There are currently no approved treatments for EB.

EB results from mutations in the genes responsible for the production of structural proteins essential for healthy skin function. Such genetic mutations cause impaired or absent function of the proteins that normally give the skin its mechanical strength. According to a 2013 article in the Journal of Investigative Dermatology, it is estimated that the incidence of EB is approximately one in 20,000, which implies that there are as many as 30,000 affected individuals in the United States and over 500,000 worldwide. EB has been categorized as encompassing five major types (EB Simplex (“EBS”), DEB, JEB, Kindler Syndrome and Aquisita) and 31 subtypes. EBS is the most common and least severe form of EB. Approximately 70% of people with EB have EBS. Patients with EBS often heal quickly with minimal or no scarring. We are developing AP101 to treat the cutaneous manifestations of the approximately 30% of EB patients that have either JEB, DEB and Kindler Syndrome. Patients with these more severe forms of EB may suffer from severe and chronic blistering, ulceration, scarring, mutilating scarring of the hands and feet, joint contractures, strictures of the esophagus and mucous membranes, a high risk of developing aggressive squamous cell carcinomas, infections and risk of premature death. Other manifestations of these more severe types of EB may include anemia, cardiomyopathy, syndactyly (fusion of the fingers and toes), renal insufficiency, dysphagia (difficulty swallowing), malnourishment, cancer, constipation, osteoporosis, muscular dystrophy and pyloric atresia.

Background on Dystrophic Epidermolysis Bullosa

DEB is the second most common variation of EB, occurring in approximately 20% of people with EB. DEB derives its name from the tendency of healing blisters to scar, which leads to contraction of the joints, fusion of the fingers and toes and contraction of the mouth membranes and narrowing of the esophagus. The intensity of DEB varies significantly, but the severity of this disease increases with age due to complications driven by scarring, the fusion of digits and wastage of skin tissue.

DEB is sub-classified into RDEB and Dominant Dystrophic EB (“DDEB”). RDEB is a more severe form of DEB. Infants affected by RDEB are typically born with widespread blistering and areas of missing skin, often caused by trauma during birth. Most frequently, blistering occurs over the entire body and affects mucous membranes such as the moist lining of the mouth and digestive tract. Healing blisters result in severe scarring. Scarring in the mouth and esophagus can make it difficult to chew and swallow food, leading to chronic malnutrition and slow growth. Additional complications of progressive scarring can include fusion of the fingers and toes, loss of fingernails and toenails, joint deformities (contractures) that restrict movement and eye inflammation leading to vision loss. Persons with RDEB have reduced life expectancy and are at increased risk of developing aggressive types of skin cancer before the age of 35. DDEB is a less common and less severe form of DEB.

Background on Junctional Epidermolysis Bullosa

Approximately 10% of people living with EB have JEB, which is sub-classified into Herlitz JEB and non-Herlitz JEB. Herlitz JEB is a more severe form of JEB. From birth or early infancy, affected individuals have blistering over large regions of the body. Blistering also affects the mucous membranes, such as the moist lining of the mouth and digestive

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tract, which often makes consumption and digestion difficult. From birth or early infancy, affected individuals have blistering over large regions of their body. Children with Herlitz JEB often die in infancy. Non-Herlitz JEB is a less severe form of JEB. The blistering associated with non-Herlitz JEB may be limited to the hands, feet, knees and elbows, and it often improves after the conclusion of the newborn period. Persons living with non-Herlitz JEB intermediate typically experience a normal lifespan.

Background on Kindler Syndrome

Kindler Syndrome is an extremely rare subtype of EB inherited in an autosomal recessive manner characterized by skin blistering and photosensitivity.

Our Solution: AP101 for the Treatment of Severe EB

AP101 is a topical product incorporating betulin and other related triterpene compounds. AP101 has been observed to stimulate keratinocyte migration and promote differentiation into mature-epithelial skin cells, thereby ensuring more rapid wound healing. We are conducting a pivotal Phase 3 clinical trial of AP101 in severe EB, including DEB and JEB, and are expecting data in the second half of 2020. We believe AP101 could become the first approved treatment for patients suffering from severe EB.

Clinical Development of AP101

We have set forth below the clinical trials conducted to date for AP101 for the treatment of severe EB.

Phase 2a Study

Our Phase 2a study of AP101 was a prospective, case-controlled study to compare the efficacy and tolerance of AP101 to SOC in EB wounds. Half of each wound was treated with AP101, together with a non-adhesive wound dressing, and the other half was treated with wound dressing alone. The primary endpoint of the study was the rate of progress of re-epithelialization from baseline to either day 14 or day 28 (depending on whether the wound was acute or chronic). A blinded assessment of efficacy was conducted by two independent experts based on a chronological series of photographs taken before start of treatment, during wound dressing changes and at the end of treatment on day 14 and day 28. Safety variables of the study were incidence, severity and causality of adverse events and assessment of tolerability by the investigators and patients and/or his/her legal representative.

Ten patients were enrolled and 12 wounds were treated with study medication. Reviewers were asked to assess which half of the wound was healing faster. Results of this trial are set forth in the table below.

Results of Blinded Efficacy Evaluation by Patient and Wound

Patient (wound #)	Blinded Efficacy Assessment Experts		Result
	Reviewer 1	Reviewer 2
1	Non-adhesive wound dressing	AP101	Undecided
2	AP101	Equal	AP101
3	AP101	AP101	AP101
4	AP101	Equal	AP101
5	Equal	Equal	Undecided
6	AP101	AP101	AP101
7	Equal	Equal	Undecided
8	AP101	Non-adhesive wound dressing	Undecided
9 (1)	AP101	AP101	AP101
9 (2)	Equal	AP101	AP101
10 (1)	AP101	AP101	AP101
10 (2)	AP101	AP101	AP101

We observed that AP101 together with the non-adhesive wound dressing significantly accelerated healing (8 of 8 decided cases; p = 0.0078, binomial test) compared to non-adhesive wound dressing only (0 of 8 decided cases). Of the four undecided cases, two consisted of opposite conclusions by the two reviewers, and the other two wounds were considered to have equal healing in both halves by the two reviewers.

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A total of 11 adverse events (“AEs”) in six patients were reported; 9 of 11 AEs (81.8%) involved the skin, 1 AE (9.1%) was classified as a flu-like syndrome, and 1 AE (9.1%) was an infection of a wound. All AEs affecting the skin were erosions within or next to the study and control wounds, respectively. None of the AEs was assessed as being related to study treatment (n = 6 not related, 54.5%; n = 5 probably not related, 45.5%). All AEs were mild to moderate in severity: 9 AEs affecting the skin were evaluated as being Common Terminology Criteria for Adverse Events (“CTCAE”) grade 1 (81.8%), only the flu-like syndrome and the infection of a wound were rated as CTCAE grade 2 (18.2%). Treatment with AP101 was not adjusted or interrupted due to AEs. No serious adverse event (“SAE”) or significant AE occurred throughout the study; none of the patients died or were lost to follow-up.

Phase 3 Trials in Partial Thickness Wounds

Before we acquired AP101, three Phase 3 studies were conducted looking at the safety and efficacy of AP101 in other partial thickness wounds (“PTWs”). This led to the approval of AP101 for the treatment of partial thickness wounds by adults by the EMA in 2016. Although we do not intend to commercialize AP101 for partial thickness wounds, we do intend to rely on these Phase 3 clinical trials in our new drug application and marketing authorization application because we consider the data from these trials supportive in the assessment of safety and efficacy of AP101 for the treatment of EB. Patients with EB also suffer from PTWs and the process that leads to wound healing involves the same cellular mechanisms as those seen in other examples of PTWs such as burns and split-thickness skin graft donor sites.

The observations of accelerated wound healing in the Phase 3 trials of PTWs contribute key clinical evidence of the biological activity and pharmacodynamic effect of AP101. In addition, we expect that the FDA, EMA and other health authorities will evaluate safety data to supplement the EASE trial data due to the small size of the study, even though the data must be interpreted with caution due to differences in study design and patient population disease characteristics. Specifically, the Phase 3 trials in PTWs enrolled adult patients who were generally healthy. Each patient had a single wound that was treated for a maximum period of 28 days. Each wound was divided into two halves and each half received topical treatment with AP101 or standard care. Their wounds healed rapidly and without long term complications. In contrast, EB patients studied in the EASE trial are predominantly adolescents, children or infants with multiple complications of their disease including anemia, malnutrition, and impaired kidney function. These disease-related complications are expected to slow the wound healing process. Furthermore, due to the underlying skin fragility caused by EB, patients may develop new wounds during the course of the study and may experience re-opening of previously healed wounds. Patients in the EASE trial are required to apply AP101 or the control gel to multiple wounds over a significant surface area of their skin for the duration of the double-blind phase of the trial (3 months), and then AP101 during the open label phase for a further 2 years. As a result, the efficacy and safety data from the EASE trial will provide the primary (pivotal) evidence required for benefit-risk assessment of AP101 in EB. Data from the Phase 3 PTW trials are expected to provide supportive evidence only.

We have presented pooled safety data for all three PTW Phase 3 trials. The rationale is based on the fact that the trials were almost identical in design. The enrolled patient population characteristics were very similar, and pooling safety data is always performed when possible to increase the number of exposed patients for a more precise estimation of the true incidence of any adverse events that might be expected when much larger numbers of patients are exposed. This approach has been endorsed by the EMA.

Phase 3 Trial in Grade 2a Burns

One of the Phase 3 trials conducted for AP101 (known as BBW-11) was a blinded, prospective, randomized, intra-individually controlled, multicenter, Phase 3 clinical trial comparing the efficacy and safety of AP101 with wound dressing to SOC treatment (an antiseptic gel plus wound dressing) in accelerating the healing of Grade 2a burns. The primary endpoint was the percentage of patients with earlier healing (at least 95% epithelialization) of the wound half treated with AP101 compared to SOC, as evaluated by the majority decision of three independent, blinded experts. Assessment of efficacy was primarily based on blinded photo evaluation.

66 patients were enrolled at ten centers in Germany, Sweden, Switzerland and the United Kingdom. Of the 66 patients, 61 patients received treatment with both AP101 and SOC. Of the 61 patients that received treatment, 50 patients completed treatment and 11 discontinued prematurely. The primary analysis evaluated only those patients for whom a difference was observed between treated wound halves (35 patients).

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As set forth in the table below, the percentage of patients showing earlier healing of AP101 treated wound half compared with SOC treated half was higher than the percentage of patients showing the opposite result (85.7% versus 14.3%, intention-to-treat population).

Primary Endpoint: Earlier Healing of Wound Half - Majority Decision of Blinded Expert Evaluation

	n (%)	95% CI	p value(1)
Patients with earlier healing of SOC treated wound half	5 (14.3)	4.8, 30.3
Patients with earlier healing of AP101 treated wound half	30 (85.7)	69.7, 95.2	<  0.0001

(1)
Based on one-sided, exact binomial test evaluating the rate of superiority of AP101 being > 0.5.

CI = confidence interval, n = number of patients with the indicated difference in wound healing.

A representative series of burn wound photographs from one of the studied patients is shown below:

 

Secondary endpoints supported the primary finding indicating faster healing and epithelialization of the wound with AP101 compared to standard of care.

Two Phase 3 Trials in Patients with Split-Thickness Skin Graft Donor Sites

AP101 was also studied in two blinded, prospective, randomized, intra-individually controlled, multicenter Phase 3 clinical trials (known as BSH-12 and BSG-12) comparing the efficacy and safety of AP101 plus SOC (non-adhesive wound dressing) versus SOC alone in accelerating the wound healing of split-thickness skin graft (“STSG”) donor sites. Since the design and conduct of these studies was identical the pooled results are presented here.

After the STSG surgery, the wound was split into halves and the two wound halves were randomized to treatment with either AP101 plus SOC or SOC alone. Treatment was applied until complete wound closure of both halves occurred but no longer than 28 days. Wound dressing change and medication application were performed at least every three or four days.

The primary endpoint of both trials was the intra-individual difference in time to wound closure (at least 95% epithelialization) between wound halves based on photo evaluation by three independent, blinded experts. 107 and 112 patients were enrolled and received treatment in the two trials respectively. However, two patients in the BSG-12 study did not provide written informed consent and are therefore removed from the analysis below.

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As set forth in the table below, wound halves treated with AP101 healed faster than the wound halves treated with SOC (mean 1.1 days, p < 0.0001, 2-sided paired t-test, based on blinded reader evaluation).

Difference in Time (Days) to Wound Closure⁽¹⁾ (total n = 217)

Study	n	Median	Min, Max	Mean	95% CI	p value(2)
BSH-12	107	-0.3	-10.0,  2.3	-1.4	-1.8,  -0.9	< 0.001
BSG-12	110	0.0	-18.3,  12.3	-0.8	-1.5,  -0.1	0.0232
Pooled	217	-0.3	-18.3,  12.3	-1.1	-1.5,  -0.7	< 0.0001

(1)
Difference in time to wound closure was set to 0 for photos rated as not evaluable. If wound closure was not observed, wound closure was set to 1 day after last photograph of the series (= “conservative +1 day approach”).

(2)
Two-sided paired t-test evaluating the mean difference as different from 0.

CI = confidence interval, n = number of patients in analysis, Max = maximum, Min = minimum.

A representative photographic series from one of the studied patients of a split-thickness skin graft donor site is shown below:

 

Pooled Safety Data From All Three Phase 3 Studies in Partial Thickness Wounds Including EBW-11, BSH-12 and BSG-12

Safety data from the three Phase 3 studies in PTWs were pooled and included 280 patients. One or more AEs were reported for a total of 35% of patients. The system organ classes with the highest incidence were infections and infestations (11%), injury, poisoning and procedural complications (8%) and skin and subcutaneous tissue disorders (10%). Most frequent AEs (by preferred term) comprised pyrexia and wound infection, skin infection, pain of skin, and pruritus, each reported at a rate of 4% to 6%.

Treatment-related AEs were reported by 25 patients (9%), including pain of skin (2.9%), pruritus (1.4%) and a few patients with other AEs, e.g., wound complication and post procedural complications. Application-site AEs were reported for the AP101 treatment half for four patients (1.4%), for the SOC treatment half for 15 patients (5.4%) and for both treatment halves (meaning that the location of the AE could not be further differentiated) for 36 patients (12.9%). In cases where the AE could be clearly ascribed to one wound half, the number of local AEs was lower in the AP101 treated wound half compared with the SOC treated wound half.

The number of patients in the pooled analysis (study BSH 12, BSG 12 and BBW 11) who experienced one or more serious SAEs was low (15 or 5.4% of the patients). The most frequently reported SAEs were wound infection (four or 1.4% of patients) and condition aggravated (two or 0.7% of patients). All other SAEs occurred in only one (0.4%) patient per event. Only one SAE was considered possibly related to treatment (wound necrosis) for which causality between treatment and event was reported as “yes: unknown” by the investigator. In this patient both wound halves had changed from Grade 2a to Grade 2b and required skin grafting resulting in prolonged hospital stay. Based on clinical experience a change in burn wound depth from Grade 2a to Grade 2b was expected for up to 30% of patients in the study. Skin grafting is the standard treatment for Grade 2b burn wounds.

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The number of patients who discontinued the study due to AEs was low (10 or 3.6% of patients) and none were considered related to treatment except for the case of wound necrosis described above.

No deaths were reported in the treatment phase of the studies. Two deaths in the 12 months follow-up period were considered not related to the treatment.

Ongoing Pivotal Phase 3 Trial of AP101 in EB (EASE)

In 2017, we commenced a double-blind, randomized, vehicle-controlled, Phase 3 study with a 24-month open-label period follow-up comparing the safety and efficacy of AP101 versus a gel vehicle in the promotion of healing of PTWs in patients with severe EB (JEB, DEB and Kindler Syndrome). Qualifying wounds must be between 10 cm² and 50 cm² in size and no younger than 21 days and no older than nine months old.

The primary endpoint is the proportion of patients with first complete closure of the EB target wound by day 45±7 days compared to vehicle based on clinical assessment by the investigator (the wound will be rated as “closed” at first appearance of complete re-epithelialization without drainage).

Key secondary endpoints are:

• Time to first complete closure of the EB target wound as evidenced by clinical assessment until the end of the double-blind phase (day 90±7 days)

• Proportion of patients with first complete closure of the EB target wound at day 90±7 days based on clinical assessment by the investigator

• The incidence of wound infection between baseline and day 90±7 days as evidenced by adverse events and/or use of topical and/or systemic antibiotics (related to wound infection)

• The maximum severity of wound infection between baseline and day 90±7 days as evidenced by adverse events and/or use of topical and/or systemic antibiotics (related to wound infection)

• Change from baseline in total body wound burden as evidenced by clinical assessment using Section I of the EB Disease Activity and Scarring Index at day 90±7 days

• Change from baseline in itching using the Itch Man Scale in patients ≥ 4 years and up to 13 years of age and the Leuven Itch Scale in patients ≥ 14 years of age before wound dressing changes at day 90±7 days

Safety endpoints include:

• Incidence, severity and relatedness of adverse events

• Local tolerability as judged by the investigator

• Safety laboratory data

• Systemic exposure to betulin

Following completion of the 90 day double blind period all patients randomized to the control gel will switch to open label treatment with AP101. Patients who had been randomized to AP101 will continue on open label treatment until the end of the study.

During the open label safety follow up period, patients will be monitored for safety evaluation by collection of adverse event information. The efficacy of AP101 during this phase of the study will be evaluated by assessment of total wound burden, impact on symptoms of disease and additional endpoints reflecting severity of disease and quality of life.

Unblinded Interim Efficacy Analysis and Safety Analysis

In January 2019, we reported that the DSMB performed a pre-specified unblinded interim efficacy analysis and recommended the continuation of the study with an increase in enrollment from 182 to 230 evaluable patients to maintain 80% conditional statistical power. This increase in sample size was based upon maintenance of 80% conditional statistical power to detect a 20% difference between treatment groups with respect to the primary efficacy endpoint.

In February 2019, the independent DSMB performed a pre-specified unblinded interim safety analysis which included review of safety and PK data from children enrolled in the trial. Following this review, the DSMB

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recommended expansion of the trial to include neonates, infants and children from ≥ 21 days of age. As a result of the COVID-19 pandemic which has had a material impact on recruitment into clinical trials globally, and given that the EASE trial was already close to full enrollment, we have taken advice from an independent expert and concluded that the statistical impact of further patient recruitment would most likely be negligible. We therefore decided to close the EASE trial to further enrollment in April 2020 and expect to report top line results from the EASE trial in the second half of 2020.

Additional Opportunity for AP101

We are also supporting an investigator-led Phase 2 study of AP101 for the treatment of severe radiation-induced dermatitis. This trial is expected to commence in the second half of 2020, with data expected in the second half of 2021.

AP103 for the Treatment of RDEB

In March 2018, we acquired the rights to a novel polymer-based topical gene therapy delivery platform for potential use in the treatment of rare genetic diseases. The technology involves the use of highly branched poly β-amino ester (“HPAE”) polymers as the topical delivery vehicle for gene therapy. Our first product candidate utilizing this platform, AP103, is currently in preclinical development for the treatment of patients with RDEB. Patients with RDEB have a defect in the COL7A1 gene resulting in the inability to produce collagen VII, which plays an important role in anchoring the dermal and epidermal layers of the skin. AP103 is the combination of this polymer technology and the COL7A1 gene. If successful, we believe this could eliminate the requirement for viruses as topical delivery vectors.

In preclinical studies in a human mouse xenograph model of EB, we observed that topical application of AP103 restored production of collagen VII. In separate preclinical studies, AP103 was observed to restore collagen VII to levels exceeding those produced by healthy human keratinocytes (cells that regenerate the outer layer of the skin). In addition, we did not observe evidence of cellular toxicity after repeated administration in these studies. Our preclinical development of AP103 is ongoing. We intend to initiate clinical development of AP103 in the second half of 2021.

In December 2018, a consortium comprised of us (as lead partner), University College Dublin, National University of Ireland (“University College Dublin”), Curran Scientific Limited and DEBRA Ireland was awarded (subject to contract negotiation) grant funding totaling €8.4 million over three years from the Disruptive Technologies Innovation Fund, part of the Irish Government’s Department of Business, Enterprise and Innovation, to develop the AP103 gene therapy platform. The grant funding will be matched by the consortium partners at various funding levels over the three-year term of the project. The grant will fund further development of the AP103 gene therapy platform from preclinical testing to proof of concept in humans. The initial funds will be used for research and development and staff costs associated with the project and, if preclinical work is successful, to fund the initial clinical development of AP103. In addition to the primary work on AP103, the funds will also support research into the development of the HPAE polymer technology for the potential treatment of other genetic diseases.

Medical Affairs

We have a medical affairs team in the United States, EMEA and Latin America. The medical affairs team maintains product registries, performs retrospective reviews of clinical data, and conducts post-marketing clinical trials as needed, to support the value propositions of our products. The medical affairs team also supports independent medical education programs and investigator-initiated studies, by providing financial grants in a number of medical and disease-related areas. The responsibilities of medical affairs personnel also include providing training and education to physicians through the dissemination of medical information, industry research and publications, providing support in connection with our post-approval clinical commitments, as well as assembling and managing scientific and medical advisory boards to obtain valuable input from experts and practitioners on a variety of medical topics relevant to our products and the diseases our products treat.

Sales, Marketing and Reimbursement

In the United States, our sales team promotes MYALEPT to healthcare professionals for the treatment of GL patients, and JUXTAPID for the treatment of adult HoFH patients. The most frequent physician call points for our products are endocrinologists (including pediatric endocrinologists) with respect to MYALEPT and lipidologists and cardiologists with respect to JUXTAPID.

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Our sales and marketing efforts are also focused on obtaining market access for our products, which primarily represents securing pricing and reimbursement approvals at acceptable levels. In the United States, we mobilize a team supporting market access initiatives with the primary responsibility of collaborating with insurance plans, health maintenance organizations and other payers on securing reimbursement and formulary status for MYALEPT and JUXTAPID. This team also anticipates authorization and reauthorization requirements and educating physicians and office staff regarding criteria to continue coverage for our products.

Outside the United States, we support this effort primarily through the work of Country Managers and local consultants. We have successfully secured market access for both lomitapide and metreleptin in certain key EU and LATAM countries. We also continue to submit market access dossiers in other EU and LATAM countries where reimbursement has not occurred to date. We intend to enter into price negotiations in such countries following approval of the relevant Health Technology Assessment (“HTA”) body. One of our primary objectives is to strengthen the value proposition for metreleptin and lomitapide for payers through the generation of market access studies to enhance patient, physician and payer knowledge of GL, HoFH, and, in the European Union, PL and the real-world burden of these diseases.

Manufacturing Supply and Distribution

We and our contract manufacturers are subject to the FDA’s Current Good Manufacturing Practice (“cGMP”) regulations in the United States and other rules and regulations prescribed by regulatory authorities outside the United States.

Lomitapide is a small molecule drug that is synthesized with readily available raw materials using conventional chemical processes. Hard gelatin capsules are prepared in 5 mg, 10 mg, 20 mg, 30 mg, 40 mg and 60 mg strengths. Metreleptin is a recombinant protein biologic that is produced using conventional fermentation, isolation and purification processing techniques. The drug product is provided globally in nominal 10 mg vials that are reconstituted prior to injection. We developed, and received approval from the EMA for new presentations in nominal 2.5 mg and 5 mg vials for commercial distribution in the European Union.

We rely on contract manufacturers to produce the drug substance for lomitapide and metreleptin and to produce the drug product for commercial supplies and for clinical trials. We manufacture the drug substance for AP101 at our facility in Germany. If AP101 receives approval we intend to supplement our internal manufacturing capacity with contract manufacturers. We have long-term supply agreements with the manufacturers of lomitapide and metreleptin. We may encounter any issue with our suppliers, for example, in the event a manufacturer notifies us that it intends to terminate its arrangement or if the manufacturer is operating in violation of the cGMP regulations. To mitigate such risks, we maintain sufficient inventory of metreleptin and lomitapide drug substance to supply our production for at least one year. Prior to appointing a new supplier, we will undertake a rigorous validation and approval process of that manufacturer.

We require supplies of bacteriostatic water for injection (“BWFI”), an approved diluent for reconstitution of metreleptin that allows for use of a reconstituted vial of metreleptin for up to three days when stored appropriately. BWFI is only available for sale and approved for use in the United States, and we or our contract manufacturers will purchase it for supply with named patient sales outside the United States and other expanded access distribution to reduce the number of metreleptin vials provided. BWFI is not required in Europe following the launch of appropriate vial sizes to accommodate the dosing needs of the patient population.

In the United States, lomitapide and metreleptin are distributed exclusively through Accredo Health Group, Inc. (“Accredo”), through a specialty pharmacy network that provides the products directly to patients and, under limited circumstances, to other U.S. purchasers. The specialty pharmacy takes title upon delivery of products. It also provides certain patient program support services. For commercial sales outside the United States, including sales of MYALEPTA and LOJUXTA, named patient sales and other expanded access distribution, we use our existing infrastructure and third party providers to distribute products either directly to the purchaser in the applicable country or to our local third party distributor or service provider for such country.

Competition

The industry in which we operate is highly competitive and subject to rapid and significant technological change. Our competitors and potential competitors include large pharmaceutical and biotechnology companies, specialty pharmaceutical and generic drug companies, academic institutions, government agencies and research institutions.

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All of these competitors currently engage in, have engaged in or may engage in the future in the development, manufacturing, marketing and commercialization of pharmaceuticals that compete with or may in the future compete with metreleptin, lomitapide or other products or product candidates we may develop or acquire. Smaller or early stage companies may also prove to be significant competitors, particularly through collaborative arrangements with large, established companies. Academic institutions, government agencies and other public and private research organizations also are conducting research activities, seeking patent protection and may commercialize products on their own or through joint ventures. The existence of these products, or other products or treatments of which we are not aware, or products or treatments that may be developed in the future, may adversely affect the marketability of products we develop.

• Lomitapide: A significant competitor product to our lomitapide product is a class of drugs known as PCSK9 inhibitors. One PCSK9 inhibitor, an Amgen, Inc. (“Amgen”) product, is currently approved and commercialized in the European Union and the United States for the same treatment indication, HoFH, as lomitapide. Sales of this PCSK9 inhibitor compete with sales of the lomitapide product and we expect that this product will continue to compete with lomitapide. However, because many therapies, including PCSK9 inhibition products, act by increasing the activity of LDL-R, HoFH patients often have an inadequate response due to their impaired LDL-R function. In addition, other companies may succeed in developing, acquiring or licensing additional pharmaceutical products that are introduced into the market and that are more effective or less costly than our products. For example, we are aware of several companies developing products that could potentially compete with lomitapide, including Regeneron Pharmaceuticals, Inc. and REGENXBIO Inc.

• Metreleptin: Our competitors are also developing products, which, if approved and depending on the labelled indication, could potentially compete with metreleptin, including Regeneron Pharmaceuticals, Inc. and Akcea Therapeutics, Inc. Although MYALEPT is the first and only product approved in the United States for the treatment of complications of leptin deficiency in patients with GL, there are a number of therapies approved to treat these complications independently that are not specific to GL. MYALEPTA also faces competition in the European Union, both for the treatment of GL and PL.

• AP101: Although there are no approved products in the United States or the European Union for the treatment of EB, some of our competitors are developing products which, if approved, and depending on the labelled indication, could potentially compete with AP101. Some companies, including Krystal Biotech, Inc. and Abeona Therapeutics Inc., are developing gene therapy treatments, while other companies, including Castle Creek Pharmaceuticals Holdings, Inc. and Wings Therapeutics, Inc., are developing non-gene therapy treatments.

Intellectual Property

We rely on patents covering inventions licensed from third parties and on other means to protect our technology, inventions and improvements that are commercially important to its business.

Our policy is to file patent applications on a worldwide basis in those jurisdictions where it is considered beneficial, depending on the subject matter and the commercialization strategy.

We have a portfolio of patents and pending patent applications covering various aspects of our lomitapide, metreleptin, AP101 and AP103 assets in a variety of jurisdictions. Our lomitapide patent portfolio includes seven Approved Drug Products with Therapeutic Equivalence Evaluations (“Orange Book”) listed patents licensed from the University of Pennsylvania (“UPenn”) claiming methods of treatment that provide protection from 2025 to 2027 in the United States and into 2025 in Europe, Australia, Canada, India, Japan, South Korea and New Zealand. A U.S. continuation application is still pending. Supplementary protection certificates (“SPCs”) have been granted in 22 European countries (including the United Kingdom, Germany, France, Italy and Spain) extending patent protection into 2028. Six additional SPCs are still pending.

The metreleptin patent portfolio consists of two issued U.S. patents claiming methods of treating lipoatrophy, two granted European patents (“EP patents”) claiming methods of treatment (validated in numerous European countries, including the United Kingdom, Germany, France, Italy and Spain), as well as pending applications in Japan and the United States, all of which have been licensed to Aegerion or are owned by Aegerion. The granted patents provide protection from 2022 to 2027 in the United States and into 2022 in the European Union. Additional applications are pending in the United States, Brazil, Canada, China, Eurasia, India, Japan and

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Mexico claiming methods of detecting anti-leptin neutralizing antibodies, and an international application is pending claiming methods of treating leptin deficient obese patients, which if granted, would expire in 2037 and 2039, respectively.

We were first granted patent protection for AP101 in Japan in 2010. Key patent grants for AP101 in Europe and the United States followed in 2013. Our AP101 patent portfolio includes one Orange Book listable U.S. patent covering oleogel compositions providing exclusivity in the United States to 2025 and two Orange Book listable U.S. patents covering treatment of EB that provide exclusivity in the United States into 2030. Our non-U.S. patents for AP101, include EP patents (validated in various European countries including the United Kingdom, Germany, France, Italy and Spain) covering the AP101 composition and methods of healing wounds with AP101, expiring in 2025 and 2030, respectively. Supplementary protection certificates have been obtained in various EU countries, extending the expiration of the composition patents in the European Union from 2025 to 2030. We have filed a further international patent application directed to the clinical formulation and methods of manufacturing and treatment therewith, which, if successfully granted in national stage jurisdictions, will provide further exclusivity to 2039, or later.

The AP103 patent portfolio includes one granted EP patent (validated in various European, countries including the United Kingdom, Germany, France, Italy and Spain) claiming HPAE polymers and polyplexes comprising HPAE polymers, and transfection methods using such polymers, as well as pending U.S. and European divisional applications. These patents and applications have been in-licensed from the University College Dublin, and are expected to provide exclusivity into 2035. We have also filed an international application covering a proprietary genus of HPAEs and novel HPAE polyplexes, as well as an international application claiming a proprietary and scalable HPAE polyplex manufacturing methods, which if granted would expire in 2039. See “—Material Contracts—University College Dublin In-License Agreement.”

JUXTAPID was granted Orphan Drug status in the United States for the treatment of HoFH and for the treatment of FCS, and LOJUXTA obtained Orphan Drug status in the European Union for the treatment of FCS. These Orphan Drug designations provide exclusivity in the United States to December 2019 (for the approved HoFH indication) and, upon approval of an FCS indication in the United States, potentially seven years of exclusivity and, upon approval of an FCS indication in the European Union, 10 years of exclusivity (plus two years on successful completion of a PIP).

Following approval by the EMA in July 2018, metreleptin is entitled to ten years of market and Orphan Drug exclusivity in the European Union (to July 2028 which can be extended a further two years upon successful completion of a PIP) for the treatment of familial partial lipodystrophy, Barraquer-Simons syndrome, Lawrence syndrome, and Berardinelli-Seip syndrome. In the United States MYALEPT has seven years of Orphan Drug exclusivity for the treatment of metabolic disorders secondary to lipodystrophy and also for the treatment of leptin deficiency secondary to PL and GL, with exclusivity specific to the approved GL indication being scheduled to expire in February 2021 (MYALEPT should also qualify for a 12-year period of exclusivity from biosimilar or interchangeable products, which will expire in 2026, under the Biologics Price Competition and Innovation Act). MYALEPT could also receive orphan designation upon submission and approval of a PL indication in the United States which would provide seven years of exclusivity for this indication following approval in the United States.

AP101 has been granted Orphan Drug status for the treatment of EB in the European Union and the United States. Should AP101 be granted approval it will be entitled to Orphan Drug exclusivity for the treatment of EB, extending seven years in the United States and ten years in the European Union from the date of approval in their respective jurisdictions. The ten-year Orphan Drug exclusivity period in the European Union can be extended a further two years upon successful completion of a PIP.

Material Contracts

The following summary of our material contracts is not intended to be a complete description of these contracts, and it is qualified in its entirety by reference to the full text of such contracts, which are filed as exhibits to the registration statement of which this prospectus forms a part. We urge you to review these exhibits in their entirety. Unless otherwise noted, each of the material contracts is not affected by the Aquisition.

The following summary excludes contracts with our principal shareholders that we entered in connection with the Acquisition, which are summarized under “Related Party Transactions.”

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Share Purchase and Transfer Agreement between Software AG – Stiftung and Dr. Armin Scheffler and Amryt Pharmaceuticals DAC

On October 16, 2015, Amryt Pharmaceuticals DAC entered into a share purchase and transfer agreement (“Birken SPA”) with Software AG – Stiftung and Dr. Armin Scheffler in their capacity as sellers (together, “Birken Sellers”) relating to the purchase of the entire issued share capital of Birken AG (now Amryt GmbH). Under the Birken SPA, the consideration for the shares of Birken AG comprised the allotment and issue of new ordinary shares in Amryt Pharmaceuticals DAC, the sum of €1,000,000 plus additional milestone and royalty payments if/when certain milestones are met. As of the date of this prospectus, we have made milestone payments of €12 million under this agreement. Remaining milestone payments are as follows:

• €3 million within 30 days of net ex-factory sales on or after marketing approval of at least €100,000;

• €10 million once net ex-factory sales/net revenue in any calendar year exceed €50 million;

• €15 million once net ex-factory sales/net revenue in any calendar year exceed €100 million; and

• €10 million on receipt of marketing approval by the EMA or the FDA for the treatment of EB.

The royalty payments payable to the Birken Sellers are as follows: (a) 9% of (i) net ex-factory sales, and (ii) net revenues in either case relating to AP101; and (b) 6% of: (i) net ex-factory sales; and (ii) net revenues relating to other betulin products, with the relevant royalty periods essentially being ten years from first commercial sale of the relevant product (other than in respect of Imlan).

University College Dublin In-License Agreement

On March 14, 2018, Amryt Genetics Limited entered into an agreement with University College Dublin relating to the patent rights for AP103. Under this agreement, University College Dublin granted an exclusive, worldwide license to the patent rights for the platform technology to develop one or more gene therapy products for therapeutic use in any disorder in humans and animals. University College Dublin retains an irrevocable, perpetual, royalty-free, worldwide right to use the licensed intellectual property for the purposes of academic teaching, publication and non-commercial research. The subject platform technology relates to a non-viral gene therapy, which offers a potential treatment for patients with EB. Preliminary data available to us suggests that the treatment could be potentially disease-modifying for patients with RDEB. We intend to conduct various preclinical studies in connection with the agreement.

In consideration of the agreement, Amryt Genetics Limited made an upfront payment of €40,000 and agreed to the following milestone payments:

• €100,000 on the successful completion of a Phase 2a proof of concept study;

• €100,000 on the successful completion of a Phase 2b study;

• €200,000 upon the first commercial sale of a gene therapy product incorporating or utilizing the licensed technology; and

• €200,000 upon the first commercial sale of each subsequent product requiring a separate marketing authorization.

Upon the sale of product, we are obligated to make royalty payments to University College Dublin as follows: (a) 2% on net sales in a calendar year up to €100 million, and (b) 3% on net sales in a calendar year in excess of €100 million. Under the agreement, we must also pay to University College Dublin a portion of the royalties we receive from our affiliates in connection with the product: (a) 30% of net royalties prior to the initiation of a Phase 1 study, (b) 20% of net royalties commencing upon the initiation of a Phase 1 study and before the completion of a Phase 2a proof of concept study, and (c) 10% of net royalties commencing upon the completion of a Phase 2a proof of concept study.

The agreement continues in full force on a product-by-product and country-by-country basis until the later of (a) the expiration of the last valid claim of patent rights, (b) the expiration of orphan drug exclusivity, or (c) 15 years after the first commercial sale of the first product by us or our affiliates or sub-licensees. University College Dublin may terminate the agreement with 30 business days’ prior written notice under the following circumstances:

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• if we are in material breach of any provision of the agreement and, after receiving notice from University College Dublin identifying a material breach, we fail to cure said material breach within 60 business days, University College Dublin may issue a notice of default. If we do not cure the material breach within 60 business days from receipt of the notice of default, then University College Dublin may terminate the agreement;

• if we become insolvent, or if an interim order is applied for or made, or a voluntary arrangement approved, or a voluntary arrangement is proposed or approved or an administration order is made, or a receiver or administrative receiver is appointed for any of our assets or undertaking or a winding-up resolution or petition is passed or presented (otherwise than for the purposes of reconstruction or amalgamation), or if any circumstances arise which entitle the court or a creditor to appoint a receiver, administrative receiver or administrator or to prevent a winding-up petition or make a winding-up order, or other similar or equivalent action is taken against or by us by reason of our insolvency or in consequence of debt, or if we make any arrangement with our creditors;

• if we or our affiliates challenge the validity of the patent applications when granted, or assist any third party to commence legal proceedings to challenge such validity;

• if, according to an independent expert’s judgment, we have failed to use commercially reasonable efforts to develop, use and exploit the intellectual property licensed under the agreement, and six months after the independent expert’s judgment we have still failed to take the specific actions to develop, use and exploit the intellectual property licensed, then University College Dublin may at any time within three months after the end of that six-month period, provide at least three months’ notice to us to terminate the agreement;

• if we fail to pay any amount due under the agreement within 30 business days having received written notice from University College Dublin of our failure to pay, and such failure to pay is not subject to a good faith dispute between the parties; and

• if we dispose of all, or a substantial part of our business involving the licensing of the intellectual property under the agreement in circumstances where we do not enter into a novation agreement pursuant to us becoming insolvent or any other circumstance described above.

We may terminate the agreement at any time by giving University College Dublin at least 60 business days’ prior written notice. Upon expiration or termination of the agreement, all rights and licenses granted to us under the agreement also terminate.

University of Pennsylvania Lomitapide License Agreement

On May 19, 2006, Aegerion entered into an agreement with UPenn for the in-license of lomitapide. Pursuant to the terms of the agreement Aegerion was granted an exclusive and worldwide license (including right to sublicense) to certain patents of UPenn and certain patents assigned to UPenn by Bristol-Myers Squibb (“Bristol-Myers”). The license is provided for the following field of use: (a) monotherapy or with other dyslipidemic therapies to treat patients with HoFH; and (b) monotherapy or in combination with other dyslipidemic therapies for treatment of patients with other severe forms of hypercholesterolemia unable to come within 15% of National Cholesterol Education Program’s (“NCEP”) goal on maximal tolerated oral therapy, as determined by the patient’s prescribing physician, with severe combined hyperlipidemia unable to come within 15% of NCEP’s non-high-density lipoprotein-cholesterol goal on maximal tolerated oral therapy, as determined by the patient’s prescribing physician, or with severe hypertriglyceridemia unable to reduce triglycerides less than 1,000 on maximal tolerated therapy.

In partial consideration of the in-license of lomitapide, Aegerion is required to pay UPenn a graduated royalty between 5% and 8% of Aegerion’s annual net sales made pursuant to the terms of the agreement. The sub-license fees are as follows: (i) 25% of sub-licensing fees received for net sales in a country that is covered by a valid claim of the licensed patent rights and (ii) 15% of all other sub-license fees.

If the indication is not HoFH or severe refractory hypercholesterolemia, then additional payments are required to be made upon the initiation of a Phase 3 trial ($300,000), the filing of a U.S. NDA ($750,000) and the grant by the FDA of approval of an NDA ($1,500,000).

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The agreement expires on a country-by-country basis upon there no longer being a valid claim of licensed patent rights. Upon the expiration in a country, the license becomes irrevocable and fully paid-up. We can also terminate the agreement at any time.

Japan Lomitapide License Agreement

On February 5, 2019, Aegerion entered into a license agreement with Recordati for the commercialization of JUXTAPID in Japan. Under the terms of the License Agreement, and subject to the conditions set forth therein, Aegerion granted to Recordati an exclusive license in Japan, for the current marketed indication for HoFH. During the term of the License Agreement, Recordati also has an exclusive right of first negotiation to any new indications for JUXTAPID in Japan that may be developed by Aegerion and the right to grant sub-licenses and to manufacture and commercialize JUXTAPID, under specific circumstances.

Pursuant to the terms of the License Agreement, and subject to the conditions set forth therein, Recordati is required to make the following payments to Aegerion: (i) $25 million as a one-time upfront payment on the effective date of the agreement (which was paid to Aegerion in the first quarter of 2019), (ii) $5 million as a one-time payment within 45 days following the date on which the Japan marketing authorization for JUXTAPID is successfully transferred to Recordati (“Completion Date”) (which was paid to Aegerion in the second quarter of 2019), (iii) quarterly royalty payments, during the term of the License Agreement, equal to 22.5% of all net sales of JUXTAPID in Japan, and (iv) 20% of all other sublicense revenues received by Recordati or any of its affiliates.

In addition, pursuant to the terms of the License Agreement, Aegerion may receive from Recordati commercial milestone payments (up to a total of $80 million) for net sales in Japan, conditioned and based upon the achievement of certain net sales levels in Japan, the first $12.5 million installment of which becomes payable at the end of the first quarter in which cumulative net sales in Japan reach $70 million, and which are payable in incremental installments thereafter at the end of each quarter in which cumulative net sales in Japan increase by $70 million (in incremental payments of $12.5 million until cumulative net sales reach $280 million and then in incremental installments of $5 million for each incremental $70 million of revenues until cumulative net sales reach $700 million).

Further, pursuant to Aegerion’s current license agreement with UPenn, UPenn is entitled to receive 15% of the $25 million upfront payment, 15% of the marketing authorization transfer milestone and any subsequent sales milestone payments received from Recordati and 25% of royalty payments received by Aegerion from Recordati under the License Agreement.

Aegerion and Recordati have also entered into a customary supply agreement under which Aegerion will supply JUXTAPID to Recordati (or its affiliate) at cost plus an agreed upon markup for an initial term of two years with automatic renewal for successive two year terms.

The initial term of the License Agreement continues until the latest of: (i) expiration of the last valid claim of the licensed patents covering JUXTAPID in Japan, (ii) expiration of data or regulatory exclusivity in relation to JUXTAPID in Japan, or (c) ten years from the Completion Date. Thereafter the term of the License Agreement will automatically renew for a single five-year term, and then thereafter for successive five-year terms unless either party provides written notice at least 18 months prior to the end of the then current renewal term. Either party may terminate the License Agreement for cause if the other party materially breaches or defaults in the performance of its obligations, and, if curable, such material breach remains uncured for 90 days (15 days for non-payment).

Metreleptin Asset Purchase Agreements

In November 2014, Aegerion entered into an asset purchase agreement relating to the acquisition from Amylin Pharmaceuticals, LLC (“Amylin”) and AstraZeneca Pharmaceuticals LP (“AstraZeneca”), an affiliate of Amylin, of certain assets and rights associated with the biological product metreleptin for injection. Under the terms of the agreement, Aegerion paid AstraZeneca $325 million upfront to acquire the global rights to develop, manufacture and commercialize metreleptin, subject to an existing distributor license with Shionogi covering Japan, South Korea and Taiwan, which Aegerion assumed upon closing. The transaction did not include the transfer of any AstraZeneca employees or facilities.

On January 9, 2015, Aegerion entered into a letter agreement with AstraZeneca and Bristol-Myers, which was subsequently amended on April 30, 2015. This agreement was entered into pursuant to Aegerion’s acquisition of metreleptin from AstraZeneca. Under the asset purchase agreement and letter agreement, Aegerion agreed to

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fulfill certain of AstraZeneca’s original obligations to Bristol-Myers including reporting, milestone and royalty payments and diligence obligations in relation to metreleptin.

Pursuant to the terms of the agreements we are required to pay 5-10% royalties to Bristol-Myers, as previously paid by AstraZeneca, on the net U.S. sales of metreleptin.

Amgen License Agreement

In connection with Aegerion’s acquisition of metreleptin in January 2015, Aegerion acquired a license agreement between Amgen and Amylin, dated February 7, 2006 (“Amgen License”) pursuant to which Aegerion obtained an exclusive worldwide license from Amgen to certain know-how and patents and patent applications covering the composition of matter and methods of use of metreleptin to develop, manufacture and commercialize a preparation containing metreleptin (“Amgen Licensed Products”).

As part of the Amgen License, Aegerion also obtained an exclusive sublicense of Amgen’s exclusive rights to certain metreleptin-related patents and patent applications owned by the Rockefeller University and exclusively licensed to Amgen under a license agreement dated April 14, 1995, as amended (“Rockefeller License”) and an exclusive sublicense of Amgen’s non-exclusive rights to certain metreleptin-related patents and patent applications owned by The Regents of the University of California and non-exclusively licensed to Amgen under a license agreement dated July 13, 2005 (“UCSF License”). Amgen retains rights to conduct research, development, manufacturing and commercialization activities with respect to products other than the Amgen Licensed Products.

Aegerion may grant sublicenses under the licenses and sublicenses granted by Amgen, subject to certain limitations, including Amgen’s right of first offer for any out-license, partnership, co-development, commercialization, co-promotion or similar agreement related to metreleptin or the Amgen Licensed Products, which expires in February 2021.

Aegerion is required to make royalty payments to Amgen and Rockefeller University on net sales of each Amgen Licensed Product on a country-by-country basis (i) at a royalty rate in the low double digits where the Amgen Licensed Product has patent protection or market exclusivity granted by a regulatory authority at the time of regulatory approval in the applicable country during the applicable royalty term, which runs on a country-by-country basis until the later of (a) the expiration of the last-to-expire valid claim covering an Amgen Licensed Product in the applicable country, (b) the expiration of any market exclusivity granted by a regulatory authority, and (c) ten years from the date on which an Amgen Licensed Product is first sold to a third party in a country after regulatory approval for the Amgen Licensed Product has been granted in such country (“Amgen Royalty Term”) or (ii) at a royalty rate in the mid-single digits to low double digits where the Amgen Licensed Product receives patent protection or market exclusivity following the time of regulatory approval in the applicable country, in either case subject to a variety of customary reductions.

Under the Amgen License, Aegerion is also required to directly meet certain payment obligations under the Rockefeller License and UCSF License. Aegerion is required to make royalty payments to Rockefeller University on net sales of each product with patent rights or know-how in the field of obesity genes, obesity gene products, and molecules that modulate or mediate their action and/or regulation on a country-by-country basis at a range of royalty rates in the low single digits depending on whether the product has an orphan product designation or not until the later to occur of expiration of (i) patent protection, (ii) any market exclusivity period granted in the applicable country, or (iii) any data exclusivity period in the applicable country (with certain limitations related to the number of units sold). Since acquiring this license agreement in January 2015, Aegerion has paid a one-time $5 million milestone payment to Rockefeller University in February 2015, which was due 12 months following the receipt of marketing approval for MYALEPT in the United States. Aegerion will also be required to pay to Rockefeller University a percentage in the low double digits of any upfront license fees or one-time fees it receives in consideration for a sublicense of the licensed rights. There are no material payment obligations outstanding under the UCSF License.

Current patent protection exists from 2022 to 2027 in the United States and into 2022 in the European Union, as well as orphan exclusivity in the European Union into 2028. Additional applications are pending in the United States, Brazil, Canada, China, India, Japan and Mexico claiming methods of detecting anti-leptin neutralizing antibodies, and an international application is pending for methods of treating leptin deficient obese patients, which if granted, would expire in 2037 and 2039, if extended upon successful completion of a PIP, respectively.

We assumed this agreement upon the Acquisition. We have the right to terminate the agreement at any time.

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National Institutes of Health (“NIH”) License Agreement

In February 2017, Aegerion entered into an in-license agreement in respect of patent rights for metreleptin with the NIH. Pursuant to the terms of the agreement Aegerion was granted an exclusive and worldwide license to (including the right to sub-license) the NIH patent rights for the use of leptin, leptin analogs and derivatives for the diagnosis, prevention and treatment of human lipodystrophy.

Upon the date of the first commercial sale of a licensed product in a country other than the United States, Aegerion agrees to pay NIH a royalty percentage of 0.4% of net sales, on a country-by-country basis, in such countries in which a valid patent claim exists. No U.S. royalty is due on the sales.

The agreement expires upon the expiration of the last to expire licensed patent rights. We can terminate the agreement at any time and NIH has the following termination and modification rights:

Other than NIH having the right to immediately terminate the agreement should we become insolvent, file for bankruptcy or receive notice of a third party’s intention to file an involuntary petition in bankruptcy, NIH may terminate the agreement if we default on any material obligation under the agreement which has not been remedied within 90 days of the notice of default being provided, including the following:

• we have not achieved certain agreed performance milestones;

• we have willfully made a false statement of, or willfully omitted, a material fact in the license application or in any report required by the agreement;

• we have committed a material breach of a covenant or agreement contained in the agreement that has not been remedied within 90 days;

• we have not been keeping the licensed products or the licensed processes reasonably available to the public after commercial use commences; or

• we cannot reasonably satisfy unmet health and safety needs.

Accredo Agreements

On December 6, 2013, Aegerion entered into a distribution agreement in respect of lomitapide with Accredo.

Pursuant to the agreement Accredo is appointed as an authorized non-exclusive distributor of record of lomitapide. Aegerion may appoint other distributors upon 60 days’ notice to Accredo (such notice must also be provided by Aegerion when other specialty pharmacies are added to the distribution network). If Aegerion exercises this right and such an appointment causes Accredo to no longer be one of three (or less) specialty pharmacies, Accredo may terminate the agreement. In addition, either party may terminate the agreement at any time upon 180 days’ written notice to the other party.

On December 6, 2013, Aegerion entered into a master services agreement in respect of metreleptin with Accredo. The agreement sets the framework of non-exclusive commercial relationship between the parties. Pursuant to the agreement, Accredo agreed to perform services on our behalf, related to metreleptin as described in one or more statements of work provided to us. Such services include, but are not limited to case management and ongoing clinical support services. In consideration for services provided, we are required to pay Accredo the fees set forth in the applicable statement of work. We have the right to terminate at any time upon 120 days’ notice and Accredo may terminate on one year’s notice.

Each of these agreements expires on September 30, 2020.

Metreleptin Contract Manufacturing Agreement

We currently have in place a contract manufacturing agreement with Sandoz GmbH in respect of metreleptin, effective as of September 30, 2010. Pursuant to the terms of the agreement, Sandoz GmbH is appointed as non-exclusive manufacturer of metreleptin. Sandoz GmbH is obligated to produce a majority of Aegerion’s metreleptin product demand and Aegerion has committed to purchase such amount and is committed to a contract value for 2020 in the amount of €11.249 million. The agreement expires on December 31, 2023. Either party may terminate the agreement for any scientific, regulatory, safety or economic reason to the end of each calendar year by giving 24 months’ prior written notice.

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Government Regulation and Product Approval

Government authorities in the United States, at the federal, state and local level, the European Union, EU Member States, and in other countries extensively regulate, among other things, the research, development, testing, manufacture, quality control, approval, labelling, packaging, storage, record-keeping, promotion, advertising, distribution, post-approval monitoring and reporting, marketing and export and import of the products. In the future, if we were to develop or acquire any other products, or any product candidates, they would also be subject to such regulations and oversight. Our products must be approved by the FDA through the NDA or the BLA process before they may be legally marketed in the United States, and must be approved by foreign regulatory authorities via various procedures before they can be marketed in the applicable country, including the EMA or the regulatory authorities of the EU Member States before they can be placed on the market in the European Union.

U.S. Regulatory Matters

Drug and Biologic Development Process

In the United States, the FDA regulates drugs under the FDCA, and regulates biologics under both the FDCA and the Public Health Service Act of 1944 (“PHSA”). The process of obtaining marketing approvals and the subsequent compliance with appropriate federal, state, local, and foreign statutes and regulations require the expenditure of substantial time and financial resources. Failure to comply with the applicable U.S. requirements at any time during the product development process, approval process or after approval, may subject a company to administrative or judicial sanctions. These sanctions could include, among other things, the FDA’s refusal to approve pending applications, withdrawal of an approval, imposition of a clinical hold, issuance of warning letters and other types of enforcement-related letters, product recalls, product seizures, changes to the conditions surrounding marketing approval such as labelling changes or changes to a REMS program, total or partial suspension of production or distribution, injunctions, civil money penalties, fines, refusals of government contracts, debarment, restitution, disgorgement of profits, or civil or criminal investigations and penalties. Any agency or judicial enforcement action could have a material adverse effect on us.

The process required by the FDA before a drug or biologic may be marketed in the United States is extensive and generally involves the following:

• completion of preclinical laboratory tests, animal studies and formulation studies according to Good Laboratory Practices and other applicable regulations;

• submission to the FDA of an investigational new drug (“IND”) application, which must become effective before human clinical trials may begin;

• performance of human clinical trials, including adequate and well-controlled trials, according to Good Clinical Practice to establish the safety and efficacy of the proposed drug for its intended use, or the safety, purity and potency of a biological product;

• approval by an independent IRB, representing each clinical site before each clinical trial may be initiated;

• submission to the FDA of an NDA or BLA;

• completion of registration batches and validation of the manufacturing process to show that the producer is capable of consistently producing quality batches of product;

• satisfactory completion of an FDA inspection of the manufacturing facility or facilities at which the product is produced to assess compliance with cGMP to assure that the facilities, methods and controls are adequate to preserve the drug’s identity, strength, quality and purity; and

• FDA review and approval of the NDA or BLA.

Once a pharmaceutical candidate is identified for development it enters the preclinical testing stage. Preclinical tests include laboratory evaluations of product chemistry, toxicity and formulation, as well as animal studies, to assess the safety and quality of the product. Animal studies must be performed in compliance with the FDA regulations and the U.S. Department of Agriculture’s Animal Welfare Act of 1966. Human clinical trials cannot commence until an IND application is submitted and becomes effective. An IND sponsor must submit the results of the preclinical tests, together with manufacturing information and analytical data, to the FDA as part of the IND. The sponsor also will include a protocol detailing, among other things, the objectives of the first phase of

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the clinical trial, the parameters to be used in monitoring safety and the effectiveness criteria to be evaluated, if the first phase lends itself to an efficacy evaluation. Preclinical testing may continue after the IND is submitted. The IND automatically becomes effective 30 days after receipt by the FDA, unless the FDA, within the 30 day time period, places the clinical trial on a clinical hold. In such a case, the IND sponsor and the FDA must resolve any outstanding concerns before the clinical trial can begin. Clinical holds also may be imposed by the FDA at any time before or during studies due to safety concerns or non-compliance, or other reasons.

All clinical trials must be conducted under the supervision of one or more qualified investigators. The conduct of clinical trials is subject to extensive regulation, including the FDA’s bioresearch monitoring regulations and GCP requirements, which establish standards for conducting, recording data from, and reporting the results of, clinical trials, and are intended to assure that the data and reported results are credible and accurate, and that the rights, safety, and well-being of study participants are protected. These regulations include the requirement that all research subjects provide informed consent. Further, an IRB must review and approve the plan for any clinical trial before it commences at any institution. An IRB considers, among other things, whether the risks to individuals participating in the trials are minimized and are reasonable in relation to anticipated benefits. The IRB also approves the information regarding the trial and the consent form that must be provided to each trial subject or his or her legal representative, and continues to provide oversight of the study until it is completed. In addition, companies sponsoring the clinical trials, investigators, and IRBs also must comply with regulations and guidelines for complying with the protocol and investigational plan, adequately monitoring the clinical trial, and timely reporting of adverse events. Foreign studies conducted under an IND must meet the same requirements that apply to studies being conducted in the United States. Data from a foreign study not conducted under an IND may be submitted in support of an NDA or BLA if the study was conducted in accordance with GCP and the FDA is able to validate the data through an onsite inspection, if necessary.

Each new clinical protocol must be submitted for FDA review, and to the IRBs for approval. Protocols detail, among other things, the objectives of the study, the primary and secondary endpoints of the study, dosing procedures, subject selection and exclusion criteria, and the parameters to be used to monitor subject safety.

Human clinical trials are typically conducted in three sequential phases that may overlap or be combined:

• Phase 1. The investigational drug is initially introduced into healthy human subjects, and tested for safety, dosage tolerance, absorption, metabolism, distribution and excretion. In the case of some products for severe or life-threatening diseases, especially when the product may be too inherently toxic to ethically administer to healthy volunteers, the initial human testing may be conducted in patients with the target diseases.

• Phase 2. This phase involves trials in a limited patient population to identify possible adverse effects and safety risks, to preliminarily evaluate the efficacy of the product for specific targeted diseases and to determine dosage tolerance and optimal dosage.

• Phase 3. This phase involves trials undertaken after preliminary evidence of effectiveness has been obtained and is intended to further evaluate dosage and clinical efficacy and safety of the drug, or the safety, purity, and potency of a biological product, in an expanded patient population, often at geographically dispersed clinical trial sites. These trials are intended to establish the overall risk/benefit ratio of the product, and to provide an adequate basis for product approval and product labelling.

Progress reports detailing developments associated with the clinical testing program must be submitted at least annually to the FDA, and safety reports must be submitted to the FDA and the investigators for serious and unexpected adverse events or animal test results that suggest a significant risk to human subjects. Phase 1, Phase 2 and Phase 3 testing may not be completed successfully within any specified period, if at all. The FDA or the sponsor may suspend or terminate a clinical trial at any time on various grounds, including a finding that the research subjects or patients are being exposed to an unacceptable health risk. Similarly, an IRB can suspend or terminate approval of a clinical trial at its institution if the clinical trial is not being conducted in accordance with the IRB’s requirements or if the drug has been associated with unexpected serious harm to patients. Further, success in either preclinical studies or early-stage clinical trials does not assure success in later-stage clinical trials. Frequently, product candidates that have shown promising results in early clinical trials have subsequently suffered significant setbacks in later clinical trials. In addition, the design of a clinical trial can determine whether its results will support approval of a product, and flaws in the design of a clinical trial may not become

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apparent until the clinical trial is well advanced. Sponsors of certain interventional clinical trials, except for Phase 1 trials, are required to submit certain clinical trial information for inclusion in the public clinical trial registry and results data bank maintained by the NIH, which are publicly available at http://clinicaltrials.gov.

Concurrent with clinical trials, companies usually complete additional studies in non-human models, and must also develop additional information about the chemistry and physical characteristics of the product, and finalize a process for manufacturing the product in commercial quantities in accordance with cGMP requirements.

The manufacturing process must be capable of consistently producing quality batches of the product candidate and, among other things, the manufacturer must develop methods for testing the identity, strength, quality and purity of the final product. In addition, appropriate packaging must be selected and tested, and stability studies must be conducted to demonstrate that the product candidate does not undergo unacceptable deterioration over its shelf life.

U.S. Review and Approval Processes

The results of product development, preclinical studies and clinical trials, along with descriptions of the manufacturing process, analytical tests conducted on the chemistry of the product, proposed labelling, and other relevant information are submitted to the FDA as part of an NDA or BLA requesting approval to market the product. The submission of an NDA or BLA generally is subject to the payment of a user fee, although NDAs or BLAs for designated Orphan Drugs are exempt from this fee.

In addition, under the U.S. Pediatric Research Equity Act of 2007, as amended, an application or supplement to an application for a drug with certain novel features (e.g., new active ingredient, new indication, new dosage form) must contain data to assess the safety and effectiveness of the drug or biologic for the claimed indications in all relevant pediatric subpopulations, and to support dosing and administration for each pediatric subpopulation for which the drug is safe and effective. The FDA may grant deferrals or full or partial waivers for submission of this data. Unless otherwise required by regulation, the act does not apply to any drug or biologic for an indication for which orphan designation has been granted.

The FDA conducts a preliminary review of all NDAs and BLAs submitted to ensure that they are sufficiently complete for substantive review before it accepts them for filing. The FDA may request additional information rather than accept an application for filing. Once the submission is accepted for filing, the FDA begins an in-depth substantive review. Under the Food and Drug Administration Amendments Act of 2007, the FDA is required to refer an NDA for a new chemical entity to an advisory committee prior to approval, or explain why such review is not necessary. The FDA is not bound by the recommendation of an advisory committee, but it frequently follows such recommendations. The approval or licensure process is lengthy and difficult, and the FDA may refuse to approve an NDA or BLA if the applicable regulatory criteria are not satisfied or may require additional clinical or other data and information. The FDA reviews an application to determine, among other things, whether a drug is safe and effective for its intended use, or whether a biologic is safe, pure, and potent, and whether its manufacturing is cGMP-compliant to assure and preserve the product’s identity, strength, quality and purity. Before approving an NDA or BLA, the FDA will typically inspect the facility or facilities where the product is manufactured. In addition, the FDA often will conduct a bioresearch monitoring inspection of the clinical trial sites involved in conducting pivotal studies to ensure data integrity and compliance with applicable GCP requirements.

Applications receive either standard review or priority review, which is reserved for a product that represents a major advance in treatment or a treatment where no adequate therapy exists. Under the U.S. Prescription Drug User Fee Act of 1992 (“PDUFA”), the FDA has ten months in which to complete its initial review of a standard new molecular entity NDA or original BLA and six months for a priority review new molecular entity NDA, BLA, or efficacy supplement. The FDA does not always meet its PDUFA goal dates and in certain circumstances the PDUFA goal date may be extended. In addition, products studied for their safety and effectiveness in treating serious or life-threatening illnesses and which provide meaningful therapeutic benefit over existing treatments, may receive accelerated approval. In that situation, the product may be approved on the basis of adequate and well-controlled clinical trials establishing that the product has an effect on a surrogate endpoint that is reasonably likely to predict clinical benefit or on the basis of an effect on a clinical endpoint other than survival or irreversible morbidity. As a condition of approval, a sponsor of a drug or biologic receiving accelerated approval must perform post-marketing studies to validate the surrogate endpoint or otherwise confirm the effect of the product on a clinical endpoint, and the product may be subject to accelerated withdrawal procedures.

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If a product receives marketing approval, the approval may be significantly limited to specific diseases, dosages or patient populations, or the indications for use may otherwise be limited, which could restrict the commercial value of the product. In addition, the FDA may impose distribution and use restrictions and other limitations on labelling and communication activities with respect to an approved product via a REMS program, which could include medication guides, patient package inserts, physician communication plans, restricted distribution methods, and patient registries.

The goal of the JUXTAPID REMS is to mitigate the risk of hepatotoxicity associated with the use of JUXTAPID by ensuring that:

(i)
prescribers are educated about the approved indication for JUXTAPID, the risk of hepatotoxicity associated with the use of JUXTAPID; and the need to monitor patients during treatment with JUXTAPID as per product labeling,

(ii)
JUXTAPID is dispensed only to patients with a clinical or laboratory diagnosis consistent with homozygous familial hypercholesterolemia (HoFH), and

(iii)
patients are informed about the risk of hepatotoxicity associated with the use of JUXTAPID and the need for baseline and periodic monitoring.

The JUXTAPID REMS includes the following Elements to Assure Safe Use: (i) certification of Healthcare Providers who prescribe JUXTAPID, (ii) certification of the pharmacies that dispense JUXTAPID, and (iii) JUXTAPID must be dispensed only to patients with evidence or other documentation of safe-use conditions. Further information can be found at http://www.juxtapidremsprogram.com.

The MYALEPT REMS elements include a Communication Plan with a Letter for Healthcare Providers (risk of severe infections), and the following Elements to Assure Safe Use: (i) certification of Healthcare Providers who prescribe MYALEPT, (ii) certification of the pharmacies that dispense MYALEPT, and (iii) MYALEPT must be dispensed only to patients with evidence or other documentation of safe-use conditions.

The goal of the MYALEPT REMS is to mitigate:

1.
the risks of serious adverse sequelae (such as severe infections, excessive weight gain, glucose intolerance, diabetes mellitus) due to the development of anti-metreleptin antibodies that neutralize endogenous leptin and/or MYALEPT, and

2.
the risk of lymphoma by:

• Educating prescribers about the development of neutralizing anti-metreleptin antibodies, the serious adverse sequelae that may result from these antibodies, and the risk for lymphoma associated with MYALEPT.

• Limiting the population exposed to MYALEPT by requiring prescriber certification, pharmacy certification, and prescriber attestation that each patient has a diagnosis consistent with the approved indication.

Further information can be found at http://myaleptrems.com.

The Hatch-Waxman Act, Marketing Exclusivity and Patent Term Restoration

The Hatch-Waxman Amendments established two abbreviated approval pathways for drug products that are in some way follow-on versions of already approved NDA products.

Generic Drugs: a generic drug is approved by means of an abbreviated NDA (“ANDA”) when its sponsor demonstrates that the proposed product is identical or bioequivalent to the approved, brand-name drug, referred to as the Reference Listed Drug (“RLD”). Generic drug applications are “abbreviated” because they are generally not required to include preclinical (animal) and clinical (human) data to establish safety and effectiveness. Instead, the applicant must show that the generic product performs in the same way as the RLD. Generally, an ANDA must contain data and information showing that the proposed generic product and RLD have the same active ingredients, in the same strength and dosage form, delivered via the same route of administration; are intended for the same uses, have the same labelling and, if applicable, are bioequivalent. An ANDA need not independently demonstrate the proposed product’s safety and effectiveness because the proposed product’s safety and effectiveness are inferred from the fact that the product is demonstrated to be the same as,

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and bioequivalent to, the RLD. These drugs are commonly referred to as “generic equivalents,” and, under state law, can typically be substituted by pharmacists under prescriptions written for the RLD.

505(b)(2) NDAs: if a product is similar to, but not the same as, an already approved product and thus is not eligible for submission of an ANDA, it may be submitted for approval via an NDA under section 505(b)(2) of the FDCA. Like an ANDA, a 505(b)(2) application is permitted to rely on the FDA’s finding that the RLD is safe and effective to the extent the products share similar features, but the sponsor must submit its own product-specific safety and effectiveness data to support any differences between the proposed and reference products.

RLD Patents: an NDA sponsor must identify to the FDA any patents that claim the drug substance, drug product or method of using the drug. These patents are among the information about the product that is listed in the FDA publication, Approved Drug Products with Therapeutic Equivalence Evaluations, also known as the “Orange Book.” The sponsor of an ANDA or 505(b)(2) application seeking to rely on an RLD must make one of several certifications regarding each listed patent, which in turn affect the timing of approval of the application.

Marketing Exclusivities in the United States

The Hatch-Waxman Act provides periods of regulatory exclusivity for products that would serve as RLDs. If a drug is a new chemical entity (“NCE”), generally meaning that the FDA has not previously approved any other drug containing the same active moiety (the molecule or ion responsible for the action of the drug substance) there is a period of up to five years from the product’s approval during which the FDA may not accept for filing any ANDA or 505(b)(2) application for a drug with the same active moiety. JUXTAPID’s NCE exclusivity expired on December 21, 2017, which means that an ANDA or 505(b)(2) NDA may be submitted for JUXTAPID.

A product that is not an NCE may qualify for three years of marketing exclusivity following approval when the application contains new clinical investigations, other than bioavailability studies, conducted or sponsored by the applicant and deemed by the FDA to be essential to the approval of the application. Three-year exclusivity is often available for changes to a previously approved drug product, such as new indications, strengths or dosage forms. Although this exclusivity period does not preclude filing or review of an ANDA or 505(b)(2) application, the FDA cannot grant final approval to the ANDA or 505(b)(2) application until three 3 years after approval of the RLD. In addition, this three-year exclusivity covers only the conditions of use associated with the new clinical investigations and, as a general matter, does not prohibit the FDA from approving ANDAs or 505(b)(2) NDAs for generic versions of the original, unmodified drug product.

Pediatric exclusivity is available in the United States under section 505A of the FDCA based on the voluntary completion of pediatric trials and submission of pediatric data in response to an FDA written request. If reports are submitted to and accepted by the FDA within statutory time limits, any periods of regulatory exclusivity or Orange Book-listed patent protections that cover the drug (other than a 30 month stay) are extended by six months. Pediatric exclusivity does not extend a patent term but instead effectively extends the regulatory period during which the FDA cannot approve an ANDA or 505(b)(2) application. Pediatric exclusivity can only extend any regulatory exclusivity or patent protection if the FDA makes its determination that the pediatric studies fairly respond to the written request prior to nine months before the expiration of the exclusivity or patent protection period.

Patent Term Restoration

The Hatch-Waxman Act established a patent restoration term of up to five years as compensation for patent term lost during product development and regulatory review. The maximum period of restoration is five years and cannot extend the remaining term of a patent beyond a total of 14 years from the product’s approval date. The patent term restoration period is generally one-half the time between the effective date of an IND and the submission date of an NDA plus the time between the submission date of an NDA and the approval of that application. Only one patent applicable to an approved drug is eligible for the extension, and the extension must be applied for prior to expiration of the patent and within 60 days of the NDA approval. The United States Patent and Trademark Office, in consultation with the FDA, reviews and approves the application for any patent term extension or restoration. A five-year patent term extension has been granted for the U.S. patent covering the composition of matter of lomitapide, extending the patent term to 2020 from the originally scheduled expiration of early 2015. With respect to metreleptin, the U.S. method-of-use patent directed to treating lipoatrophy has been elected for a 1,445-day patent term extension that will extend its expiration date to 2027.

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The Biologics Price Competition and Innovation Act

The BPCI Act, enacted in 2010 as part of the Patient Protection and Affordable Care Act (“PPACA”) authorizes the FDA to license a biological product that is highly similar (“biosimilar”) to, and possibly interchangeable with, a PHSA-licensed reference biological product through an abbreviated pathway. The objectives of the BPCI Act are conceptually similar to those of the Hatch-Waxman Act, which established abbreviated pathways for the approval of small molecule drug products. The BPCI Act establishes criteria for determining that a product is biosimilar to an already-licensed biologic (a reference product), and establishes a process by which an abbreviated BLA for a biosimilar product is submitted, reviewed and approved. The BPCI Act provides periods of exclusivity that protect a reference product from biosimilar competition. Under the BPCI Act, innovator manufacturers of original reference products are granted 12 years of exclusive use before biosimilar versions of such products can be licensed for marketing in the United States. This means that the FDA may not approve an application for a biosimilar version of a reference product until 12 years after the date of approval of the reference product, although a biosimilar application may be submitted 4 years after the date of licensure of the reference product. In addition, the BPCI Act establishes procedures that require the biosimilar applicant to provide information about its application and product to the reference product sponsor, allow information about potentially relevant patents to be shared and permit litigation over patents in advance of approval. The BPCI Act also provides a period of exclusivity for the first biosimilar to be determined by the FDA to be interchangeable with the reference product. The Directors believe that under the BPCI Act, metreleptin has 12 years of exclusivity in the United States until February 24, 2026.

The FDA has not yet issued proposed regulations setting forth its interpretation of the BPCI Act’s provisions but has issued guidance documents related to BPCI Act implementation concerning biosimilarity and interchangeability, BLA submission requirements, and exclusivity. The Directors anticipate that contours of the BPCI Act will be further defined through issuance of additional guidance documents by the FDA, proposed regulations, and decisions in the course of considering specific applications.

Like pediatric exclusivity for drug products approved under the FDCA, pediatric exclusivity for products approved under the BPCI Act is only available if the FDA determines the submitted pediatric study met the terms of its written request prior to 9 months before the expiration of either the 12-year reference product or the seven-year Orphan Drug exclusivity periods. The BPCI Act sets forth a complex mechanism for resolving patent disputes that involves a step-wise exchange of information prior to the initiation of a patent infringement lawsuit against a biosimilar or interchangeable product sponsor. Unlike the Hatch-Waxman Act, the BPCI Act provides no automatic stay on approval of a biosimilar or interchangeable product application based on patents.

Orphan Drug Designation

Under the U.S. Orphan Drug Act of 1983, the FDA may grant Orphan Drug Designation to a drug or biological product intended to treat a rare disease or condition, which is generally a disease or condition that affects fewer than 200,000 individuals in the United States, or for which there is no reasonable expectation that development and production costs will be recovered from sales of the drug for such disease or condition in the U.S. Orphan Drug Designation must be requested before submitting an NDA or BLA. After the FDA grants Orphan Drug Designation, the identity of the therapeutic agent and its potential orphan use are disclosed publicly by the FDA. If a product that has Orphan Drug Designation subsequently receives FDA approval and is the first drug approved for the disease for which it has such designation, the product is entitled to orphan product exclusivity, which means that the FDA may not approve any other applications to market the same drug for the same indication for seven years, except in limited circumstances such as a demonstration that the subsequent drug is clinically superior or the inability of the existing manufacturer to supply the market.

Expedited Development and Review Programs

The FDA’s Fast Track program facilitates the development and review of drugs that are intended to treat a serious or life-threatening disease or condition and demonstrate the potential to address unmet medical needs. Under the program, the sponsor of a new drug may request that the FDA designate the drug for a specific indication as a Fast Track product concurrent with or after the filing of the IND for the product candidate. A drug that receives Fast Track Designation may be eligible for more frequent meetings with the FDA to discuss the development; more frequent written correspondence from the FDA about the design of the proposed clinical trials; and rolling review of its NDA. Drugs with Fast Track Designation may also become eligible for Priority

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Review within a six month time frame from the time the complete NDA is accepted, as opposed to the ten month time frame for a standard review. The FDA grants priority review to products that demonstrate the potential to significantly improve safety or effectiveness of treatment, prevention, or diagnosis of a serious condition. We were granted Fast Track Designation by the FDA for AP101 in September 2019 following the opening of the IND in the United States. Fast Track Designation is expected to result in a priority-review process thereby shortening the review timeline for AP101, conducted by the FDA, upon completion of the Phase 3 clinical trial.

Rare Pediatric Disease Priority Review Voucher

Certain drugs may also be candidates for Rare Pediatric Disease designation by the FDA. In order to qualify for such designation, a sponsor must demonstrate that the proposed indication is for the treatment or prevention of a rare disease or condition that affects fewer than 200,000 individuals in the United States, or that affects more than 200,000 individuals in the United States and there is no reasonable expectation that the cost of developing and making available in the United States a drug for this type of disease or condition will be recovered from sales in the United States for that product, and is a serious or life-threatening disease in which the serious or life-threatening manifestations primarily affect individuals aged from birth to 18 years. Under the FDCA, a sponsor who receives approval of an NDA for a product for the prevention or treatment of a rare pediatric disease and meets certain additional criteria, may qualify for a Rare Pediatric Disease PRV. A PRV can be redeemed to receive priority review under an expedited timeframe for a subsequent marketing application for a different product. A PRV may also be sold or transferred from the initial sponsor to another sponsor and may be further transferred any number of times before it is used. Pursuant to the U.S. 21^st Century Cures Act of 2006, the FDA’s authority to award rare pediatric disease vouchers has been extended until September 30, 2020, and until September 30, 2022 for products that receive Rare Pediatric Disease designation by September 30, 2020. We were granted a Rare Pediatric Disease Designation in August 2018.

Post-Approval Requirements

Following FDA approval of a drug product, our activities, as well as those of our third-party manufacturers, remain subject to ongoing regulation and close monitoring by the FDA. The FDA may withdraw an approval, following notice and an opportunity for a hearing, if, among other things, compliance with certain regulatory standards is not maintained or if safety or efficacy problems occur after the product reaches the market. Later discovery of previously unknown problems with a product may result in restrictions on the product or even complete withdrawal of the product from the market. If new safety issues are identified following approval, the FDA may require the NDA sponsor to take certain measures, such as revising the approved labelling to reflect the new safety information, conducting post-market studies or clinical trials to assess the new safety information, and/or implementing or changing a REMS program to mitigate newly-identified risks. These are often referred to as Phase 4 or post-marketing studies, and may involve additional clinical trials, nonclinical testing and surveillance programs to monitor the safety of approved products which have been commercialized. After approval, most changes to the approved product, such as adding new indications, manufacturing changes and additional labelling claims, require supplemental applications for FDA review and approval. Manufacturers and other entities involved in the manufacture and distribution of approved drugs or licensed biologics are required to register their establishments with the FDA and certain state agencies, and are subject to periodic unannounced inspections by the FDA and certain state agencies for compliance with cGMP requirements and other laws. In its approvals of MYALEPT and JUXTAPID, the FDA required extensive post-marketing requirements and commitments. See “Clinical Development Post-Approval Obligations” of this section for our post-marketing commitments to applicable regulatory authorities.

Manufacturing, Packaging and Distribution

Companies engaged in manufacturing drug products or their components must comply with applicable cGMP requirements and product-specific regulations enforced by the FDA and other regulatory agencies. The FDA’s cGMPs are intended to ensure that pharmaceutical products are safe and that they consistently meet applicable requirements and specifications. Compliance with cGMP requires adherence to requirements relating to methods, facilities and controls used in the manufacturing, processing and packaging of a pharmaceutical product, including with respect to personnel, facilities, equipment, control of components and drug product containers and closures, production and process controls, packaging and labelling controls, holding and distribution, laboratory

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controls, and records and reporting requirements. If, after receiving approval, a company makes a material change in manufacturing equipment, location, or process (all of which are, to some degree, incorporated in the NDA or BLA) additional regulatory review and approval may be required.

The distribution of pharmaceutical products is subject to additional requirements and regulations, including extensive record-keeping, licensing, storage and security requirements intended to prevent the unauthorized sale of pharmaceutical products.

In addition, companies manufacturing or distributing drug products pursuant to FDA approvals are subject to record-keeping requirements; requirements on reporting of adverse experiences with the drug, and providing the FDA with updated safety and efficacy or safety, purity, and potency information for drugs and biologics, respectively; compliance within REMS program reporting obligations; drug and biologic sampling and distribution requirements; compliance with certain electronic records and signature requirements, and compliance with the FDA promotion and advertising requirements. As discussed more fully below, the FDA strictly regulates labelling, advertising, promotion and other types of information regarding marketed products that are placed on the market. Drugs and biologics may be promoted only for their approved indications and in accordance with the provisions of the approved labelling.

The FDA also conducts regular, periodic visits to inspect facilities following the initial approval of a product. Accordingly, manufacturers must continue to spend time, money and effort to maintain cGMP compliance. The Directors cannot be certain that our future third party manufacturers will consistently comply with cGMP and other applicable FDA regulatory requirements.

If an FDA investigator identifies conditions in a manufacturer’s facilities that do not comply with applicable regulatory requirements, the FDA investigator may issue observations through a Notice of Inspectional Observations, commonly referred to as a “Form FDA 483” report. If observations in the Form FDA 483 report are not addressed in a timely manner and to the FDA’s satisfaction, the FDA may issue a Warning Letter or proceed directly to other forms of enforcement action. The failure to provide adequate and timely corrective actions can result in further enforcement action, as described below.

Title II of the Drug Quality and Security Act (“DQSA”), known as the Drug Supply Chain Security Act, calls for the establishment of a nationwide electronic system that tracks certain prescription drugs at each point in the supply chain in order to prevent the introduction of counterfeit, adulterated, or mislabeled drugs into the market. Implementation began in 2015 and is scheduled to be completed by 2023. The FDA has issued regulations and guidance implementing the DQSA, which require manufacturers, distributors and dispensers to comply with various regulatory requirements related to product identification, product tracing, product verification, detection and response, notification and wholesaler licensing.

If there are problems with our products, such as adverse events of unanticipated severity or frequency, problems with the facility where the product is manufactured, or non-compliance with the FDCA or comparable laws, then there may be restrictions imposed on the product, the manufacturing facility or them, or we may be required to take other action such as recall or withdrawal of the product from the market or suspension of manufacturing. If our, our respective product candidates, or the manufacturing facilities for our respective product candidates fail to comply with applicable regulatory requirements, or undesirable side effects caused by such products are identified, a governmental authority may:

• issue safety alerts, “Dear Doctor” letters, press releases or other communications containing warnings about such product;

• mandate modifications to promotional materials or require us to provide corrective information to healthcare practitioners;

• require that we conduct post-marketing studies;

• require us to enter into a consent decree, which can include imposition of various fines, reimbursements for inspection costs, required due dates for specific actions and penalties for noncompliance;

• seek an injunction or impose civil or criminal penalties or monetary fines;

• suspend marketing of, withdraw regulatory approval of or recall such product;

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• suspend any ongoing clinical studies;

• refuse to approve pending applications or supplements to applications filed by us;

• suspend or impose restrictions on operations, including costly new manufacturing requirements; or

• seize or detain products, refuse to permit the import or export of products or require us to initiate a product recall.

The occurrence of any event or penalty described above may inhibit our ability to commercialize its products and generate revenue.

Promotional Activities and Interactions with Healthcare Providers and Patients

The FDA and other regulatory agencies strictly regulate promotional claims about prescription drug and biological products through, among other things, standards and regulations for direct-to-consumer advertising, communications regarding unapproved uses, industry-sponsored scientific and educational activities, and promotional activities involving the internet and social media. A product generally cannot be commercially promoted before it is approved. In general, after approval, a drug product may not be promoted for uses that are not approved by the FDA, the EC, and the regulatory authorities of the EU Member States or such other regulatory agencies as reflected in the product’s prescribing information. Moreover, the promotion of prescription-only medicinal products to non-healthcare professionals is prohibited in the European Union. In the United States, healthcare professionals are generally permitted to prescribe drugs and biologics for “off-label” uses (i.e., uses not described in the drug’s labelling) because the FDA does not regulate the practice of medicine. However, the FDA and the FTC regulate the advertising and promotion of prescription drug and biological products to ensure that claims made with respect to such products are consistent with regulatory approvals, are adequately substantiated by reasonable data, and are neither false nor misleading in any respect. With respect to off-label uses, FDA regulations impose stringent restrictions on manufacturers’ communications regarding off-label uses. A manufacturer may not promote a drug or biologic for off-label use, but under very specific conditions, it may be permissible for a manufacturer to engage in non-promotional, truthful, non-misleading communication regarding off-label information. If a company is found to have made product claims that are false, misleading, unsubstantial or that promote off-label uses, it may become subject to adverse publicity and enforcement action by the FDA, the DOJ, or the Office of the Inspector General of the United States Department of Health & Human Services, as well as state authorities. This could subject a company to a range of penalties that could have a significant commercial impact, including civil and criminal fines and agreements that materially restrict the manner in which a company promotes or distributes drug or biological products. The federal government has levied large civil and criminal fines against companies for alleged improper promotion, and has also requested that companies enter into consent decrees or permanent injunctions under which specified promotional conduct is changed or curtailed. For example, to resolve investigations conducted by the DOJ and SEC regarding Aegerion’s U.S. commercial activities and disclosures related to JUXTAPID, in September 2017 Aegerion was required, among other things, to pay approximately $40.1 million in aggregate penalties, which includes $7.2 million in restitution payable over three years beginning January 2018, a civil penalty of $4.1 million to be paid to the SEC pursuant to the SEC judgment, and $28.8 million to be paid pursuant to the settlement agreement with the DOJ. See Note 15 “Commitments and Contingencies” in Consolidated Statements of Aegerion for December 31, 2018 included in this prospectus.

Other Laws Regulating Prescription Drug and Biological Products

From time to time, legislation is drafted, introduced and passed in U.S. Congress that could significantly change the statutory provisions governing the testing, approval, manufacturing and marketing of products regulated by the FDA. In addition to new legislation, FDA regulations and guidance are often revised or interpreted by the agency in ways that may significantly affect our business and products. Changes in regulations, statutes or the interpretation of existing regulations could impact our business in the future by requiring, for example: (a) changes to its manufacturing arrangements; (b) additions or modifications to product labelling; (c) the recall or discontinuation of its products; or (d) additional record-keeping requirements.

Manufacturing, sales, promotion and other activities following product approval are also subject to regulation by numerous regulatory authorities in the United States in addition to the FDA, including the CMS, other divisions of the United States Department of Health & Human Services, the DOJ, the Drug Enforcement Administration

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(“DEA”), the Consumer Product Safety Commission, the FTC, the Occupational Safety & Health Administration, the Environmental Protection Agency and state and local governments.

Under the Controlled Substances Act, manufacturers and distributors of controlled substances must maintain registration with the DEA and comply with various regulatory requirements, including with respect to maintaining records and inventory, reporting to the DEA, and meeting certain security and operational safeguards.

U.S. Pricing and Reimbursement Environment

Significant uncertainty exists regarding the coverage and reimbursement status of products approved by the FDA and other government authorities. Sales of pharmaceutical products depend in significant part on the availability and adequacy of third party reimbursement. Third party payers include government health administrative authorities, managed care providers, private health insurers and other organizations. Third party payers are increasingly challenging the prices charged for, examining the medical necessity of, and assessing the cost-effectiveness of medicinal products and services. Coverage may also be more limited than the purposes for which the product is approved by the FDA or comparable foreign regulatory authorities. Further, one payer’s determination to provide coverage for a product does not assure that other payers will also provide coverage for the product.

Government and private third party payers have a variety of methodologies for paying for drugs and biologics. Payers are increasingly considering new metrics as the basis for reimbursement rates, such as average sales price, average manufacturer price or actual acquisition cost.

Private Payers

Coverage of drugs and biologics by private health insurance varies. Private payers may use a variety of utilization management techniques designed to control costs, including requiring pre-approval of coverage for drug therapies before reimbursing healthcare providers or patients that use such therapies. In addition, a payer’s decision to provide coverage for a drug product does not imply that an adequate reimbursement rate will be provided. Coverage may not be sufficient to maintain price levels high enough to realize an appropriate return on investment in product development.

Medicare

In the United States, the Medicare program provides health insurance for people who are 65 or older, as well as certain people with disabilities and other conditions irrespective of their age. The Medicare program is funded by the federal government and administered by the CMS. The U.S. Medicare Prescription Drug, Improvement, and Modernization Act of 2003, established the Medicare Part D program to provide a voluntary prescription drug benefit to Medicare beneficiaries. Medicare Part D is a voluntary prescription drug benefit through which beneficiaries may enroll in prescription drug plans offered by private entities under contract with CMS for coverage of certain outpatient prescription drugs. Medicare Part D generally provides coverage for medically necessary self-administered drugs. Coverage and reimbursement for outpatient drugs under Part D is not standardized. Part D prescription drug plan sponsors are not required to pay for all covered Part D drugs, and each drug plan can develop its own drug formulary that identifies which drugs the plan will cover and to what extent so long as the plan includes drugs from each therapeutic category and class of covered drugs, though not necessarily all the drugs in each category or class.

Although the availability of coverage under Medicare Part D may increase demand for MYALEPT and JUXTAPID, any negotiated prices for our products covered by a Part D prescription drug plan likely will be lower than the prices it might otherwise obtain. In order for MYALEPT and JUXTAPID to remain on or be included on the formularies of Part D prescription drug plans, we may have to offer discounts on the price of those products. In addition, manufacturers, are required to provide a 70% discount on brand name prescription drugs utilized by Medicare Part D beneficiaries when those beneficiaries reach the so-called “donut hole” in their drug benefits in a particular year (i.e., a coverage gap between initial coverage and catastrophic coverage). Any reduction in payment that results from the MMA may result in a similar reduction in payments from non-governmental payers because private payers use Medicare coverage policies and payment limitations in setting their own rates.

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The Directors believe that investigations and enforcement actions by certain government agencies have caused a reduction in contributions to these third party patient organizations, which may prevent these organizations from providing adequate financial assistance, including assistance with co-payment obligations, to individuals who would otherwise be unable to afford our products. As a result, Medicare Part D patients may not be able to afford their out-of-pocket co-payments for our products. In 2017, for example, the Directors believe that a considerable number of JUXTAPID patients who were covered by Medicare Part D ceased treatment with JUXTAPID, due to reductions in contributions to patient assistance programs operated by independent charitable 501(c)(3) organizations, which resulted in prior sources of financial support for these patients being less available.

We may develop products that, once approved, may be administered by a physician. Under currently applicable U.S. law, certain products not usually self-administered (including injectable drugs) may be eligible for coverage under Medicare through Medicare Part B. Medicare Part B is the part of Medicare that covers outpatient services and supplies, including certain pharmaceutical products that are medically necessary to treat a beneficiary’s health condition. As a condition of receiving Medicare Part B reimbursement for a manufacturer’s eligible drugs, the manufacturer is required to participate in other government healthcare programs, including the Medicaid Drug Rebate Program and the Public Health Service 340B Drug Pricing Program, discussed below.

Medicaid

Medicaid is a health insurance program with mandatory coverage for certain low-income children, families, pregnant women, and people with disabilities. States also have the option of expanding Medicaid coverage to low-income individuals generally and many states have done so. Medicaid is jointly funded by the federal and state governments, but administered by the states.

We participate in the Medicaid Drug Rebate Program. The Medicaid Drug Rebate Program requires pharmaceutical manufacturers to enter into and have in effect a national rebate agreement with the Secretary of the Department of Health and Human Services as a condition for states to receive federal matching funds for the manufacturer’s outpatient drugs furnished to Medicaid patients. Under the Medicaid Drug Rebate Program, we are required to, pay a rebate for each unit of our product reimbursed by a state Medicaid program as a condition of having federal funds made available to the states for our drugs under Medicaid and Medicare Part B. Those rebates are based on pricing data that we report on a monthly and quarterly basis to CMS, the federal agency that administers the Medicaid Drug Rebate Program. We may also participate in state Medicaid supplemental rebate programs which require payment of an incremental rebate to state Medicaid programs for covered utilization of our products. Price reductions as well as price increases that exceed the rate of inflation for our products may result in increasing the rebates we are required to pay under the Medicaid Drug Rebate Program or state Medicaid supplemental rebate programs and the discounts we will be required to offer under the Public Health Service 340B drug pricing discount program, known as the “340 B Program.” As a result of the substantial price increase for MYALEPT in February 2015, the Directors expect a significant gross-to-net adjustment for Medicaid rebates which will offset the majority of revenues from Medicaid and negatively impact net product sales in future quarters, since Medicaid rebates directly reduce our net product sales. The degree of such impact on our overall financial performance will depend on the percentage of MYALEPT patients that have Medicaid as their primary insurance coverage and the quantity of units ordered per patient. To date, approximately 35% of patients prescribed MYALEPT have been Medicaid beneficiaries. The number of patients prescribed MYALEPT in the future who are Medicaid beneficiaries could be higher than historical rates.

To maintain coverage of our products under the Medicaid Drug Rebate Program and Medicare Part B, it will be required to extend significant discounts to certain “covered entities” (defined by statute to include certain types of hospitals and other healthcare providers that receive federal grants) that purchase products under the 340B Program. The 340B Program requires participating manufacturers to agree to charge such covered entities no more than the 340B “ceiling price” for the manufacturers’ covered outpatient drugs. The 340B ceiling price is calculated using a statutory formula, which is based on the average manufacturer price and rebate amount for the covered outpatient drug as calculated under the Medicaid Drug Rebate Program. Orphan drugs (those designated under section 526 of the FDCA, such as MYALEPT and JUXTAPID) are exempt from the ceiling price requirements with respect to drugs purchased by certain covered entities (i.e., rural referral centers, sole community hospitals, critical access hospitals and free-standing cancer hospitals). The PPACA also obliges the

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Health Resources and Services Administration, the agency which administers the 340B Program, to promulgate various regulations and implement processes to improve the integrity of the 340B Program. The status of new and pending regulations and guidance is uncertain under the new presidential administration.

Recent regulations have granted Medicare Part B plans authority to apply new cost control measures to steer patients toward lower-priced drug products prior to covering non-preferred, more expensive products. This could potentially have the result of reducing coverage of our products under Medicare Part B. Additionally, President Trump’s administration has proposed to establish an international pricing index that would tie domestic prices for certain drugs and biologics to the prices in other countries with more aggressive drug price regulation. Such regulatory changes could have the effect of lowering the level of coverage or reimbursement for our products under Medicare Part B.

Drug products are subject to discounted pricing when purchased by federal agencies via the Federal Supply Schedule (“FSS”). FSS participation is required for a drug product to be covered and reimbursed by certain federal agencies and for coverage under Medicaid, Medicare Part B and the 340B Program. FSS pricing is negotiated periodically with the Department of Veterans Affairs. FSS pricing is intended not to exceed the price that a manufacturer charges its most-favored non-federal customer for its product. In addition, prices for drugs purchased by the Veterans Administration, Department of Defense (including drugs purchased by military personnel and dependents through the Tricare Retail Pharmacy program), Coast Guard and PHS are subject to a cap on pricing (known as the federal ceiling price) and may be subject to an additional discount if pricing increases more than the rate of inflation. We have had an FSS contract in place for its products since 2016. We also participate in the Tricare Retail Pharmacy program, under which we pay quarterly rebates on utilization of MYALEPT and JUXTAPID when the products are dispensed through the Tricare Retail Pharmacy network to Tricare beneficiaries.

The marketability of any products for which we will receive regulatory approval for commercial sale may suffer if the government and third party payers fail to provide adequate coverage and reimbursement. An increasing emphasis on cost containment measures in the United States has increased and the Directors expect will continue to increase the pressure on pharmaceutical pricing. Coverage policies and third party reimbursement rates may change at any time. Even if favorable coverage and reimbursement status is attained for one or more products for which we receive regulatory approval, less favorable coverage policies and reimbursement rates may be implemented in the future.

In recent years, additional laws have resulted in direct or indirect reimbursement reductions for certain Medicare providers, including:

• The U.S. Budget Control Act of 2011, among other things, created measures for spending reductions by Congress. A Joint Select Committee on Deficit Reduction, tasked with recommending a targeted deficit reduction of at least $1.2 trillion for the years 2013 through 2021, was unable to reach required goals, thereby triggering the legislation’s automatic reduction to several government programs. These changes included aggregate reductions to Medicare payments to providers of up to 2% per fiscal year, which went into effect in April 2013 and will remain in effect through 2025 unless additional Congressional action is taken.

• The U.S. American Taxpayer Relief Act of 2012, among other things, reduced Medicare payments to several providers, and increased the statute of limitations period for the government to recover overpayments to providers from three to five years.

• The U.S. Middle Class Tax Relief and Job Creation Act of 2012 required that the CMS reduce the Medicare clinical laboratory fee schedule by 2% in 2013, which served as a base for 2014 and subsequent years. In addition, effective January 1, 2014, CMS also began bundling the Medicare payments for certain laboratory tests ordered while a patient received services in a hospital outpatient setting.

These laws, and future state and federal healthcare reform measures may be adopted in the future, any of which may result in additional reductions in Medicare and other healthcare funding and otherwise affect the prices we may obtain for any product candidates for which we may obtain regulatory approval or the frequency with which any such product candidate is prescribed or used.

Further, in the United States, the cost of pharmaceuticals continues to generate substantial governmental and third party payer interest. There have been several recent U.S. congressional inquiries and proposed federal and

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proposed and enacted state legislation designed to, among other things, bring more transparency to drug pricing, review the relationship between pricing and manufacturer patient programs, reduce the costs of drugs under Medicare and reform government program reimbursement methodologies for drug products. Individual state legislatures have become increasingly aggressive in passing legislation and implementing regulations designed to control pharmaceutical and biological product pricing. Some of these measures include price or patient reimbursement constraints, discounts, restrictions on certain product access, marketing cost disclosure and transparency measures, and in some cases measures designed to encourage importation from other countries and bulk purchasing. In addition, regional health care authorities and individual hospitals are increasingly using bidding procedures to determine what pharmaceutical products and which suppliers will be included in their prescription drug and other health care programs. These measures could reduce the ultimate demand for our products, once approved, or put pressure on our product pricing.

Other Regulatory and Liability Matters

We may be subject to numerous environmental, health and safety laws and regulations, including those governing laboratory procedures and the handling, use, storage, treatment and disposal of hazardous materials and wastes. From time to time and in the future, our operations may involve the use of hazardous and flammable materials, including chemicals and biological materials, and may also produce hazardous waste products. Even if we contract with third parties for the disposal of these materials and waste products, we cannot completely eliminate the risk of contamination or injury resulting from these materials.

We will maintain insurance to cover us for costs and expenses we may incur due to injuries to its employees, but this insurance may not provide adequate coverage against potential liabilities.

U.S. Healthcare Reform

Our revenues and operations could be affected by changes in healthcare spending and policy in the United States. We operate in, a highly regulated industry and new laws, regulations or judicial decisions, or new interpretations of existing laws, regulations or decisions, related to health care availability, the method of delivery or payment for health care products and services could negatively impact our business, operations and financial condition. As noted above, the U.S. Congress, federal agencies and state legislatures from time to time propose and adopt initiatives aimed at cost containment, which could impact our ability to sell our products profitably. For example, the PPACA substantially changed the way healthcare is financed by both governmental and private insurers. The law contains a number of provisions that affect coverage and reimbursement of drug products and/or that could potentially reduce the demand for our products such as:

• increasing drug rebates under state Medicaid programs for brand name prescription drugs and extending those rebates to Medicaid managed care;

• requiring drug manufacturers to provide a 50% discount on Medicare Part D brand name prescription drugs sold to Medicare beneficiaries whose prescription drug costs cause the beneficiaries to be subject to the Medicare Part D coverage cap (i.e., the so-called donut hole); and

• expanding programs designed to test innovative payment models, service delivery models, or value-based arrangements.

Some of the provisions of the U.S. Affordable Care Act of 2010 have yet to be implemented, and there have been legal and political challenges to certain aspects of the Affordable Care Act. On January 20, 2017, President Donald J. Trump signed an Executive Order directing federal agencies with authorities and responsibilities under the PPACA to waive, defer, grant exemptions from, or delay the implementation of any provision of the PPACA that would impose a fiscal or regulatory burden on states, individuals, healthcare providers, health insurers, or manufacturers of pharmaceuticals or medical devices. On October 13, 2017, President Trump signed an Executive Order terminating the cost-sharing subsidies that reimburse insurers under the PPACA. Several state Attorneys General filed suit to stop the administration from terminating the subsidies, but their request for a restraining order was denied by a federal judge in California on October 25, 2017. Further, on January 22, 2018, President Trump signed a continuing resolution on appropriations for fiscal year 2018 that delayed the implementation of certain Affordable Care Act-mandated fees, including the so-called “Cadillac” tax on certain high cost employer-sponsored insurance plans, the annual fee imposed on certain health insurance providers based on market share, and the medical device excise tax on non-exempt medical devices. In July 2018, CMS

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briefly suspended further collections and payments to and from certain Affordable Care Act qualified health plans and health insurance issuers under the Affordable Care Act risk adjustment program pending the outcome of federal district court litigation regarding the method CMS uses to determine this risk adjustment. Congress is continuing to consider legislation that would alter other aspects of the Affordable Care Act.

The ultimate content, timing or effect of any healthcare reform legislation on the U.S. healthcare industry is unclear. On December 14, 2018, a United States District Court judge in Texas ruled that the Affordable Care Act is unconstitutional in its entirety because the “individual mandate” was repealed by Congress as part of the Tax Cuts and Jobs Act of 2017. On December 18, 2019, the United States Court of Appeals for the Fifth Circuit affirmed the district court’s ruling declaring the Affordable Care Act’s individual mandate unconstitutional and remanded for the district court to conduct analysis in the first instance on which provisions of the statute are severable from the individual mandate and thus remain intact. The pending litigation is likely to continue for the foreseeable future, with the district court’s ruling likely to be appealed again to the Fifth Circuit and, ultimately, to the Supreme Court. In the meantime, the litigation creates additional uncertainty around the Affordable Care Act’s ultimate fate.

The Directors anticipate that the Affordable Care Act, if substantially maintained in its current form, will continue to result in additional downward pressure on coverage and the price that we may receive for any approved product, and could seriously harm our business. Any reduction in reimbursement from Medicare and other government programs may result in a similar reduction in payments from private payers. The implementation of cost containment measures or other healthcare reforms may prevent us from being able to generate revenue, attain profitability, or commercialize our products.

Other legislative changes have been proposed and adopted since the Affordable Care Act was enacted. For example, in August 2011, President Barack H. Obama signed into law the Budget Control Act of 2011, which, among other things, created the Joint Select Committee on Deficit Reduction to recommend to Congress proposals in spending reductions. This committee did not achieve a targeted deficit reduction of at least $1.2 trillion for fiscal years 2012 through 2021, triggering the legislation’s automatic reduction to several government programs. This includes aggregate reductions to Medicare payments to providers of up to 2% per fiscal year, which went into effect beginning on April 1, 2013 and will stay in effect through 2027 unless additional Congressional action is taken. In January 2013, the American Taxpayer Relief Act of 2012 was signed into law, which, among other things, further reduced Medicare payments to several types of providers, including hospitals, and increased the statute of limitations period for the government to recover overpayments to providers from three to five years.

Other Laws Affecting Us in the United States

Healthcare providers, physicians and third party payers will play a primary role in the recommendation and prescription of any products for which we obtain marketing approval. Our future arrangements with third party payers, healthcare providers and physicians may expose us to broadly applicable fraud and abuse and other healthcare laws and regulations that may constrain the business or financial arrangements and relationships through which we market, sell and distribute any drugs for which we obtain marketing approval. In the United States, these laws include, without limitation, state and federal anti-kickback, false claims, physician transparency, and patient data privacy and security laws and regulations, including but not limited to those described below.

The U.S. Anti-Kickback Statute makes it illegal for any person, including a prescription drug manufacturer (or a party acting on its behalf), to knowingly and willfully solicit, receive, offer or pay any remuneration, directly or indirectly, in cash or in kind, that is intended to induce or reward referrals, including the purchase, recommendation, order or prescription of a particular drug, for which payment may be made under a federal healthcare program, such as Medicare or Medicaid. Violations of this law are punishable by up to five years in prison, criminal fines, administrative civil money penalties and exclusion from participation in federal healthcare programs. The term remuneration has been interpreted broadly to include anything of value.

There are a number of statutory exceptions and regulatory safe harbors protecting some common activities from prosecution. The exceptions and safe harbors are drawn narrowly and practices that involve remuneration that may be alleged to be intended to induce prescribing, purchasing or recommending may be subject to scrutiny if they do not qualify for an exception or safe harbor. Failure to meet all of the requirements of a particular applicable statutory exception or regulatory safe harbor does not make the conduct per se illegal under the

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Anti-Kickback Statute. Instead, the legality of the arrangement will be evaluated on a case-by-case basis based on a cumulative review of all of its facts and circumstances. Our practices may not in all cases meet all of the criteria for protection under a statutory exception or regulatory safe harbor. The statutory exceptions and regulatory safe harbors are also subject to change and some safe harbors, such as the safe harbor for certain rebate arrangements, are currently under review for amendments or reversal.

The intent standard under the federal Anti-Kickback Statute was amended by the Patient Protection and Affordable Care Act, as amended by the Health Care and Education Reconciliation Act of 2010, to a stricter standard such that a person or entity no longer needs to have actual knowledge of the statute or specific intent to violate it in order to have committed a violation. Several courts have also interpreted the Anti-Kickback Statute’s intent requirement to mean that if any one purpose of an arrangement involving remuneration is to induce referrals of federal healthcare covered business, the statute has been violated, even if there are additional, legitimate purposes for the remuneration. In addition, the Affordable Care Act also codified case law that a claim including items or services resulting from a violation of the federal Anti-Kickback Statute constitutes a false or fraudulent claim for purposes of the federal False Claims Act (“FCA”).

The Federal False Claims Act imposes civil penalties, including through civil whistle-blower or qui tam actions, against individuals or entities (including manufacturers) for, among other things, knowingly presenting, or causing to be presented false or fraudulent claims for payment by a federal healthcare program or making a false statement or record material to payment of a false claim or avoiding, decreasing or concealing an obligation to pay money to the federal government. The government may deem manufacturers to have “caused” the submission of false or fraudulent claims by, for example, providing inaccurate billing or coding information to customers or promoting a product off-label. Claims which include items or services resulting from a violation of the federal Anti-Kickback Statute are false or fraudulent claims for purposes of the False Claims Act. Our future marketing and activities relating to the reporting of wholesaler or estimated retail prices for our products, the reporting of prices used to calculate Medicaid rebate information and other information affecting federal, state and third party reimbursement for our products, and the sale and marketing of its products and any future product candidates, are subject to scrutiny under this law.

HIPAA imposes criminal and civil liability for knowingly and willfully executing a scheme, or attempting to execute a scheme, to defraud any healthcare benefit program, including private payers, or falsifying, concealing or covering up a material fact or making any materially false statements in connection with the delivery of or payment for healthcare benefits, items or services.

HIPAA, as amended by the Health Information Technology for Economic and Clinical Health Act of 2009 (“HITECH”), and their respective implementing regulations impose, among other things, specified requirements on covered entities and their business associates relating to the privacy and security of individually identifiable health information including mandatory contractual terms and required implementation of technical safeguards of such information. HITECH also created new tiers of civil monetary penalties, amended HIPAA to make civil and criminal penalties directly applicable to business associates, and gave state attorneys general new authority to file civil actions for damages or injunctions in federal courts to enforce the federal HIPAA laws and seek attorneys’ fees and costs associated with pursuing federal civil actions. Like the Anti-Kickback Statute, the Affordable Care Act amended the intent standard for certain healthcare fraud statutes under HIPAA such that a person or entity no longer needs to have actual knowledge of the statute or specific intent to violate it in order to have committed a violation.

The Physician Payments Sunshine Act, enacted as part of the PPACA, imposes new annual reporting requirements for certain manufacturers of drugs, devices, biologics and medical supplies for which payment is available under Medicare, Medicaid, or the Children’s Health Insurance Program, for certain payments and “transfers of value” provided to physicians and teaching hospitals, as well as ownership and investment interests held by physicians and their immediate family members. This information is made publicly available on a CMS website, and failure to report accurately could result in penalties.

Analogous state and foreign fraud and abuse laws and regulations, such as state anti-kickback and false claims laws, may be broader in scope and apply regardless of payer. Such laws are enforced by various state agencies and through private actions. Some state laws require pharmaceutical companies to comply with the pharmaceutical industry’s voluntary compliance guidelines and the relevant federal government compliance guidance, require drug manufacturers to report information related to payments and other transfers of value to

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physicians and other healthcare providers, and restrict marketing practices or require disclosure of marketing expenditures. State and foreign laws also govern the privacy and security of health information in some circumstances. Such data privacy and security laws may differ from each other in significant ways and often are not pre-empted by HIPAA, thus complicating compliance efforts.

In order to distribute products commercially companies must comply with state laws that require the registration of manufacturers and wholesale distributors of drug and biological products in a state, including, in certain states, manufacturers and distributors who ship products into the state even if such manufacturers or distributors have no place of business within the state. Some states also impose requirements on manufacturers and distributors to establish the pedigree of product in the chain of distribution, including some states that require manufacturers and others to adopt new technology capable of tracking and tracing product as it moves through the distribution chain. Several states have enacted legislation requiring pharmaceutical and biotechnology companies to establish marketing compliance programs, file periodic reports with the state, make periodic public disclosures on sales, marketing, pricing, clinical trials and other activities, and/or register their sales representatives, as well as to prohibit pharmacies and other healthcare entities from providing certain physician prescribing data to pharmaceutical and biotechnology companies for use in sales and marketing, and to prohibit certain other sales and marketing practices. All of our activities are potentially subject to federal and state consumer protection and unfair competition laws.

The scope and enforcement of each of these laws is uncertain and subject to rapid change in the current environment of healthcare reform, especially in light of the lack of applicable precedent and regulations. If our operations are found to be in violation of any of these laws or any other related governmental regulations that may apply to us, we may be subject to significant civil, criminal and administrative penalties, damages, fines, imprisonment, disgorgement, exclusion of drugs from government funded healthcare programs, such as Medicare and Medicaid, reputational harm, additional oversight and reporting obligations and the curtailment or restructuring of our operations. For example, in September 2017, Aegerion entered into a settlement agreement with the DOJ (as further described under “—Legal Proceedings” in this section) to resolve investigations conducted by the DOJ and the SEC regarding Aegerion’s U.S. commercial activities and disclosures related to JUXTAPID, and on January 30, 2018, a U.S. District Court judge sentenced Aegerion after the judge accepted Aegerion’s guilty criminal plea. Aegerion was required, among other things, to pay approximately $40.1 million in aggregate penalties over three years, and was put on probation for three years. Aegerion is also subject to a CIA, DPA related to HIPAA compliance, and a civil consent decree (which took effect in March 2019) with the FDA and the DOJ relating to the JUXTAPID REMS program.

EU Regulatory Matters

Following the United Kingdom’s formal departure from the European Union on January 31, 2020, the United Kingdom entered a transition period until December 31, 2020 (which can be extended by agreement), during which time EU pharmaceutical laws will remain applicable to the United Kingdom as if it remained an EU Member State. After the transition period, however, changes may be forthcoming depending on the terms of the United Kingdom and European Union’s future relationship that are negotiated (if any).

Drug and Biologic Development Process

The conduct of clinical trials is currently governed by the EU Clinical Trials Directive 2001/20/EC (“Clinical Trials Directive”) and will be replaced by the EU Clinical Trials Regulation (EU) No. 536/2014 (“Clinical Trials Regulation”) once it comes into effect. National laws and regulations, and the applicable Good Clinical Practice and Good Laboratory Practice standards must also be respected during the conduct of the trials, including the International Council for Harmonization of Technical Requirements for Pharmaceuticals for Human Use (ICH) guidelines on Good Clinical Practice (“GCP”). Under the current regime, before a clinical trial can be initiated, it must be approved in each EU Member State where there is a site at which the trial is to be conducted by two separate entities: the National Competent Authority (“NCA”) and one or more Ethics Committees. The NCA of the EU Member States in which the clinical trial will be conducted must authorize the conduct of the trial, and the independent Ethics Committee must grant a positive opinion in relation to the conduct of the clinical trial in the relevant EU Member State before the commencement of the trial. Any substantial changes to trial protocol or other information submitted with the clinical trial applications must be submitted to or approved by the relevant NCA and Ethics Committees. Under the current regime all suspected unexpected serious adverse reactions to the investigated drug that occur during the clinical trial must be reported to the NCA and to the Ethics Committees

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of the EU Member State where they occur. However, under the new Clinical Trials Regulation a more harmonized procedure will apply. Clinical trial authorization and other applications or notifications will be submitted through a centralized EU clinical trials portal, and a new application procedure for the authorization of clinical trials where one national regulatory authority (the reporting EU Member State proposed by the applicant) takes the lead in reviewing the application and the other regulatory authorities have only a limited involvement will be implemented. The coming into effect of the Clinical Trials Regulation has been postponed several times due to technical difficulties with the underlying IT systems that are still ongoing. Currently it is expected to come into force in or after December 2020.

During the development of a medicinal product, the EMA and national regulators within the European Union provide the opportunity for dialogue and guidance on the development program. At the EMA level, this is usually done in the form of scientific advice or protocol assistance in the case of an orphan designated development product, which is given by the Committee for Medicinal Products for Human Use (“CHMP”) on the recommendation of the Scientific Advice Working Party (SAWP). A fee is incurred with each scientific advice procedure but is significantly reduced for designated orphan medicines. Advice from the EMA is typically provided based on questions concerning, for example, quality (chemistry, manufacturing and controls testing), nonclinical testing and clinical studies, and pharmacovigilance plans and risk-management programs. Advice is not legally binding with regard to any future marketing authorization application of the product concerned.

The approval process for clinical trials in other countries outside the United States and the European Union varies from country to country, and the time may be longer or shorter than that required for FDA approval. In all cases, clinical trials are conducted in accordance with GCP and the applicable regulatory requirements and the ethical principles that have their origin in the Declaration of Helsinki.

Drug Marketing Authorization

In the European Union, drugs are subject to extensive pre- and post-market regulation by regulatory authorities at both the European Union and national levels. In the European Union, after completion of all required clinical testing, medicinal products may only be placed on the market after a related marketing authorization has been granted. Marketing authorizations can be obtained through, amongst others, a centralized or decentralized procedure. All of our products are currently regulated under the centralized procedure outlined below.

The centralized procedure provides for the grant of a single marketing authorization by the EC that is valid for all EU Member States and, after national implementing decisions, the three additional member states of the European Economic Area (Iceland, Liechtenstein and Norway). The centralized procedure is compulsory for certain medicinal products, including medicinal products derived from biotechnological processes, orphan medicinal products, advanced therapy medicinal products and products with a new active substance indicated for the treatment of AIDS, cancer, neurodegenerative disorders, diabetes, auto-immune and viral diseases. It is optional for medicinal products containing a new active substance not yet authorized in the European Economic Area before May 20, 2004, that constitute significant therapeutic, scientific or technical innovations, or for which the grant of a marketing authorization through the centralized procedure would be in the interest of public health at EU level. Under the centralized procedure, the timeframe for the evaluation of a marketing authorization application by the CHMP is, in principle, 210 days from receipt of a valid application for marketing authorization. However, this timeline excludes clock stops, when additional written or oral information is to be provided by the applicant in response to questions asked by the CHMP, so the overall process typically takes a year or more. Applications may be eligible for accelerated assessment if the CHMP decides the product is of major interest for public health and therapeutic innovation. The accelerated assessment timeline may also be subject to clock stops, or reverted back to the standard 210-day timeline by the CHMP in the event of any significant outstanding issues. On request, the CHMP can reduce the time frame to 150 days if the applicant provides sufficient justification for an accelerated assessment. The CHMP will provide a positive opinion regarding the application only if it meets certain quality, safety and efficacy requirements. However, the EC has final authority for granting the marketing authorization within 67 days after receipt of the CHMP opinion. Although the United Kingdom has left the European Union on January 31, 2020, the relevant EU laws on marketing authorization procedures and other pharmaceutical laws still apply until the end of the transition period, which is currently expected to last until the end of 2020 (but which can be extended by agreement of the parties). Further changes may be forthcoming in the scope of the centralized approval procedure as the terms of the future relationship are negotiated between the United Kingdom and the European Union.

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The decentralized marketing authorization procedure permits companies to file identical applications for marketing authorization to the competent authorities in several EU Member States simultaneously for a medicinal product that has not yet been authorized in any EU Member State. This procedure is available for medicinal products not falling within the mandatory scope of the centralized procedure. The competent authority of a single EU Member State, the reference member state, is appointed to review the application and provide an assessment report. The competent authorities of the other EU Member States, the concerned member states, are subsequently required to grant marketing authorization for their territories on the basis of this assessment. The only exception to this is where the competent authority of an EU Member State considers that there are concerns of potential serious risk to public health related to authorization of the product. In these circumstances the matter is submitted to the Heads of Medicines Agencies for review. Where a medicinal product has already been authorized for marketing in an EU Member State, this national authorization can be recognized in another EU Member State through the mutual recognition procedure. If a medicinal product falls under the optional scope of the centralized procedure, the applicant has the choice of using either the centralized or the national (decentralized/mutual recognition) procedure.

All new marketing authorization applications must include a risk management plan, describing the risk management system that the company will put in place and documenting measures to prevent or minimize the risks associated with the product. The regulatory authorities may also impose specific obligations as a condition of the marketing authorization. Risk management plans and periodic safety update repors (“PSURs”) are routinely available to third parties requesting access, subject to limited redactions.

Marketing authorizations have an initial duration of five years. After these five years, the authorization may be renewed on the basis of a re-evaluation of the risk-benefit balance. Once renewed, the marketing authorization is valid for an unlimited period unless the EC or the national competent authority decides, on justified grounds relating to pharmacovigilance, to proceed with one additional five-year renewal. Applications for renewal must be made to the EMA at least nine months before the five-year period expires.

Episalvan was granted a marketing authorization under the centralized procedure for the treatment of partial-thickness skin wounds in adults and is subject to additional monitoring (which aims to enhance reporting of suspected adverse drug reactions for drugs for which the clinical evidence base is less well developed). LOJUXTA and MYALEPTA were each granted a marketing authorization under exceptional circumstances by the EC under the centralized procedure, and are also subject to additional monitoring. So-called “marketing authorizations under exceptional circumstances” are intended for medicinal products where the applicant can demonstrate that it is not possible to provide comprehensive clinical data on efficacy and safety under normal conditions of use. For instance, because the indications for which the product in question is intended are encountered so rarely that the applicant cannot reasonably be expected to provide comprehensive evidence, or in the present state of scientific knowledge, comprehensive information cannot be provided, or it would be contrary to generally accepted principles of medical ethics to collect such information. Consequently, marketing authorizations under exceptional circumstances may be granted subject to certain specific obligations. In the case of LOJUXTA and MYALEPTA:

The LOJUXTA marketing authorization under exceptional circumstances includes a commitment to conduct a long term observational study to collect information on the safety and effectiveness outcomes of patients treated with LOJUXTA. The EMA has required that all patients taking LOJUXTA in the European Union be encouraged to participate in the study, and that the study period be open-ended. In the study, physicians will follow each patient to evaluate serum lipid control, hepatic effects, tumor occurrence, teratogenicity, gastrointestinal adverse reactions, events associated with coagulopathy, major adverse cardiovascular events and death. The EMA also required that a vascular imaging study be conducted to determine the impact of lomitapide on vascular endpoints. In addition an appropriate risk-management educational program to inform healthcare professionals and patients must be established prior to the product being launched in each country.

The MYALEPTA authorization under exceptional circumstances includes a commitment to establish a registry including all patients with GL or PL treated with MYALEPTA to further evaluate the long-term safety and effectiveness of MYALEPTA under normal conditions of clinical practice, along with a commitment to conduct an efficacy and safety study to further investigate the effect of MYALEPTA on poor metabolic control once background therapy is maximized in patients with familial or acquired PL. In order to address the potential safety concerns and/or lack of efficacy related to immunogenicity of MYALEPTA, the EMA also requires an integrated

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analysis of immunogenicity measured accordingly to validated assays, including samples from all available historical samples from previous studies with patients with GL or PL and samples obtained from patients that will be included in the efficacy and safety study in PL patients, the pediatric investigation plan (“PIP”) study and the patients registry.

The LOJUXTA and MYALEPTA exceptional circumstances marketing authorizations both require an annual reassessment of the risk/benefit balance for each of LOJUXTA and MYALEPTA by the CHMP. A negative assessment could potentially result in the relevant marketing authorization being suspended or revoked. The renewal of a marketing authorization of a medicinal product under exceptional circumstances, however, follows the same rules as a “normal” marketing authorization. Thus, a marketing authorization under exceptional circumstances is granted for an initial five years, after which the authorization will become valid indefinitely, unless the EMA decides that safety grounds merit one additional five-year renewal.

It is anticipated that the AP103 product candidate will be regulated as an advanced therapy medicinal product (“ATMP”) under the EU Regulation (EC) No. 1394/2007 on advanced therapy medicinal products, or the ATMP Regulation. ATMPs comprise gene therapy medicinal products, somatic cell therapy medicinal products and tissue engineered products, which are cells or tissues that have undergone substantial manipulation and that are administered to human beings in order to regenerate, repair or replace a human tissue. Pursuant to the ATMP Regulation, the Committee for Advanced Therapies (“CAT”) is responsible in conjunction with the CHMP for the evaluation of ATMPs. The CAT is primarily responsible for the scientific evaluation of ATMPs and prepares a draft opinion on the quality, safety and efficacy of each ATMP for which a marketing authorization application is submitted. The CAT’s opinion is then taken into account by the CHMP when giving its final recommendation regarding the authorization of a product in view of the balance of benefits and risks identified. Although the CAT’s draft opinion is submitted to the CHMP for final approval, the CHMP may depart from the draft opinion, if it provides detailed scientific justification. The CHMP and CAT are also responsible for providing guidelines on ATMPs and have published numerous guidelines, including specific guidelines on gene therapies and cell therapies. These guidelines provide additional guidance on the factors that the EMA will consider in relation to the development and evaluation of ATMPs and include, among other things, the pre-clinical studies required to characterize ATMPs; the manufacturing and control information that should be submitted in a marketing authorization application; and post-approval measures required to monitor patients and evaluate the long term efficacy and potential adverse reactions of ATMPs. Although such guidelines are not legally binding, compliance with them is often necessary to gain and maintain approval for product candidates.

New Chemical Entity Exclusivity

New chemical entities approved in the European Union, sometimes referred to as new active substances, qualify for eight years of data exclusivity from the date of notification of the marketing authorization and ten years of market protection. The data exclusivity, if granted, is the period of eight years during which an applicant cannot rely on the marketing authorization holder’s pharmacological, toxicological and clinical data in support of another marketing authorization for the purposes of submitting an application, obtaining marketing authorization or placing the product on the market, i.e., generics, hybrids, biosimilars cannot be validated by a regulatory agency. After this period has expired a generic or biosimilar marketing authorization application may be submitted, and the innovator’s data may be referenced in the application. However, even if the generic product or biosimilar products is authorized it cannot be marketed in the European Union during the ten year marketing protection period.

The overall ten-year marketing protection period will be extended to a maximum of 11 years if, during the first eight years of those 10 years, the marketing authorization holder obtains an authorization for one or more new therapeutic indications which, during the scientific evaluation prior to their authorization, are determined to bring a significant clinical benefit in comparison with currently approved therapies.

MYALEPTA is entitled to 10 years of market exclusivity by the European Union from its approval in July 2018. On July 31, 2013 lomitapide was granted a centralized marketing authorization from the European Commission. Lomitapide has eight years’ data exclusivity and 10 years’ marketing exclusivity in the European Union from July 31, 2013.

Orphan Designation and Exclusivity

The EMA grants Orphan Drug Designation to promote the development of products that diagnose, prevent or treat life-threatening or chronically debilitating conditions affecting not more than five in 10,000 people in the

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European Union. In addition, Orphan Drug Designation can be granted if, for economic reasons, the medicinal product would be unlikely to be developed without incentives and if there is no other satisfactory method approved in the European Union of diagnosing, preventing, or treating the condition, or if such a method exists, the proposed medicinal product is a significant benefit to patients affected by the condition. An application for Orphan Drug Designation (which is not a marketing authorization, as not all orphan-designated medicines reach the authorization application stage) must be submitted first before an application for marketing authorization of the medicinal product is submitted. The EMA’s Committee for Orphan Medicinal Products reassesses the Orphan Drug Designation of a product in parallel with the review for a marketing authorization; for a product to benefit from market exclusivity it must maintain its Orphan Drug Designation at the time of marketing authorization review by the EMA and approval by the European Commission. Additionally, any marketing authorization granted for an orphan medicinal product must only cover the therapeutic indication(s) that are covered by the Orphan Drug Designation. Upon the grant of a marketing authorization, Orphan Drug Designation provides up to ten years of market exclusivity in the orphan indication. During this ten-year period, with a limited number of exceptions, the regulatory authorities of the EU Member States and the EMA may not accept applications for marketing authorization, accept an application to extend an existing marketing authorization or grant marketing authorization for other similar medicinal products for the same therapeutic indication. The market exclusivity period may be extended by two additional years for an orphan-designated condition when the results of specific studies are reflected in the Summary of Product Characteristics (“SmPC”) addressing the pediatric population and completed in accordance with a fully compliant PIP. Conversely, the ten year market exclusivity period may be reduced to six years if at the end of the fifth year, it is established that a product no longer fulfils the criteria for an Orphan Drug Designation, i.e., the condition prevalence or financial returns criteria under Article 3 of Regulation (EC) No. 141/2000 on orphan medicinal products. When the period of orphan market exclusivity for an indication ends, the Orphan Drug Designation for that indication expires as well. Orphan exclusivity runs in parallel with normal rules on data exclusivity and market protection.

In 2012, metreleptin was granted four Orphan Drug Designations by the European Commission for the treatment of acquired and congenital GL (Lawrence syndrome and Berardinelli-Seip syndrome respectively), and acquired and familial PL (Barraquer-Simons syndrome). Metreleptin is entitled to ten years of market exclusivity by the European Union from its approval as MYALEPTA in July 2018. Despite the prevalence rate, lomitapide does not have Orphan Drug exclusivity in the European Union for the treatment of HoFH because the EMA views the relevant condition, for Orphan Drug purposes, to include both HoFH and the more prevalent HeFH.

In the European Union, certain patents relating to LOJUXTA may qualify for a supplemental protection certificate that would extend patent protection for up to five years after patent expiration upon marketing authorization in the European Union. Grant of such supplemental protection certificate is, however, subject to strict conditions and it is not automatic. Aegerion has applied for such protection in the countries in which LOJUXTA is approved, on a country-by-country basis, and in some countries, supplemental protection has been granted to extend patent protection to July or August of 2028, while in other countries, the applications are still pending.

Pediatric Development

In the European Union, companies developing a new medicinal product are obligated to study their product in children and must therefore agree on a PIP with the EMA’s Pediatric Committee (PDCO). These companies must conduct pediatric clinical trials in accordance with that PIP, unless a waiver applies, e.g. because the relevant disease or condition occurs only in adults. The marketing authorization application for the product must include the results of pediatric clinical trials conducted in accordance with the PIP, unless a waiver applies, or a deferral has been granted, in which case the pediatric clinical trials must be completed at a later date. Products that are granted a marketing authorization on the basis of the pediatric clinical trials conducted in accordance with the PIP are eligible for a six month extension of the protection under a supplementary protection certificate (if any is in effect at the time of approval) or, in the case of orphan medicinal products, a two year extension of the orphan market exclusivity. This pediatric reward is subject to specific conditions and is not automatically available when data in compliance with the PIP are developed and submitted.

Post-Approval Regulation

Similar to the United States, both marketing authorization holders and manufacturers of medicinal products are subject to comprehensive regulatory oversight by the EMA, the European Commission and/or the competent regulatory authorities of the EU Member States. This oversight applies both before and after grant of

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manufacturing licenses and marketing authorizations. It includes control of compliance with EU good manufacturing practices rules, manufacturing authorizations, pharmacovigilance rules and requirements governing advertising, promotion, sale, and distribution, recordkeeping, importing and exporting of medicinal products.

Failure by us or by any of our third-party partners, including suppliers, manufacturers and distributors to comply with EU laws and the EU Member State laws implementing Directive 2001/83/EC on medicinal products for human use and other core legislation relating to pharmaceutical products, and other EU Member State laws that apply to the conduct of clinical trials, manufacturing approval, marketing authorization of medicinal products and marketing of such products, both before and after grant of marketing authorization, manufacturing of medicinal products, statutory health insurance, bribery and anti-corruption or with other applicable regulatory requirements may result in administrative, civil or criminal penalties. These penalties could include delays or refusal to authorize the conduct of clinical trials or to grant marketing authorization, product withdrawals and recalls, product seizures, suspension, withdrawal or variation of the marketing authorization, total or partial suspension of production, distribution, manufacturing or clinical trials, operating restrictions, injunctions, suspension of licenses, fines and criminal penalties.

The holder of an EU marketing authorization for a medicinal product must also comply with EU pharmacovigilance legislation and its related regulations and guidelines, which entail many requirements for conducting pharmacovigilance, or the assessment and monitoring of the safety of medicinal products.

These pharmacovigilance rules can impose on holders of marketing authorizations the obligation to conduct a labor intensive collection of data regarding the risks and benefits of marketed medicinal products and to engage in ongoing assessments of those risks and benefits, including the possible requirement to conduct additional clinical studies or post-authorization safety studies to obtain further information on a medicine’s safety, or to measure the effectiveness of risk-management measures, which may be time consuming and expensive and could impact our profitability. Marketing authorization holders must establish and maintain a pharmacovigilance system and appoint an individual qualified person for pharmacovigilance who is responsible for oversight of that system. Key obligations include expedited reporting of suspected serious adverse reactions and submission of PSURs in relation to medicinal products for which they hold marketing authorizations The EMA reviews PSURs for medicinal products authorized through the centralized procedure. If the EMA has concerns that the risk-benefit profile of a product has varied, it can adopt an opinion advising that the existing marketing authorization for the product be suspended, withdrawn or varied. The Agency can advise that the marketing authorization holder be obliged to conduct post-authorization Phase 4 safety studies. The EMA opinion is submitted to the European Commission for its consideration. If the Commission agrees with the opinion, it can adopt a decision varying the existing marketing authorization. Failure by the marketing authorization holder to fulfill the obligations for which the European Commission’s decision provides can undermine the on-going validity of the marketing authorization.

More generally, non-compliance with pharmacovigilance obligations can lead to the variation, suspension or withdrawal of the marketing authorization for the product or imposition of financial penalties or other enforcement measures.

The manufacturing process for medicinal products in the European Union is highly regulated and regulators may shut down manufacturing facilities that they believe do not comply with regulations. Manufacturing requires a manufacturing authorization, and the manufacturing authorization holder must comply with various requirements set out in the applicable EU laws, regulations and guidance, including Directive 2001/83/EC, Directive 2003/94/EC, Regulation (EC) No 726/2004 and the European Commission Guidelines for Good Manufacturing Practice (“GMP”). These requirements include compliance with EU GMP standards when manufacturing medicinal products and active pharmaceutical ingredients, including the manufacture of active pharmaceutical ingredients outside of the European Union with the intention to import the active pharmaceutical ingredients into the European Union. Similarly, the distribution of medicinal products into and within the European Union is subject to compliance with the applicable EU laws, regulations and guidelines, including the requirement to hold appropriate authorizations for distribution granted by the competent authorities of the EU Member States. The manufacturer or importer must have a qualified person who is responsible for certifying that each batch of product has been manufactured in accordance with GMP, before releasing the product for commercial distribution in the European Union or for use in a clinical trial. Manufacturing facilities are subject to periodic inspections by the competent authorities for compliance with GMP.

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We and our third-party manufacturers are subject to GMP, which are extensive regulations governing manufacturing processes, stability testing, record keeping and quality standards as defined by the EMA, the European Commission, the competent authorities of EU Member States and other regulatory authorities. Companies may be subject to civil, criminal or administrative sanctions. These include suspension of manufacturing authorization in case of non-compliance with the European Union or EU Member States’ requirements governing the manufacturing of medicinal products.

Advertising and Promotion

The advertising and promotion of our products is also subject to EU laws concerning promotion of medicinal products, interactions with physicians, misleading and comparative advertising and unfair commercial practices. In addition, other national legislation of individual EU Member States may apply to the advertising and promotion of medicinal products and may differ from one country to another. These laws require that promotional materials and advertising in relation to medicinal products comply with the product’s SmPC as approved by the competent regulatory authorities. The SmPC is the document that provides information to physicians concerning the safe and effective use of the medicinal product. It forms an intrinsic and integral part of the marketing authorization granted for the medicinal product. Promotion of a medicinal product that does not comply with the SmPC is considered to constitute off-label promotion. The off-label promotion of medicinal products is prohibited in the European Union. The applicable laws at the EU level and in the individual EU Member States also prohibit the direct-to-consumer advertising of prescription-only medicinal products. Violations of the rules governing the promotion of medicinal products in the European Union could be penalized by administrative measures, fines and imprisonment. These laws may further limit or restrict the advertising and promotion of our products to the general public and may also impose limitations on its promotional activities with healthcare professionals.

Pricing and Reimbursement Environment

Even if a product obtains a marketing authorization in the European Union, there can be no assurance that reimbursement for such product will be secured on a timely basis or at all. The EU Member States are free to restrict the range of medicinal products for which their national health insurance systems provide reimbursement, and to control the prices and reimbursement levels of medicinal products for human use. An EU Member State may approve a specific price or level of reimbursement for the medicinal product, or alternatively adopt a system of direct or indirect controls on the profitability of the company responsible for placing the medicinal product on the market, including volume-based arrangements, caps and reference pricing mechanisms.

Reference pricing used by various EU Member States and parallel distribution, or arbitrage between low-priced and high-priced member states, can further reduce prices. In some countries, we may be required to conduct a clinical study or other studies that compare the cost-effectiveness of our product candidates, if any, to other available therapies in order to obtain or maintain reimbursement or pricing approval. There can be no assurance that any country that has price controls or reimbursement limitations for pharmaceutical products will allow favorable reimbursement and pricing arrangements for any of our products. Historically, products launched in the European Union do not follow price structures of the United States and generally published and actual prices tend to be significantly lower. This has largely proven to be the case for lomitapide and the Directors expect this to be the case for metreleptin. Publication of discounts by third party payers or authorities may lead to further pressure on the prices or reimbursement levels within the country of publication and other countries.

The HTA of medicinal products is becoming an increasingly common part of the pricing and reimbursement procedures in some EU Member States, including France, Germany, Ireland, Italy and Sweden. The HTA process, which governed by the national laws of these countries, is the procedure according to which the assessment of the public health impact, therapeutic impact, and the economic and societal impact of use of a given medicinal product in the national healthcare systems of the individual country is conducted. HTA generally focuses on the clinical efficacy and effectiveness, safety, cost, and cost-effectiveness of individual medicinal products as well as their potential implications for the healthcare system. Those elements of medicinal products are compared with other treatment options available on the market. The outcome of HTA regarding specific medicinal products will often influence the pricing and reimbursement status granted to medicinal products by the regulatory authorities of individual EU Member States. A negative HTA of one of our products by a leading and recognized HTA body could not only undermine our ability to obtain reimbursement for such product in the EU Member State in which such negative assessment was issued, but also in other EU Member States. For example, EU Member States that

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have not yet developed HTA mechanisms could rely to some extent on the HTA performed in other countries with a developed HTA framework, when adopting decisions concerning the pricing and reimbursement of a specific medicinal product.

On January 31, 2018, the European Commission adopted a proposal for a regulation on health technology assessment. This legislative proposal is intended to boost cooperation among EU Member States in assessing health technologies, including new medicinal products, and providing the basis for cooperation at the EU level for joint clinical assessments in these areas. The proposal provides that EU Member States will be able to use common HTA tools, methodologies and procedures across the European Union, working together in four main areas, including joint clinical assessment of the innovative health technologies with the most potential impact for patients, joint scientific consultations whereby developers can seek advice from HTA authorities, identification of emerging health technologies to identify promising technologies early, and continuing voluntary cooperation in other areas. Individual EU Member States will continue to be responsible for assessing non-clinical (e.g., economic, social, ethical) aspects of health technology, and making decisions on pricing and reimbursement. The European Commission has stated that the role of the draft HTA regulation is not to influence pricing and reimbursement decisions in the individual EU Member States, but there can be no assurance that the draft HTA regulation will not have effects on pricing and reimbursement decisions if and when the draft HTA regulation comes into force.

To obtain reimbursement or pricing approval in some countries, including the EU Member States, we may be required to conduct studies that compare the cost-effectiveness of our product candidates to other therapies that are considered the local standard of care. There can be no assurance that any country will allow favorable pricing, reimbursement and market access conditions for any of our products, or that we will be feasible to conduct additional cost-effectiveness studies, if required.

In certain of the EU Member States, products, such as MYALEPTA for GL and PL, that are designated as orphan medicinal products may be exempted or waived from having to provide certain clinical, cost-effectiveness and other economic data in connection with their filings for pricing/reimbursement approval. As noted above, LOJUXTA was not granted an Orphan Drug Designation by the EMA for the treatment of HoFH. As such, it is not eligible for benefits related to Orphan Drug Designation. As a result, we may not be able to provide all of the data required to obtain pricing/reimbursement approvals in certain EU Member States, which has and could, in the future, result in delays of pricing/reimbursement approvals for LOJUXTA, LOJUXTA not obtaining pricing/reimbursement approval at all, or LOJUXTA obtaining approvals at less than acceptable levels or with significant restrictions on use or reimbursement.

Many countries outside the United States and the European Union have pricing and reimbursement approvals and regimes that are comparable to those in the key EU markets.

European Data Laws

The collection and use of personal health data and other personal information in the European Union is governed by the provisions of the GDPR, which came into force in May 2018 and related implementing laws in individual EU Member States. In addition, following the United Kingdom’s formal departure from the European Union on January 31, 2020, the United Kingdom entered a transition period until December 31, 2020, during which time the GDPR will remain applicable to the United Kingdom, alongside the DPA 2018. At the end of the transition period, there is uncertainty regarding the future relationship between the United Kingdom and the European Union in relation to data protection. Once the United Kingdom leaves the European Union and the transition period has expired, the United Kingdom will become a “third country” for the purposes of data protection law. A “third country” is a country other than the EU Member States and the three additional European Economic Area countries (Norway, Iceland and Liechtenstein) that have adopted a national law implementing the GDPR. Under the GDPR, personal data can only be transferred to third countries in compliance with specific conditions for cross-border data transfers. Appropriate safeguards are required to enable transfers of personal data from the EU and EEA Member States. This status has a number of significant practical consequences, in particular for international data transfers, competent supervisory authorities and enforcement of the GDPR. The GDPR increased responsibility and liability in relation to personal data that we process.

We put in place additional mechanisms to ensure compliance with the GDPR and the DPA 2018 during 2018. The GDPR imposes a number of strict obligations and restrictions on the ability to process (processing includes collection, analysis and transfer of) personal data, including health data from clinical trials and adverse event

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reporting. The GDPR also includes requirements relating to the consent of the individuals to whom the personal data relates, the information provided to the individuals prior to processing their personal data or personal health data, notification of data processing obligations to the national data protection authorities and the security and confidentiality of the personal data. The GDPR also prohibits the transfer of personal data to countries outside of the European Union that are not considered by the European Commission to provide an adequate level of data protection, except if the data controller meets very specific requirements. These countries include the United States, and following the end of the transition period, it may include the United Kingdom if no adequacy decision is given prior to this. In the United Kingdom, the DPA 2018 supplements the GDPR, and in particular sets out specific requirements related to the processing of "special categories of personal data,'' including personal data related to health, genetic information and personal data related to criminal offenses or convictions.

Failure to comply with the requirements of the GDPR and the related national data protection laws of the EU Member States and the United Kingdom may result in significant monetary fines, other administrative penalties and a number of criminal offenses (punishable by uncapped fines) for organizations and in certain cases their directors and officers as well as civil liability claims from individuals whose personal data was processed. Data protection authorities from the different EU Member States and the United Kingdom may still implement certain variations, enforce the GDPR and national data protection laws differently, and introduce additional national regulations and guidelines, which adds to the complexity of processing personal data in the European Union and the United Kingdom. Guidance developed at both EU level and at the national level in individual EU Member States and the United Kingdom concerning implementation and compliance practices are often updated or otherwise revised.

There is, moreover, a growing trend towards required public disclosure of clinical trial data in the European Union which adds to the complexity of obligations relating to processing health data from clinical trials. Such public disclosure obligations are provided in the new EU Clinical Trials Regulation, EMA disclosure initiatives and voluntary commitments by industry. Failing to comply with these obligations could lead to government enforcement actions and significant penalties against us, harm to our reputation, and adversely impact our business and operating results. The uncertainty regarding the interplay between different regulatory frameworks, such as the Clinical Trials Regulation and the General Data Protection Regulation, further adds to the complexity that we face with regard to data protection regulation.

Promotional Activities

In the European Union, interactions between pharmaceutical companies and physicians are also governed by strict laws, regulations, industry self-regulation codes of conduct and physicians’ codes of professional conduct both at EU level and in the individual EU Member States. The provision of benefits or advantages to physicians to induce or encourage the prescription, recommendation, endorsement, purchase, supply, order or use of medicinal products is prohibited in the European Union. The provision of benefits or advantages to physicians is also governed by the national anti-bribery laws of the EU Member States. One example is the UK Bribery Act 2010. This Act applies to any company incorporated in or “carrying on business” in the United Kingdom, irrespective of where in the world the alleged bribery activity occurs. Violation of these laws could result in substantial fines and imprisonment.

Payments made to physicians in certain EU Member States must be publicly disclosed. Moreover, agreements with physicians must often be the subject of prior notification and approval by the physician’s employer, his/her regulatory professional organization, and/or the authorities of the individual EU Member States. These requirements are provided in the national laws, industry codes, or professional codes of conduct, applicable in the EU Member States. Failure to comply with these requirements could result in reputational risk, public reprimands, administrative penalties, fines or imprisonment.

Expanded Access Outside the United States and the European Union

In certain countries, drug products approved in the United States or the European Union can be accessed by patients before the drug has obtained marketing approval in such country. Various forms of this access include sale of product, often to the government, on a named patient basis and provision of the product free of charge on a named patient basis or a compassionate use basis. Each country has its own laws and regulations that apply to these forms of access. Aegerion has made lomitapide available in Brazil, South Africa and other countries that allow such access and plan to continue to consider access to additional countries. When Aegerion acquired

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metreleptin from AstraZeneca in January 2015, there were a number of patients receiving metreleptin therapy free of charge in certain countries outside the United States that allow use of a drug before marketing approval has been obtained in such country. Where permitted in accordance with applicable requirements, Aegerion has continued to make metreleptin available free of charge under such program, which has resulted in significant costs to Aegerion. In 2016, Aegerion began generating revenues from named patient sales of metreleptin in certain markets where named patient sales of metreleptin are possible and to the extent permitted by applicable law and local regulatory authorities. Where appropriate Aegerion has supported the transfer of patients from free of charge supply to locally approved pre-authorization funding programs.

Additional Laws and Regulations Governing International Operations

For other countries outside of the European Union and the United States, such as countries in Eastern Europe, Latin America or Asia, the requirements governing the conduct of clinical trials, product licensing, pricing and reimbursement vary from country to country. In addition, the clinical trials must be conducted in accordance with GCP requirements and the applicable regulatory requirements and the ethical principles that have their origin in the Declaration of Helsinki.

If we fail to comply with applicable foreign regulatory requirements, we may be subject to, among other things, fines, suspension or withdrawal of regulatory approvals, product recalls, seizure of products, operating restrictions and criminal prosecution.

The UK Bribery Act, the FCPA and other anti-corruption laws generally prohibit us and our employees and intermediaries from authorizing, promising, offering or providing, directly or indirectly, improper or prohibited payments, or anything else of value, to government officials or other persons to obtain or retain business or gain some other business advantage. Under the UK Bribery Act, we may also be liable for failing to prevent a person associated with us from committing a bribery offense. We and our commercial partners operate in a number of jurisdictions that pose a high risk of potential UK Bribery Act or FCPA violations, and we also participate in collaborations and relationships with third parties whose corrupt or illegal activities could potentially subject us to liability under the UK Bribery Act, FCPA or local anti-corruption laws, even if we did not explicitly authorize or have actual knowledge of such activities. The FCPA also requires us, as a public company, to make and keep books and records that accurately and fairly reflect all of our transactions and to devise and maintain an adequate system of internal accounting controls.

Compliance with the FCPA is expensive and difficult, particularly in countries in which corruption is a recognized problem. In addition, the FCPA presents particular challenges in the pharmaceutical industry, because, in many countries, hospitals are operated by the government, and doctors and other hospital employees are considered foreign officials. Certain payments to hospitals in connection with clinical trials and other work have been deemed to be improper payments to government officials and have led to FCPA enforcement actions against other companies.

Various laws, regulations and executive orders also restrict the use and dissemination outside of the United States, or the sharing with certain non-U.S. nationals, of information classified for national security purposes, as well as certain products and technical data relating to those products. If we are successful in expanding our presence outside of the United States, such expansion may require us to dedicate additional resources to comply with these laws, and these laws may preclude us from developing, manufacturing, or selling certain products and product candidates outside of the United States, which could limit our growth potential and increase our development costs.

We will be subject to compliance with the anti-bribery laws of other countries, including Brazil. Our activities outside the United States or those of its employees, licensees, distributors, manufacturers, clinical research organizations, or other third parties who act on its behalf or with whom we do business could subject us to investigation or prosecution under foreign or U.S. laws. For example, federal and São Paulo authorities in Brazil are each conducting an investigation to determine whether there have been any violations of Brazilian laws related to the sales of JUXTAPID in Brazil.

The failure to comply with laws governing international business practices may result in substantial civil and criminal penalties and suspension or debarment from government contracting. The SEC also may suspend or bar issuers from trading securities on U.S. exchanges for violations of the FCPA’s accounting provisions.

Our present and future business has been and will continue to be subject to various other laws and regulations. Various laws, regulations and recommendations relating to safe working conditions, laboratory practices, the experimental use

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of animals, and the purchase, storage, movement, import and export and use and disposal of hazardous or potentially hazardous substances, including radioactive compounds and infectious disease agents, used or that we may use in the future in connection with our development work are or may be applicable to our activities. Certain agreements we enter into involving exclusive license rights or acquisitions may be subject to national or supranational antitrust regulatory control, the effect of which cannot be predicted. The extent of government regulation, which might result from future legislation or administrative action, cannot accurately be predicted.

Employees

As of December 31, 2019, we had 169 employees, excluding temporary workers, 22% of whom are based in Dublin, Ireland, 31% in the United States, and 47% elsewhere globally. None of our employees has engaged in any labor strikes. We have no collective bargaining agreements with our employees, but we maintain company agreements (Betriebsvereinbarungen) with respect to certain topics at our Niefern site. Our German entity, Amryt GmbH, has a works counsel that works with the Amryt GmbH management team.

Property and Facilities

Our principal executive office is in Dublin, Ireland, where we lease office space under leases that expire in 2040. Our U.S. headquarters are in Boston, Massachusetts, where we lease office space under a lease that expires in 2027. We also have additional offices, research facilities and manufacturing facilities in the United States and in Germany. We believe these facilities are adequate for our current needs and that additional or replacement space will be available on commercially reasonable terms as needed.

Legal Proceedings

Other than as outlined below, we are not subject to any material legal proceedings.

Investigations in Brazil

Federal prosecutors in Brazil are conducting an investigation to determine whether there have been violations of Brazilian laws related to the sales of JUXTAPID in Brazil. In July 2016, the Ethics Council of Interfarma fined Aegerion’s subsidiary in Brazil (“Aegerion Brazil”) approximately $0.5 million for violations to its Code of Conduct, to which Aegerion Brazil is bound due to its affiliation with Interfarma. Also, the Board of Directors of Interfarma imposed an additional penalty of suspension of Aegerion Brazil’s membership, without suspension of Aegerion Brazil’s membership contribution, for a period of 180 days for Aegerion Brazil to demonstrate the implementation of effective measures to cease alleged irregular conduct, or exclusion of Aegerion Brazil’s membership in Interfarma if such measures are not implemented. Aegerion Brazil paid the fine of approximately $0.5 million during the third quarter of 2016. In March 2017, after the suspension period ended, Interfarma’s Board of Directors decided to reintegrate Aegerion Brazil, enabling it to participate regularly in Interfarma activities, subject to meeting certain obligations. In March 2019, the Board of Directors of Interfarma agreed that Aegerion Brazil had successfully met all of the requirements imposed by the association, and the investigation was closed.

In June 2017, the Federal Public Prosecutor of the City of São José dos Campos, State of São Paulo, in connection with its criminal investigation into former employees of Aegerion Brazil, requested that a Brazilian federal court provide federal investigators with access to the bank records of certain individuals and entities, including Aegerion Brazil, certain former Aegerion Brazil employees, a Brazilian patient association, and 462 Brazilian physicians. The Federal Trial Court Judge issued a decision on July 12, 2018 authorizing the access to the banking records on the terms that the Federal Public Prosecutor of the City of São José dos Campos had requested. On July 16, 2018, Aegerion Brazil filed a motion for clarification of the decision that authorized the breach of the banking secrecy, which was denied by the Federal Court Judge. The Public Prosecutor in São José dos Campos continues to gather information in connection with this investigation. At this time, the Company does not know whether the inquiry of the Public Prosecutor in São José dos Campos will result in the commencement of any formal proceeding against Aegerion, but if Aegerion’s activities in Brazil are found to violate any laws or governmental regulations, Aegerion may be subject to significant civil lawsuits to be filed by the Public Prosecution office, and administrative penalties imposed by Brazilian regulatory authorities and additional damages and fines. Under certain circumstances, Aegerion could be barred from further sales to federal and/or state governments in Brazil, including sales of JUXTAPID and/or MYALEPT, due to penalties

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imposed by Brazilian regulatory authorities or through civil actions initiated by federal or state public prosecutors. Additionally, the SEC conducted inquiries with Aegerion concerning the investigations by Brazilian authorities and, in July 2019, the SEC concluded the investigation and no enforcement action was made against Aegerion. The Company cannot determine if a loss is probable as a result of the investigations and inquiry in Brazil and whether the outcome will have a material adverse effect on the Company’s business.

Claim for Refund in Italy

The Agenzia Italiana del Farmaco (“AIFA”), also known as the Italian Medicines Agency, is the public institution responsible for the regulation of pharmaceuticals in Italy. AIFA has claimed that Amryt Pharma Italy S.r.l. refund approximately €4 million (of a total of €6.8 million inclusive of VAT) related to sales of Lojuxta in Italy during 2018. AIFA alleges that it classified Lojuxta incorrectly as an “Orphan” product and thus claims the repayment of funds paid to Amryt Pharma Italy. We adhere to the view that Lojuxta has all the characteristics to be considered an “Orphan” product, and we intend to defend against AIFA’s claim for a refund. We expect that the dispute will be reviewed by the Italian Courts in due course.

Our History

Our predecessor company was incorporated under the Companies Act 1985 and registered in England and Wales on December 20, 2004 under the name Elm Partners plc. We changed our name to Hamilton Partners plc on December 4, 2006, to Sterling Green Group plc on April 26, 2007, and to Fastnet Oil & Gas plc on June 8, 2012.

On April 18, 2016, we completed a reverse merger into AIM-quoted Fastnet Equity plc. Concurrently with the completion of this reverse merger, we also acquired Birken AG, which offered a range of derma-cosmetic products, including treatments for sensitive, allergy-prone and dry skin. We subsequently changed our name to Amryt Pharma plc.

We were incorporated under the Companies Act 2006 and registered in England and Wales on July 17, 2019 as a private company limited by shares under the name Amryt Pharma Holdings Limited. Amryt was re-registered as a public limited company, Amryt Pharma Holdings plc, on September 24, 2019.

On September 24, 2019, Amryt Pharma Holdings plc became the new parent company of Amryt Pharma plc pursuant to a scheme of arrangement between Amryt Pharma plc and its shareholders under Part 26 of the Companies Act 2006 (“Companies Act”), which is referred to in this prospectus as the “Scheme.” Amryt Pharma Holdings plc changed its name to Amryt Pharma plc.

Since September 25, 2019, our ordinary shares have traded on AIM under the symbol “AMYT” and on EGE under the symbol “AYP.” Our registered office is located at Dept 920a 196 High Road, Wood Green, London N22 8HH, United Kingdom, and our principal office is located at 90 Harcourt Street, Dublin 2, Ireland. We maintain a website at https://www.amrytpharma.com. The reference to our website is an inactive textual reference only and the information contained in, or that can be accessed through, our website is not a part of this prospectus.

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MANAGEMENT

Executive Officers and Directors

The following table presents information about our executive officers and directors:

Name	Age	Position
Executive Officers
Dr. Joseph A. Wiley	49	Chief Executive Officer
Rory P. Nealon	52	Chief Financial Officer and Chief Operating Officer
Non-Executive Directors
Raymond T. Stafford	73	Chairman of the Board
George P. Hampton, Jr.	50	Non-executive Director
Dr. Alain H. Munoz	71	Non-executive Director
Donald K. Stern	74	Non-executive Director
Dr. Patrick V.J.J. Vink	57	Non-executive Director
Stephen T. Wills	63	Non-executive Director

The business address for each of our executive officers and directors is c/o Amryt Pharma plc, 90 Harcourt Street, Dublin 2, Ireland.

The following are biographies of our executive officers and directors:

Dr. Joseph A. Wiley. Dr. Wiley founded Amryt and has served as Chief Executive Officer since 2015. He has over 20 years of experience in the pharmaceutical, medical and venture capital industries. Prior to Amryt, Dr. Wiley opened and led the European office of Sofinnova Ventures Inc. He was previously a medical director at Astellas Pharma Limited. Prior to joining Astellas, he held investment roles at Spirit Capital SA, Inventages Venture Capital Investment Inc. and Aberdeen Asset Managers Private Equity Limited. Dr. Wiley trained in general medicine at Trinity College Dublin, specializing in neurology. He holds a Masters of Business Administration from INSEAD and is also a Member of the Royal College of Physicians in Ireland.

Rory P. Nealon. Mr. Nealon founded Amryt and has served as Chief Financial Officer and Chief Operating Officer since 2015. He was previously a board member of Trinity Biotech Plc where he joined as Chief Financial Officer in January 2003 and subsequently was appointed as Chief Operations Officer in November 2007, a position he held until leaving Trinity Biotech Plc in 2014. Prior to joining Trinity Biotech Plc, Mr. Nealon served as Chief Financial Officer of Conduit Plc, an Irish directory services provider with operations in Ireland, the UK, Austria and Switzerland. Prior to joining Conduit Plc, Mr. Nealon was an associate director for AIB Capital Markets, a subsidiary of AIB Group plc, the Irish banking group. Mr. Nealon holds a Bachelor of Commerce degree from University College Dublin, and is a Fellow of the Institute of Chartered Accountants in Ireland, a member of the Institute of Taxation in Ireland, and a member of the Institute of Corporate Treasurers in the UK.

Raymond T. Stafford. Mr. Stafford has been a director of Amryt since 2016. He has worked in the pharmaceutical industry for more than 30 years. He has served as Chairman, Chief Executive Officer and majority shareholder of the Tosara Group which owned, manufactured and marketed the successful international brand Sudocrem, and was ultimately integrated into the U.S.-based, NYSE-listed company Forest Laboratories, Inc. in 1988. Mr. Stafford held numerous senior positions within such corporations, including Chief Executive Officer of Forest UK and Ireland as well as Chief Executive Officer of Forest Laboratories Europe since 1999. Mr. Stafford retired in 2014 following the sale of Forest Laboratories, Inc. to Actavis Plc (now Allergan plc) in a $28 billion transaction where Mr. Stafford was Executive Vice President of Global Marketing. Separately, Mr. Stafford also founded one of Ireland’s current leading multi-channel sales, marketing and distribution service providers approved by the Irish Medicines Board (now, The Health Products Regulatory Authority) to service the wholesale and retail trade.

George P. Hampton, Jr. Mr. Hampton has been a director of Amryt since 2019. He joined Currax Pharmaceuticals in April of 2019 as Chief Executive Officer and serves on its board of directors. Prior to joining Currax, Mr. Hampton served as executive vice president, primary care business unit for Horizon Pharmaceuticals (HZNP), a public biopharmaceutical company. In this role he was tasked with leading the company’s forward-looking strategy, as well as establishing operational goals for the business. Previously, Mr. Hampton

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served as executive vice president, global orphan business unit and international operations for Horizon Pharmaceuticals. He has more than 25 years of experience as a successful executive in the pharmaceutical and biotechnology field on both a national and international scale including specific expertise in rare disease (ACTIMMUNE, RAVICTI, PROCYSBI), autoimmune (HUMIRA), primary care, orthopedic (CELEBREX), diabetes (BYETTA), anti-infectives and cardiovascular spaces. This includes roles of increasing responsibility in sales, marketing and operations at G.D. Searle, Abbott (now AbbVie), Amylin and Horizon Pharmaceuticals. Mr. Hampton earned his Bachelor of Science from Miami University in Oxford, Ohio. He serves on the board of IMAC (Nasdaq: IMAC) regeneration medical centers.

Dr. Alain H. Munoz. Dr. Munoz has been a director of Amryt since 2019. He is an entrepreneur and independentmanagement consultant in the pharmaceutical and biotechnology industry and has over 30 years of experience inthe industry at the executive level. Dr. Munoz worked with the Fournier Group as Research and Developmentdirector and thereafter as Senior Vice President of the Pharmaceutical Division. Prior to serving at Fournier, heserved at Sanofi Group, first as director in the cardiovascular and anti-thrombotic products department, andthereafter as Vice President of international development. Dr. Munoz is qualified in cardiology andanesthesiology from the University Hospital of Montpellier, France where he was head of the clinical cardiologydepartment. He has been a member of the Scientific Committee of the French drug agency, is advisor to KurmaPartners, and serves on the scientific advisory board of Valneva SA. In addition, he is an independent boardmember of Oxthera AB, Auris Medical Holding AG (Nasdaq: EARS) and Zealand Pharma A/S (Nasdaq: ZEAL).Mr. Munoz received an undergraduate degree from International Institute for Management Development, adoctorate from the University of Montpellier and a graduate degree from Centre Hospitalier UniversitairePitie-Salpetriere.

Donald K. Stern. Mr. Stern has been a director of Amryt since 2019. He was previously a director of Novelion,Aegerion’s former parent company, and was a member of Aegerion’s board of directors from September 2015 toOctober 2016. Mr. Stern serves as Managing Director of Corporate Monitoring & Consulting Services atAffiliated Monitors, Inc., a consulting firm providing independent integrity monitoring services and complianceservices across a wide range of regulated industries and professions. He is also Of Counsel to the Boston lawfirmof Yurko, Salvesen & Remz. He has had a diverse and distinguished legal career, evenly split between privatepractice and public service. Prior to joining Affiliated Monitors, Inc., Mr. Stern was a partner at three major lawfirms: Cooley LLP, Bingham McCutchen LLP and Hale & Dorr LLP (now Wilmer Cutler Pickering Hale andDorr LLP). Mr. Stern also served as the United States Attorney for the District of Massachusetts, the Chief LegalCounsel to Governor Michael S. Dukakis and the Chief of the Government Bureau in the Massachusetts AttorneyGeneral’s office. Mr. Stern holds a Masters in Laws from University of Pennsylvania Law School, a Juris Doctordegree from Georgetown University Law Center and a Bachelor of Arts from Hobart College.

Dr. Patrick V.J.J. Vink. Dr. Vink has been a director of Amryt since 2019. He has significant experience as a senior executive, having worked in the pharmaceutical industry for more than 30 years. Dr. Vink serves as Chairman at Acacia Pharma Group plc and Targovax ASA, both publicly listed biopharma companies based in the United Kingdom and Norway. Dr. Vink also serves as Chairman of venture capital-backed NMD Pharma, a neurology biopharmaceutical company in Denmark and F2G Ltd, a rare fungal disease UK and Austria based company. In addition, Dr. Vink is a board member at Santhera AG and Spero Therapeutics, Inc. and in 2019 began working with Athyrium as a Senior Advisor. While serving in these capacities, Dr. Vink has been involved in initial public listings and geographic expansions and has contributed to the achievement of significant development and commercial milestones. Earlier in his career he held several leadership positions across the industry, including Head of Global Biopharmaceuticals for the Sandoz division of the Novartis Group, Vice President International Business for Biogen Inc., and Head of Worldwide Marketing, Cardiovascular and Thrombosis at Sanofi-Synthelabo Ltd. Dr. Vink also served as a member of the Executive Committee of the European Federation of Pharmaceutical Industries and Associations from 2013 to 2015. Dr. Vink graduated as a medical doctor from the University of Leiden, Netherlands in 1988 and obtained his Masters of Business Administration in 1992 at the University of Rochester.

Stephen T. Wills. Mr. Wills became a director of Amryt in 2019. He currently serves as the Chief Financial Officer (since 1997), and Chief Operating Officer (since 2011) of Palatin Technologies, Inc. (NYSE: PTN), a biopharmaceutical company developing targeted, receptor-specific peptide therapeutics for the treatment of diseases with significant unmet medical need and commercial potential. Mr. Wills serves on the boards of

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directors of MediWound Ltd. (Nasdaq: MDWD), a biopharmaceutical company focused on treatment in the fields of severe burns, chronic and other hard to heal wounds, since April 2017, and as Chairman since January 2018, and of Gamida Cell Ltd. (Nasdaq: GMDA), a leading cellular and immune therapeutics company, since March 2019 (audit and finance committee member). Mr. Wills also has served on the board of trustees and executive committee of The Hun School of Princeton, a college preparatory day and boarding school, since 2013, and its Chairman since June 2018. Mr. Wills served on the board of directors of Caliper Corporation, a psychological assessment and talent development company, since March 2016, and as Chairman from December 2016 to December 2019, when Caliper was acquired by PSI. Mr. Wills served as Executive Chairman and Interim Principal Executive Officer of Derma Sciences, Inc., a provider of advanced wound care products, from December 2015 to February 2017, when Derma Sciences was acquired by Integra Lifesciences (Nasdaq: IART). Previously, Mr. Wills served on the board of directors of Derma Sciences as the lead director and chairman of the audit committee from June 2000 to December 2015. Mr. Wills served as the Chief Financial Officer of Derma Sciences from 1997 to 2000. Mr. Wills served as the President and Chief Operating Officer of Wills, Owens & Baker, P.C., a public accounting firm, from 1991 to 2000. Mr. Wills, a certified public accountant, earned his Bachelor of Science in accounting from West Chester University, and a Master of Science in taxation from Temple University.

Family Relationships

There are no family relationships among any of the directors or senior management.

Composition of Board of Directors

Our Board consists of seven members. The Board has determined that six of its seven directors, Raymond Stafford, Stephen Wills, Donald Stern, George Hampton, Patrick Vink and Alain Munoz, do not have a relationship that would interfere with the exercise of independent judgment in carrying out the responsibilities of director and that each of these directors is “independent” as that term is defined under the rules of the Nasdaq. As a foreign private issuer, we are not required to meet the Nasdaq rule that our Board be comprised of a majority of independent directors. However, we voluntarily comply and intend to continue to comply with this requirement.

In accordance with our Articles of Association, unless otherwise determined by special resolution of our shareholders, the directors shall not be fewer than two or more than seven in number. Each of the directors is subject to compulsory retirement and at each annual general meeting which occurs after September 25, 2021, one-third of the current directors (or, if their number is not a multiple of three, the number nearest to but not greater than one-third, subject to a minimum of one) shall retire from office by rotation. See “Description of Share Capital and Certain Corporate Matters.”

A registration rights agreement to which we are a party contains provisions regarding the composition of our Board. See “Related Party Transactions—Agreements with Principal Shareholders—Registration Rights Agreement” for further information.

Role of the Board in Risk Oversight

Our Board is primarily responsible for the oversight of our risk management activities and has delegated to the audit committee the responsibility to assist our Board in this task. While our Board oversees our risk management, our management is responsible for day-to-day risk management processes. We believe this division of responsibilities is the most effective approach for addressing the risks we face. Our Board expects our management to consider risk and risk management in each business decision, to proactively develop and monitor risk management strategies and processes for day-to-day activities and to effectively implement risk management strategies adopted by the Board.

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Corporate Governance Practices

As a “foreign private issuer,” as defined by the SEC, although we are permitted to follow certain governance practices of the United Kingdom instead of those otherwise required under the Nasdaq rules for domestic issuers, we intend to follow the Nasdaq corporate governance rules applicable to foreign private issuers, though we may in the future decide to use the foreign private issuer exemption with respect to some or all of the other Nasdaq corporate governance rules. While we voluntarily follow most Nasdaq corporate governance rules, we intend to take advantage of the following limited exemptions:

• Exemption from filing quarterly reports on Form 10-Q and current reports on Form 8-K disclosing significant events within four days of their occurrence.

• Exemption from Section 16 rules regarding sales of ordinary shares by insiders, which will provide less data in this regard than to shareholders of U.S. companies that are subject to the Exchange Act.

• Exemption from the Nasdaq rules applicable to domestic issuers requiring disclosure within four business days of any determination to grant a waiver of the code of business conduct and ethics to directors and officers. Although we will require approval from our Board of any such waiver, we may choose not to disclose the waiver in the manner set forth in the Nasdaq rules, as permitted by the foreign private issuer exemption.

• Exemption from the requirements that director nominees are selected, or recommended for selection by our Board, either by (1) independent directors constituting a majority of our Board’s independent directors in a vote in which only independent directors participate, or (2) a committee comprised solely of independent directors, and that a formal written charter or board resolution, as applicable, addressing the nominations process is adopted.

Furthermore, Nasdaq Rule 5615(a)(3) provides that a foreign private issuer, like our company, may rely on home country corporate governance practices in lieu of certain of the rules in the Nasdaq Rule 5600 Series and Rule 5250(d), provided that we nevertheless comply with the Nasdaq’s Notification of Noncompliance requirement (Rule 5625), the Voting Rights requirement (Rule 5640) and that we have an audit committee that satisfies Rule 5605(c)(3), consisting of committee members who meet the independence requirements of Rule 5605(c)(2)(A)(ii). Although we generally intend to follow Nasdaq corporate governance rules, we do not intend to follow Nasdaq Rule 5620(c) regarding quorum requirements applicable to meetings of shareholders. Such quorum requirements are not required under English law. In accordance with generally accepted business practice, our Articles of Association provide alternative quorum requirements that are generally applicable to meetings of shareholders.

We intend to take all actions necessary to maintain compliance as a foreign private issuer under the applicable corporate governance requirements of the Sarbanes-Oxley Act, the rules adopted by the SEC and the Nasdaq corporate governance rules and listing standards.

Committees of Board of Directors

The Board has three standing committees: an Audit Committee, a Remuneration Committee and a Compliance Committee.

Audit Committee

The Audit Committee has responsibility for, among other things, the monitoring of the financial integrity of our financial statements and the involvement of our auditors in that process. The Committee focuses in particular on compliance with accounting policies and ensuring that an effective system of internal and external audit and financial control is maintained, including oversight of the annual audit and the extent of the non-audit work undertaken by our external auditors, as well as advising on the appointment of external auditors. The Audit Committee comprises three members, who are independent non-executive directors who are financially sophisticated and experienced: Stephen Wills, Raymond Stafford and Donald Stern. The Committee is chaired by Stephen Wills. Our Board has determined that Mr. Wills qualifies as an “audit committee financial expert” as defined by applicable SEC rules and has the requisite financial sophistication as defined under applicable Nasdaq rules and regulations. The Board has determined that all of the members of the Audit Committee satisfy the “independence” requirements set forth in Rule 10A-3 under the Exchange Act. The Audit Committee is governed by terms of reference that comply with the Nasdaq rules for a charter of such body.

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Among the responsibilities of the Audit committee are:

• monitoring the integrity of our financial statements, including our annual and half-yearly reports, interim management statements, preliminary results announcements and any other formal announcement relating to our financial performance;

• advising on and recommending the appointment of the independent auditors;

• evaluating our independent auditors’ qualifications, performance and independence, and presenting its conclusions to the full Board on at least an annual basis;

• reviewing and challenging where necessary our accounting policies, methods and applications of accounting standards;

• ensuring that we maintain an effective system of internal and external audit and financial control;

• risk management; and

• reviewing and considering the scope of the annual audit and the extent of the non-audit work undertaken by our independent auditors.

The Audit Committee will meet as often as one or more members of the Audit Committee deem necessary, but in any event will meet at least two times a year at the appropriate times in the financial reporting and audit cycle.

Remuneration Committee

The Remuneration Committee has responsibility for determining senior management compensation. The Remuneration Committee comprises three members, who are independent non-executive directors: George Hampton, Alain Munoz and Patrick Vink. The Committee is chaired by George Hampton. Under the SEC and Nasdaq rules, there are heightened independence standards for members of the Remuneration Committee, including a prohibition against the receipt of any compensation from us other than as a standard board member. Although foreign private issuers are not required to meet this heightened standard, we voluntarily comply and intend to continue to comply with this heightened standard. The Remuneration Committee is governed by terms of reference that comply with the Nasdaq rules for a charter of such body.

The responsibilities of the Remuneration Committee include:

• proposing and recommending specific remuneration packages for each of the executive directors, including pension rights and any compensation payments;

• recommending and monitoring the level and structure of remuneration for senior management, and the implementation of share option, or other performance-related schemes;

• determining and monitoring policy on and setting levels of remuneration for executive directors and senior management;

• determining policy on termination of senior management;

• determining performance-related pay, pension arrangements, share incentive plans and reporting and disclosure;

• reviewing the Company’s arrangements for its employees to raise concerns, in confidence, about possible wrongdoing in financial reporting or other matters; and

• establishing the selection criteria for, selecting, appointing and setting the terms of reference for, any remuneration consultants who advise the Remuneration Committee.

The Remuneration Committee will meet as often as one or more members of the Remuneration Committee deem necessary, but in any event will meet at least two times a year at the appropriate times in the financial reporting and audit cycle.

Compliance Committee

The Compliance Committee has responsibility for overseeing our compliance with laws, regulations, internal procedures and industry standards the violation of which may cause us significant business, regulatory or

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reputational damage, as well as legal and business trends and public policy issues. The Compliance Committee comprises three members, who are independent non-executive directors: Donald Stern, Patrick Vink and Stephen Wills. The Committee is chaired by Donald Stern.

The responsibilities of the Compliance Committee include oversight of the development and implementation of compliance and ethics policies and practices.

The Compliance Committee will meet as often as one or more members of the Compliance Committee deem necessary, but in any event will meet at least two times a year at the appropriate times in the financial reporting and audit cycle.

Code of Ethics

We have adopted a Code of Ethics that is applicable to all of our employees, executive officers and directors. The Audit Committee will be responsible for overseeing the Code of Ethics and will be required to approve any waivers of the Code of Ethics for employees, executive officers and directors. We expect that any amendments to the Code of Ethics, or any waivers of its requirements, will be disclosed on our website.

Conflicts of Interest

Our Board, in the manner set out in the Companies Act, may authorize any interest of a director that conflicts, or may conflict, with our interests as a company, provided that the conflict is not a direct transaction or arrangement between the director and us as a company. Subject to specified exceptions in our Articles of Association, a director shall not vote nor be counted in the quorum on any resolution of the Board in which that director is materially interested.

Compensation

2019 Director Remuneration

For the fiscal year ended December 31, 2019, the aggregate compensation accrued or paid to the members of our Board and our executive officers for services in all capacities was $2.8 million. The following table sets forth the approximate remuneration paid during the year ended December 31, 2019 to our directors.

	Base Salary and Fees	Bonus	Pension Contributions	Other Benefits	2019 Total
	US$ in thousands
Raymond T. Stafford	$61	$—	$—	$—	$61
Dr. Joseph A. Wiley	588	703	50	31	1,372
George P. Hampton, Jr.(1)	17	—	—	—	17
Dr. Alain H. Munoz(1)	15	—	—	—	15
Donald K. Stern(1)	21	—	—	—	21
Dr. Patrick V.J.J. Vink(1)	16	—	—	—	16
Stephen T. Wills(1)	23	—	—	—	23
Rory P. Nealon(2)	411	583	37	18	1,049
Harry Stratford(2)	82	—	—	—	82
James Culverwell(2)	58	—	—	—	58
Markus Zeiner(2)	47	—	—	—	47
Total(2)	$1,339	$1,286	$87	$49	$2,761

(1)
Mr. Hampton, Mr. Munoz, Mr. Stern, Dr. Vink and Mr. Wills were all appointed to the Board on September 24, 2019 and their salaries reflect the period from the date of appointment to December 31, 2019.

(2)
Mr. Nealon, Mr. Statford, Mr. Culverwell and Mr. Zeiner resigned from the Board on September 24, 2019 upon completion of the Acquisition. Mr. Nealon’s remuneration is for the full financial year as he continued in his roles as Chief Financial Officer and Chief Operating Officer, and the salaries of the other directors that resigned on September 24, 2019 reflect their salaries from January 1, 2019 to September 24, 2019.

In 2019, a total of 6,093,939 share options were issued to our Chief Executive Officer, Dr. Wiley. 316,039 share options were granted to Dr. Wiley on May 21, 2019 at a strike price of £0.7584. A further 5,777,900 share options were granted to Dr. Wiley on November 5, 2019 at a strike price of £1.215. Mr. Nealon was a director

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until September 2019. He continues to act in his role as Chief Financial Officer and Chief Operating Officer. A total of 251,915 share options were granted to Mr. Nealon at a strike price of £0.7584 on May 21, 2019, while he was a member of the Board.

Directors’ Service Agreements and Letters of Appointment

Raymond T. Stafford

We entered into a letter of appointment with Raymond Stafford on August 27, 2019 under which he was appointed a non-executive director with effect from September 24, 2019. Mr. Stafford was also appointed as a member of the Audit Committee from that date. The appointment is subject to our Articles of Association, and pursuant to terms of the letter of appointment may be terminated at any time upon three months’ written notice served by either party. Mr. Stafford is entitled to an annual fee of $77,500 for his role as non-executive chairman, a fee of $10,000 for his role as member of the Audit Committee, and reimbursement of reasonable expenses. Mr. Stafford is also subject to a six-month non-compete restrictive covenant.

George P. Hampton, Jr.

We entered into a letter of appointment with George Hampton on August 27, 2019 under which he was appointed a non-executive director with effect from September 24, 2019. Mr. Hampton was also appointed as a member and chair of the Remuneration Committee from that date. The appointment is subject to our Articles of Association, and pursuant to terms of the letter of appointment may be terminated at any time upon three months’ written notice served by either party. Mr. Hampton is entitled to an annual fee of $50,000 for his role as non-executive director, a fee of $15,000 for his role as chair and member of the Remuneration Committee, and reimbursement of reasonable expenses. Mr. Hampton is also subject to a six-month non-compete restrictive covenant.

Dr. Alain H. Munoz

We entered into a letter of appointment with Alain Munoz on August 27, 2019 under which he was appointed a non-executive director with effect from September 24, 2019. Dr. Munoz was also appointed as a member of the Remuneration Committee from that date. The appointment is subject to our Articles of Association, and pursuant to terms of the letter of appointment may be terminated at any time upon three months’ written notice served by either party. Dr. Munoz is entitled to an annual fee of $50,000 for his role as non-executive director, a fee of $7,500 for his role as member of the Remuneration Committee and reimbursement of reasonable expenses. Dr. Munoz is also subject to a six-month non-compete restrictive covenant.

Donald K. Stern

We entered into a letter of appointment with Donald Stern on August 27, 2019 under which he was appointed a non-executive director with effect from September 24, 2019. Mr. Stern was also appointed as a member of the Audit Committee and a member and chair of the Compliance Committee from that date. The appointment is subject to our Articles of Association, and pursuant to terms of the letter of appointment may be terminated at any time upon three months’ written notice served by either party. Mr. Stern is entitled to an annual fee of $50,000 for his role as non-executive director, a fee of $10,000 for his role as member of the Audit Committee, a fee of $20,000 for his role as the chair of the Compliance Committee and reimbursement of reasonable expenses. Mr. Stern is also subject to a six-month non-compete restrictive covenant.

Dr. Patrick V.J.J. Vink

We entered into a letter of appointment with Patrick Vink on August 27, 2019 under which he was appointed a non-executive director with effect from September 24, 2019. Dr. Vink was also appointed as a member of the Remuneration Committee and the Compliance Committee from that date. The appointment is subject to our Articles of Association, and pursuant to terms of the letter of appointment may be terminated at any time upon three months’ written notice served by either party. Dr. Vink is entitled to an annual fee of $50,000 for his role as non-executive director, a fee of $10,000 for his role as member of the Compliance Committee and reimbursement of reasonable expenses. Dr. Vink is also subject to a six-month non-compete restrictive covenant.

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Stephen T. Wills

We entered into a letter of appointment with Stephen Wills on August 27, 2019 under which he was appointed a non-executive director with effect from September 24, 2019. Mr. Wills was also appointed as a member and chair of the Audit Committee and a member of the Compliance Committee from that date. The appointment is subject to our Articles of Association, and pursuant to terms of the letter of appointment may be terminated at any time upon three months’ written notice served by either party. Mr. Wills is entitled to an annual fee of $50,000 for his role as non-executive director, a fee of $20,000 for his role as chair of the Audit Committee, a fee of $7,500 for his role as a member of the Remuneration Committee, a fee of $10,000 for his role as member of the Compliance Committee and reimbursement of reasonable expenses. Mr. Wills is also subject to a six-month non-compete restrictive covenant.

A registration rights agreement to which we are a party contains provisions regarding the terms of appointment of our Board. See “Related Party Transactions—Agreements with Principal Shareholders—Registration Rights Agreement” for further information.

Equity Compensation Arrangements

The Share Option Plan

Our Employee Share Option Plan (“Share Option Plan”), was adopted on September 23, 2019 and effective from September 24, 2019. Prior to such date, we granted options under a prior employee share option plan, which had the same terms and conditions as the Share Option Plan. On September 24, 2019, all options held under our prior share option plan were rolled over into options to subscribe for our ordinary shares with the key terms including strike price, vesting and the expiration date of such rolled over options remaining the same as they were on the issue date of the options under the prior share option plan.

On November 28, 2017, a total of 343,521 share options were granted to Dr. Wiley at an exercise price of £1.2072 per share, on May 21, 2019, a total of 316,039 share options were granted to Dr. Wiley at an exercise price of £0.7584 per share, and on November 5, 2019, a total of 5,777,900 share options were granted to Dr. Wiley at an exercise price of £1.215 per share.

On November 28, 2017, a total of 137,408 share options were granted to Mr. Nealon at an exercise price of £1.2072 per share, on May 21, 2019, a total of 251,915 share options were granted to Mr. Nealon at an exercise price of £0.7584 per share and, on November 5, 2019, a total of 4,437,500 share options were granted to Mr. Nealon at an exercise price of £1.215 per share.

No new share options were granted to directors in 2018.

Eligibility, Awards and Administration

The Share Option Plan provides for the grant of share options to our directors, employees and consultants eligible to receive awards. The Board may grant options to our directors, employees or consultants or the directors, employees or consultants of any associated company (companies in which we hold a 20% or greater interest). The maximum number of shares over which options may be in issue at any one time pursuant to the Share Option Plan cannot currently exceed 15% of our share capital from time to time (“Option Limit”). On January 1 in each calendar year, the then Option Limit will automatically increase by five per cent. of the Company’s issued share capital from time to time. The Option Limit from time to time shall decrease by the number of our ordinary shares in relation to which options are exercised (such decrease to be calculated by reference to the percentage that the number of ordinary shares in relation to which options are exercised bears to our issued share capital immediately prior to such exercise).

Options granted under the Share Option Plan typically contain a three-year vesting period and typically have seven years within which they must be exercised. When options are granted, the Board may at its discretion attach conditions to the exercise of the options, which must be satisfied before an option may be exercised.

Before the date of adoption of the Share Option Plan, we had 4,308,805 options in issue to purchase ordinary shares granted to employees and consultants of which 42,485 have since lapsed. On September 24, 2019, these options were rolled over into new options to subscribe for our ordinary shares. On November 5, 2019, we issued 10,215,400 additional share options. Of such outstanding options, as at December 31, 2019, 2,468,310 had fully vested and were exercisable and 12,013,410 were not yet exercisable.

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Lapse of Options

Options lapse on the last date upon which any part thereof may be exercised under the terms of the Share Option Plan, which date shall be determined by the Board and specified in the option certificate issued to a participant in connection with the grant of an option, but in no event shall such date be later than the date preceding the tenth anniversary of the date of grant of an option (“Final Option Date”).

To the extent then exercisable, options also lapse on the occurrence of certain events unless exercised within certain specified periods (or, if earlier, by no later than the Final Option Date), subject to the exercise by the Board of its discretion, as follows:

• on death, one year from the date of death;

• on the option holder ceasing to meet the requirements of a participant in the Share Option Plan due to retirement or resignation or early retirement due to disability or ill health, on the expiration of one year after having resigned or retired; or

• on resignation, retirement or dismissal for reasons other than termination summarily for serious misconduct, 90 days after such event. On termination summarily for serious misconduct, options lapse immediately on such termination.

If options were not already exercisable on the occurrence of the events referred to above, the options will lapse (subject to the discretion of the Board). The Board may at its discretion extend the periods set out above.

Options shall also lapse in the event of a liquidator being appointed.

Certain Transactions

Under the Share Option Plan, on a merger, takeover or other re-organization resulting in a change of control of us (or the Board considers this is about to occur), each option shall accelerate and become exercisable in full as of a date specified by the Board and subject to such conditions as the Board may at its discretion determine. A reverse takeover by us of another company will not result in an acceleration of option vesting. The Remuneration Committee may also make such other comparable arrangements to replace any options (whether exercisable or not) as it determines in its discretion.

Adjustments

Under the Share Option Plan, in the event of a consolidation, sub-division or reduction of our share capital, or any other variation in our share capital, the Board may make such adjustment to the relevant exercise price, the number, and/or the class of shares subject to each option as it deems appropriate. If shareholders are granted rights to subscribe for further ordinary shares (such rights being related to the number of ordinary shares held by them respectively) the Board shall at its absolute discretion decide whether the granting of such rights and the subscriptions made thereunder shall result in the depletion in the value of each ordinary share and the Board may make such adjustment to the exercise price, the number and/or class of shares subject to each option as it deems appropriate.

Amendment and Termination

The Share Option Plan may be terminated at any time by resolution of our Board. Subsequent to any termination of the Share Option Plan, we shall not grant any further options, but no such termination shall affect or modify any subsisting rights or obligations of participants in respect of any options, and notwithstanding such termination, we shall continue to administer and manage the Share Option Plan in accordance with the rules of the Share Option Plan.

We may at any time by resolution of the Board vary, amend or revoke any of the provisions of the Share Option Plan in such manner as the Board sees fit.

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The following table summarizes the outstanding number of our ordinary shares and options held by directors and executive officers as of March 31, 2020:

Name	Ordinary Shares	% Ownership of Share Capital	Share Options	Grant Date	Exercise Price Stg£	Expiration Date
Dr. Joseph A. Wiley	3,507,080	2.0%	343,521	Nov 28, 2017	1.2072	Nov 28, 2024
			316,039	May 21, 2019	0.758	May 21, 2026
			5,777,900	Nov 5, 2019	1.215	Nov 5, 2026
Rory P. Nealon	1,610,770	0.9%	137,408	Nov 28, 2017	1.2072	​Nov 28, 2024
			251,915	May 21, 2019	0.758	May 21, 2026
			4,437,500	Nov 5, 2019	1.215	Nov 5, 2026
George P. Hampton	—	—	—	—	—	—
Dr. Alain H. Munoz	—	—	—	—	—	—
Raymond T. Stafford	1,301,001	0.8%	—	—	—	—
Donald K. Stern	—	—	—	—	—	​—
Stephen T. Wills	—	—	—	—	—	—

Bonus Payments

All executive directors and senior management are eligible for a discretionary annual bonus. Annual cash bonuses are paid on the achievement of pre-set strategic objectives. The Remuneration Committee in conjunction with the Board reviews and sets the objectives at the start of each calendar year.

Pension, Retirement or Similar Benefits

We operate defined contribution schemes in various locations where employees are based. Contributions to the defined contribution schemes are recognized in our statement of comprehensive income in the period in which the related services are received from the employee. Under these schemes we have no obligation, either legal or constructive, to pay further contributions in the event that the fund does not hold sufficient assets to meet our benefit commitments.

As of December 31, 2019, we had accrued $206,000 in pension benefits which was paid to the pension administrators in early 2020. With the exception of Dr. Wiley and Mr. Nealon, no director or secretary has any pension or retirement benefits. In addition, no director or senior manager is entitled to any pension, retirement or related benefit upon termination of his employment.

Insurance and Indemnification

To the extent permitted by law, each of our directors, alternate directors, officers or employees is entitled to be indemnified out of our assets against all losses or liabilities which he or she may sustain or incur in or about the execution of the duties of his or her office or otherwise in relation thereto. We maintain directors’ and officers’ insurance to insure such persons against certain liabilities.

To the extent permitted by law, the directors have the power to purchase and maintain insurance for or for the benefit of any persons who are or were at any time:

• directors, alternate directors, officers or employees of a group company; or

• trustees of any pension fund in which our employees or employees of any other group company are interested, including in each case insurance against any liability incurred by such persons in respect of any act or omission in the actual or purported execution and/or discharge of their duties and/or in the exercise or purported exercise of their powers and/or otherwise in relation to their duties, powers or offices in relation to us or any other group company or pension fund.

Insofar as indemnification of liabilities arising under the Securities Act may be permitted to the Board, executive officers, or persons controlling us pursuant to the foregoing provisions, we are informed that, in the opinion of the SEC, such indemnification is against public policy as expressed in the Securities Act and is therefore unenforceable.

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RELATED PARTY TRANSACTIONS

The following is a summary of related party transactions, since January 1, 2017 to date, in which we have been a participant and in which the amount involved exceeded or will exceed $120,000, and in which any of our then directors, executive officers or holders of more than 5% of any class of our voting securities at the time of such transaction, or any members of their immediate family, had or will have a direct or indirect material interest.

Agreements with Principal Shareholders

We entered into a number of agreements with our principal shareholders in connection with the Acquisition. The following summary of these agreements is not intended to be a complete description of these agreements, and it is qualified in its entirety by reference to the full text of such agreements, which are filed as exhibits to the registration statement of which this prospectus forms a part. We urge you to review these exhibits in their entirety.

The Restructuring Support Agreement

On May 20, 2019, we entered into a Restructuring Support Agreement (as subsequently amended on June 12, 2019, the “Restructuring Support Agreement”) with (i) Aegerion and Aegerion Pharmaceuticals Holdings, Inc. (together, “Debtors”), (ii) Novelion, as holder of 100% of the outstanding equity interests of Aegerion and holder of 100% by principal amount of claims under Aegerion’s Amended and Restated Loan Credit Agreement dated March 15, 2018, (iii) the holders of 100% in principal amount under Aegerion’s Bridge Loan Credit Agreement dated November 8, 2018 (“Bridge Loan Credit Agreement”), and (iv) holders of in excess of 67% in principal amount of Aegerion’s 2% Senior Unsecured Convertible Notes due 2019. The holders of Aegerion debt party to the Restructuring Support Agreement are collectively referred to herein as the “Consenting Lenders.”

Under the Restructuring Support Agreement, we shall, among other things, negotiate in good faith the documents necessary to complete the transactions in connection with the Acquisition. The Debtors agreed to, among other things, (a) use reasonable best efforts to take any actions necessary in connection with the transaction; (b) commence the Debtors’ bankruptcy cases and file and seek approval of “first day” motions; and (c) file and prosecute confirmation of the Plan of Reorganization.

Each of the Consenting Lenders agreed to, among other things, (a) vote to accept the Plan of Reorganization and otherwise support the transactions; (b) not object or impede confirmation or implementation of the Plan of Reorganization or the transactions, including by soliciting an alternative transaction; (c) not support any other Chapter 11 plan or other restructuring or reorganization of the Debtors other than the Plan of Reorganization; and (d) not take any action that would interfere with consummation of the transactions and take any and all necessary, appropriate, or advisable action in furtherance of the transactions.

Each Consenting Lender also agreed that, if the Restructuring Support Agreement was terminated pursuant to the specified provisions contained therein on or after the date Aegerion received a proposal for an alternative transaction that is still outstanding at the time of such termination, it would vote against, and use its reasonable best efforts to oppose, approval of an alternative transaction unless, following a written inquiry by the Consenting Lenders regarding whether we would be willing to complete the transactions in accordance with the definitive documents, we did not confirm within five business days following such inquiry (subject to withdrawal at any time upon five business days’ notice) that we would be willing to consummate the transactions if the documents are re-executed by the other parties thereto.

Plan Funding Agreement

On May 20, 2019, we entered into the Plan Funding Agreement with Aegerion setting forth the terms and conditions of the Acquisition.

In consideration of the Acquisition, Aegerion’s former creditors received our ordinary shares (including shares represented by ADSs and zero cost warrants that may be exercised at any time), constituting approximately 61.6% of our outstanding share capital prior to our $60 million dollar equity issuance in September 2019 (“September 2019 Equity Raise”). The number of our ordinary shares that were issued as consideration was calculated by multiplying the aggregate amount of all issued and outstanding ordinary shares of Amryt immediately prior to the closing of the Acquisition by 1.59, after giving effect to the transactions, but before giving effect to equity underlying the issuance of the Convertible Notes, the September 2019 Equity Raise, our

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ordinary shares that may be issuable in satisfaction of the CVRs if the relevant milestones are achieved on the terms of the CVRs, and equity that was reserved for issuance under any management equity compensation plan adopted by us.

The Plan Funding Agreement contains reciprocal representations and warranties made by us and Aegerion relating to organization, qualification, due authorization of the parties and authority, consents and approvals, capitalization, financial statements, no undisclosed liabilities, recent events, contracts and commitments, real property, intellectual property, privacy and data security, legal compliance, permits, environmental compliance and conditions, litigation, tax matters, insurance, illegal or improper payments, related party transactions, brokers’ fees, employees and healthcare compliance matters.

Under the terms of the Plan Funding Agreement:

• We were not required to take any action which is prohibited or restricted by the Takeover Code in relation to any alternative transaction. Similarly, we were not required to refrain from any action which is required by the Takeover Code in relation to any alternative transaction.

• Following the approval by the U.S. Bankruptcy Court of the Plan Funding Agreement Approval Motion on June 28, 2019, Aegerion had a 55-day period to solicit an alternative transaction. Subject to the limitations of the Plan Funding Agreement¸ Aegerion was also entitled to respond to unsolicited proposals that Aegerion determined reasonably likely to result in a superior transaction.

• At any time prior to and after the completion of the 55-day period, Aegerion was entitled to receive offers from competing bidders and was able to respond to any such offer if its board deemed that the offer constituted or was likely to constitute a superior proposal.

The Plan Funding Agreement included termination rights for both us and Aegerion and related provisions that allocated fees and expenses among the parties if the Plan Funding Agreement was terminated prior to closing.

Upon termination of the Restructuring Support Agreement, we had the right to terminate the Plan Funding Agreement for any reason, so long as the termination of the Restructuring Support Agreement was not principally due to our material uncured breach. Aegerion also had a right to terminate the Plan Funding Agreement upon termination of the Restructuring Support Agreement, so long as the termination of the Restructuring Support Agreement was not principally due to Aegerion’s or the Consenting Lenders’ material uncured breach.

In specified circumstances, upon termination, we had the obligation to pay Aegerion a termination fee of $2,050,000. Additionally, upon a termination in other specified circumstances, Aegerion was obligated to pay us an expense reimbursement amount equal to all of our reasonable and documented fees and expenses incurred in connection with the negotiation, preparation and implementation of the documents for the transactions, up to $4,000,000. In addition, if Aegerion disclosed an alternative transaction and did not withdraw it and Aegerion entered into an agreement for an alternative transaction with a third party within one year of the date of the Plan Funding Agreement, Aegerion was liable to pay us a termination fee of $11,850,000 upon consummation of such transaction.

Backstop Agreement

On July 10, 2019, we entered into the Backstop Agreement with Highbridge MSF International Ltd., Highbridge SCF Special Situations SPV, L.P., 1992 Tactical Credit Master Fund, L.P., Athyrium Opportunities II Acquisition 2 LP, Athyrium Opportunities III Acquisition 2 LP, Whitebox Relative Value Partners, LP, Whitebox GT Fund, LP; Whitebox Multi-Strategy Partners, LP, Pandora Select Partners, LP, Nineteen77 Global Multi-Strategy Alpha Master Limited and Nineteen77 Global Convertible Bond Master Limited (each a “Backstop Party” and collectively, “Backstop Parties”), whereby each Backstop Party agreed to subscribe for a specified percentage of any unsubscribed shares in the September 2019 Equity Raise. In consideration for the Backstop Parties’ undertaking to subscribe for our ordinary shares, we agreed to pay the Backstop Parties the sum of $3 million in cash as commission.

The obligations of each Backstop Party were conditional on the satisfaction of, among others, the following conditions: (a) the rights attaching to our ordinary shares not having been altered at the date of execution of the Restructuring Support Agreement; (b) the approval of specified resolutions by our shareholders; and (c) a final order confirming the Plan of Reorganization having been entered (in form and substance reasonably satisfactory to the Backstop Parties and to us), the Effective Date, having occurred, and all conditions precedent to the

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occurrence of the Effective Date having been satisfied or waived by the Backstop Parties. The Backstop Parties, acting unanimously, had the ability to waive in whole or in part all or any of the conditions.

DIP Funding Agreement

On June 28, 2019 Aegerion (as borrower) entered into a $20 million aggregate maximum principal amount post-petition super-priority secured debtor-in-possession credit facility (“DIP Funding Agreement”) with the Athyrium Funds and certain affiliates of Highbridge, as lenders, and Cantor Fitzgerald Securities, as administrative agent. The DIP Funding Agreement was fully and unconditionally guaranteed on a super-priority senior secured basis by Aegerion’s U.S. subsidiaries (other than Aegerion Securities Corporation) (“DIP Subsidiary Guarantors”).

The loans under the DIP Funding Agreement bore interest at a rate of 12.5% per annum, to be paid in cash on a monthly basis. The DIP Funding Agreement matured on the earliest to occur of: (a) the Effective Date; (b) the date that is 150 days after May 20, 2019 (being October 17, 2019), subject to a 60 day extension in accordance with the Plan Funding Agreement; and (c) the date on which the obligations under the DIP Funding Agreement are accelerated and became due and payable following the occurrence of an event of default.

The DIP Funding Agreement contained negative covenants restricting Aegerion and its subsidiaries, including limitations on, or related to, liens, investments, the incurrence of indebtedness, fundamental changes, dispositions, restricted payments, changes in business, transactions with affiliates, prepayments and modifications of debt, negative pledges, changes to organizational documents, use of proceeds, compliance with laws and orders of the bankruptcy court, ownership of subsidiaries, Chapter 11 claims, revisions of orders and applications to the bankruptcy court, and adequate protection.

The DIP Funding Agreement contained events of default, including failure to make payments when due, breach of covenants, breach of representations or warranties, cross-defaults, judgments, ERISA, invalidity of loan documents, change of control, failure of liens to be perfected, dissolution or liquidation, discontinuation of business, maintenance of independent directors, and bankruptcy matters including failure to confirm the approved Plan, non-compliance or revocation or modification of bankruptcy orders, and non-compliance with the Restructuring Support Agreement.

The DIP Funding Agreement was repayable from time to time and contained mandatory repayments from the proceeds of certain dispositions of assets, casualty events, and the incurrence of indebtedness. However, the DIP Funding Agreement facility was never utilized by Aegerion, therefore no repayments were required to be made.

Registration Rights Agreement

On the closing of the Acquisition, we entered into a Registration Rights Agreement with affiliates of Athyrium (“Athyrium Parties”) and affiliates of Highbridge (“Highbridge Parties”). Pursuant to the agreement, we were to use commercially reasonable efforts to list our ordinary shares, or ADSs representing such shares, on the Nasdaq within 90 days of the closing of the Acquisition. The agreement also provided the Athyrium Parties and the Highbridge Parties with the right to demand share registration up to two times in any 12-month period and a right to participate in future registration statements filed by us. We are obligated to pay any expenses relating to such registrations and indemnify the Athyrium Parties and the Highbridge Parties (and their respective partners, members, directors, offices and professional advisers, among others) participating in such registration against specified liabilities that may arise in connection with such registration. Furthermore, each of the Athyrium Parties and the Highbridge Parties participating in such registration will indemnify us and certain other persons against specified limited liabilities that may arise in connection with such registration.

The agreement provided the Athyrium Parties and the Highbridge Parties with specified rights to nominate directors and designate observers to the Board. The agreement specified that, on closing of the Acquisition, the initial Board would be made up of seven members. The Board was to initially consist of Joseph Wiley, our Chief Executive Officer, one independent chairman and one independent director proposed by us, one independent director proposed by the Athyrium Parties, one non-independent director proposed by the Athyrium Parties and two independent directors proposed by the Highbridge Parties. The Highbridge Parties have the right to subsequently select a non-independent director to replace one of its nominated independent directors, subject to the shareholding requirements set out below. The agreement also provided that Joseph Wiley would continue as Chief Executive Officer for no less than two years after the closing of the Acquisition, unless he: (a) resigns;

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(b) is removed from the position by a vote of a majority of the Board, which majority must include the vote of at least one of the directors nominated by us; or (c) is removed for cause under the terms of his employment agreement.

The initial Board is subject to compulsory retirement and will be put up for re-election at our first annual general meeting to be held at least 24 months after the closing of the Acquisition. For so long as each of the Athyrium Parties or the Highbridge Parties (or their respective affiliates) respectively hold at least 10% of our issued share capital, the Athyrium Parties and the Highbridge Parties (as applicable) are each entitled to nominate a replacement of the non-independent director (as applicable) selected by them on his or her resignation or retirement. Any such director shall serve on the Board until our next annual general meeting, where such director’s appointment will be subject to approval by an ordinary resolution of our shareholders.

The agreement will also grant the Athyrium Parties or the Highbridge Parties the right to designate a board observer to attend Board meetings under the Secured Credit Facility (as described below), subject to restrictions on attendance when specified matters are discussed. Each of the Athyrium Parties and the Highbridge Parties have the ability to terminate this right, and this right will be terminated when the Secured Credit Facility is either refinanced or paid off in full. To date, only Athyrium has exercised this right.

Secured Credit Facility

On September 24, 2019, we entered into the Secured Credit Facility. The Secured Credit Facility is fully and unconditionally guaranteed on a senior secured basis by us and, to the extent permitted by law, each of our and Aegerion’s existing and subsequently acquired or formed direct and indirect subsidiaries (other than certain immaterial non-U.S. subsidiaries and Aegerion Securities Corporation) (“Facility Guarantors”). Proceeds from the Secured Credit Facility were used to refinance Aegerion’s existing secured bridge loan in the principal amount of approximately $50 million held by certain funds managed by Athyrium and Highbridge, respectively, and our existing €20 million (in principal) secured loan facility with EIB.

The loans under the Secured Credit Facility bear interest at a rate of: (a) 11% per annum paid in cash on a quarterly basis; or (b) 6.5% per annum paid in cash plus 6.5% per annum paid in kind on a quarterly basis. The Secured Credit Facility will mature five years after issuance, unless earlier repaid.

The Secured Credit Facility contains the following negative covenants: (a) limitations on liens; (b) limitations on disposition of assets (including restrictions on dispositions of material assets and exclusive licensing transactions in material territories but permitting, among other things, (i) dispositions consisting of licenses of AP101 and AP103 assets outside of the United States and European territories, (ii) dispositions of obsolete and surplus property that are immaterial, and (iii) a general basket for dispositions less than $250,000 individually and $2,500,000 in the aggregate); (c) limitations on consolidations and mergers; (d) limitations on loans and investments (with restrictions on investments and indebtedness to non-Facility Guarantor subsidiaries to be agreed); (e) limitations on indebtedness (with exceptions permitting, among other things, the indebtedness under the CVR deed poll); (f) limitations on transactions with affiliates (with exceptions permitting, among other things, certain de minimis transactions, certain transactions for the provision of inter-company services among Facility Guarantors and other transactions with non-Facility Guarantor affiliates in the ordinary course of business on an arm’s-length basis not to exceed an amount to be agreed upon, including Transactions with non-Facility Guarantor affiliates in respect of intercompany services charged on a cost plus 10% basis); (g) compliance with ERISA; (h) limitations on restricted payments (including restrictions on dividends (stock or cash), share repurchases, spin-off and split-off transactions and payments to non- Facility Guarantors); (i) limitations on changes in business, changes in structure, changes in accounting, name and jurisdiction of organization, amendments to organizational documents that are adverse to the lenders, any amendments, modifications or waivers to the CVR deed poll; (j) limitations on material changes to other indebtedness documents; (k) limitations on restricted debt payments; (l) no negative pledges or burdensome agreements; and (m) certain other customary restrictions.

The Secured Credit Facility contains the following events of default under the Secured Credit Facility: (a) failure to pay principal, interest or any other amount when due; (b) representations and warranties incorrect in any material respect when made or deemed made; (c) failure to comply with covenants; (d) cross-default to other

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indebtedness; (e) failure to satisfy or stay execution of judgments; (f) bankruptcy or insolvency; (g) actual or asserted invalidity or impairment of any part of the credit documentation (including the failure of any lien to remain perfected); (h) ERISA default; (i) failure to conduct business; (j) change of ownership or control; and (k) liquidation or dissolution.

Repayments of the Secured Credit Facility are subject to a prepayment premium as follows: (a) 105% of the amount repaid plus interest that would have accrued on such amount repaid through the second anniversary of the closing of the Acquisition discounted at a rate equal to the yield on U.S. Treasury notes with a maturity closest to the second anniversary of the closing of the Acquisition plus 50 basis points in years one and two; (b) 105% of the amount repaid in year three; (c) 101% of the amount repaid in year four; and (d) thereafter, 100%. Such premium will apply to repayments after acceleration, but will not apply to prepayments from insurance and condemnation proceeds.

The Secured Credit Facility also contains a covenant requiring the maintenance of minimum liquidity of $10 million at all times, subject to an add back with respect to cash expenditures made to French pricing authorities related to the MYALEPTA product. The Secured Credit Facility requires mandatory prepayments for certain specified debt issuances, asset sales or other dispositions of assets (including exclusive licensing transactions and insurance and condemnation proceeds), subject to certain exceptions and thresholds.

Convertible Notes

On September 24, 2019, we issued $125 million aggregate principal amount of Convertible Notes due 2025 to certain creditors of Aegerion. The Convertible Notes are fully and unconditionally guaranteed on a senior unsecured basis by us and, to the extent permitted by law, each of our and Aegerion’s existing and subsequently acquired or formed direct and indirect subsidiaries (other than certain immaterial non-U.S. subsidiaries and Aegerion Securities Corporation) (“Notes Guarantors”). The Convertible Notes are issued pursuant to an indenture by and among Aegerion and the Notes Guarantors and a trustee for the Convertible Notes.

The Convertible Notes bear interest at a rate of 5% per annum, payable in cash semi-annually. The Convertible Notes will mature approximately five and a half years after issuance, unless earlier repurchased, redeemed or converted.

The Convertible Notes and guarantees are the general senior unsecured obligations of Aegerion and the Notes Guarantors, respectively, ranking equally in right of payment with all of the existing and future liabilities of such entities that are not so subordinated.

Convertible Note holders are entitled to convert their Convertible Notes, at their option, at any time prior to the close of business on the second scheduled trading day immediately prior to the maturity date, unless the Convertible Notes have been previously repurchased or redeemed. The Convertible Notes are initially convertible into 386.75 ordinary shares for each $1,000 principal amount of Convertible Notes, which is equivalent to an initial conversion price of approximately $2.59 per ordinary share. The conversion rate is subject to customary anti-dilution adjustments as well as adjustments following certain fundamental changes (as set out below) and adjustments to account for the final conversion share price. Upon conversion, Aegerion may choose to pay or deliver, as the case may be, cash, ordinary shares or a combination of cash and our ordinary shares.

The Convertible Notes have the following events of default: (a) a default in the payment when due of the principal of, or the redemption price or repurchase price for, any Convertible Note; (b) a default for 30 days in the payment when due of interest on any Convertible Note; (c) the failure to deliver, when required, a repurchase notice; (d) a default in the obligation to convert a Convertible Note upon the exercise of the conversion right with respect thereto and such failure continues for five business days; (e) a default in obligations with respect to successors to us or Aegerion; (f) a default in other obligations or agreements under the Convertible Notes or the indenture where such default is not cured or waived within 60 days after notice; (g) certain defaults with respect to any one or more mortgages, agreements or other instruments under which there is outstanding, or by which there is secured or evidenced, any indebtedness for money borrowed of at least $12 million (or its foreign currency equivalent) subject to a 60-day grace period; (h) one or more final judgments for the payment of at least $12 million in the aggregate, where such judgment is not timely discharged or stayed, subject to a 60-day grace period; and (i) certain events of bankruptcy, insolvency and reorganization with respect to Aegerion, us or any of our or their significant subsidiaries.

Aegerion has the right to redeem all or any portion of the Convertible Notes on or after the third anniversary of issuance, provided the per share volume-weighted average price of our ordinary shares exceeds 150% of the

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conversion price on: (a) each of at least 20 trading days (whether or not consecutive) during the 30 consecutive trading days ending on, and including, the trading day immediately before the date Aegerion sends the notice of such redemption; and (b) the trading day immediately before the date Aegerion sends such notice. Aegerion may also redeem the Convertible Notes upon certain changes in tax law or regulation. The redemption price will be an amount in cash equal to 100% of the principal amount of the Convertible Notes redeemed, plus any accrued and unpaid interest.

Upon the occurrence of events deemed a fundamental change, Convertible Note holders may require Aegerion to repurchase all or a portion of their Convertible Notes at a repurchase price of 100% of the principal amount of the Convertible Notes, plus any accrued and unpaid interest. With certain exceptions, a fundamental change will occur if: (a) a “person” or “group” (within the meaning of Section 13(d)(3) of the Exchange Act), other than us, any of our wholly owned subsidiaries or their respective employee benefit plans, has filed any report with the SEC indicating that such person or group has become the direct or indirect beneficial owner of our ordinary shares representing more than 50% of the voting power of all of our then-outstanding ordinary shares; (b) the consummation of (i) any sale, lease or other transfer, in one transaction or a series of Transactions, of all or substantially all of the assets of us and our subsidiaries, taken as a whole, to any person, other than one or more of our wholly owned subsidiaries; or (ii) any transaction or series of related transactions in connection with which (whether by means of merger, consolidation, share exchange, combination, reclassification, recapitalization, acquisition, liquidation or otherwise) all of our ordinary shares are exchanged for, converted into, acquired for, or constitute solely the right to receive, other securities, cash or other property (other than changes resulting solely from a subdivision or combination, or a change in par value, of our ordinary shares); (c) our shareholders approve any plan or proposal for the liquidation or dissolution of our company; or (d) our ordinary shares cease to be traded on AIM or another permitted exchange.

If a fundamental change occurs or if Aegerion initiates a redemption of the Convertible Notes, the conversion rate will increase for any conversion occurring in connection with such fundamental change or redemption. The increase in the conversion rate will be determined based on a schedule set forth in the indenture and is subject to adjustment in accordance with the final conversion share price.

Zero Cost Warrant

We agreed, for certain Aegerion creditors who wished to restrict their percentage share interest in our issued share capital, to issue to the relevant Aegerion creditor, as an alternative to our ordinary shares, an equivalent number of new zero cost warrants to subscribe for our ordinary shares to be constituted on the terms of the zero cost warrant. The relevant Aegerion creditor is entitled at any time to exercise the zero cost warrants, at which point in time we would issue to that Aegerion creditor the relevant number of fully paid ordinary shares in return for the exercise of the zero cost warrants.

In accordance with the terms of the zero cost warrant, each zero cost warrant entitles the holder thereof to subscribe for one ordinary share at any time after our admission to trade on AIM. In no event shall any holder of zero cost warrants have the right to subscribe for zero additional consideration for, nor shall we issue to such holder of zero cost warrants, ordinary shares to the extent that such subscription would result in the holder of zero cost warrants and its affiliates, including groups that include the holder of zero cost warrants and its affiliates, together beneficially owning more than their prescribed maximum percentage to be set in the zero cost warrant. Any issue of our ordinary shares in contravention of this condition will be null and void.

The zero cost warrants constitute our direct and unsecured obligations and rank pari passu and without any preference among themselves (save for any obligations to be preferred by law) at least equally with our other present and future unsecured and unsubordinated obligations. The zero cost warrants are not transferrable except with our prior written consent.

On September 24, 2019, certain of Aegerion’s creditors elected to receive 13,976,722 warrants to subscribe for our ordinary shares as consideration for the Acquisition.

On November 14, 2019, we repurchased a combined 4,864,656 ordinary shares from Highbridge Tactical Master Fund L.P., Highbridge SCF Special Situations SPV, L.P. and Nineteen77 Global Multi Strategy Alpha Master Limited. In exchange for the ordinary shares, these institutions were issued an equivalent number of zero cost warrants. Each warrant entitles the holder to subscribe for one ordinary share at zero cost. These ordinary shares are now held as treasury shares. On December 19, 2019, Highbridge MSF International Ltd exercised 1,645,105 zero cost warrants in exchange for 1,645,105 ordinary shares.

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Interim Subscription Agreements

On August 27, 2019, we entered into the following subscription agreements in the total amount of $8 million:

• a subscription agreement between Amryt, Highbridge MSF International Ltd. and Highbridge SCF Special Situations SPV, L.P. (together, “Highbridge Subscribers”) pursuant to which the Highbridge Subscribers agreed to subscribe for 907,193 ordinary shares for an aggregate subscription price of $1 million and, subject to the GM Resolutions being passed, a further 2,765,901 ordinary shares for an aggregate subscription price of $3 million;

• a subscription agreement between Amryt and Raymond Stafford pursuant to which Raymond Stafford agreed to subscribe for 918,273 Amryt ordinary shares for an aggregate subscription price of $1 million; and

• a subscription agreement between Amryt and Stonepine Capital, LP pursuant to which Stonepine Capital, LP agreed to subscribe for 459,136 Amryt ordinary shares for an aggregate subscription price of $500,000.

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PRINCIPAL AND REGISTERED HOLDERS

The following table sets forth information relating to the beneficial ownership of our ordinary shares as of March 31, 2020, by:

• each person, or group of affiliated persons, known to us to beneficially own 5% or more of our outstanding ordinary shares;

• each member of our Board and each of our executive officers; and

• all Board members and executive officers as a group.

Beneficial ownership is determined in accordance with the rules of the SEC. These rules generally attribute beneficial ownership of securities to persons who possess sole or shared voting power or investment power with respect to those securities as well as any ordinary shares that the individual has the right to acquire within 60 days of March 31, 2020 through the exercise of any option, warrant or other right. The percentage ownership information shown in the table prior to this offering is based upon 171,695,160 ordinary shares, which includes 154,498,887 ordinary shares outstanding as of March 31, 2020 (not including 4,864,656 treasury shares), plus 17,196,273 zero cost warrants that may be exercised at any time. The table below does not give effect to options to purchase an aggregate of 1,320,000 ordinary shares that we intend to grant to our non-executive directors immediately after the effectiveness of the registration statement of which this prospectus forms a part.

Except as otherwise indicated, all of the shares reflected in the table are ordinary shares and all persons listed below have sole voting and investment power with respect to the shares beneficially owned by them, subject to applicable community property laws. The information is not necessarily indicative of beneficial ownership for any other purpose.

In computing the number of ordinary shares beneficially owned by a person and the percentage ownership of that person, we deemed outstanding ordinary shares subject to options and warrants held by that person that are immediately exercisable or exercisable within 60 days of March 31, 2020. We did not deem these shares outstanding, however, for the purpose of computing the percentage ownership of any other person. Beneficial ownership representing less than 1% is denoted with an asterisk (*). Except as otherwise indicated in the table below, addresses of the directors, executive officers and named beneficial owners are in care of Amryt Pharma plc, Dept 920a 196 High Road, Wood Green, London, N22 8HH, United Kingdom.

	Ordinary Shares Beneficially Owned Prior to this Offering		Number of Shares Being Registered in the Offering	Ordinary Shares Beneficially Owned After this Offering**
Name of beneficial owner	Number	Percentage		Number	Percentage
5% or greater shareholders (not including current directors or officers):
Athyrium Funds(1)	64,796,247	37.7%	64,796,247	0	—
Highbridge(2)	36,342,967	21.2%	36,342,967	0	—
UBS(3)	13,932,851	8.1%	13,932,851	0	—
Novelion Therapeutics Inc.	12,490,250	7.3%	12,490,250	0	—
Edgepoint Capital	12,126,650	7.1%	12,126,650	0	—
Software AG-Stiftung	10,212,153	5.9%	10,212,153	0	—
Executive officers and directors:
Dr. Joseph A. Wiley(4)	3,757,850	2.2%	3,507,080	250,770	*
Rory P. Nealon(5)	1,742,453	1.0%	1,610,770	131,083	*
Ray T. Stafford	1,363,501	*	1,363,501	0	—
Donald K. Stern	—	—
Dr. Alain H. Munoz	—	—
George P. Hampton	—	—
Dr. Patrick V.J.J. Vink	—	—
Stephen T. Wills	—	—
All executive officers and directors as a group (eight persons)	6,801,304	4.0%	6,481,351	381,853	*
Other Registered Holder
Axa Investments	6,494,164	3.8%	6,494,164	0	—

*
Less than 1%.

**
Unlike an initial public offering, any disposition by the Registered Holders of the Registered Shares represented by ADSs is not being underwritten by any investment bank. The Registered Holders may or may not elect to dispose of Registered Shares represented by ADSs as and to the extent that they may individually determine. Such dispositions, if any, will be made through brokerage transactions on Nasdaq or other securities exchanges in the United States at prevailing market prices, and the post-offering ownership figures in these columns represent the lowest level of ownership that would exist if the Registered Holders sold 100% of the Registered Shares owned by them, which may or may not happen.

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(1)
Consists of an aggregate of 64,796,247 ordinary shares that are registered hereby, including 43,286,346 ordinary shares and 21,509,901 ordinary shares issuable upon conversion of Convertible Notes held by the following affiliates of Athyrium: Athyrium Opportunities II Acquisition 2 LP, Athyrium Opportunities III Acquisition 2 LP, Athyrium Opportunities II Acquisition LP and Athyrium Opportunities III Acquisition LP.

(2)
Consists of an aggregate of 36,342,967 ordinary shares that are registered hereby, including 10,954,293 ordinary shares, 12,422,154 ordinary shares issuable upon conversion of Convertible Notes, and 12,966,520 ordinary shares issuable upon exercise of zero cost warrants held by the following affiliates of Highbridge: Highbridge MSF International Ltd., Highbridge SCF Special Situations SPV, L.P. and Highbridge Tactical Credit Master Fund, L.P.

(3)
Consists of an aggregate of 13,932,851 ordinary shares that are registered hereby, including 6,309,224 ordinary shares, 3,393,874 ordinary shares issuable upon conversion of Convertible Notes, and 4,229,753 ordinary shares issuable upon exercise of zero cost warrants held by the following affiliates of UBS: Nineteen77 Global Multi-Strategy Alpha Master Limited and Nineteen77 Global Convertible Bond Master Limited.

(4)
Consists of 3,507,080 ordinary shares that are registered hereby, as well as (i) 171,760 share options exercisable at £1.2072 per share, which expire on November 28, 2024 and (ii) 79,010 share options exercisable at £0.758 per share, which expire on May 21, 2026, that are not registered hereby.

(5)
Consists of 1,610,770 ordinary shares that are registered hereby, as well as (i) 68,704 share options exercisable at £1.2072 per share, which expire on November 28, 2024 and (ii) 62,979 share options exercisable at £0.758 per share, which expire on May 21, 2026, that are not registered hereby.

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DESCRIPTION OF SHARE CAPITAL AND CERTAIN CORPORATE MATTERS

The following information is a summary of the material terms of our share capital, consisting of ordinary shares with a nominal (i.e., par) value of £0.06 each, as specified in our articles of association (‘‘Articles”). We are incorporated as a public company with limited liability and our affairs are governed by our Articles and the laws of England and Wales.

The following description summarizes the most important rights attached to our share capital that are currently in effect and are expected to be in effect upon effectiveness of this registration statement. Because it is only a summary, it does not contain all of the information that may be important to you. For a complete description of the rights attaching to our share capital, you should refer to the Articles, a copy of which is included as an exhibit to the registration statement of which this prospectus forms a part, and to the applicable provisions of the Companies Act.

We are not permitted under English law to hold our own shares unless they are repurchased by us and held in treasury.

General

We were incorporated under the Companies Act on July 17, 2019 as a private company limited by shares under the name Amryt Pharma Holdings Limited, with company number 12107859.

We were re-registered as a public limited company on September 13, 2019 under the name Amryt Pharma Holdings plc. On September 24, 2019, in connection with the scheme of arrangement under which we acquired Aegerion, we became the parent company of our legacy businesses and changed our name to Amryt Pharma plc. The principal legislation under which we operate, and under which the share capital has been (and will be) created, is the Companies Act and regulations made thereunder.

Share Capital

As of January 1, 2020, our issued share capital consisted of 159,363,543 fully paid ordinary shares, including 4,864,656 ordinary shares held as treasury shares. Our share capital was issued in the following transactions:

• On the date of our incorporation, being July 17, 2019, we issued one ordinary share.

• On September 12, 2019, we issued one redeemable share with a nominal value of £49,999.94. This has subsequently been redeemed on November 7, 2019 and, upon redemption, the redeemable share was cancelled and our issued share capital was diminished accordingly by the nominal value of the redeemable share.

• On September 24, 2019, we issued 53,149,070 ordinary shares to former holders of Amryt Pharma Holdings Limited. The ordinary shares were issued by resolution of the Board, as authorized pursuant to a resolution passed by the holders of our ordinary shares at the general meeting held on September 23, 2019.

• On September 24, 2019, we issued 77,027,423 ordinary shares (including 48,739,975 ordinary shares represented by 9,747,995 ADSs) and 8,065,000 zero cost warrants to former creditors of Aegerion as consideration for the Acquisition. The ordinary shares and warrants were issued by resolution of the Board, as authorized pursuant to a resolution passed by the holders of our ordinary shares at the general meeting held on September 23, 2019.

• On September 24, 2019, we issued 27,541,944 ordinary shares (including 1,693,275 ordinary shares represented by 338,655 ADSs) and 5,911,722 zero cost warrants in connection with a $60 million equity offering to new and existing investors and former creditors of Aegerion. The ordinary shares and warrants were issued by resolution of the Board, as authorized pursuant to a resolution passed by the holders of our ordinary shares at our general meeting held on September 23, 2019.

• On November 14, 2019, we repurchased 4,864,656 ordinary shares from Highbridge Tactical Master Fund L.P., Highbridge SCF Special Situations SPV, L.P. and Nineteen77 Global Multi Strategy Alpha Master Limited. In exchange for the ordinary shares, these institutions were issued an equivalent number of zero cost warrants. Each warrant entitles the holder to subscribe for one ordinary share at zero cost. The repurchased ordinary shares are now held as treasury shares. On December 19, 2019, these holders elected to exercise 1,645,105 warrants in exchange for 1,645,105 ordinary shares.

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• On December 19, 2019, Highbridge MSF International Ltd exercised 1,645,105 warrants in exchange for 1,645,105 ordinary shares.

Rights of Holders

The following summarizes the rights of holders of our ordinary shares:

• subject to any special rights or restrictions as to voting attached to any shares, on a show of hands every holder who (being an individual) is present in person or by proxy not being himself or herself a holder or (being a corporation) is present by a representative or by proxy not being himself or herself a holder shall have one vote and on a poll every holder who is present in person or by proxy shall have one vote for every share of which he or she is the holder; and

• holders have the right to receive notice of, attend and vote at general meetings; the right to participate in our profits and receive such dividends as are recommended by the directors, pro rata according to the amount paid up on the shares during the period in respect of which the dividend is paid; and, on a winding up or return of capital or otherwise, the right to repayment of the amounts paid up or credited as paid up on the shares and the right to participate pro rata in any excess assets.

Share Registration

We are required by the Companies Act to keep a register of shareholders. Under English law, shares are taken to be allotted when a person acquires the unconditional right to be included in the company’s register of holders in respect of the shares and the shares are deemed to be issued when the name of the holder is entered into the company’s register of holders. The register of holders therefore is prima facie evidence of the identity of our holders and the shares that they hold. The register of holders generally provides limited, or no, information regarding the ultimate beneficial owners of our ordinary shares. The register of holders is maintained by our registrar, Link Asset Services Limited.

Holders of our ADSs will not be treated as shareholders and their names will therefore not be entered in our register of holders. The depositary, the custodian or their nominees will be the holder of the ordinary shares underlying our ADSs. For discussion on our ADSs and ADS holder rights see “Description of American Depositary Shares” in this prospectus. Holders of our ADSs have a right to receive the ordinary shares underlying their ADSs as discussed in “Description of American Depositary Shares”. Each ADS represents the right to receive, and to exercise the beneficial ownership interests in, five ordinary shares that are on deposit with the depositary and/or custodian.

Under the Companies Act, we must enter an allotment of any shares in our register of holders as soon as practicable and in any event within two months of the allotment. We are also required under the Companies Act to register a transfer of any shares (or give the transferee notice of and reasons for refusal) as soon as practicable and in any event within two months after the date on which the transfer is lodged with us.

We, any of our holders and any other aggrieved person may apply to court for rectification of the register of holders if:

• the name of any person, without sufficient cause, is entered in or omitted from our register of holders; or

• there is a default or unnecessary delay in entering on the register the fact of any person having ceased to be a holder.

Preemptive Rights

English law generally provides holders with preemptive rights when new shares are issued for cash; however, it is possible for the articles of association, or holders in a general meeting by way of a special resolution, to exclude preemptive rights. Such an exclusion of preemptive rights may be for a maximum period of up to five years from the date of adoption of the articles of association, if the exclusion is contained in the articles of association, or from the date of the holder resolution, if the exclusion is by holder resolution. In either case, this exclusion would need to be renewed by our holders upon its expiration (i.e., at least every five years). Typically UK public companies seek the disapplication of preemption rights (in relation to a specified aggregate nominal

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amount) on an annual basis at their annual general meeting. There are no provisions in the Articles which prescribe any right of preemption in relation to offers for subscription of securities in the same class. We are subject to the statutory preemption rights set out in the Companies Act.

Options and Warrants

Directors may issue options or warrants if authorized to do so by the company’s articles or an ordinary resolution of the shareholders. We may issue shares upon the exercise of options or warrants without further shareholder authorization, up to the relevant authorized limit.

Options

As of December 31, 2019, there were options to purchase 14,481,720 ordinary shares outstanding with a weighted average exercise price of £1.16 per ordinary share. The options generally lapse after seven years from the date of the grant. The outstanding options were issued under the Share Option Plan.

Warrants

As of December 31, 2019, there were two categories of warrants to purchase our ordinary shares outstanding:

• Warrants issued on September 24, 2019 to purchase 345,542 ordinary shares with a weighted average exercise price of £1.44 per ordinary share. These warrants generally lapse after five years from the date of the grant. These warrants were issued to Shore Capital and Davy in exchange for warrants granted to such parties on April 19, 2016 in connection with the placement undertaken by us on that date.

• Warrants to purchase 17,196,273 ordinary shares, with an exercise price of zero. These warrants were issued (i) to certain Aegerion creditors who expressed a wish to restrict their percentage share interest in our issued share capital and who elected to take warrants in lieu of ordinary shares, and (ii) to certain investors in exchange for the repurchase of 4,864,656 ordinary shares by us from these investors on November 14, 2019 and who subsequently, on December 19, 2019, elected to exercise 1,645,105 warrants in exchange for 1,645,105 ordinary shares.

Contingent Value Rights

On September 24, 2019, we issued CVRs with a potential value of $85 million if all three triggering events under the CVRs occur to holders of shares and options pursuant to the terms of the scheme of arrangement and the Acquisition. The following CVRs were issued by resolution of the Board, as authorized pursuant to a resolution passed by our shareholders at the general meeting held on September 23, 2019:

• 53,149,070 EMA CVRs, which entitle their holders to a payment of up to $15 million in the aggregate (in satisfaction of which the Company may elect to issue loan notes or ordinary shares) upon the EMA issuing a qualifying approval (i.e., an approval or marketing authorization issued by the EMA in relation to the sale by us of AP101 to consumers for medical purposes which satisfies a certain criteria in respect of AP101) if such qualifying approval is obtained by December 31, 2021. The amount payable reduces on a straight-line basis if the qualifying approval is obtained after December 31, 2021 but prior to July 1, 2022;

• 53,149,070 FDA CVRs, which entitle their holders to a payment of up to $35 million in the aggregate (in satisfaction of which the Company may elect to issue loan notes or ordinary shares) upon the FDA issuing a qualifying approval (i.e., an approval or marketing authorization issued by the FDA in relation to the sale by us of AP101 to consumers for medical purposes which satisfies a certain criteria in respect of AP101) if qualifying approval is obtained by December 31, 2021. The amount payable reduces on a straight-line basis if the qualifying approval is obtained after December 31, 2021 but prior to July 1, 2022; and

• 53,149,070 Revenue CVRs, which entitle their holders to a payment of up to $35 million in the aggregate (in satisfaction of which the Company may elect to issue loan notes or ordinary shares) upon the generation of certain revenues from sales of AP101 in trailing 12-month revenues in any period prior to June 30, 2024.

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Articles of Association

Our Articles were adopted on September 23, 2019 (effective from September 24, 2019).

The following is a description of our Articles as currently in effect. Unless noted otherwise, references in this prospectus to the Articles refer to the Articles as currently in effect.

Application of Contractual Agreements with Holders

The Articles are subject to any contractual agreement we entered into with any of the holders of our shares (in their capacity as our shareholders only).

Shares and Rights Attaching to Them

Holders of our ordinary shares have the right to receive notice of, attend and vote at general meetings, the right to participate in our profits and, on a winding up or return of capital or otherwise, the right to the amounts paid up or credited as paid up to the shares and the right to participate pro rata in any excess assets.

Variation of Share Rights

The rights attached to any class may, subject to the provisions of the Companies Act, be varied in such manner (if any) as may be provided by such rights or in the absence of such variation rights either with the consent in writing of the holders of not less than three-quarters in nominal value of the issued shares of the class or with the sanction of a special resolution passed at a separate general meeting of the holders of the shares of the class.

Increase in Share Capital

We may from time to time by ordinary resolution increase our capital by such sum to be divided into shares of such amounts and carrying such rights as the resolution may prescribe.

Consolidation, Cancellation and Subdivision

Subject to the provisions of the Companies Act, we may, by ordinary resolution:

• consolidate and divide all or any of our share capital into shares of larger nominal value than our existing shares;

• cancel any shares which, at the date of the passing of the resolution, have not been taken or agreed to be taken; and

• sub-divide our shares into shares of smaller nominal value than our existing shares.

Reduction

Subject to the provisions of the Companies Act, we may by special resolution reduce our share capital or any capital revaluation reserve or share premium account in any manner. We completed a capital reduction of our share premium account effected by a resolution passed by our shareholders at the general meeting held on September 23, 2019, and which was formally approved at the UK High Court of Justice on November 5, 2019.

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Votes of Members

Votes Attaching to Shares

Subject to any special rights or restrictions as to voting attached to any shares, on a show of hands every holder who (being an individual) is present in person or by proxy not being himself or herself a holder or (being a corporation) is present by a representative or by proxy not being himself or herself a holder shall have one vote and on a poll every holder who is present in person or by proxy shall have one vote for every share of which he or she is the holder.

Votes on a Poll

At a general meeting, a resolution put to the vote of the meeting shall be decided on a show of hands, unless before or upon the declaration of the result of the show of hands a poll is demanded by (i) the chairperson of the meeting, (ii) not less than five holders having the right to vote at the meeting, (iii) holder(s) representing not less than one tenth of the total voting rights of all the holders having the right to vote at the meeting; or (iv) holder(s) of shares conferring a right to vote, being shares on which an aggregate sum has been paid up to not less than one tenth of the total sum paid up on all the shares conferring that right. On a poll, votes may be given either personally or by proxy. On a poll, a holder entitled to more than one vote need not cast all the votes in the same way.

Restrictions on Voting

No holder shall, unless determined otherwise by the directors, be entitled to vote at any general meeting in respect of any share held by him or her, either personally or by proxy, or to exercise any privilege as a member, if:

• any call or other sum presently payable by him or her to us in respect of the shares remains unpaid; or

• the holder has been served with a notice under section 793 of the Companies Act and he or she has failed to provide us with information concerning interests in those shares required to be provided under the Companies Act.

Dividends

We may by ordinary resolution at a general meeting declare dividends out of profits available for distribution in accordance with the respective rights of holders but no such dividend shall be payable other than in accordance with the Companies Act or exceed the amount recommended by the directors. There is no fixed date on which entitlement to dividends arises.

All dividends shall be declared and paid according to the amounts paid-up on the shares in respect of which the dividend is paid. All dividends shall be apportioned and paid pro rata according to the amount paid up on the shares during any portion or portions of the period in respect of which the dividend is paid.

Insofar as, in the opinion of the directors, our profits justify such payments, the directors may pay interim dividends on shares of any class. Subject to their judgment, the directors may also pay half yearly or at other suitable intervals, any dividend which may be payable at a fixed rate.

The directors may deduct from any dividend or other monies payable on or in respect of a share all sums of money (if any) presently payable by the holder to us on account of calls or otherwise in relation to our shares. The directors may withhold dividends payable on shares after there has been failure to provide us with information concerning interests in those shares required to be provided under the Articles or the Companies Act until such failure has been remedied.

No dividend or other monies payable in respect of a share shall bear interest as against us unless otherwise provided by the rights attached to the share. Any dividend unclaimed after a period of 12 years from the date of its declaration shall be forfeited and cease to remain owing by us and shall then belong to us absolutely.

We may upon the recommendation of the directors by ordinary resolution at a general meeting direct payment of a dividend in whole or in part by the distribution of specific assets (and in particular of paid up shares or debentures of any other company) and the directors shall give effect to such resolution.

There are no dividend restrictions or procedures for non-resident holders.

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Uncertificated Shares

Subject to the Companies Act, the directors may permit title to shares of any class to be issued or held otherwise than by a certificate and to be transferred by means of a “relevant system” (i.e., the CREST System) without a certificate. The directors have power to implement such arrangements as they think fit in order for any class of shares to be a participating security (subject always to the Uncertificated Securities Regulations 2001 (SI 2001 No. 01/378), as amended (“CREST Regulations”)).

Change of Control

There are no provisions in the Articles which set out any ownership threshold above which share ownership must be disclosed. See, however, “Other UK Law Considerations” below.

Forfeiture or Lien

Notice on Failure to Pay a Call

If a holder fails to pay any call or “installment” of a call on the due date of payment, the directors may, at any time after the failure, serve a notice on him or her requiring payment and shall state that in the event of non-payment in accordance with such notice the shares on which the call was made will be liable to be forfeited.

Lien on Partly-Paid Shares

We shall have a first and paramount lien on every share (not being a fully paid share) for all monies (whether presently payable or not) called or payable at a fixed time in respect of such share.

Sale of Shares Subject to Lien

We may sell in such manner as the directors think fit any share on which we have a lien 14 days after a notice in writing stating and demanding payment of the sum presently payable and giving notice of intention to sell in default.

Distributions on Winding Up

If we shall be wound up, the liquidator may, with the authority of a special resolution and subject to any sanction required by the Companies Act, divide among the holders in specie the whole or any part of our assets and whether or not the assets shall consist of property of one kind or shall consist of property of different kinds, and may for such purpose set such value as he or she deems fair upon any one or more class or classes or property and may determine how such divisions shall be carried out as between the holders or different classes of holders. The liquidator may, with the like authority, vest any part of the assets in trustees upon such trusts for the benefit of holders as the liquidator shall think fit but so that no holder shall be compelled to accept any shares in respect of which there is a liability.

Capitalization of Profits and Reserves

The directors may, with the authority of an ordinary resolution at a general meeting, capitalize any sum standing to the credit of any of our reserve accounts (including our share premium account and capital redemption reserve) or any sum standing to the credit of our profit and loss account.

Such capitalization shall be effected by appropriating such sum to the holders of ordinary shares in proportion to their holdings of ordinary shares and applying such sum on their behalf in paying up in full unissued shares to be allotted as fully paid to those holders who would have been entitled to that sum if it were distributed by way of dividend.

Transfer of Shares

Transfers of shares may be effected by transfer in writing in any usual or common form or in any other form acceptable by the directors or as required by any rules from time to time made by any operator of a relevant system as defined by the CREST Regulations. The instrument of transfer shall be signed by or on behalf of the transferor and (except in the case of fully paid shares) by or on behalf of the transferee. The transferor shall be deemed to remain the holder of the shares until the name of the transferee is entered in the register in respect of such shares. All instruments of transfer which are registered may be retained by us.

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The directors may refuse to register any transfer of any share which is not a fully paid share provided that the directors shall not refuse to register any transfer or renunciation of partly paid shares in breach of AIM Rules. The directors may also decline to register a transfer of shares (except for certain types of transfer) after there has been a failure to provide us with information concerning interests in those shares required to be provided under the Articles or the Companies Act until such failure has been remedied.

Conversion

There are no provisions in the Articles which prescribe any rights of conversion in relation to any class of shares.

Holder Meetings

In accordance with the Companies Act, we are required in each year to hold an annual general meeting in addition to any other meeting held in that year and within six months of the end of any financial period provided that not more than 15 months has elapsed between the date of one annual general meeting and the next. The annual general meeting shall be convened whenever and wherever the Board sees fit, subject to the requirements of the Companies Act, as described in “—Differences in Corporate Law—Annual General Meeting” and “—Differences in Corporate Law—Notice of General Meetings” in this prospectus.

The directors may, whenever they think fit, and shall on requisition in accordance with the Companies Act, proceed to convene a general meeting.

Notices

An annual general meeting shall be called by at least 21 days’ notice and all other general meetings shall be called by at least 14 days’ notice in each case (exclusive of the day on which the notice is served (or deemed served) and the day for which the notice is given).

The notice must be in writing and must specify the place, day and time of the meeting, the general nature of the business and, in the case of an annual general meeting, shall specify the meeting as such. We may give notice by electronic communication and/or by making it available on our website.

Directors

Number of Directors

Unless otherwise determined by special resolution of the shareholders, the directors shall not be fewer than two or more than seven in number.

Directors’ Renumeration

The ordinary remuneration of the executive directors shall from time to time be determined by the directors who may delegate their authority.

Directors’ Expenses

The directors are entitled to be paid all expenses properly incurred in attending meetings of the Board or of any committee of the Board or holders’ meetings or otherwise in connection with our business.

Retirement by Rotation

At each of our annual general meetings that occurs after September 25, 2021, one-third of the directors for the time being (or, if their number is not a multiple of three, the number nearest to but not greater than one-third, subject to a minimum of one) shall retire from office by rotation. A director retiring by rotation shall be eligible for re-election.

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Restrictions on Voting

A director shall not vote (save as provided in the Articles) in respect of any contract or arrangement or any other proposal in which he or she has a material interest. A director shall not be counted in the quorum at a meeting in relation to any resolution on which he or she is not entitled to vote. A director shall (in the absence of some other material interest than is indicated above) be entitled to vote (and be counted in the quorum) in respect of any resolution:

• relating to the giving of any security, guarantee or indemnity:

• to him or her in relation to money lent or obligations incurred by him or her at the request of or for the benefit of us or any of our subsidiaries; or

• to a third party in relation to our or any of our subsidiaries’ debt or obligation for which he himself or she herself has assumed responsibility in whole or part by the giving of security under a guarantee or indemnity;

• where we or any of our subsidiaries is offering securities in which offer the director is or is to be interested directly or as a participant in the underwriting or sub-underwriting;

• relating to another company in which he or she does not hold an interest in shares representing 1% or more of either class of the equity share capital, or the voting rights in such company;

• relating to a superannuation fund, retirement benefits scheme, share option scheme or share incentive scheme under which he or she may benefit; or

• concerning the purchase and/or maintenance of any insurance policy under which he or she may benefit.

Indemnities

To the extent permitted by law, each of our directors, alternate directors, officers or employees is entitled to be indemnified out of our assets against all losses or liabilities which he or she may sustain or incur in or about the execution of the duties of his or her office or otherwise in relation thereto. To the extent permitted by law, the directors have the power to purchase and maintain insurance for or for the benefit of any persons who are or were at any time:

• directors, alternate directors, officers or employees of a group company; or

• trustees of any pension fund in which our employees or employees of any other group company are interested, including in each case insurance against any liability incurred by such persons in respect of any act or omission in the actual or purported execution and/or discharge of their duties and/or in the exercise or purported exercise of their powers and/or otherwise in relation to their duties, powers or offices in relation to us or any other group company or pension fund.

Borrowing Powers

The directors may exercise all of our powers to borrow money, to guarantee and to mortgage or charge our undertaking, property, assets and uncalled capital and, subject to the Companies Act, to issue debentures and other securities, whether outright or as collateral security, for any of our or any third party’s debt, liability or obligation.

Disclosure of Interest in Shares

We may give notice pursuant to article 78 of the Articles and section 793 of the Companies Act to any person whom we know or have reasonable cause to believe:

• to be interested in our shares; or

• to have been so interested at any time in the three years immediately preceding the date on which the notice is to be issued.

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The notice may require the person to:

• confirm that fact or (as the case may be) to state whether or not it is the case; and

• if he or she holds, or has during that time held, any such interest, to give such further information as may be required in accordance with section 793 of the Companies Act (including particulars of the interest (past or present) and the identity of the persons interested in the shares in question).

If we have served a disclosure notice on a person appearing to be interested in specified shares and we have not received the information required in the disclosure notice within the relevant period (as defined below) after service of the disclosure notice, subject to the other provisions of the Articles, the holder holding the specified shares shall not be entitled to vote at general meetings and, in addition, if the holder holds 0.25% or more of any class of shares, such holder shall not be entitled to receive any dividend in respect of such shares or to transfer or agree to transfer such shares or rights therein. For the purposes of this paragraph, “relevant period” shall be: (i) 28 days, if a holder holds less than 0.25% of any class of shares; or (ii) 14 days, if a holder holds 0.25% or more of any class of shares.

Other UK Law Considerations

Notification of Voting Rights

A holder of our shares, as a holder in a public company incorporated in the United Kingdom whose shares are admitted to trading on AIM is required pursuant to Chapter 5 of the Disclosure Guidance and Transparency Rules of the UK Financial Conduct Authority to notify us of the percentage of his or her voting rights if the percentage of voting rights which he or she holds as a holder or through his or her direct or indirect holding of financial instruments (or a combination of such holdings) reaches, exceeds or falls below 3%, 4%, 5%, and each 1% threshold thereafter up to 100%, as a result of an acquisition or disposal of shares or financial instruments or as a result of events changing the breakdown of the voting rights.

Mandatory Purchases and Acquisitions

Pursuant to Sections 979 to 991 of the Companies Act, where a takeover offer has been made for us and the offeror has acquired or unconditionally contracted to acquire not less than 90% in value of the shares to which the offer relates and, where the shares to which the offer relates are voting shares, not less than 90% of the voting rights carried by those shares, the offeror may give notice to the holder of any shares to which the offer relates which the offeror has not acquired or unconditionally contracted to acquire that he or she wishes to acquire, and is entitled to so acquire, those shares on the same terms as the general offer. Such notice must be sent within three months of the last day on which the offer can be accepted in the prescribed manner. The squeeze-out of the minority holders can be completed at the end of six weeks from the date the notice has been given, subject to the minority holders failing to successfully lodge an application to the court to prevent such squeeze-out any time prior to the end of those six weeks, following which the offeror can execute a transfer of the outstanding shares in its favor and pay the consideration to us, which we would then hold on trust for the outstanding minority holders. The consideration offered to the outstanding minority holders whose shares are compulsorily acquired under the Companies Act must, in general, be the same as the consideration that was available under the takeover offer.

Sell Out

The Companies Act also gives our minority holders a right to be bought out in certain circumstances by an offeror who has made a takeover offer for all of our shares. The holder of shares to which the offer relates, and who has not otherwise accepted the offer, may require the offeror to acquire his or her shares if, prior to the expiration of the acceptance period for such offer, the offeror has acquired or unconditionally agreed to acquire (i) not less than 90% in value of the voting shares, and (ii) not less than 90% of the voting rights carried by those shares. The offeror may impose a time limit on the rights of minority holders to be bought out that is not less than three months after the end of the acceptance period. If a holder exercises his or her rights to be bought out, the offeror is required to acquire those shares on the terms of the offer or on such other terms as may be agreed.

Purchase of Own Shares

Under English law, a limited company may only purchase its own shares out of the distributable profits of the company or the proceeds of a fresh issue of shares made for the purpose of financing the purchase, provided that they are not restricted from doing so by their articles. A limited company may not purchase its own shares if, as

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a result of the purchase, there would no longer be any issued shares of the company other than redeemable shares or shares held as treasury shares. Shares must be fully paid in order to be repurchased. Subject to the foregoing, we may purchase or may enter into any contract under which we will or may purchase, any of our own shares, including any redeemable shares. We completed a capital reduction of our share premium account which was formally approved at the UK High Court of Justice on November 5, 2019 following which we now have positive distributable reserves. Further, pursuant to an authority conferred by our shareholders at a general meeting on September 23, 2019, we agreed to repurchase 4,864,656 ordinary shares from Highbridge Tactical Master Fund L.P., Highbridge SCF Special Situations SPV, L.P. and Nineteen77 Global Multi Strategy Alpha Master Limited. In exchange for the ordinary shares, these institutions were issued an equivalent number of zero cost warrants. Each warrant entitles the holder to subscribe for one ordinary share at zero cost. These ordinary shares are now held as treasury shares.

Distributions and Dividends

Under the Companies Act, before a company can lawfully make a distribution or dividend, it must ensure that it has sufficient distributable reserves (on a non-consolidated basis). A company’s profits available for the purpose of making a distribution are its accumulated, realized profits, so far as not previously utilized by distribution or capitalization, less its accumulated, realized losses, so far as not previously written off in a reduction or reorganization of capital. The requirement to have sufficient distributable reserves before a distribution or dividend can be paid applies to us and to each of our subsidiaries that has been incorporated under English law.

It is not sufficient that we have distributable profits available for the purpose of making a distribution. As a public company, an additional capital maintenance requirement is imposed on us to ensure that the net worth of the company is at least equal to the amount of its capital. A public company can only make a distribution:

• if, at the time that the distribution is made, the amount of its net assets (that is, the aggregate of the company’s assets less the aggregate of its liabilities) is no less than the aggregate of its called up share capital and undistributable reserves; and

• if, and to the extent that, the distribution itself, at the time that it is made, does not reduce the amount of the net assets to less than that total.

City Code on Takeovers and Mergers

As a public company incorporated in England and Wales with our registered office in England and Wales which has shares admitted to AIM, we are subject to the UK City Code on Takeovers and Mergers (“City Code”), which is issued and administered by the UK Panel on Takeovers and Mergers (“Panel”). The City Code provides a framework within which takeovers of companies subject to it are conducted. In particular, the City Code contains certain rules in respect of mandatory offers. Under Rule 9 of the City Code, if a person:

• acquires an interest in our shares which, when taken together with shares in which he or she or persons acting in concert with him or her are interested, carries 30% or more of the voting rights of our shares; or

• who, together with persons acting in concert with him or her, is interested in shares that in the aggregate carry not less than 30% and not more than 50% of the voting rights of our shares, and such persons, or any person acting in concert with him or her, acquires additional interests in shares that increase the percentage of shares carrying voting rights in which that person is interested,

the acquirer and depending on the circumstances, its concert parties, would be required (except with the consent of the Panel) to make a cash offer for our outstanding shares at a price not less than the highest price paid for any interests in the shares by the acquirer or its concert parties during the previous 12 months.

Exchange Controls

There are no governmental laws, decrees, regulations or other legislation in the United Kingdom that may affect the import or export of capital, including the availability of cash and cash equivalents for use by us, or that may affect the remittance of dividends, interest, or other payments by us to non-resident holders of our ordinary shares or ADSs, other than withholding tax requirements. There is no limitation imposed by English law or in the Articles on the right of non-residents to hold or vote shares. However, where a holder has a registered address within the United Kingdom and has not notified us of an address within the United Kingdom at which notices may be given to him or her, such holder shall not be entitled to receive any notice.

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Differences in Corporate Law

The applicable provisions of the Companies Act and other laws applicable to English public limited companies and their shareholders differ from laws applicable to U.S. corporations and their shareholders. Set forth below is a summary of certain differences between the provisions of the Companies Act applicable to us and the General Corporation Law of the State of Delaware relating to shareholders’ rights and protections. This summary is not intended to be a complete discussion of the respective rights and it is qualified in its entirety by reference to Delaware law and English law.

	England and Wales	Delaware
Number of Directors	Under the Companies Act, a public limited company must have at least two directors and the number of directors may otherwise be fixed by or in the manner provided in the company’s articles of association.	Under Delaware law, a corporation must have at least one director and the number of directors shall otherwise be fixed by or in the manner provided in the bylaws.
Removal of Directors	Under the Companies Act, shareholders may remove a director without cause by an ordinary resolution (which is passed by a simple majority of those voting in person or by proxy at a general meeting) irrespective of any provisions of any service contract the director has with the company, provided 28 clear days’ notice of the resolution has been given to the company and the company must, where practicable, give its shareholders notice of such resolution in the same manner and at the same time as it gives notice of the meeting. Where that is not practicable, the company must give its shareholders notice at least 14 days before the meeting. On receipt of notice of an intended resolution to remove a director, the company must forthwith send a copy of the notice to the director concerned. Certain other procedural requirements under the Companies Act must also be followed such as allowing the director to make representations against his or her removal either at the meeting or in writing.	Under Delaware law, any director or the entire board of directors may be removed, with or without cause, by the holders of a majority of the shares then entitled to vote at an election of directors, except (a) unless the certificate of incorporation provides otherwise, in the case of a corporation whose board of directors is classified, shareholders may effect such removal only for cause, or (b) in the case of a corporation having cumulative voting, if less than the entire board of directors is to be removed, no director may be removed without cause if the votes cast against his or her removal would be sufficient to elect him or her if then cumulatively voted at an election of the entire board of directors, or, if there are classes of directors, at an election of the class of directors of which he or she is a part.
Vacancies on the Board of Directors	Under English law, the procedure by which directors, other than a company’s initial directors, are appointed is generally set out in the company’s articles of association, provided that where two or more persons are appointed as directors of a public limited company by resolution of the shareholders, resolutions appointing each director must be voted on individually.	Under Delaware law, vacancies and newly created directorships may be filled by a majority of the directors then in office (even though less than a quorum) or by a sole remaining director unless (a) otherwise provided in the certificate of incorporation or by-laws of the corporation or (b) the certificate of incorporation directs that a particular class of stock is to elect such director, in which case a majority of the other directors elected by such class, or a sole remaining director elected by such class, will fill such vacancy.

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	England and Wales	Delaware
Annual General Meeting	Under the Companies Act, a public limited company must hold an annual general meeting in each six-month period beginning with the day following the company’s annual accounting reference date.	Under Delaware law, the annual meeting of stockholders shall be held at such place, on such date and at such time as may be designated from time to time by the board of directors or as provided in the certificate of incorporation or by the bylaws.
General Meeting	Under the Companies Act, a general meeting of the shareholders of a public limited company may be called by the directors.   In addition, shareholders holding at least 5% of the paid-up capital of the company carrying voting rights at general meetings (excluding any paid-up capital held as treasury shares) can require the directors to call a general meeting and, if the directors fail to do so within a certain period, the requisitionists (or any of them representing more than one-half of the total voting rights of all of them) may convene a general meeting.	Under Delaware law, special meetings of the stockholders may be called by the board of directors or by such person or persons as may be authorized by the certificate of incorporation or by the bylaws.
Notice of General Meetings	Under the Companies Act, at least 21 clear days’ notice must be given for an annual general meeting and any resolutions to be proposed at the meeting, subject to a company’s articles of association providing for a longer period. Subject to a company’s articles of association providing for a longer period, at least 14 clear days’ notice is required for any other general meeting. In addition, certain matters, such as the removal of directors or auditors, require special notice, which is 28 clear days’ notice. The shareholders of a company may in all cases consent to a shorter notice period, the proportion of shareholders’ consent required being 100% of those entitled to attend and vote in the case of an annual general meeting and, in the case of any other general meeting, a majority in number of the members having a right to attend and vote at the meeting, being a majority who together hold not less than 95% in nominal value of the shares giving a right to attend and vote at the meeting (excluding any shares held in the company as treasury shares).	Under Delaware law, unless otherwise provided in the certificate of incorporation or bylaws, written notice of any meeting of the stockholders must be given to each stockholder entitled to vote at the meeting not less than ten nor more than 60 days before the date of the meeting and shall specify the place, date, hour and purpose or purposes of the meeting.

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	England and Wales	Delaware
Quorum	Subject to the provisions of a company’s articles of association, the Companies Act provides that two shareholders present at a meeting (in person, by proxy or authorized representative under the Companies Act) shall constitute a quorum for companies with more than one shareholder.	The certificate of incorporation or bylaws may specify the number of shares, the holders of which shall be present or represented by proxy at any meeting in order to constitute a quorum, but in no event shall a quorum consist of less than one-third of the shares entitled to vote at the meeting. In the absence of such specification in the certificate of incorporation or bylaws, a majority of the shares entitled to vote, present in person or represented by proxy, shall constitute a quorum at a meeting of stockholders.
Proxy	Under the Companies Act, a shareholder may appoint another person to attend, speak and vote at any general meeting on their behalf by proxy.	Under Delaware law, at any meeting of stockholders, a stockholder may designate another person to act for such stockholder by proxy, but no such proxy shall be voted or acted upon after three years from its date, unless the proxy provides for a longer period. A director of a Delaware corporation may not issue a proxy representing the director’s voting rights as a director.
Preemptive Rights	Under the Companies Act, “equity securities,” being (i) shares in the company other than shares that, with respect to dividends and capital, carry a right to participate only up to a specified amount in a distribution, referred to as ordinary shares, or (ii) rights to subscribe for, or to convert securities into, ordinary shares, proposed to be allotted for cash must be offered first to the existing holders of ordinary shares in the company in proportion to the respective nominal value of their holdings, unless an exception applies or a special resolution disapplying such preemptive rights has been passed by shareholders in a general meeting or the articles of association provide for the disapplication of such preemptive rights in each case in accordance with the provisions of the Companies Act.	Under Delaware law, shareholders have no preemptive rights to subscribe to additional issues of stock or to any security convertible into such stock unless, and except to the extent that, such rights are expressly provided for in the certificate of incorporation.

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	England and Wales	Delaware
Authority to Allot	Under the Companies Act, the directors of a public limited company must not allot shares or grant rights to subscribe for or to convert any security into shares unless an exception applies or an ordinary resolution has been passed by shareholders in a general meeting authorizing such allotment or the articles of association provide for such authorization, in each case subject to a specified maximum nominal value and in accordance with the provisions of the Companies Act.	Under Delaware law, if the corporation’s charter or certificate of incorporation so provides, the board of directors has the power to authorize the issuance of stock. The board of directors may authorize capital stock to be issued for consideration consisting of cash, any tangible or intangible property or any benefit to the corporation or any combination thereof. It may determine the amount of such consideration by approving a formula. In the absence of actual fraud in the transaction, the judgment of the directors as to the value of such consideration is conclusive.
Liability of Directors and Officers	Under the Companies Act, any provision, whether contained in a company’s articles of association or any contract or otherwise, that purports to exempt a director of a company, to any extent, from any liability that would otherwise attach to him or her in connection with any negligence, default, breach of duty or breach of trust in relation to the company is void.	Under Delaware law, a corporation’s certificate of incorporation may include a provision eliminating or limiting the personal liability of a director to the corporation and its stockholders for damages arising from a breach of fiduciary duty as a director. However, no provision can limit the liability of a director for:
	In addition, any provision by which a company directly or indirectly provides an indemnity, to any extent, for a director of the company or of an associated company against any liability attaching to him or her in connection with any negligence, default, breach of duty or breach of trust in relation to the company of which he or she is a director is also void except as permitted by the Companies Act, which provides exceptions for the company to (a) purchase and maintain insurance against such liability; (b) provide a “qualifying third party indemnity” (being an indemnity against liability incurred by the director to a person other than the company or an associated company or criminal proceedings in which he or she is convicted); and (c) provide a “qualifying pension scheme indemnity” (being an indemnity against liability incurred in connection with the company’s activities as trustee of an occupational pension plan).	•	any breach of the director’s duty of loyalty to the corporation or its stockholders;
		•	acts or omissions not in good faith or that involve intentional misconduct or a knowing violation of law;
		•	intentional or negligent payment of unlawful dividends or stock purchases or redemptions; or
		•	any transaction from which the director derives an improper personal benefit.

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	England and Wales	Delaware
Voting Rights	For an English company it is usual for the articles of association to provide that, unless a poll is demanded by the shareholders of a company or is required by the chairman of the meeting or the company’s articles of association, shareholders shall vote on all resolutions on a show of hands. On a show of hands every shareholder has one vote (regardless of the number of ordinary shares held) and, subject to the company's articles of association, every proxy appointed by more than one shareholder has one vote unless they have been instructed by different shareholders to vote in different ways (in which case they will have one vote for and one vote against a resolution). Under the Companies Act, a provision of a company's articles is void if it has the effect of making ineffective a demand for a poll by (a) not fewer than five shareholders having the right to vote on the resolution; (b) any shareholder(s) representing not less than 10% of the total voting rights of all the shareholders having the right to vote on the resolution (excluding any voting rights attaching to treasury shares); or (c) any shareholder(s) holding shares in the company conferring a right to vote on the resolution (excluding any voting rights attaching to treasury shares) being shares on which an aggregate sum has been paid up equal to not less than 10% of the total sum paid up on all the shares conferring that right. A company’s articles of association may provide more extensive rights for shareholders to call a poll.   Under English law, ordinary resolutions require the affirmative vote of a simple majority (more than 50%) of the votes cast by shareholders present, in person or by proxy, at the meeting. Special resolutions require the affirmative vote of not less than 75% of the votes cast by shareholders present, in person or by proxy, at the meeting.	Delaware law provides that, unless otherwise provided in the certificate of incorporation, each stockholder is entitled to one vote for each share of capital stock held by such stockholder.

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	England and Wales		Delaware
Shareholder Vote on Certain Transactions	The Companies Act provides for schemes of arrangement, which are arrangements or compromises between a company and any class of shareholders or creditors and used in certain types of reconstructions, amalgamations, capital reorganizations or takeovers. These arrangements require:		Generally, under Delaware law, unless the certificate of incorporation provides for the vote of a larger portion of the stock, completion of a merger, consolidation, sale, lease or exchange of all or substantially all of a corporation’s assets or dissolution requires:
	•	the approval at a shareholders’ or creditors’ meeting convened by order of the court, of a majority in number of shareholders or creditors or a class thereof representing 75% in value of the capital held by, or debt owed to, the class of shareholders or creditors, or class thereof present and voting, either in person or by proxy; and	•	the approval of the board of directors; and
			•	approval by the vote of the holders of a majority of the outstanding stock or, if the certificate of incorporation provides for more or less than one vote per share, a majority of the votes of the outstanding stock of the corporation entitled to vote on the matter.
	•	the approval of the court.

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	England and Wales		Delaware
Standard of Conduct for Directors	Under English law, a director owes various statutory and fiduciary duties to the company, including:		Delaware law does not contain specific provisions setting forth the standard of conduct of a director. The scope of the fiduciary duties of directors is generally determined by the courts of the State of Delaware. In general, directors have a duty to act without self-interest, on a well-informed basis and in a manner they reasonably believe to be in the best interest of the stockholders.   Directors of a Delaware corporation owe fiduciary duties of care and loyalty to the corporation and to its shareholders. The duty of care generally requires that a director act in good faith, with the care that an ordinarily prudent person would exercise under similar circumstances. Under this duty, a director must inform himself or herself of all material information reasonably available regarding a significant transaction. The duty of loyalty requires that a director act in a manner he or she reasonably believes to be in the best interests of the corporation. He or she must not use his corporate position for personal gain or advantage. In general, but subject to certain exceptions, actions of a director are presumed to have been made on an informed basis, in good faith and in the honest belief that the action taken was in the best interests of the corporation. However, this presumption may be rebutted by evidence of a breach of one of the fiduciary duties. Delaware courts have also imposed a heightened standard of conduct upon directors of a Delaware corporation who take any action designed to defeat a threatened change in control of the corporation.   In addition, under Delaware law, when the board of directors of a Delaware corporation approves the sale or break-up of a corporation, the board of directors may, in certain circumstances, have a duty to obtain the highest value reasonably available to the shareholders.
	•	to act in the way he or she considers, in good faith, would be most likely to promote the success of the company for the benefit of its members as a whole (and in doing so have regard (amongst other matters) to: (i) the likely consequences of any decision in the long-term, (ii) the interests of the company’s employees, (iii) the need to foster the company’s business relationships with suppliers, customers and others, (iv) the impact of the company’s operations on the community and the environment, (v) the desirability to maintain a reputation for high standards of business conduct and (vi) the need to act fairly as between members of the company);
	•	to avoid a situation in which he or she has, or can have, a direct or indirect interest that conflicts, or possibly conflicts, with the interests of the company;
	•	to act in accordance with the company’s constitution and only exercise his or her powers for the purposes for which they are conferred;
	•	to exercise independent judgment;
	•	to exercise reasonable care, skill and diligence;
	•	not to accept benefits from a third party conferred by reason of his or her being a director or doing, or not doing, anything as a director; and
	•	a duty to declare any interest that he or she has, whether directly or indirectly, in a proposed or existing transaction or arrangement with the company.

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	England and Wales	Delaware
Stockholder Suits	Under English law, generally, the company, rather than its shareholders, is the proper claimant in an action in respect of a wrong done to the company or where there is an irregularity in the company’s internal management. Notwithstanding this general position, the Companies Act provides that (i) a court may allow a shareholder to bring a derivative claim (that is, an action in respect of and on behalf of the company) in respect of a cause of action arising from a director’s negligence, default, breach of duty or breach of trust and (ii) a shareholder may bring a claim for a court order where the company’s affairs have been or are being conducted in a manner that is unfairly prejudicial to some of its shareholders.	Under Delaware law, a stockholder may initiate a derivative action to enforce a right of a corporation if the corporation fails to enforce the right itself. The complaint must:
		•	state that the plaintiff was a stockholder at the time of the transaction of which the plaintiff complains or that the plaintiffs shares thereafter devolved on the plaintiff by operation of law; and
		•	allege with particularity the efforts made by the plaintiff to obtain the action the plaintiff desires from the directors and the reasons for the plaintiff’s failure to obtain the action; or
		•	state the reasons for not making the effort.
		Additionally, the plaintiff must remain a stockholder through the duration of the derivative suit. The action will not be dismissed or compromised without the approval of the Delaware Court of Chancery.

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DESCRIPTION OF AMERICAN DEPOSITARY SHARES

American Depositary Shares

Citibank, N.A. (“Citibank”) has agreed to act as the depositary for the ADSs. Citibank’s depositary offices are located at 388 Greenwich Street, New York, New York 10013. The ADSs represent ownership interests in securities that are on deposit with the depositary. The ADSs may be represented by certificates that are commonly known as American Depositary Receipts (“ADRs”). The depositary typically appoints a custodian to safekeep the securities on deposit. In this case, the custodian is Citibank, N.A. (London), located at Citigroup Centre, Canary Wharf, London E14 5LB, United Kingdom.

Citibank has been appointed as depositary bank pursuant to a deposit agreement. A copy of the deposit agreement is on file with the SEC under cover of a registration statement on Form F-6. You may obtain a copy of the deposit agreement from the SEC’s Public Reference Room at 100 F Street, N.E., Washington, D.C. 20549 and from the SEC’s website (https://www.sec.gov). Please refer to registration number 333-233844 when retrieving such copy.

This section contains a summary description of the material terms of the ADSs and of your material rights as an owner of ADSs. Please remember that summaries by their nature lack the precision of the information summarized and that the rights and obligations of an owner of ADSs will be determined by reference to the terms of the deposit agreement and not by this summary. We urge you to review the deposit agreement in its entirety.

Each ADS represents the right to receive, and to exercise the beneficial ownership interests in, five ordinary shares that is on deposit with the depositary and/or custodian. An ADS also represents the right to receive, and to exercise the beneficial interests in, any other property received by the depositary or the custodian on behalf of the owner of the ADS but that has not been distributed to the owners of ADSs because of legal restrictions or practical considerations. We and the depositary may agree to change the ADS-to-ordinary share ratio by amending the deposit agreement. This amendment may give rise to, or change, the depositary fees payable by ADS owners. The custodian, the depositary and their respective nominees will hold all deposited property for the benefit of the holders and beneficial owners of ADSs. The deposited property does not constitute the proprietary assets of the depositary, the custodian or their nominees. Beneficial ownership in the deposited property will under the terms of the deposit agreement be vested in the beneficial owners of the ADSs. The depositary, the custodian and their respective nominees will be the record holders of the deposited property represented by the ADSs for the benefit of the holders and beneficial owners of the corresponding ADSs. A beneficial owner of ADSs may or may not be the holder of ADSs. Beneficial owners of ADSs will be able to receive, and to exercise beneficial ownership interests in, the deposited property only through the registered holders of the ADSs, the registered holders of the ADSs (on behalf of the applicable ADS owners) only through the depositary, and the depositary (on behalf of the owners of the corresponding ADSs) directly, or indirectly, through the custodian or their respective nominees, in each case upon the terms of the deposit agreement.

If you become an owner of ADSs, you will become a party to the deposit agreement and therefore will be bound to its terms and to the terms of any ADR that represents your ADSs. The deposit agreement and the ADR specify our rights and obligations as well as your rights and obligations as owner of ADSs and those of the depositary. As an ADS holder you appoint the depositary to act on your behalf in certain circumstances. The deposit agreement and the ADRs are governed by New York law. However, our obligations to the holders of ordinary shares will continue to be governed by the laws of England and Wales, which may be different from the laws in the United States.

In addition, applicable laws and regulations may require you to satisfy reporting requirements and obtain regulatory approvals in certain circumstances. You are solely responsible for complying with such reporting requirements and obtaining such approvals. Neither the depositary, the custodian, we or any of their or our respective agents or affiliates shall be required to take any actions whatsoever on your behalf to satisfy such reporting requirements or obtain such regulatory approvals under applicable laws and regulations.

As an owner of ADSs, you will not be treated as a shareholder and you will not have direct shareholder rights. The depositary will hold on your behalf the shareholder rights attached to the ordinary shares underlying your ADSs. As an owner of ADSs, you will be able to exercise the shareholders rights for the ordinary shares

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represented by your ADSs through the depositary only to the extent contemplated in the deposit agreement. To exercise any shareholder rights not contemplated in the deposit agreement you will, as an ADS owner, need to arrange for the cancellation of your ADSs and become a direct shareholder.

The manner in which you own the ADSs (e.g., in a brokerage account vs. as registered holder, or as holder of certificated vs. uncertificated ADSs) may affect your rights and obligations, and the manner in which, and extent to which, the depositary’s services are made available to you. As an owner of ADSs, you may hold your ADSs either by means of an ADR registered in your name, through a brokerage or safekeeping account, or through an account established by the depositary in your name reflecting the registration of uncertificated ADSs directly on the books of the depositary (commonly referred to as the “direct registration system” or “DRS”). The direct registration system reflects the uncertificated (book-entry) registration of ownership of ADSs by the depositary. Under the direct registration system, ownership of ADSs is evidenced by periodic statements issued by the depositary to the holders of the ADSs. The direct registration system includes automated transfers between the depositary and The Depositary Trust Company (“DTC”), the central book-entry clearing and settlement system for equity securities in the United States. If you decide to hold your ADSs through your brokerage or safekeeping account, you must rely on the procedures of your broker or bank to assert your rights as ADS owner. Banks and brokers typically hold securities such as the ADSs through clearing and settlement systems such as DTC. The procedures of such clearing and settlement systems may limit your ability to exercise your rights as an owner of ADSs. Please consult with your broker or bank if you have any questions concerning these limitations and procedures. All ADSs held through DTC will be registered in the name of a nominee of DTC. This summary description assumes you have opted to own the ADSs directly by means of an ADS registered in your name and, as such, refers to you as the “holder.” When “you” is used, it is assumed that the reader owns ADSs and will own ADSs at the relevant time.

The registration of the ordinary shares in the name of the depositary or the custodian shall, to the maximum extent permitted by applicable law, vest in the depositary or the custodian the record ownership in the applicable ordinary shares with the beneficial ownership rights and interests in such ordinary shares being at all times vested with the beneficial owners of the ADSs representing the ordinary shares. The depositary or the custodian shall at all times be entitled to exercise the beneficial ownership rights in all deposited property, in each case only on behalf of the holders and beneficial owners of the ADSs representing the deposited property.

Dividends and Distributions

As a holder of ADSs, you generally have the right to receive the distributions made on the securities deposited with the custodian. Your receipt of these distributions may be limited, however, by practical considerations and legal limitations. Holders of ADSs will receive such distributions under the terms of the deposit agreement in proportion to the number of ADSs held as of the specified record date, after deduction the applicable fees, taxes and expenses.

Distributions of Cash

If any cash distributions for the securities on deposit with the custodian are made, the funds will be deposited with the custodian. Upon receipt of confirmation of the deposit of the requisite funds, the depositary will arrange for the funds received in a currency other than U.S. dollars to be converted into U.S. dollars and for the distribution of the U.S. dollars to the holders, subject to the laws and regulations of England and Wales. The conversion into U.S. dollars will take place only if practicable and if the U.S. dollars are transferable to the United States. The depositary will apply the same method for distributing the proceeds of the sale of any property (such as undistributed rights) held by the custodian in respect of securities on deposit.

The distribution of cash will be made net of the fees, expenses, taxes and governmental charges payable by holders under the terms of the deposit agreement. The depositary will hold any cash amounts it is unable to distribute in a non-interest bearing account for the benefit of the applicable holders and beneficial owners of ADSs until the distribution can be effected or the funds that the depositary holds must be escheated as unclaimed property in accordance with the laws of the relevant states of the United States.

Distributions of Shares

Whenever a free distribution of ordinary shares for the securities on deposit with the custodian is made, the applicable number of ordinary shares will be deposited with the custodian. Upon receipt of confirmation of such deposit, the depositary will either distribute to holders new ADSs representing the ordinary shares deposited or

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modify the ADS-to-ordinary shares ratio, in which case each ADS you hold will represent rights and interests in the additional ordinary shares so deposited. Only whole new ADSs will be distributed. Fractional entitlements will be sold and the proceeds of such sale will be distributed as in the case of a cash distribution.

The distribution of new ADSs or the modification of the ADS-to-ordinary share ratio upon a distribution of ordinary shares will be made net of the fees, expenses, taxes and governmental charges payable by holders under the terms of the deposit agreement. In order to pay such taxes or governmental charges, the depositary may sell all or a portion of the new ordinary shares so distributed.

No such distribution of new ADSs will be made if it would violate a law (e.g., the U.S. securities laws) or if it is not operationally practicable. If the depositary does not distribute new ADSs as described above, it may sell the ordinary shares received upon the terms described in the deposit agreement and will distribute the proceeds of the sale as in the case of a distribution of cash.

Distributions of Rights

Whenever we intend to distribute rights to subscribe for additional ordinary shares, we will give notice to the depositary and will assist the depositary in determining whether it is lawful and reasonably practicable to distribute rights to subscribe for additional ADSs to holders.

The depositary will establish procedures to distribute rights to subscribe for additional ADSs to holders and to enable such holders to exercise such rights if it is lawful and reasonably practicable to make the rights available to holders of ADSs, and if we provide all of the documentation contemplated in the deposit agreement (such as opinions to address the lawfulness of the transaction). You may have to pay fees, expenses, taxes and other governmental charges to subscribe for the new ADSs upon the exercise of your rights. The depositary is not obligated to establish procedures to facilitate the distribution and exercise by holders of rights to purchase new ordinary shares other than in the form of ADSs. The depositary will not distribute the rights to you if:

• no timely request that the rights be distributed to you is received or if we request that the rights not be distributed to you;

• we fail to deliver satisfactory documents to the depositary; or

• it is not reasonably practicable to distribute the rights.

The depositary will sell the rights that are not exercised or not distributed if such sale is lawful and reasonably practicable. The proceeds of such sale will be distributed to holders as in the case of a cash distribution. If the depositary is unable to sell the rights, it will allow the rights to lapse.

Elective Distributions

Whenever we intend to distribute a dividend payable at the election of shareholders either in cash or in additional shares, we will give prior notice thereof to the depositary and will indicate whether it wishes the elective distribution to be made available to you. In such case, we will assist the depositary in determining whether such distribution is lawful and reasonably practicable.

The depositary will make the election available to you only if it is reasonably practicable and if it has received all of the documentation contemplated in the deposit agreement. In such case, the depositary will establish procedures to enable you to elect to receive either cash or additional ADSs, in each case as described in the deposit agreement.

If the election is not made available to you, you will receive either cash or additional ADSs, depending on what a shareholder in England and Wales would receive upon failing to make an election, as more fully described in the deposit agreement.

Other Distributions

Whenever we intend to distribute property other than cash, ordinary shares or rights to purchase additional ordinary shares, we will notify the depositary in advance and will indicate whether we wish such distribution to be made to you. In such case, we will assist the depositary in determining whether such distribution is lawful and reasonably practicable.

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If it is reasonably practicable to distribute such property to you and if we provide to the depositary all of the documentation contemplated in the deposit agreement, the depositary will distribute the property to the holders in a manner it deems practicable.

The distribution will be made net of fees, expenses, taxes and governmental charges payable by holders under the terms of the deposit agreement. In order to pay such taxes and governmental charges, the depositary may sell all or a portion of the property received.

The depositary will not distribute the property to you and will sell the property if:

• we do not timely request that the property be distributed to you or if we request that the property not be distributed to you;

• we do not deliver satisfactory documents; or

• the depositary determines that all or portion of the distribution to you is not reasonably practicable.

The proceeds of such a sale will be distributed to holders as in the case of a cash distribution.

Redemption

Whenever we decide to redeem any of the ordinary shares on deposit with the custodian, we will notify the depositary in advance. If it is practicable and if we provide all of the documentation contemplated in the deposit agreement, the depositary will provide notice of the redemption to the holders.

The custodian will be instructed to surrender the ordinary shares being redeemed against payment of the applicable redemption price. The depositary will convert the redemption funds received into U.S. dollars upon the terms of the deposit agreement and will establish procedures to enable holders to receive the net proceeds from the redemption upon surrender of their ADSs to the depositary. You may have to pay fees, expenses, taxes and other governmental charges upon the redemption of your ADSs. If less than all ADSs are being redeemed, the ADSs to be retired will be selected by lot or on a pro rata basis, as the depositary may determine.

Changes Affecting Ordinary Shares

The ordinary shares held on deposit for your ADSs may change from time to time. For example, there may be a change in nominal or par value, split-up, cancellation, consolidation or any other reclassification of such ordinary shares or a recapitalization, reorganization, merger, consolidation or sale of assets of our company.

If any such change were to occur, your ADSs would, to the extent permitted by law and the deposit agreement, represent the right to receive the property received or exchanged in respect of the ordinary shares held on deposit. The depositary may in such circumstances deliver new ADSs to you, amend the deposit agreement, the ADRs and the applicable Registration Statement(s) on Form F-6, call for the exchange of your existing ADSs for new ADSs and take any other actions that are appropriate to reflect as to the ADSs the change affecting the ordinary shares. If the depositary may not lawfully distribute such property to you, the depositary may sell such property and distribute the net proceeds to you as in the case of a cash distribution.

Issuance of ADSs Upon Deposit of Ordinary Shares

The depositary may create ADSs on your behalf if you or your broker deposit ordinary shares with the custodian. The depositary will deliver these ADSs to the person you indicate only after you pay any applicable issuance fees and any charges and taxes payable for the transfer of the ordinary shares to the custodian. Your ability to deposit ordinary shares and receive ADSs may be limited by the legal considerations in the United States and England and Wales applicable at the time of deposit.

The issuance of ADSs may be delayed until the depositary or the custodian receives confirmation that all required approvals have been given and that the ordinary shares have been duly transferred to the custodian. The depositary will only issue ADSs in whole numbers.

When you make a deposit of ordinary shares, you will be responsible for transferring good and valid title to the depositary. As such, you will be deemed to represent and warrant that:

• the ordinary shares are duly authorized, validly issued, fully paid, non-assessable and legally obtained;

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• all preemptive (and similar) rights, if any, with respect to such ordinary shares have been validly waived or exercised;

• you are duly authorized to deposit the ordinary shares;

• the ordinary shares presented for deposit are free and clear of any lien, encumbrance, security interest, charge, mortgage or adverse claim, and are not, and the ADSs issuable upon such deposit will not be, “restricted securities” (as defined in the deposit agreement);

• the ordinary shares presented for deposit have not been stripped of any rights or entitlements; and

• the deposit of shares does not violate any applicable provision of English law.

If any of the representations or warranties are incorrect in any way, we and the depositary may, at your cost and expense, take any and all actions necessary to correct the consequences of the misrepresentations.

Transfer, Combination and Split Up of ADRs

As an ADR holder, you will be entitled to transfer, combine or split up your ADRs and the ADSs evidenced thereby. For transfers of ADRs, you will have to surrender the ADRs to be transferred to the depositary and also must:

• ensure that the surrendered ADR is properly endorsed or otherwise in proper form for transfer;

• provide such proof of identity and genuineness of signatures as the depositary deems appropriate;

• provide any transfer stamps required by the State of New York or the United States; and

• pay all applicable fees, charges, expenses, taxes and other government charges payable by ADR holders pursuant to the terms of the deposit agreement, upon the transfer of ADRs.

To have your ADRs either combined or split up, you must surrender the ADRs in question to the depositary with your request to have them combined or split up, and you must pay all applicable fees, charges and expenses payable by ADR holders, pursuant to the terms of the deposit agreement, upon a combination or split up of ADRs.

Withdrawal of Ordinary Shares Upon Cancellation of ADSs

As a holder, you will be entitled to present your ADSs to the depositary for cancellation and then receive the corresponding number of underlying ordinary shares at the custodian’s offices. Your ability to withdraw the ordinary shares held in respect of the ADSs may be limited by the legal considerations in the United States and England and Wales applicable at the time of withdrawal. In order to withdraw the ordinary shares represented by your ADSs, you will be required to pay to the depositary the fees for cancellation of ADSs and any charges and taxes payable upon the transfer of the ordinary shares. You assume the risk for delivery of all funds and securities upon withdrawal. Once canceled, the ADSs will not have any rights under the deposit agreement.

If you hold ADSs registered in your name, the depositary may ask you to provide proof of identity and genuineness of any signature and such other documents as the depositary may deem appropriate before it will cancel your ADSs. The withdrawal of the ordinary shares represented by your ADSs may be delayed until the depositary receives satisfactory evidence of compliance with all applicable laws and regulations. Please keep in mind that the depositary will only accept ADSs for cancellation that represent a whole number of securities on deposit.

You will have the right to withdraw the securities represented by your ADSs at any time except as a result of:

• temporary delays that may arise because (i) the transfer books for the ordinary shares or ADSs are closed, or (ii) ordinary shares are immobilized on account of a shareholders’ meeting or a payment of dividends;

• obligations to pay fees, taxes and similar charges;

• restrictions imposed because of laws or regulations applicable to ADSs or the withdrawal of securities on deposit; and

• other circumstances specifically contemplated by Section I.A.(I) of the General Instructions to Form F-6 (as such General Instructions may be amended from time to time).

The deposit agreement may not be modified to impair your right to withdraw the ordinary shares represented by your ADSs except to comply with mandatory provisions of law.

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Voting Rights

As a holder, you generally have the right under the deposit agreement to instruct the depositary to exercise the voting rights for the ordinary shares represented by your ADSs. The voting rights of holders of ordinary shares are described in “Description of Share Capital and Certain Corporate Matters” in this prospectus. At our request, the depositary will distribute to you any notice of shareholders’ meeting received from us together with information explaining how to instruct the depositary to exercise the voting rights of the ordinary shares represented by ADSs. In lieu of distributing such materials, the depositary bank may distribute to holders of ADSs instructions on how to retrieve such materials upon request.

If the depositary timely receives voting instructions from a holder of ADSs, it will endeavor to vote (or cause the custodian to vote) the securities (in person or by proxy) represented by the holder’s ADSs as follows:

• If voting at the shareholders’ meeting by show of hands: The depositary will vote (or cause the custodian to vote) all the securities represented by ADSs in accordance with the voting instructions received from a majority of the ADS holders who provided voting instructions.

• If voting at the shareholders’ meeting by poll: The depositary will vote (or cause the custodian to vote) the securities represented by ADSs in accordance with the voting instructions received from the holders of ADSs.

Securities for which no voting instructions have been received will not be voted (except as otherwise contemplated in the deposit agreement). Please note that the ability of the depositary to carry out voting instructions may be limited by practical and legal limitations and the terms of the securities on deposit. It cannot be assured you that you will receive voting materials in time to enable you to return voting instructions to the depositary in a timely manner.

Fees and Charges

As an ADS holder, you will be required to pay the following fees under the terms of the deposit agreement:

Service	Fees
Issuance of ADSs (e.g., an issuance of ADS upon a deposit of ordinary shares, upon a change in the ADS(s)-to-ordinary shares ratio, or for any other reason), excluding ADS issuances as a result of distributions of ordinary shares)	Up to U.S. 5¢ per ADS issued
Cancellation of ADSs (e.g., a cancellation of ADSs for delivery of deposited property, upon a change in the ADS(s)-to-ordinary shares ratio, or for any other reason)	Up to U.S. 5¢ per ADS cancelled
Distribution of cash dividends or other cash distributions (e.g., upon a sale of rights and other entitlements)	Up to U.S. 5¢ per ADS held
Distribution of ADSs pursuant to (i) stock dividends or other free stock distributions, or (ii) exercise of rights to purchase additional ADSs	Up to U.S. 5¢ per ADS held
Distribution of securities other than ADSs or rights to purchase additional ADSs (e.g., upon a spin-off)	Up to U.S. 5¢ per ADS held
ADS services	Up to U.S. 5¢ per ADS held on the applicable record date(s) established by the depositary bank
Registration of ADS transfers (e.g., upon a registration of the transfer of registered ownership of ADSs, upon a transfer of ADSs into DTC and vice versa, or for any other reason)	Up to U.S. 5¢ per ADS (or fraction thereof) transferred

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Service	Fees
Conversion of ADSs of one series for ADSs of another series (e.g., upon conversion of Partial Entitlement ADSs for Full Entitlement ADSs, or upon conversion of Restricted ADSs (each as defined in the deposit agreement) into freely transferable ADSs, and vice versa).	Up to U.S. 5¢ per ADS (or fraction thereof) converted

As an ADS holder you will also be responsible to pay certain charges such as:

• taxes (including applicable interest and penalties) and other governmental charges;

• the registration fees as may from time to time be in effect for the registration of ordinary shares on the share register and applicable to transfers of ordinary shares to or from the name of the custodian, the depositary bank or any nominees upon the making of deposits and withdrawals, respectively;

• certain cable, telex and facsimile transmission and delivery expenses;

• the fees, expenses, spreads, taxes and other charges of the depositary bank and/or service providers (which may be a division, branch or affiliate of the depositary bank) in the conversion of foreign currency;

• the reasonable and customary out-of-pocket expenses incurred by the depositary bank in connection with compliance with exchange control regulations and other regulatory requirements applicable to ordinary shares, ADSs and ADRs; and

• the fees, charges, costs and expenses incurred by the depositary bank, the custodian, or any nominee in connection with the ADR program.

ADS fees and charges for (i) the issuance of ADSs and (ii) the cancellation of ADSs are charged to the person for whom the ADSs are issued (in the case of ADS issuances) and to the person for whom ADSs are cancelled (in the case of ADS cancellations). In the case of ADSs issued by the depositary into DTC, the ADS issuance and cancellation fees and charges may be deducted from distributions made through DTC, and may be charged to the DTC participant(s) receiving the ADSs being issued or the DTC participant(s) holding the ADSs being cancelled, as the case may be, on behalf of the beneficial owner(s) and will be charged by the DTC participant(s) to the account of the applicable beneficial owner(s) in accordance with the procedures and practices of the DTC participants as in effect at the time. ADS fees and charges in respect of distributions and the ADS service fee are charged to the holders as of the applicable ADS record date. In the case of distributions of cash, the amount of the applicable ADS fees and charges is deducted from the funds being distributed. In the case of (i) distributions other than cash and (ii) the ADS service fee, holders as of the ADS record date will be invoiced for the amount of the ADS fees and charges and such ADS fees and charges may be deducted from distributions made to holders of ADSs. For ADSs held through DTC, the ADS fees and charges for distributions other than cash and the ADS service fee may be deducted from distributions made through DTC, and may be charged to the DTC participants in accordance with the procedures and practices prescribed by DTC and the DTC participants in turn charge the amount of such ADS fees and charges to the beneficial owners for whom they hold ADSs. In the case of (i) registration of ADS transfers, the ADS transfer fee will be payable by the ADS Holder whose ADSs are being transferred or by the person to whom the ADSs are transferred, and (ii) conversion of ADSs of one series for ADSs of another series, the ADS conversion fee will be payable by the Holder whose ADSs are converted or by the person to whom the converted ADSs are delivered.

In the event of refusal to pay the depositary fees, the depositary may, under the terms of the deposit agreement, refuse the requested service until payment is received or may set off the amount of the depositary fees from any distribution to be made to the ADS holder. Note that the fees and charges you may be required to pay may vary over time and may be changed by us and by the depositary. You will receive prior notice of such changes. The depositary may reimburse us for certain expenses we incur in respect of the ADR program, by making available a portion of the ADS fees charged in respect of the ADR program or otherwise, upon such terms and conditions as we and the depositary agree from time to time.

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Amendments and Termination

We may agree with the depositary to modify the deposit agreement at any time without your consent. We will undertake to give holders 30 days’ prior notice of any modifications that would materially prejudice any of their substantial rights under the deposit agreement. We will not consider to be materially prejudicial to your substantial rights any modifications or supplements that are reasonably necessary for the ADSs to be registered under the Securities Act or to be eligible for book-entry settlement, in each case without imposing or increasing the fees and charges you are required to pay. In addition, we may not be able to provide you with prior notice of any modifications or supplements that are required to accommodate compliance with applicable provisions of law.

You will be bound by the modifications to the deposit agreement if you continue to hold your ADSs after the modifications to the deposit agreement become effective. The deposit agreement cannot be amended to prevent you from withdrawing the ordinary shares represented by your ADSs (except as permitted by law). We have the right to direct the depositary to terminate the deposit agreement. Similarly, the depositary may in certain circumstances on its own initiative terminate the deposit agreement. In either case, the depositary must give notice to the holders at least 30 days before termination. Until termination, your rights under the deposit agreement will be unaffected.

Termination

After termination, the depositary will continue to collect distributions received (but will not distribute any such property until you request the cancellation of your ADSs) and may sell the securities held on deposit. After the sale, the depositary will hold the proceeds from such sale and any other funds then held for the holders of ADSs in a non-interest-bearing account. At that point, the depositary will have no further obligations to holders other than to account for the funds then held for the holders of ADSs still outstanding (after deduction of applicable fees, taxes and expenses).

Books of Depositary

The depositary will maintain ADS holder records at its depositary office. You may inspect such records at such office during regular business hours but solely for the purpose of communicating with other holders in the interest of business matters relating to the ADSs and the deposit agreement.

The depositary will maintain in New York facilities to record and process the issuance, cancellation, combination, split-up and transfer of ADSs. These facilities may be closed from time to time, to the extent not prohibited by law.

Transmission of Notices, Reports and Proxy Soliciting Material

The depositary will make available for your inspection at its office all communications that it receives from us as a holder of deposited securities that are generally made available to holders of deposited securities. Subject to the terms of the deposit agreement, the depositary will send you copies of those communications or otherwise make those communications available to you upon a request from us.

Limitations on Obligations and Liabilities

The deposit agreement limits our obligations and the depositary’s obligations to you. Please note the following:

• We and the depositary are obligated only to take the actions specifically stated in the deposit agreement without negligence or bad faith.

• The depositary disclaims any liability for any failure to carry out voting instructions, for any manner in which a vote is cast or for the effect of any vote, provided it acts in good faith and in accordance with the terms of the deposit agreement.

• The depositary disclaims any liability for any failure to determine the lawfulness or practicality of any action, for the content of any document forwarded to you on our behalf or for the accuracy of any translation of such a document, for the investment risks associated with investing in ordinary shares, for the validity or worth of the ordinary shares, for any tax consequences that result from the ownership of ADSs, for the credit-worthiness of any third party, for allowing any rights to lapse under the terms of the deposit agreement, for the timeliness of any of our notices or for our failure to give notice.

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• We and the depositary will not be obligated to perform any act that is inconsistent with the terms of the deposit agreement.

• We and the depositary disclaim any liability if we or the depositary are prevented or forbidden from or subject to any civil or criminal penalty or restraint on account of, or delayed in, doing or performing any act or thing required by the terms of the deposit agreement, by reason of any provision, present or future of any law or regulation, or by reason of present or future provision of any provision of our Articles of Association, or any provision of or governing the securities on deposit, or by reason of any act of God or war or other circumstances beyond our control.

• We and the depositary disclaim any liability by reason of any exercise of, or failure to exercise, any discretion provided for in the deposit agreement or in our Articles of Association or in any provisions of or governing the securities on deposit.

• We and the depositary further disclaim any liability for any action or inaction in reliance on the advice or information received from legal counsel, accountants, any person presenting ordinary shares for deposit, any holder of ADSs or authorized representatives thereof, or any other person believed by us in good faith to be competent to give such advice or information.

• We and the depositary also disclaim liability for the inability by a holder to benefit from any distribution, offering, right or other benefit that is made available to holders of ordinary shares but is not, under the terms of the deposit agreement, made available to you.

• We and the depositary may rely without any liability upon any written notice, request or other document believed to be genuine and to have been signed or presented by the proper parties.

• We and the depositary also disclaim liability for any consequential or punitive damages for any breach of the terms of the deposit agreement.

• No disclaimer of any Securities Act liability is intended by any provision of the deposit agreement.

• Nothing in the deposit agreement gives rise to a partnership or joint venture, or establishes a fiduciary relationship, among us, the depositary bank and you as ADS holder.

• Nothing in the deposit agreement precludes Citibank (or its affiliates) from engaging in transactions in which parties adverse to us or the ADS owners have interests, and nothing in the deposit agreement obligates Citibank to disclose those transactions, or any information obtained in the course of those transactions, to us or to the ADS owners, or to account for any payment received as part of those transactions.

Taxes

You will be responsible for the taxes and other governmental charges payable on the ADSs and the ordinary shares represented by the ADSs. We, the depositary and the custodian may deduct from any distribution the taxes and other governmental charges payable by holders and may sell any and all property on deposit to pay the taxes and governmental charges payable by holders. You will be liable for any deficiency if the sale proceeds do not cover the taxes that are due.

The depositary may refuse to issue ADSs; to deliver, transfer, split and combine ADRs; or to release securities on deposit until all taxes and charges are paid by the applicable holder. The depositary and the custodian may take reasonable administrative actions to obtain tax refunds and reduced tax withholding for any distributions on your behalf. However, you may be required to provide to the depositary and to the custodian proof of taxpayer status and residence and such other information as the depositary and the custodian may require to fulfill legal obligations. You are required to indemnify us, the depositary and the custodian for any claims with respect to taxes based on any tax benefit obtained for you.

Foreign Currency Conversion

The depositary will arrange for the conversion of all foreign currency received into U.S. dollars if such conversion is practical, and it will distribute the U.S. dollars in accordance with the terms of the deposit agreement. You may have to pay fees and expenses incurred in converting foreign currency, such as fees and expenses incurred in complying with currency exchange controls and other governmental requirements.

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If the conversion of foreign currency is not practical or lawful, or if any required approvals are denied or not obtainable at a reasonable cost or within a reasonable period, the depositary may take the following actions in its discretion:

• Convert the foreign currency to the extent practical and lawful and distribute the U.S. dollars to the holders for whom the conversion and distribution is lawful and practical.

• Distribute the foreign currency to holders for whom the distribution is lawful and practical.

• Hold the foreign currency (without liability for interest) for the applicable holders.

Governing Law and Waiver of Jury Trial

The deposit agreement, the ADRs and the ADSs will be interpreted in accordance with the laws of the State of New York. The rights of holders of ordinary shares (including ordinary shares represented by ADSs) are governed by the laws of England and Wales.

AS A PARTY TO THE DEPOSIT AGREEMENT, YOU IRREVOCABLY WAIVE YOUR RIGHT, TO THE FULLEST EXTENT PERMITTED BY APPLICABLE LAW, TO TRIAL BY JURY IN ANY LEGAL PROCEEDING ARISING OUT OF THE DEPOSIT AGREEMENT OR THE ADRs AGAINST US AND/OR THE DEPOSITARY.

The deposit agreement provides that, to the extent permitted by law, ADS holders waive the right to a jury trial of any claim they may have against us or the depositary arising out of or relating to our shares, the ADSs or the deposit agreement, including any claim under U.S. federal securities laws. If we or the depositary opposed a jury trial demand based on the waiver, the court would determine whether the waiver was enforceable in the facts and circumstances of that case in accordance with applicable case law. However, you will not be deemed, by agreeing to the terms of the deposit agreement, to have waived our or the depositary's compliance with U.S. federal securities laws and the rules and regulations promulgated thereunder.

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SHARES AND AMERICAN DEPOSITARY SHARES ELIGIBLE FOR FUTURE SALE

Although our ordinary shares are currently listed for trading on AIM and EGE, to date there has not been any public market for ADSs representing our ordinary shares. Future sales of substantial amounts of ADSs representing our shares in the United States or of our ordinary shares on AIM or EGE, or the perception that such sales may occur, could adversely affect prevailing market prices of such ADSs and of our ordinary shares. As of March 31, 2020, we had outstanding 159,363,543 ordinary shares (including 4,864,656 treasury shares), plus 17,196,273 zero cost warrants that may be exercised at any time for an equal number of ordinary shares. Of the shares registered hereby, 34,034,130 shares are represented by 6,806,826 ADSs. Upon the effectiveness of the registration statement of which this prospectus forms a part, holders of ordinary shares that are not represented by ADSs will be able to deposit such shares with the depositary in exchange for ADSs representing such shares at the ratio of five ordinary shares per ADS. ADSs representing the shares registered hereby will be freely tradeable on the effective date of the registration statement of which this prospectus forms a part.

Ordinary shares not registered hereby may not be sold publicly in the United States (including in the form of ADSs) unless they are registered or one of the exemptions from U.S. registration requirements set forth below is available.

Rule 144

In general, under Rule 144, beginning 90 days after the date of this prospectus, a person (or persons whose ordinary shares are aggregated) who is not considered to have been an affiliate at any time during the 90 days preceding a sale and who has beneficially owned the ordinary shares proposed to be sold for at least six months, including the holding period of any prior owner other than an affiliate, is entitled to sell those ordinary shares without restriction, subject to our compliance with the reporting obligations under the Exchange Act. In addition, under Rule 144, a person (or persons whose ordinary shares are aggregated) who is not considered to have been an affiliate at any time during the 90 days preceding a sale and who has beneficially owned the ordinary shares proposed to be sold for at least one year, including the holding period of any prior owner other than an affiliate, is entitled to sell those ordinary shares immediately upon the effectiveness of the registration statement of which this prospectus forms a part without restriction and without regard to whether we are in compliance with our reporting obligations under the Exchange Act.

In general, under Rule 144, beginning 90 days after the date of this prospectus, a person who is an affiliate and has beneficially owned our ordinary shares for at least six months is entitled to sell within any three-month period a number of our ordinary shares that does not exceed the greater of 1% of the number of our ordinary shares then outstanding, in the form of ADSs or otherwise, and the average weekly trading volume of the ordinary shares, in the form of ADSs or otherwise, on the Nasdaq during the four calendar weeks preceding the filing of a notice on Form 144 in connection with the sale.

Any such sales by an affiliate are also subject to manner of sale provisions, notice requirements and our compliance with reporting obligations under the Exchange Act.

Rule 701

In general, under Rule 701, any of our employees, directors, officers, consultants or advisors who purchase shares from us in connection with a compensatory share or option plan or other written agreement before the effectiveness of the registration statement of which this prospectus forms a part is entitled to resell such shares 90 days after the date of this prospectus in reliance on Rule 144, without having to comply with the holding period requirements or other restrictions contained in Rule 701.

The SEC has indicated that Rule 701 will apply to typical share options granted by an issuer before it becomes subject to the reporting requirements of the Exchange Act, along with the shares acquired upon exercise of such options, including exercises after the date of this prospectus. Securities issued in reliance on Rule 701 are restricted securities and, subject to the contractual restrictions described below, beginning 90 days after the date of this prospectus, may be sold by persons other than “affiliates,” as defined in Rule 144, subject only to the manner of sale provisions of Rule 144 and by “affiliates” under Rule 144 without compliance with its one-year minimum holding period requirement.

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Regulation S

Regulation S under the Securities Act provides that ordinary shares owned by any person may be sold without registration in the United States, provided that the sale is effected in an offshore transaction and no directed selling efforts are made in the United States (as these terms are defined in Regulation S), subject to certain other conditions. In general, this means that our ordinary shares may be sold in some other manner outside the United States without requiring registration in the United States.

Equity Plans

As of December 31, 2019, options to purchase a total of 14,481,720 ordinary shares were issued and outstanding. Of the total number of issued and outstanding options, 3,008,654 will be vested upon the effectiveness of the registration statement of which this prospectus forms a part.

We intend to file a registration statement on Form S-8 under the Securities Act to register up to 25.75 million ordinary shares, in the aggregate, issued or reserved for issuance under our Share Option Plan. The registration statement on Form S-8 will become effective automatically upon filing. Ordinary shares issued upon exercise of a share option and registered pursuant to the Form S-8 registration statement will, subject to vesting provisions and Rule 144 volume limitations applicable to our affiliates, be available for sale in the open market immediately.

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INCOME TAX CONSIDERATIONS

Material U.S. Federal Income Tax Considerations

The following describes the material U.S. federal income tax consequences relating to the acquisition, ownership and disposition of the ADSs. This section does not purport to be a comprehensive description of all of the U.S. federal income tax considerations that may be relevant to a particular person’s decision to acquire the ADSs. This discussion is based on the U.S. Internal Revenue Code of 1986, as amended (“Code”), and U.S. Treasury regulations promulgated thereunder (“Treasury Regulations”), as well as judicial and administrative interpretations thereof as in effect as of the date of this prospectus. All of the foregoing authorities are subject to change or differing interpretations, which change or differing interpretations could apply retroactively and could affect the tax consequences described below, and there can be no assurance that the U.S. Internal Revenue Service (“IRS”), or U.S. courts will agree with the tax consequences described in this section. We undertake no obligation to publicly update or otherwise revise this section whether as a result of new Treasury Regulations, Code sections, judicial and administrative interpretations or otherwise.

This description applies only to U.S. Holders (as defined below) that purchase ADSs representing our ordinary shares and hold the ADSs as capital assets within the meaning of Section 1221 of the Code (generally, property held for investment). This section does not address any U.S. federal estate and gift tax, alternative minimum tax or Medicare tax on net investment income consequences, or any U.S. state or local or non-U.S. tax consequences. This section also does not address the tax considerations that may be relevant to certain types of investors subject to special treatment under U.S. federal income tax laws, such as:

• banks and other financial institutions;

• insurance companies;

• regulated investment companies or real estate investment trusts;

• dealers or traders in securities or currencies that use a mark-to-market method of accounting;

• broker-dealers;

• tax exempt organizations, retirement plans, individual retirement accounts and other tax deferred accounts;

• persons holding the ADSs as part of a straddle, hedging, conversion or integrated transaction for U.S. federal income tax purposes;

• U.S. expatriates;

• U.S. Holders whose functional currency is not the U.S. dollar;

• any entity or arrangement classified as partnership for U.S. federal income tax purposes or investors therein;

• persons who own or are deemed to own, directly or constructively, 10% or more of the total combined voting power of all classes of our voting stock or 10% or more of the total value of shares of all classes of our stock;

• persons subject to special tax accounting rules as a result of any item of gross income with respect to the ADSs being taken into account in an applicable financial statement; and

• persons that held, directly, indirectly or by attributions, ownership interest in us prior to the effectiveness of the registration statement of which this prospectus forms a part.

THE DESCRIPTION OF U.S. FEDERAL INCOME TAX CONSEQUENCES SET OUT BELOW IS FOR GENERAL INFORMATION ONLY. PROSPECTIVE INVESTORS SHOULD CONSULT THEIR TAX ADVISORS REGARDING THE APPLICATION OF THE U.S. FEDERAL TAX RULES TO THEIR PARTICULAR CIRCUMSTANCES AS WELL AS THE STATE, LOCAL, NON-U.S. AND OTHER TAX CONSEQUENCES TO THEM OF THE ACQUISITION, OWNERSHIP AND DISPOSITION OF THE ADSs.

As used in this discussion, the term “U.S. Holder” means a beneficial owner of the ADSs that is for U.S. federal income tax purposes:

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• a citizen or individual resident of the United States;

• a corporation (or other entity treated as a corporation) created or organized in or under the laws of the United States, any state thereof or the District of Columbia;

• an estate, the income of which is subject to U.S. federal income taxation regardless of its source; or

• trust that (1) is subject to the primary supervision of a court within the United States and the control of one or more U.S. persons for all substantial decisions of the trust or (2) has a valid election in effect under applicable Treasury Regulations to be treated as a U.S. person.

The U.S. federal income tax treatment of a partner in an entity or arrangement treated as a partnership for U.S. federal income tax purposes that holds ADSs generally will depend on the status of the partner and the activities of the partnership. Partnerships considering an investment in the ADSs and partners in such partnerships should consult their tax advisors regarding the specific U.S. federal income tax consequences to them of the acquisition, ownership and disposition of the ADSs.

The discussion below assumes that the representations contained in the deposit agreement and any related agreement are true and that the obligations in such agreements will be complied with in accordance with their terms. The discussion below also assumes that we are not treated as a U.S. domestic corporation under Section 7874 of the Code as a result of the Acquisition. See “Risk Factors—Risks Related to Ownership of our ADSs and Ordinary Shares and Our Prospective Nasdaq Listing.”

ADSs

For U.S. federal income tax purposes, U.S. Holders of ADSs generally will be treated as the beneficial owners of the underlying shares represented by the ADSs and an exchange of ADSs for our shares generally will not be subject to U.S. federal income tax.

The U.S. Treasury Department and the IRS have expressed concerns that U.S. Holders of ADSs may be claiming foreign tax credits in situations where an intermediary in the chain of ownership between the holder of an ADS and the issuer of the security underlying the ADS has taken actions that are inconsistent with the U.S. Holder of the ADS being treated as the beneficial owner of the underlying security. Such actions also may be inconsistent with the claiming of the reduced rate of tax applicable to certain dividends received by non-corporate U.S. Holders of ADSs, including individual U.S. Holders. Accordingly, the availability of foreign tax credits or the reduced U.S. federal income tax rate for “qualified dividend income,” each discussed below, could be affected by actions taken by intermediaries in the chain of ownership between the holder of an ADS and us, if as a result of such actions the U.S. Holder of an ADS is not properly treated as the beneficial owner of the underlying share.

U.S. Holders should consult their own tax advisers concerning the U.S. federal, state, local and non-U.S. tax consequences of acquiring, owning and disposing of ADSs in their particular circumstances.

Dividends and Other Distributions

Subject to the passive foreign investment company (“PFIC”), rules discussed below, the gross amount of any distribution made by us to a U.S. Holder with respect to the ADSs (including the amount of any taxes withheld therefrom) generally will be included in such holder’s gross income as non-U.S. source dividend income in the year actually or constructively received by the depositary, but only to the extent that the distribution is paid out of our current or accumulated earnings and profits (as determined under U.S. federal income tax principles). As a non-U.S. company, we do not maintain calculations of our earnings and profits under U.S. federal income tax principles. Therefore, it is expected that any distributions generally will be reported to U.S. Holders as dividends. Any dividends that we pay will not be eligible for the dividends-received deduction allowed to qualifying corporations under Section 243 of the Code.

With respect to certain non-corporate U.S. Holders, including individual U.S. Holders, dividends paid on the ADSs may be eligible to be taxed at favorable rates applicable to “qualified dividend income,” provided that (1) the ADSs are readily tradable on an established securities market in the United States, or we are eligible for the benefits of an approved qualifying income tax treaty with the United States that includes an exchange of information program (2) we are not a PFIC (as discussed below) with respect to the relevant U.S. Holder for either our taxable year in which the dividend is paid or the preceding taxable year (3) certain minimum holding

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period and other requirements are met and (4) the relevant U.S. Holder is not under any obligation to make related payments with respect to persons in substantially similar or related property. Such favorable rates may not, however, be available if we are treated as a “surrogate foreign corporation” within the meaning of Section 7874 of the Code.

Under a published IRS Notice, common or ordinary shares, or ADSs representing such shares, are considered to be readily tradable on an established securities market in the United States if they are listed on the Nasdaq, as our ADSs are expected to be. U.S. Holders should consult their tax advisors regarding the availability of the favorable rate applicable to qualified dividend income for any dividends we pay with respect to the ADSs, including such availability if we are treated as a “surrogate foreign corporation” within the meaning of Section 7874 of the Code.

The amount of any distribution paid in a currency other than U.S. dollars will be included in a U.S. Holder’s income in an amount equal to the U.S. dollar value of such currency calculated by reference to the exchange rate in effect on the date the distribution is actually or constructively received by the depositary, regardless of whether the payment is in fact converted into U.S. dollars at that time. If the distribution is converted into U.S. dollars on the date of receipt, a U.S. Holder generally should not be required to recognize foreign currency gain or loss in respect of the distribution. A U.S. Holder may have foreign currency gain or loss if the distribution is converted into, or exchanged for, U.S. dollars after the date of receipt.

Any dividends we pay to U.S. Holders generally will constitute non-U.S. source “passive category” income for U.S. foreign tax credit limitation purposes. However, if 50% or more of our stock is treated as held by US persons, we will be treated as a “United States-owned foreign corporation.” In that case, dividends may be treated, for foreign tax credit limitation purposes, as income from sources outside the United States to the extent attributable to our non-U.S. source earnings an profits, and as income from sources within the United States to the extent attributable to our U.S. source earnings and profits. If any United Kingdom taxes are withheld with respect to dividends paid to a U.S. Holder with respect to the ADSs, subject to certain conditions and limitations provided in the Code and the applicable Treasury Regulations (including a minimum holding period requirement), such taxes may be treated as non-U.S. taxes eligible for credit against such U.S. holder’s U.S. federal income tax liability (to the extent not exceeding the withholding rate applicable to the U.S. Holder). In lieu of claiming a foreign tax credit, U.S. Holders may, at their election, deduct non-U.S. taxes, including any United Kingdom taxes withheld from dividends on the ADSs, in computing their taxable income, subject to generally applicable limitations under U.S. federal income tax law. An election to deduct non-U.S. taxes instead of claiming foreign tax credits applies to all non-U.S. taxes paid or accrued in the taxable year. If a refund of the tax withheld is available under the laws of the United Kingdom or under an applicable income tax treaty, the amount of tax withheld that is refundable will not be eligible for such credit against a U.S. Holder’s U.S. federal income tax liability (and will not be eligible for the deduction against U.S. federal taxable income). If the dividends constitute qualified dividend income as discussed above, the amount of the dividend taken into account for purposes of calculating the U.S. foreign tax credit limitation generally will be limited to the gross amount of the dividend, multiplied by the reduced rate applicable to the qualified dividend income, divided by the highest rate of tax normally applicable to dividends.

The rules relating to the determination of the U.S. foreign tax credit and the deduction of non-U.S. taxes are complex, and U.S. Holders should consult their tax advisors to determine whether and to what extent a credit or deduction may be available in their particular circumstances.

Taxable Dispositions of the ADSs

Subject to the PFIC rules discussed below, a U.S. Holder generally will recognize taxable gain or loss on any sale, exchange or other taxable disposition of an ADS in an amount equal to the difference between the sum of the fair market value of any property and the amount of cash received in such disposition and the holder’s tax basis in the ADS. The U.S. Holder’s tax basis in the ADSs generally will equal the cost of the ADSs to the U.S. Holder. The gain or loss generally will be capital gain or loss, and generally will be a long term capital gain or loss if the U.S. Holder has held the ADS for more than one year at the time of disposition. For certain non-corporate taxpayers (including individuals), long term capital gains are subject to tax at reduced rates. The deductibility of capital losses is subject to limitations.

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Any gain or loss that a U.S. Holder recognizes on a sale or other taxable disposition of an ADS generally will be treated as U.S. source income or loss for U.S. foreign tax credit limitation purposes. U.S. Holders should consult their tax advisors regarding the proper treatment of any gain or loss in their particular circumstances, including the effects of any applicable income tax treaties.

Passive Foreign Investment Company Considerations

Based on the current and anticipated value of our assets and the nature and composition of our income and assets, we do not expect to be a PFIC for our current taxable year ending December 31, 2020, or in the foreseeable future. However, the determination of PFIC status is based on an annual determination that cannot be made until the close of a taxable year, involves extensive factual investigation, including ascertaining the fair market value of all of our assets on a quarterly basis and the active or passive character of each item of income that we earn, and is subject to uncertainty in several respects. Changes in the nature or composition of our income or assets, the structure of our operation or the value of our assets may cause us to become a PFIC. The determination of the value of our assets may depend in part upon the value of our goodwill not reflected on our balance sheet (which may depend upon the market value of the ADSs from time to time, which may be volatile). Accordingly, we cannot assure you that we will not be a PFIC for our current taxable year ending December 31, 2020, or for any future taxable year. If we are a PFIC for any year during which a U.S. Holder holds the ADSs, we generally would continue to be treated as a PFIC with respect to that U.S. Holder for all succeeding years during which the U.S. Holder holds the ADSs, even if we ceased to meet the threshold requirements for PFIC status in any particular year, unless the U.S. Holder has made a “deemed sale” election under the PFIC Rules when we cease to be a PFIC.

A non-U.S. corporation such as us will be treated as a PFIC for U.S. federal income tax purposes for any taxable year if, applying applicable look-through rules, either:

• at least 75% of its gross income for such year is “passive income” for purposes of the PFIC rules; or

• at least 50% of the value of its assets (determined based on a quarterly average) during such year is attributable to assets that produce or are held for the production of passive income.

For this purpose, passive income generally includes dividends, interest, royalties and rents other than certain royalties and rents derived in the active conduct of a trade or business and not derived from a related person. We will be treated as owning a proportionate share of the assets and earning a proportionate share of the income of any other corporation in which we own, directly or indirectly, more than 25% by value of the stock.

If we were a PFIC for any taxable year during which a U.S. Holder holds ADSs, then, unless such U.S. Holder makes a “mark-to-market” election (as discussed below), such U.S. Holder generally would be subject to special adverse tax rules with respect to any “excess distribution” that it receives from us and any gain that it recognizes from a sale or other disposition, including, in certain circumstances, a pledge, of ADSs. For this purpose, distributions that a U.S. Holder receives in a taxable year that are greater than 125% of the average annual distributions that it received during the shorter of the three preceding taxable years or your holding period for the ADSs will be treated as an excess distribution. Under these rules:

• the excess distribution or recognized gain would be allocated ratably over the U.S. Holder’s holding period for the ADSs;

• the amount of the excess distribution or recognized gain allocated to the taxable year of distribution or gain, and to any taxable years in the U.S. Holder’s holding period prior to the first taxable year in which we were treated as a PFIC, would be treated as ordinary income; and

• the amount of the excess distribution or recognized gain allocated to each other taxable year would be subject to the highest tax rate in effect for individuals or corporations, as applicable, for each such year and the resulting tax will be subject to the interest charge generally applicable to underpayments of tax.

If we were a PFIC for any taxable year during which a U.S. Holder holds ADSs and any of our non-U.S. subsidiaries or other corporate entities in which we own equity interests is also a PFIC, the U.S. Holder would be treated as owning a proportionate amount (by value) of the shares of each such non-U.S. entity classified as a PFIC, each such entity referred to as a lower-tier PFIC, for purposes of the application of these rules. U.S. Holders should consult their own tax advisor regarding the application of the PFIC rules to any of our lower-tier PFICs.

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If we were a PFIC for any taxable year during which a U.S. Holder holds ADSs, then in lieu of being subject to the tax and interest-charge rules discussed above, the U.S. Holder may make an election to include gain on the ADSs as ordinary income under a mark-to-market method, provided that our ADSs constitute “marketable stock.” Marketable stock is stock that is regularly traded on a qualified exchange or other market, as defined in applicable Treasury Regulations. Shares or ADSs generally will be considered regularly traded during any calendar year during which they are traded, other than in de minimis quantities, on at least 15 days during each calendar quarter. Any trades that have as their principal purpose meeting this requirement will be disregarded. We expect that the ADSs, but not our shares, will be listed on the Nasdaq, which is a qualified exchange or other market for these purposes.

Consequently, if the ADSs are listed on the Nasdaq and are regularly traded, we expect that the mark-to-market election would be available to U.S. Holders of ADSs if we were to become a PFIC, but no assurances are given in this regard.

Because a mark-to-market election cannot be made for any lower-tier PFICs that we may own (unless the shares in such lower-tier PFIC are themselves treated as marketable stock), if we were a PFIC for any taxable year, a U.S. Holder that makes the mark-to-market election may continue to be subject to the tax and interest charges under the general PFIC rules with respect to such U.S. Holder’s indirect interest in any investments held by us that are treated as an equity interest in a PFIC for U.S. federal income tax purposes.

In certain circumstances, a shareholder in a PFIC may avoid the adverse tax and interest-charge regime described above by making a “qualified electing fund” election to include in income its share of the corporation’s income on a current basis. However, a U.S. Holder may make a qualified electing fund election with respect to the ADSs only if we agree to furnish such U.S. Holder annually with a PFIC annual information statement as specified in the applicable Treasury Regulations. We do not intend to provide such information, and thus we do not expect that a U.S. Holder will be able to make a qualified electing fund election.

If a U.S. Holder owns ADSs during any year in which we are a PFIC, such U.S. Holder (including, potentially, indirect holders) generally will be required to file an IRS Form 8621 with such holder’s U.S. federal income tax return for that year.

U.S. Holders should consult their own tax advisors regarding the application of the PFIC rules to their ownership of the ADSs.

Information Reporting and Backup Withholding

Dividend payments with respect to the ADSs and proceeds from a sale, exchange, redemption or other taxable disposition of the ADSs made within the United States or through certain U.S. related financial intermediaries may be subject to information reporting to the IRS and possible U.S. backup withholding. Backup withholding will not apply, however, to a U.S. Holder that furnishes a correct taxpayer identification number and makes any other required certification on IRS Form W-9 or that is otherwise exempt from backup withholding. U.S. Holders of the ADSs should consult their tax advisors regarding the application of the U.S. information reporting and backup withholding rules.

Backup withholding is not an additional tax. Amounts withheld as backup withholding may be credited against such U.S. Holder’s U.S. federal income tax liability, and such holder may obtain a refund of any excess amounts withheld under the backup withholding rules by filing an appropriate claim for refund with the IRS and furnishing any required information in a timely manner.

Certain U.S. Holders may be required to comply with certain reporting requirements relating to the ADSs, including filing IRS Form 8938, with respect to the holding of certain foreign financial assets, including stock of foreign issuers (such as us), either directly or through certain foreign financial institutions, if the aggregate value of all such assets exceeds $50,000 on the last day of the tax year or $75,000 at any time during the tax year. U.S. Holders who fail to report the required information could be subject to substantial penalties. U.S. Holders should consult their own tax advisors regarding the application of these rules to their ownership of the ADSs.

THE DISCUSSION ABOVE IS A GENERAL SUMMARY. IT DOES NOT COVER ALL TAX MATTERS THAT MAY BE IMPORTANT TO YOU. PROSPECTIVE INVESTORS SHOULD CONSULT THEIR TAX ADVISORS REGARDING THE APPLICATION OF THE U.S. FEDERAL TAX RULES TO THEIR PARTICULAR CIRCUMSTANCES AS WELL AS THE STATE, LOCAL, NON-U.S. AND OTHER TAX CONSEQUENCES TO THEM OF THE ACQUISITION, OWNERSHIP AND DISPOSITION OF THE ADSs.

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United Kingdom Tax Considerations

The following is a general summary of certain UK tax considerations relating to the ownership and disposal of the ADSs and does not address all possible tax consequences relating to an investment in the ADSs. It is based on current UK tax law and generally published HM Revenue & Customs (“HMRC”) practice as at the date of this prospectus supplement, both of which are subject to change, possibly with retrospective effect.

Save as provided otherwise, this summary applies only to persons who are resident (and, in the case of individuals, domiciled) in the United Kingdom for tax purposes and who are not resident for tax purposes in any other jurisdiction and do not have a permanent establishment or fixed base in any other jurisdiction with which the holding of ADSs is connected (“UK Holders”). Persons (a) who are not resident (or, if resident are not domiciled) in the United Kingdom for tax purposes, including those individuals and companies who trade in the United Kingdom through a branch, agency or permanent establishment in the United Kingdom to which the ADSs are attributable, or (b) who are resident or otherwise subject to tax in a jurisdiction outside the United Kingdom, are recommended to seek the advice of professional advisers in relation to their taxation obligations.

This summary is for general information only and is not intended to be, nor should it be considered to be, legal or tax advice to any investor. It does not address all of the tax considerations that may be relevant to specific investors in light of their particular circumstances or to investors subject to special treatment under UK tax law. In particular, this summary:

• only applies to the absolute beneficial owners of the ADSs and any dividends paid in respect of the ordinary shares represented by the ADSs where the dividends are regarded for UK tax purposes as that person’s own income (and not the income of some other person); and

• (a) only addresses the principal UK tax consequences for investors who hold the ADSs as capital assets/investments, (b) does not address the tax consequences that may be relevant to certain special classes of investor such as dealers, brokers or traders in shares or securities and other persons who hold the ADSs otherwise than as an investment, (c) does not address the tax consequences for holders that are financial institutions, insurance companies, collective investment schemes, pension schemes, charities or tax-exempt organizations, (d) assumes that the holder is not an officer or employee of the Company (or of any related company) and has not (and is not deemed to have) acquired the ADSs by reason of an office or employment, and (e) assumes that the holder does not control or hold (and is not deemed to control or hold), either alone or together with one or more associated or connected persons, directly or indirectly (including through the holding of the ADSs), an interest of 10% or more in the issued share capital (or in any class thereof), voting power, rights to profits or capital of the Company, and is not otherwise connected with the Company.

This summary further assumes that a holder of ADSs will be treated as the beneficial owner of the underlying ordinary shares for UK direct tax purposes.

POTENTIAL INVESTORS IN THE ADSs SHOULD SATISFY THEMSELVES PRIOR TO INVESTING AS TO THE OVERALL TAX CONSEQUENCES, INCLUDING, SPECIFICALLY, THE CONSEQUENCES UNDER UK TAX LAW AND HMRC PRACTICE OF THE ACQUISITION, OWNERSHIP AND DISPOSAL OF THE ADSs (OR UNDERLYING ORDINARY SHARES), IN THEIR OWN PARTICULAR CIRCUMSTANCES BY CONSULTING THEIR OWN TAX ADVISERS.

Taxation of dividends

Withholding Tax

Dividend payments in respect of the ordinary shares represented by the ADSs may be made without withholding or deduction for or on account of UK tax.

Income Tax

Dividends received by individual UK Holders will be subject to UK income tax on the amount of the dividend paid.

The first £2,000 of dividend income received by an individual UK Holder in a tax year will be subject to a nil rate of tax regardless of the amount of the individual’s other taxable income.

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Dividend income in excess of the £2,000 to which the nil rate of tax applies will be taxed at the rate of 7.5% to the extent that the dividend, when treated as the top slice of the relevant UK Holder’s income, does not exceed the basic rate income tax limit, at the rate of 32.5% to the extent that the dividend, when treated as the top slice of the relevant UK Holder’s income, exceeds the basic rate income tax limit but does not exceed the higher rate income tax limit, and at the rate of 38.1% to the extent that the dividend, when treated as the top slice of the relevant UK Holder’s income, exceeds the higher rate income tax limit.

An individual holder of ADSs who is not a UK Holder will not be chargeable to UK income tax on dividends paid by the Company, unless such holder carries on (whether solely or in partnership) a trade, profession or vocation in the United Kingdom through a branch or agency in the United Kingdom to which the ADSs (or underlying ordinary shares) are attributable. In these circumstances, such holder may, depending on his or her individual circumstances, be chargeable to UK income tax on dividends received from the Company.

Corporation Tax

A UK Holder within the charge to UK corporation tax may be entitled to exemption from UK corporation tax in respect of dividend payments. If the conditions for the exemption are not satisfied, or such UK Holder elects for an otherwise exempt dividend to be taxable, UK corporation tax will be chargeable on the gross amount of any dividends. If potential investors are in any doubt as to their position, they should consult their own professional advisers.

A corporate holder of ADSs that is not a UK Holder will not be subject to UK corporation tax on dividends received from the Company, unless it carries on a trade in the United Kingdom through a permanent establishment to which the ADSs (or underlying ordinary shares) are attributable. In these circumstances, such holder may, depending on its individual circumstances and if the exemption from UK corporation tax discussed above does not apply, be chargeable to UK corporation tax on dividends received from the company.

Taxation of disposals

UK Holders

A disposal or deemed disposal of ADSs by an individual UK Holder may, depending on his or her individual circumstances, give rise to a chargeable gain or to an allowable loss for the purpose of UK capital gains tax. The principal factors that will determine the capital gains tax position on a disposal of ADSs are the extent to which the holder realizes any other capital gains in the tax year in which the disposal is made, the extent to which the holder has incurred capital losses in that or any earlier tax year and the level of the annual allowance of tax-free gains in that tax year (“annual exemption”). The annual exemption for individuals for the 2019/2020 tax year is £12,000. On March 23, 2020, an order was made setting the annual exemption for individuals for the 2020/2021 tax year at £12,300. If, after all allowable deductions, an individual UK Holder’s total taxable income (which, for the avoidance of doubt, includes any dividend income within the £2,000 nil rate band described above) for the year exceeds the basic rate income tax limit, a taxable capital gain accruing on a disposal of ADSs will be taxed at 20%. If, after all allowable deductions, an individual UK Holder’s total taxable income for the year does not exceed the basic rate income tax limit, and assuming the individual does not have any other taxable capital gains in the tax year that would use up the remaining basic rate allowance, a taxable capital gain accruing on a disposal of ADSs will be taxed at 10% on an amount that, when treated as the top slice of the relevant UK Holder’s income/gains, does not exceed the basic rate income tax and at 20% on the remainder.

A disposal of ADSs by a corporate UK Holder may give rise to a chargeable gain or an allowable loss for the purposes of UK corporation tax.

Any gains or losses in respect of currency fluctuations over the period of holding the ADSs would also be brought into account on the disposal.

Non-UK Holders

An individual holder who is not a UK Holder will not be liable to UK capital gains tax on capital gains realized on the disposal of his or her ADSs unless such holder carries on (whether solely or in partnership) a trade, profession or vocation in the United Kingdom through a branch or agency in the United Kingdom to which the ADSs (or underlying ordinary shares) are attributable. In these circumstances, such holder may, depending on his or her individual circumstances, be chargeable to UK capital gains tax on chargeable gains arising from a

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disposal of his or her ADSs. Furthermore, an individual holder of ADSs who has ceased to be resident for tax purposes in the United Kingdom for a period of less than five years and who disposes of ADSs during that period may be liable on his or her return to the United Kingdom to UK tax on any capital gain realized (subject to any available exemption or relief).

A corporate holder of ADSs that is not a UK Holder will not be liable for UK corporation tax on chargeable gains realized on the disposal of its ADSs unless it carries on a trade in the United Kingdom through a permanent establishment to which the ADSs (or underlying ordinary shares) are attributable. In these circumstances, a disposal of ADSs by such holder may give rise to a chargeable gain or an allowable loss for the purposes of UK corporation tax.

Stamp Duty and Stamp Duty Reserve Tax

Issue of Ordinary Shares

No UK stamp duty or stamp duty reserve tax (“SDRT”) is payable on the issue of the underlying ordinary shares in the company.

Transfer of Ordinary Shares

Neither UK stamp duty nor SDRT should arise on transfers of the underlying ordinary shares (including instruments transferring ordinary shares and agreements to transfer ordinary shares) on the basis that the ordinary shares are admitted to trading on AIM, provided the following requirements are (and continue to be) met:

• the ordinary shares are admitted to trading on AIM but are not listed on any market (with the term “listed” being construed in accordance with section 99A of the UK Finance Act 1986); and

• AIM continues to be accepted as a “recognized growth market” (as construed in accordance with section 99A of the UK Finance Act 1986).

In the event that either of the above requirements is not met, stamp duty or SDRT will generally apply to transfers of, or agreements to transfer, ordinary shares. Where applicable, the purchaser normally pays the stamp duty or SDRT.

Issue and Transfer of ADSs

No UK stamp duty or SDRT is payable on the issuance of the ADS. Based on current HMRC published practice, no UK stamp duty should be payable on a written instrument transferring an ADS or on a written agreement to transfer an ADS, on the basis that a depositary receipt is not regarded as “stock” or a “marketable security” for UK stamp duty purposes.

No SDRT will be payable on an agreement to transfer an ADS, as a depositary receipt is not considered a “chargeable security” for the purposes of SDRT.

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PLAN OF DISTRIBUTION

The registration statement of which this prospectus forms a part has been filed in part in respect of our obligations under a Registration Rights Agreement, dated September 25, 2019, concerning an aggregate of 67,207,159 ordinary shares held by investors identified herein, which includes 12,966,520 shares issuable upon the exercise of zero cost warrants by such investors. We are also registering hereby an aggregate of 54,113,792 ordinary shares held by other shareholders identified herein, as well as 4,229,753 shares issuable upon the exercise of zero cost warrants by investors identified herein and 37,325,929 shares issuable upon the exercise of convertible notes by investors identified herein. All such shares are referred to collectively herein as the Registered Shares, and the holders of all such shares are identified in this prospectus as the Registered Holders. Any Registered Shares offered and sold in the United States by the Registered Holders will be in the form of ADSs. The Registered Holders are also permitted to sell shares not represented by ADSs in private or offshore transactions, including on AIM and EGE, which resales are not covered by this prospectus. Unlike an initial public offering, any resale by the Registered Holders of the Registered Shares represented by ADSs is not being underwritten by any investment bank. We expect that the opening public price of our ADSs on Nasdaq will be determined by reference to the most recent trading price of our ordinary shares on AIM, as adjusted for the currency exchange rate and an ADS-to-share ratio of 1 to 5. The Registered Holders may, or may not, elect to sell Registered Shares represented by ADSs as and to the extent that they may individually determine.

The Registered Holders may dispose of all or a portion of the Registered Shares from time to time directly or through one or more underwriters, broker-dealers or agents. If ADSs representing our shares are sold through underwriters or broker-dealers, the Registered Holders will be responsible for any applicable underwriting discounts or commissions or agent’s commissions. ADSs representing our shares may be sold on Nasdaq or any other national securities exchange or quotation service on which the securities may be listed or quoted at the time of disposition, in the over-the-counter market or in transactions otherwise than on these exchanges or systems or in the over-the-counter market and in one or more transactions at fixed prices, at prevailing market prices at the time of the disposition, at varying prices determined at the time of disposition, or at negotiated prices. These dispositions may be effected in transactions that may involve crosses or block transactions. The Registered Holders may use any one or more of the following methods when disposing of shares:

• ordinary brokerage transactions and transactions in which the broker-dealer solicits purchasers;

• block trades in which the broker-dealer will attempt to sell the shares as agent but may position and resell a portion of the block as principal to facilitate the transaction;

• purchases by a broker-dealer as principal and resale by the broker-dealer for its account;

• an exchange distribution in accordance with the rules of the applicable exchange;

• privately negotiated transactions and offshore transactions;

• settlement of short sales entered into after the effective date of the registration statement of which this prospectus is a part;

• broker-dealers may agree with the Registered Holders to sell a specified number of such shares at a stipulated price per share;

• through the writing or settlement of options or other hedging transactions, whether such options are listed on an options exchange or otherwise;

• a combination of any such methods of disposition; and

• any other method permitted pursuant to applicable law.

The Registered Holders also may resell all or a portion of the Registered Shares in offshore transactions or open market transactions in reliance upon Rule 144 under the Securities Act, as permitted by that rule, or Section 4(a)(1) under the Securities Act, if available, rather than under this prospectus, provided that they meet the criteria and conform to the requirements of those provisions.

Broker-dealers engaged by the Registered Holders may arrange for other broker-dealers to participate in dispositions. If the Registered Holders effect such transactions by selling ADSs representing our shares to or through underwriters, broker-dealers or agents, such underwriters, broker-dealers or agents may receive applicable commissions in the form of discounts, concessions or commissions from the Registered Holders or commissions

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from purchasers of ADSs representing our shares for whom they may act as agent or to whom they may sell as principal. Such commissions will be in amounts to be negotiated, but, except as set forth in a supplement to this prospectus, in the case of an agency transaction will not be in excess of a customary brokerage commission in compliance with FINRA Rule 2121 and Supplementary Material .01 and Supplementary Material .02 thereto.

In connection with dispositions of ADSs representing Registered Shares, the Registered Holders may enter into hedging transactions with broker-dealers or other financial institutions, which may in turn engage in short sales of ADSs representing Registered Shares in the course of hedging in positions they assume. The Registered Holders may also sell ADSs representing Registered Shares short and, if such short sale shall take place after the date that the registration statement of which this prospectus forms a part is declared effective, the Registered Holders may deliver ADSs representing Registered Shares to close out short positions and to return borrowed shares in connection with such short sales. The Registered Holders may also loan or pledge ADSs representing Registered Shares to broker-dealers that in turn may sell such shares, to the extent permitted by applicable law. The Registered Holders may also enter into option or other transactions with broker-dealers or other financial institutions or the creation of one or more derivative securities which require the delivery to such broker-dealer or other financial institution of Registered Shares, which shares such broker-dealer or other financial institution may resell pursuant to this prospectus (as supplemented or amended to reflect such transaction). Notwithstanding the foregoing, the Registered Holders have been advised that they may not use Registered Shares to cover short sales of our shares (or ADSs representing shares) made prior to the date the registration statement of which this prospectus forms a part has been declared effective by the SEC.

The Registered Holders may, from time to time, pledge or grant a security interest in some or all of the warrants or ADSs representing Registered Shares owned by them and, if they default in the performance of their secured obligations, the pledgees or secured parties may offer and sell the ADSs representing Registered Shares from time to time pursuant to this prospectus or any amendment to this prospectus under Rule 424(b)(3) or other applicable provision of the Securities Act, amending, if necessary, the list of Registered Holders to include the pledgee, transferee or other successors in interest as Registered Holders under this prospectus. The Registered Holders also may transfer and donate the ADSs representing Registered Shares in other circumstances in which case the transferees, donees, pledgees or other successors in interest will be the selling beneficial owners for purposes of this prospectus.

The Registered Holders and any broker-dealer or agents participating in the distribution of the ADSs representing Registered Shares may be deemed to be “underwriters” within the meaning of Section 2(a)(11) of the Securities Act in connection with such dispositions. In such event, any commissions paid, or any discounts or concessions allowed to, any such broker-dealer or agent and any profit on the resale of the ADSs representing our Registered Shares purchased by them may be deemed to be underwriting commissions or discounts under the Securities Act. Registered Holders who are “underwriters” within the meaning of Section 2(a)(11) of the Securities Act will be subject to the applicable prospectus delivery requirements of the Securities Act including Rule 172 thereunder and may be subject to certain statutory liabilities of, including but not limited to, Sections 11, 12 and 17 of the Securities Act and Rule 10b-5 under the Securities Exchange Act of 1934, as amended, or the Exchange Act.

Each Registered Holder has informed us that it is not a registered broker-dealer and does not have any written or oral agreement or understanding, directly or indirectly, with any person to distribute ADSs representing Registered Shares. Upon our being notified in writing by a Registered Holder that any material arrangement has been entered into with a broker-dealer for the sale of ADSs representing Registered Shares through a block trade, special offering, exchange distribution or secondary distribution or a purchase by a broker or dealer, a supplement to this prospectus will be filed, if required, pursuant to Rule 424(b) under the Securities Act, disclosing (i) the name of each such Registered Holder and of the participating broker-dealer(s), (ii) the number of ADSs representing Registered Shares involved, (iii) the price at which such ADSs representing Registered Shares were sold, (iv) the commissions paid or discounts or concessions allowed to such broker-dealer(s), where applicable, (v) that such broker-dealer(s) did not conduct any investigation to verify the information set out or incorporated by reference in this prospectus, and (vi) other facts material to the transaction.

Each Registered Holder and any other person participating in such distribution will be subject to applicable provisions of the Exchange Act and the rules and regulations thereunder, including, without limitation, to the extent applicable, Regulation M of the Exchange Act, which may limit the timing of purchases and sales of any of ADSs representing Registered Shares by the Registered Holder and any other participating person. To the extent applicable, Regulation M may also restrict the ability of any person engaged in the distribution of the

200

ADSs representing Registered Shares to engage in market-making activities with respect thereto. All of the foregoing may affect the marketability of ADSs representing Registered Shares and the ability of any person or entity to engage in market-making activities with respect thereto.

We will pay all expenses of the registration of ADSs representing our shares pursuant to the registration rights agreement with respect thereto, including, without limitation, SEC filing fees and expenses of compliance with state securities or “blue sky” laws, if any; provided, however, that each Registered Holder will pay all underwriting discounts and selling commissions, if any, incurred by such Registered Holder in connection with the disposition of Registered Shares.

We are not party to any arrangement with any Registered Holder or any broker-dealer with respect to disposition of ADSs or Registered Shares, other than the Registration Rights Agreement described in elsewhere in this prospectus. Therefore, we will not have any input if, when and how any Registered Holder elects to dispose of ADSs representing such Registered Holder’s Registered Shares or the price or prices at which any such disposition may occur, and there can be no assurance that any Registered Holder will exchange its Registered Shares for ADSs or dispose of any or all of the ADSs representing such shares even if so exchanged pursuant to the deposit agreement. We will not receive proceeds from any disposition of Registered Shares in the form of ADSs by the Registered Holders.

To date, there has not been a public market for ADSs representing our shares. We offer no assurances that an active trading market for ADSs representing our shares will develop or, if developed, be maintained.

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EXPENSES OF THIS OFFERING

The following table sets forth the costs and expenses that we expect to incur in connection with this registration. All amounts are estimates except for the SEC registration fee and the Nasdaq Global Select Market listing fee.

Item	Amount to be Paid
SEC registration fee	$   *
Nasdaq Global Select Market listing fee	$*
Printing and engraving expenses	$*
Legal fees and expenses	$*
Accounting fees and expenses	$*
Miscellaneous expenses	$*
Total	$*

*
To be provided by amendment.

202

LEGAL MATTERS

The validity of the issuances of the shares underlying the ADSs offered in this prospectus and certain other matters of English law and U.S. federal law will be passed upon by Gibson, Dunn & Crutcher UK LLP and Gibson, Dunn & Crutcher LLP, respectively.

EXPERTS

The audited financial statements of Amryt included in this prospectus have been so included in reliance on the report of Grant Thornton, independent registered public accountants upon the authority of said firm as experts in accounting and auditing. The registered business address of Grant Thornton is 13-18 City Quay, Dublin 2, Ireland.

The consolidated financial statements of Aegerion Pharmaceuticals, Inc. and its subsidiaries (“Aegerion”) as of December 31, 2018 and 2017, and for the years then ended, included in this prospectus, have been audited by Deloitte & Touche LLP, independent auditors, as stated in their report appearing herein (which report expresses an unqualified opinion on the consolidated financial statements and includes emphasis of matter paragraphs referring to Aegerion's ability to continue as a going concern, transactions with Aegerion’s former parent and bankruptcy proceedings). Such consolidated financial statements have been so included in reliance upon the report of such firm given upon their authority as experts in accounting and auditing. The registered business address of Deloitte & Touche LLP is 200 Berkeley Street, Boston, Massachusetts 02116.

CHANGES IN REGISTRANT’S CERTIFYING ACCOUNTANT

At the general meeting of our shareholders held on July 10, 2018, Grant Thornton was approved to replace BDO LLP as chartered accountants and statutory auditor in the United Kingdom of Amryt for the 2018 reporting year. The appointment of Grant Thornton was the result of Board discussions completed on August 27, 2018 and the recommendation of Grant Thornton by our audit committee. BDO LLP resigned as our statutory auditor in the United Kingdom on August 27, 2018.

BDO LLP performed statutory audits of our financial statements, prepared under International Financial Reporting Standards as adopted by the European Union, for each of the fiscal years ending December 31, 2017 and 2016, respectively, in accordance with International Standards on Auditing (U.K. and Ireland). Neither BDO LLP’s reports relating to the statutory audits, nor the historic financial statements, prepared under International Financial Reporting Standards as adopted by the European Union, are included or incorporated by reference in the Prospectus constituting a part of this Registration Statement. BDO LLP’s statutory audit reports did not contain an adverse opinion or a disclaimer of opinion, and they were not qualified or modified as to uncertainty, audit scope or accounting principles, although BDO LLP stated in each of their statutory audit reports that:

“This report is made solely to the company’s members, as a body, in accordance with Chapter 3 of Part 16 of the Companies Act 2006. Our audit work has been undertaken so that we might state to the company’s members those matters we are required to state to them in an auditor’s report and for no other purpose. To the fullest extent permitted by law, we do not accept or assume responsibility to anyone other than the company and the company’s members as a body, for our audit work, for this report, or for the opinions we have formed.”

During the two years ended December 31, 2017 and the subsequent interim period through August 27, 2018, there were no disagreements over any matter of accounting principles or practices, financial statement disclosure or auditing scope or procedures, which disagreements if not resolved to BDO LLP’s satisfaction would have caused it to make reference to the subject matter of the disagreement in connection with its auditors’ reports; and (3) no “reportable event” as described in Item 16F(a)(1)(v) of Form 20-F occurred.

We have provided BDO LLP with a copy of this prospectus prior to its filing with the SEC and requested that it furnish us with a letter addressed to the SEC stating whether or not they agree with the above disclosure. A copy of that letter is filed as exhibit 16.1 to the registration statement of which this prospectus forms a part.

Furthermore, in the two years ended December 31, 2017, and the subsequent interim period through August 27, 2018, we did not consult with Grant Thornton regarding either (i) the application of accounting principles to a specified transaction, either completed or proposed, or the type of audit opinion that might be rendered with respect to our consolidated financial statements or (ii) any matter that was the subject of a disagreement as defined in Item 16F(a)(1)(iv) of Form 20-F or a reportable event as defined in Item 16F(a)(1)(v) of Form 20-F.

203

ENFORCEMENT OF CIVIL LIABILITIES

We are incorporated and currently existing under the laws of England and Wales. In addition, certain of our directors and officers reside outside of the United States and most of the assets of our non-U.S. subsidiaries are located outside of the United States. As a result, it may be difficult for investors to effect service of process on us or those persons in the United States or to enforce in the United States judgments obtained in United States courts against us or those persons based on the civil liability or other provisions of the United States securities laws or other laws.

In addition, uncertainty exists as to whether the courts of England and Wales would:

• recognize or enforce judgments of United States courts obtained against it or its directors or officers predicated upon the civil liabilities provisions of the securities laws of the United States or any state in the United States; or

• entertain original actions brought in England and Wales against us or our directors or officers predicated upon the securities laws of the United States or any state in the United States.

We are informed by Gibson, Dunn & Crutcher UK LLP that there is currently no treaty between (i) the United States and (ii) England and Wales providing for reciprocal recognition and enforcement of judgments of United States courts in civil and commercial matters (although the United States and the United Kingdom are both parties to the New York Convention on the Recognition and Enforcement of Foreign Arbitral Awards) and that a final judgment for the payment of money rendered by any general or state court in the United States based on civil liability, whether or not predicated solely upon the United States securities laws, would not be automatically enforceable in England and Wales. We also understand from Gibson, Dunn & Crutcher UK LLP that any final and conclusive monetary judgment for a definite sum obtained against us in United States courts would be treated by the courts of England and Wales as a cause of action in itself and sued upon as a debt at common law so that no retrial of the issues would be necessary, provided that:

• the appropriate procedural requirements in England and Wales for recognition and enforcement of such a judgment were followed;

• the relevant U.S. court had jurisdiction over the original proceedings according to English conflicts of laws principles at the time when proceedings were initiated;

• the courts of England and Wales had jurisdiction over the matter on enforcement, and the defendant either submitted to such jurisdiction or was resident or carrying on business within such jurisdiction and was duly served with process;

• the U.S. judgment was final and conclusive on the merits in the sense of being final and unalterable in the court that pronounced it and being for a definite sum of money;

• the judgment given by the courts was not in respect of penalties, taxes, fines or similar fiscal or revenue obligations (or otherwise based on a U.S. law that an English court considers to relate to a penal, revenue or other public law); if, on the contrary, this were the case, an English court may sever the relevant part from an otherwise unenforceable judgment;

• the judgment was not procured by fraud;

• recognition or enforcement of the judgment in England and Wales would not be contrary to public policy or the Human Rights Act 1998;

• the proceedings pursuant to which judgment was obtained were not contrary to natural justice or incompatible with the principles of notice in England and Wales;

• the U.S. judgment was not obtained in breach of an anti-suit injunction or an applicable alternative dispute resolutions provision;

• the U.S. judgment was not arrived at by doubling, trebling or otherwise multiplying a sum assessed as compensation for the loss or damages sustained and not being otherwise in breach of Section 5 of the UK Protection of Trading Interests Act 1980, or is a judgment based on measures designated by the Secretary of State under Section 1 of that Act; if, on the contrary, this were the case, an English court may sever the relevant part from an otherwise unenforceable judgment;

204

• there is not a prior decision of an English court or the court of another jurisdiction on the issues in question between the same parties; and

• the English enforcement proceedings were commenced within the limitation period.

Whether these requirements are met in respect of a judgment based upon the civil liability provisions of the United States securities laws, including whether the award of monetary damages under such laws would constitute a penalty, is an issue for the English court making such decision.

Subject to the foregoing, investors may be able to enforce in England and Wales judgments in civil and commercial matters that have been obtained from U.S. federal or state courts. Nevertheless, no assurance can be made that those judgments will be recognized or enforceable in England and Wales. Although the pendency of an appeal does not mean a judgment is not final or conclusive, the English courts may stay execution pending a possible appeal.

If an English court gives judgment for the sum payable under a U.S. judgment, the English judgment will be enforceable by methods generally available for this purpose. These methods generally permit the English court discretion to prescribe the manner of enforcement. In addition, it may not be possible to obtain an English judgment or to enforce that judgment if the judgment debtor is or becomes subject to any insolvency or similar proceedings, or if the judgment debtor has any set-off or counterclaim against the judgment creditor. Also note that, in any enforcement proceedings, the judgment debtor may raise any counterclaim that could have been brought if the action had been originally brought in England unless the subject of the counterclaim was in issue and denied in the U.S. proceedings.

205

WHERE YOU CAN FIND MORE INFORMATION

We have filed with the SEC a registration statement (including amendments and exhibits to the registration statement) on Form F-1 under the Securities Act with respect to the ADSs offered in this prospectus. This prospectus, which constitutes a part of the registration statement, does not contain all of the information set forth in the registration statement or the exhibits and schedules filed therewith. For further information with respect to us and our ordinary shares and ADSs, reference is made to the registration statement and the exhibits and schedules filed therewith. Statements contained in this prospectus regarding the contents of any contract or any other document that is filed as an exhibit to the registration statement are not necessarily complete, and each such statement is qualified in all respects by reference to the full text of such contract or other document filed as an exhibit to the registration statement. The SEC maintains a website that contains reports, proxy and information statements and other information regarding registrants that file electronically with the SEC. The address is https://www.sec.gov. We currently make available to the public our annual and interim reports, as well as certain information regarding our corporate governance and other matters on our website https://www.amrytpharma.com/. The reference to our website address does not constitute incorporation by reference of the information contained on or available through our website, and you should not consider it to be a part of this prospectus.

Upon the effectiveness of the registration statement of which this prospectus forms a part, we will become subject to the information reporting requirements of the Exchange Act applicable to foreign private issuers. Accordingly, we will be required to file reports and other information with the SEC, including annual reports on Form 20-F and periodic reports on Form 6-K. Those reports may be inspected without charge at the locations described above. As a foreign private issuer, we will be exempt from the rules under the Exchange Act related to the furnishing and content of proxy statements, and our officers, directors and principal shareholders will be exempt from the reporting and short-swing profit recovery provisions contained in Section 16 of such act. In addition, we will not be required under the Exchange Act to file periodic reports and financial statements with the SEC as frequently or as promptly as U.S. companies whose securities are registered thereunder.

As a foreign private issuer, we are also exempt from the requirements of Regulation FD (Fair Disclosure) which, generally, are meant to ensure that select groups of investors are not privy to specific information about an issuer before other investors. We are, however, still subject to the anti-fraud and anti-manipulation rules of the SEC, such as Rule 10b-5 of the Exchange Act. Since many of the disclosure obligations required of us as a foreign private issuer are different than those required by U.S. domestic reporting companies, our shareholders, potential shareholders and the investing public in general should not expect to receive information about us in the same amount and at the same time as information is received from, or provided by, U.S. domestic reporting companies.

We will send the depositary a copy of all notices of shareholders meetings and other reports, communications and information that are made generally available to shareholders. The depositary will, if we so request, mail to all registered holders of ADSs a notice containing the information (or a summary of the information) contained in any notice of a meeting of our shareholders received by the depositary from us or will make available to all registered holders of ADSs such notices and all such other reports and communications received by the depositary from us.

206

Amryt Pharma plc
INDEX TO FINANCIAL STATEMENTS

	Page
Financial Information of Amryt Pharma plc
Report of Independent Registered Public Accounting Firm	F-2
Consolidated Statement of Financial Position as of December 31, 2019 and December 31, 2018	F-3
Consolidated Statement of Comprehensive Loss for the years ended December 31, 2019 and December 31, 2018	F-4
Consolidated Statement of Cash Flows for the years ended December 31, 2019 and December 31, 2018	F-5
Consolidated Statement of Changes in Equity for the years ended December 31, 2019 and December 31, 2018	F-6
Notes to Consolidated Financial Statements	F-7

Consolidated Statement of Financial Position as of March 31, 2020 and December 31, 2019	F-48
Condensed Consolidated Statement of Comprehensive Loss for the periods ended March 31, 2020 and March 31, 2019	F-49
Condensed Consolidated Statement of Cash Flows for the periods ended March 31, 2020 and March 31, 2019	F-50
Condensed Consolidated Statement of Changes in Equity for the periods ended March 31, 2020 and March 31, 2019	F-51
Notes to Consolidated Financial Statements	F-53

	Page
Financial Information of Aegerion Pharmaceuticals, Inc.
Independent Auditors’ Report	F-68
Consolidated Balance Sheets as of December 31, 2018 and 2017	F-70
Consolidated Statements of Operations for the years ended December 31, 2018 and 2017	F-71
Consolidated Statements of Comprehensive Loss for the years ended December 31, 2018 and 2017	F-72
Consolidated Statements of Shareholders’ Equity (Deficit) for the years ended December 31, 2018 and 2017	F-73
Consolidated Statements of Cash Flows for the years ended December 31, 2018 and 2017	F-74
Notes to Consolidated Financial Statements	F-75

	Page
Financial Information of Aegerion Pharmaceuticals, Inc. (Debtor-in-Possession) (Unaudited)
Consolidated Balance Sheets as of June 30, 2019 and December 31, 2018	F-101
Consolidated Statements of Operations for the six months ended June 30, 2019 and 2018	F-102
Consolidated Statements of Comprehensive Loss for the six months ended June 30, 2019 and 2018	F-103
Consolidated Statements of Changes in Shareholders’ Deficit for the six months ended June 30, 2019 and 2018	F-104
Consolidated Statements of Cash Flows for the six months ended June 30, 2019 and 2018	F-105
Notes to Consolidated Financial Statements	F-106

F-1

REPORT OF INDEPENDENT REGISTERED PUBLIC ACCOUNTING FIRM

Board of Directors and Shareholders
Amryt Pharma Plc

Opinion on the financial statements

We have audited the accompanying consolidated statements of financial position of Amryt Pharma Plc and its subsidiaries (the “Company”) as of December 31, 2019 and 2018, and the related consolidated statements of comprehensive income, cash flows, and changes in equity for each of the two years in the period ended December 31, 2019, and the related notes (collectively referred to as the “financial statements”). In our opinion, the financial statements present fairly, in all material respects, the financial position of the Company as of December 31, 2019 and 2018, and the results of its operations and its cash flows for each of the two years in the period ended December 31, 2019, in conformity with International Financial Reporting Standards as issued by the International Accounting Standards Board.

Basis for opinion

These financial statements are the responsibility of the Company’s management. Our responsibility is to express an opinion on the Company’s financial statements based on our audits. We are a public accounting firm registered with the Public Company Accounting Oversight Board (United States) (“PCAOB”) and are required to be independent with respect to the Company in accordance with the U.S. federal securities laws and the applicable rules and regulations of the Securities and Exchange Commission and the PCAOB.

We conducted our audits in accordance with the standards of the PCAOB. Those standards require that we plan and perform the audit to obtain reasonable assurance about whether the financial statements are free of material misstatement, whether due to error or fraud. The Company is not required to have, nor were we engaged to perform, an audit of its internal control over financial reporting. As part of our audits we are required to obtain an understanding of internal control over financial reporting but not for the purpose of expressing an opinion on the effectiveness of the Company’s internal control over financial reporting. Accordingly, we express no such opinion.

Our audits included performing procedures to assess the risks of material misstatement of the financial statements, whether due to error or fraud, and performing procedures that respond to those risks. Such procedures included examining, on a test basis, evidence regarding the amounts and disclosures in the financial statements. Our audits also included evaluating the accounting principles used and significant estimates made by management, as well as evaluating the overall presentation of the financial statements. We believe that our audits provide a reasonable basis for our opinion.

/s/ Grant Thornton
GRANT THORNTON

Dublin, Ireland

We have served as the Company’s auditor since 2018.

April 20, 2020

F-2

Amryt Pharma plc
 
Consolidated Statement of Financial Position

		Year ended December 31,
		2019	2018
	Note	US$’000
Assets
Non-current assets
Goodwill	12	$30,813	$—
Intangible assets	12	350,953	60,297
Property, plant and equipment	13	3,036	1,098
Other non-current assets		2,306	149
Total non-current assets		387,108	61,544
Current assets
Trade and other receivables	14	36,387	5,927
Inventories	15	43,623	2,137
Cash and cash equivalents, including restricted cash	16	67,229	11,226
Total current assets		147,239	19,290
Total assets		534,347	80,834
Equity and liabilities
Equity attributable to owners of the parent
Share capital	17	11,918	25,198
Share premium	17	2,422	68,233
Other reserves		248,656	(24,865)
Accumulated deficit		(133,674)	(72,263)
Total equity		129,322	(3,697)
Non-current liabilities
Contingent consideration and contingent value rights	6	102,461	47,316
Deferred tax liability	18	18,921	6,161
Long term loan	19	81,610	19,011
Convertible notes	20	96,856	—
Provisions and other liabilities	22	4,963	—
Total non-current liabilities		304,811	72,488
Current liabilities
Trade and other payables	21	76,596	12,043
Provisions and other liabilities	22	23,618	—
Total current liabilities		100,214	12,043
Total liabilities		405,025	84,531
Total equity and liabilities		$534,347	$80,834

F-3

Amryt Pharma plc
 
Consolidated Statement of Comprehensive Loss

		Year ended December 31,
		2019	2018
	Note	US$’000
Revenue	3	$58,124	$17,095
Cost of sales	4	(42,001)	(6,266)
Gross profit		16,123	10,829
Research and development expenses		(15,827)	(10,703)
Selling, general and administrative expenses		(35,498)	(17,342)
Restructuring and acquisition costs	6	(13,038)	—
Share based payment expenses	5	(841)	(821)
Impairment charge	12	(4,670)	—
Operating loss before finance expense	7	(53,751)	(18,037)
Non-cash change in fair value of contingent consideration	6	(6,740)	(10,566)
Non-cash contingent value rights finance expense	6	(1,511)	—
Net finance expense — other	9	(4,759)	(1,841)
Loss on ordinary activities before taxation		(66,761)	(30,444)
Tax credit/(charge) on loss on ordinary activities	10	1,226	(43)
Loss for the year attributable to the equity holders of the Company		(65,535)	(30,487)
Exchange translation differences which may be reclassified through profit or loss		781	(77)
Total other comprehensive profit/(loss)		781	(77)
Total comprehensive loss for the year attributable to the equity holders of the Company		$(64,754)	$(30,564)
Loss per share
Loss per share - basic and diluted, attributable to ordinary equity holders of the parent (US$)	11	$(0.86)	$(0.67)

F-4

Amryt Pharma plc
 
Consolidated Statement of Cash Flows

		Year ended December 31,
		2019	2018
	Note	US$’000
Cash flows from operating activities
Loss on ordinary activities after taxation		$(65,535)	$(30,487)
Net finance expense — other	9	4,759	1,841
Depreciation and amortization	12, 13	12,655	367
Amortization of inventory fair value step-up	4, 7	10,367	—
Loss on disposal of fixed assets		43	—
Share based payment expenses	5	841	821
Non-cash change in fair value of contingent consideration	6	6,740	10,566
Non-cash contingent value rights finance expense	6	(1,511)	—
Impairment of intangible asset	12	4,670	—
Deferred taxation credit		(1,665)	—
Movements in working capital and other adjustments:
Change in trade and other receivables	14	(4,732)	(532)
Change in trade and other payables	21	(6,356)	3,051
Change in provision and other liabilities	22	4,922	—
Change in inventories	15	(5,894)	(928)
Change in non-current assets		177	(153)
Net cash flow used in operating activities		(37,497)	(15,454)
Cash flow from investing activities
Net cash received on acquisition of subsidiary	6	24,985	—
Payments for property, plant and equipment	13	(578)	(80)
Payments for intangible assets	12	(74)	(155)
Deposit interest received		92	6
Net cash flow from (used in) investing activities		24,425	(229)
Cash flow from financing activities
Proceeds from issue of equity instruments - net of expenses	17	63,009	—
Proceeds from long term borrowings net of debt issue costs	19	31,176	5,914
Repayment of long term debt	19	(21,990)	—
Interest paid	19	(6,253)	(283)
Payment of deferred consideration		—	(2,366)
Net cash flow from financing activities		65,942	3,265
Exchange and other movements		3,133	(767)
Net change in cash and cash equivalents		56,003	(13,185)
Cash and cash equivalents at beginning of year		11,226	24,411
Restricted cash at end of year	16	2,032	1,362
Cash at bank available on demand at end of year	16	65,197	9,864
Total cash and cash equivalents at end of year	16	$67,229	$11,226

F-5

Amryt Pharma plc
 
Consolidated Statement of Changes in Equity

		Share capital	Share premium	Warrant reserve	Treasury shares	Share based payment reserve	Merger reserve	Reverse acquisition reserve	Equity component of convertible notes	Other distributable reserves	Currency translation reserve	Accumulated deficit	Total
	Note	US$'000
Balance at January 1, 2018		$25,198	$68,233	$—	$—	$5,659	$42,627	$(73,914)	$—	$—	$26	$(41,783)	$26,046
Loss for the year		—	—	—	—	—	—	—	—	—	—	(30,487)	(30,487)
Foreign exchange translation reserve		—	—	—	—	—	—	—	—	—	(77)	—	(77)
Total comprehensive loss		—	—	—	—	—	—	—	—	—	(77)	(30,487)	(30,564)
Transactions with owners
Share based payment expense	5	—	—	—	—	821	—	—	—	—	—	—	821
Share based payment expense – Lapsed		—	—	—	—	(7)	—	—	—	—	—	7	—
Total transactions with owners		—	—	—	—	814	—	—	—	—	—	7	821
Balance at December 31, 2018		25,198	68,233	—	—	6,473	42,627	(73,914)	—	—	(51)	(72,263)	(3,697)
Balance at January 1, 2019		25,198	68,233	—	—	6,473	42,627	(73,914)	—	—	(51)	(72,263)	(3,697)
Loss for the year		—	—	—	—	—	—	—	—	—	—	(65,535)	(65,535)
Foreign exchange translation reserve		—	—	—	—	—	—	—	—	—	781	—	781
Total comprehensive loss		—	—	—	—	—	—	—	—	—	781	(65,535)	(64,754)
Transactions with owners
Share consolidation	17	(21,262)	21,262	—	—	—	—	—	—	—	—	—	—
Issue of shares in August 2019 equity fund raise	17	533	7,467	—	—	—	—	—	—	—	—	—	8,000
Issue costs associated with August 2019 equity fund raise	17	—	(1,886)	—	—	—	—	—	—	—	—	—	(1,886)
Acquisition of subsidiary without a change of control	17	(495)	(3,726)	—	—	—	—	—	—	(2,969)	7,190	—	—
Issue of shares and warrants in consideration of Aegerion Acquisition	17	5,759	132,392	14,464	—	—	—	—	—	—	—	—	152,615
Issue of shares and warrants in equity fund raise	17	2,059	47,338	10,603	—	—	—	—	—	—	—	—	60,000
Issue costs associated with September 2019 equity fund raise	17	—	(2,575)	(530)	—	—	—	—	—	—	—	—	(3,105)
Issue of convertible notes	20	—	—	—	—	—	—	—	29,210	—	—	—	29,210
Issue of contingent value rights	6	—	—	—	—	—	—	—	—	(47,902)	—	—	(47,902)
Transfer to distributable reserves	17	—	(268,505)	—	—	—	—	—	—	268,505	—	—	—
Treasury shares acquired in consideration for additional warrants	17	—	—	7,534	(7,534)	—	—	—	—	—	—	—	—
Issue of shares in exchange for warrants in December 2019	17	126	2,422	(2,548)	—	—	—	—	—	—	—	—	—
Share based payment expense	5	—	—	—	—	841	—	—	—	—	—	—	841
Share based payment expense – Lapsed		—	—	—	—	(4,124)	—	—	—	—	—	4,124	—
Total transactions with owners		(13,280)	(65,811)	29,523	(7,534)	(3,283)	—	—	29,210	217,634	7,190	4,124	197,773
Balance at December 31, 2019		$11,918	$2,422	$29,523	$(7,534)	$3,190	$42,627	$(73,914)	$29,210	$217,634	$7,920	$(133,674)	$129,322

F-6

1. General information

We are a global, commercial-stage biopharmaceutical company dedicated to commercializing and developing novel therapeutics to treat patients suffering from serious and life-threatening rare diseases.

As used herein, references to “we,” “us,” “Amryt” or the “Group” in these consolidated financial statements shall mean Amryt Pharma plc and its global subsidiaries, collectively. References to the “Company” in these consolidated financial statements shall mean Amryt Pharma plc.

Amryt Pharma plc is a company incorporated in England and Wales. The Company is listed on the AIM market of the London Stock Exchange (ticker: AMYT) and the Euronext Growth Exchange of the Irish Stock Exchange (ticker: AYP).

Aegerion Pharmaceuticals, Inc. (“Aegerion”), a former subsidiary of Novelion Therapeutics Inc., is a rare and orphan disease company with a diversified offering of multiple commercial and development stage assets. The acquisition of Aegerion by Amryt in September 2019 has given Amryt an expanded commercial footprint to market two U.S. and EU approved products, lomitapide (JUXTAPID (U.S.) / LOJUXTA (EU)) and metreleptin (MYALEPT (U.S.) / MYALEPTA (EU)).

On July 10, 2019, the shareholders of the Company approved a resolution to give authority to the Company to undertake a consolidation of the existing ordinary shares in the capital of the Company under which every six existing ordinary shares were consolidated into one ordinary share. The number of shares in issue at December 31, 2018 has been adjusted to reflect this share consolidation on July 10, 2019 for the purposes of the loss per share calculation. The number of share options outstanding at January 1, 2018 and the share options granted and lapsing during the year ended December 31, 2018 have been restated to reflect the 2019 share consolidation.

On September 20, 2019, Amryt registered FILSUVEZ as the trademark name for the Group’s lead development asset, AP101, in the European Union. On February 18, 2020, Amryt also registered this trademark name in the United States and is in the process of registering the FILSUVEZ trademark in other key jurisdictions.

2. Accounting policies

Basis of preparation

The consolidated financial statements of the Company and its subsidiaries (“Group”) have been prepared in accordance with International Financial Reporting Standards (“IFRS”) as issued by the International Accounting Standards Board (“IASB”). Except for the new accounting standards to IFRS that have been adopted by the Group, effective January 1, 2019, the financial statements have been prepared using the same accounting policies as 2018.

The consolidated financial statements were authorized for issue by the Company’s Board of Directors on April 20, 2020.

Basis of going concern

Having considered the Group’s current financial position and cash flow projections, the Board of Directors believes that the Group will be able to continue in operational existence for at least the next 12 months from the date of approval of these consolidated financial statements and that it is appropriate to continue to prepare the consolidated financial statements on a going concern basis.

As part of their inquiries, the Board of Directors reviewed budgets, projected cash flows, and other relevant information for 12 months from the date of approval of the consolidated financial statements for the year ended December 31, 2019.

A key consideration for the impact on going concern is the acquisition of Aegerion, which was completed in September 2019. This acquisition represents a significant step forward for Amryt and has created value for Amryt with immediate effect post-deal close through enhanced scale of the combined Group, which has the potential to drive revenues and deliver operational synergies through a combination of medical, commercial, clinical, development and regulatory infrastructure. Additionally, Amryt completed a US$60,000,000 fundraising as part of the acquisition of Aegerion.

F-7

Basis of consolidation

The consolidated financial statements comprise the financial statements of the Group for the years ended December 31, 2019 and 2018. Subsidiaries are entities controlled by the Company. Where the Company has control over an investee, it is classified as a subsidiary. The Company controls an investee if all three of the following elements are present: power over an investee, exposure or rights to variable returns from its involvement with the investee and the ability to use its power to affect those variable returns. Control is reassessed whenever facts and circumstances indicate that there may be a change in any of these elements of control.

Subsidiaries are fully consolidated from the date that control commences until the date that control ceases. Accounting policies of subsidiaries have been changed where necessary to ensure consistency with the policies adopted by the Group. Intergroup balances and any unrealized gains or losses, income or expenses arising from intergroup transactions are eliminated in preparing the consolidated financial statements.

Merger reserve

The merger reserve was created on the acquisition of Amryt Pharmaceuticals DAC (“Amryt DAC”) by Amryt Pharma plc in April 2016. Ordinary shares in Amryt Pharma plc were issued to acquire the entire issued share capital of Amryt DAC. Under section 612 of the Companies Act 2006, the premium on these shares has been included in a merger reserve.

Presentation of balances

Beginning January 1, 2018 (the earliest period presented), the Company changed its reporting currency from Euros (“€”) to U.S. dollars (“US$”) to align with the new functional currency of the Company, subsequent to the Aegerion acquisition in September 2019, and to provide greater clarity to users of these consolidated financial statements. The change in reporting currency was applied retrospectively beginning January 1, 2018 using the following procedures:

• assets and liabilities were translated from their Euro functional currency to U.S. dollars using the exchange rate in effect at the balance sheet date;

• income and expenditure was translated at the average rate of exchange prevailing for the relevant period; and

• opening shareholders’ equity at January 1, 2018 was translated at the historic rate on that date and any other movements in shareholders’ equity during the year have been translated using the rates prevailing on the date of the transaction.

Any differences which arose due to the change in reporting currency have been posted to the currency translation reserve.

The following table discloses the major exchange rates of those currencies other than the functional currency of US$ that are utilized by the Group:

Foreign currency units to 1 US$	€	£	CHF	SEK	NOK	DKK
Average period to December 31, 2019	0.8932	0.7836	0.9938	9.4533	8.7976	6.6690
At December 31, 2019	0.8929	0.7624	0.971	9.3282	8.8046	6.6698

Foreign currency units to 1 US$	€	£	CHF	SEK	NOK	DKK
Average period to December 31, 2018	0.8455	0.7485	0.9763	8.6784	8.1289	6.2997
At December 31, 2018	0.8739	0.7833	0.9976	9.0855	8.5654	6.5700

(€ = Euro; £ = Pounds Sterling, CHF = Swiss Franc, SEK = Swedish Kroner, NOK = Norwegian Kroner, DKK = Danish Kroner)

F-8

Changes in accounting policies and disclosures

New standards and amendments to IFRS effective as of January 1, 2019 that are relevant to the Group have been reviewed by the Group. These standards and amendments are described in more detail below.

Adoption of new standards issued and effective as of January 1, 2019

Impact of initial application of IFRS 16 Leases

IFRS 16 replaced IAS 17, Leases, and the related interpretations. The Group adopted IFRS 16 effective January 1, 2019 by applying the modified retrospective approach. The Group also elected various practical expedients, including the election to not separate lease and non-lease components, the election for leases of low value assets, and the election to not record leases with an initial term of 12 months or less on the statement of financial position. As a result of these elections, each lease component and any associated non-lease components are accounted for as a single lease, and leases with a total maximum term of 12 months and leases for underlying assets of low value will be exempt from balance sheet recognition.

Under IFRS 16, at the commencement date of a lease, a lessee will recognize a liability to make lease payments (i.e. the lease liability) and an asset representing the right to use the underlying asset during the lease term (i.e. the right-of-use asset).

Lessees will be required to separately recognize the interest expense on the lease liability and the depreciation expense on the right-of-use asset. Under IFRS 16 lessees will also be required to remeasure the lease liability upon the occurrence of certain events (e.g. a change in lease term or a change in future lease payments resulting from a change in an index or rate used to determine those payments). The lessee will generally recognize the amount of the re-measurement of the lease liability as an adjustment to the right-of-use asset.

Upon the initial application of IFRS 16 as of January 1, 2019, the Group recognized right-of-use asset and lease liabilities of US$874,000. The lease liabilities were discounted using the discount rates, which were arrived at using a methodology to calculate incremental borrowing rates across the Group as of January 1, 2019. The weighted average discount rate was 6.64%. Additionally, as a result of the adoption of IFRS 16, total amount of depreciation recognized related to the right-of-use assets was US$321,000 during the year ended December 31, 2019; total amount of interest expense recognized on the lease liability was US$36,000 during the year ended December 31, 2019.

The impact on the opening Retained earnings is considered immaterial, hence no adjustments were made to the Retained earnings as a result of adoption of IFRS 16.

In the current year, the Group has applied a number of amendments to IFRS and Interpretations issued by the IASB that are effective for annual period begins on or after January 1, 2019. These amendments and interpretations do not have significant impact on the disclosures or the amounts reported in these consolidated financial statements.

• IAS 19 Employee Benefits (Amendment on Employee Benefits Plan, Amendment, Curtailment or Settlement)

• IFRIC 23 Uncertainty over Income Tax Payments

• IFRS 9 Prepayment Features with Negative Compensation (Amendment to IFRS 9)

• IAS 28 Long-term Interests in Associates and Joint Ventures (Amendment to IAS 28)

• Annual improvements to IFRS 2015-2017 Cycle

Standards issued but not yet effective

There were a number of standards and interpretations which were in issue at December 31, 2019 but were not effective at December 31, 2019 and have not been adopted for these financial statements.

• Definition of Business (Amendment to IFRS 3 Business Combination)

• IFRS 17 Insurance Contracts

• Definition of Material (Amendments to IAS 1 and 8)

• Conceptual Framework for Financial Reporting

F-9

These amendments are not expected to have significant impact on disclosures or amounts reported in the consolidated financial statements in the period of initial application.

Critical accounting judgements and key sources of estimation uncertainty

The preparation of financial statements in conformity with IFRS requires management to make judgements, estimates and assumptions that affect the application of policies and amounts reported in the financial statements and accompanying notes. The estimates and associated assumptions are based on historical experience and various other factors that are believed to be reasonable under the circumstances, the results of which form the basis of making the judgements about the carrying value of assets and liabilities that are not readily apparent from other sources. Actual results may differ from these estimates.

The estimates and underlying assumptions are reviewed on an ongoing basis. Revisions to accounting estimates are recognized in the period in which the estimate is revised if the revision affects only that period or in the period of the revision and future periods if the revision affects both current and future periods.

The critical accounting policies which involve significant estimates, assumptions or judgements, the actual outcome of which could have a material impact on the Group’s results and financial position outlined below, are as follows:

Valuation of convertible notes

In conjunction with the accounting for financial instruments, the Group recorded compound financial instruments related to the convertible notes that were issued on September 24, 2019. In determining the classification of the convertible notes, the Group assessed the fixed-for-fixed criteria and considered that this was met and the number of shares that can be converted by holders of the notes is fixed. The compound financial instrument consists of a liability component and an equity component. The liability component is valued using an estimated discounted cash flow calculation based on the future contractual cash flows in the contract which are discounted at a rate of interest an identical financial instrument without a conversion feature would be subject to. Factors that are considered in estimating the prevailing market rate of interest include or are not limited to:

• loan term and maturity;

• repayment profile during the loan term other than interest;

• level of loan security; and

• principal amount of the loan.

Valuation of acquired assets

In conjunction with the accounting for business combinations, the Group recorded intangible assets such as in connection with the Aegerion acquisition, primarily related to developed technology on the commercially marketed products, and inventories which include raw materials and finished goods. The identifiable intangible assets and inventories are measured at their respective fair values as of the acquisition date. When significant identifiable intangible assets and inventories are acquired, the Group determines the fair values of these assets as of the acquisition date. The models used in valuing these intangible assets and inventories require the use of significant estimates and assumptions including but not limited to:

Intangible assets

• estimates of revenues and operating profits related to the products or product candidates;

• the probability of success for unapproved product candidates considering their stages of development;

• the time and resources needed to complete the development and approval of product candidates;

• projecting regulatory approvals;

• developing appropriate discount rates and probability rates by project; and

• tax implications, including the forecasted effective tax rate.

F-10

Inventories

• estimates of saleable inventory and non-saleable inventory, which was determined by a sales forecast and production timeline; and

• expected selling price and estimated costs of disposal.

The Group believes the fair values used to record intangible assets and inventories acquired in connection with a business combination are based upon reasonable estimates and assumptions given the facts and circumstances as of the acquisition date.

Valuation of contingent value rights (“CVRs”)

The Group issued CVRs for payments to its shareholders based on the occurrence of two milestones related to AP101, its pipeline product. The CVRs have pre-determined payouts, based on the occurrence of a future event. If the event does not occur, the CVR expires as worthless. The fair value of the CVRs is estimated as of September 24, 2019, based on the following key assumptions:

• expected timing of achievement of the two milestones (U.S. Food and Drug Administration (“FDA”) approval and European Medicines Agency approval) related to AP101;

• probabilities of achievements;

• revenue forecast related to AP101; and

• the appropriate discount rate selected to measure the risks inherent in the future cash flows.

The Group believes the fair value of the CVRs is based upon reasonable estimates and assumptions given the facts and circumstances as of the valuation date.

Impairment of intangible assets and goodwill

The impairment assessment for intangible assets requires management to make significant judgements and estimates to determine the fair value of the assets. Management periodically evaluates and updates the estimates based on the conditions which influence these variables. A detailed discussion of the impairment methodology applied and key assumptions used by the Group in the context of long-lived assets is provided in Note 12, Intangible assets and goodwill, to the consolidated financial statements. The assumptions and conditions for determining impairment of intangible assets reflect management’s best assumptions and estimates, but these items involve inherent uncertainties described above, many of which are not under management’s control. As a result, the accounting for such items could result in different estimates or amounts if management used different assumptions or if different conditions occur in future accounting periods.

Goodwill represents the difference between the purchase price and the fair value of the identifiable tangible and intangible net assets acquired in a business combination. Goodwill is not amortized, but instead is reviewed for impairment on an annual basis or when an event becomes known that could trigger an impairment. To perform the annual impairment test of goodwill, the Group has identified the Group as a whole as a single cash generating unit (“CGU”). CGUs reflect the lowest level at which goodwill is monitored for internal management purposes. At least once a year, the Group compares the recoverable amount of the Group’s CGU to the CGU’s carrying amount. The recoverable amount (value in use) of a CGU is determined using a discounted cash flow approach based upon the cash flow expected to be generated by the CGU. In case that the value in use of the CGU is less than its carrying amount, the difference is at first recorded as an impairment of the carrying amount of the goodwill. The assumptions utilized in the impairment test are dependent on management’s estimates, in particular in relation to the forecasting of future cash flows, the discount rates applied to those cash flows, the expected long-term growth rate of the applicable businesses and terminal values. As a result, the accounting for such items could result in different estimates or amounts if management used different assumptions or if different conditions occur in future accounting periods.

Contingent consideration

Contingent consideration arising as a result of business combinations is initially recognized at fair value using a probability adjusted present value model. The fair value of the contingent consideration is updated at each reporting date. The key judgements and estimates applied by management in the determination of the fair value

F-11

of the contingent consideration relate to the determination of an appropriate discount rate, the assessment of market size and opportunity and probability assessments based on market data for the chance of success of the commercialization of an orphan drug. A detailed discussion of the methodology applied and key input assumptions used by the Group is provided in Note 6, Business combinations and asset acquisitions, to the consolidated financial statements. The fair value of the contingent consideration uses management’s best estimates and judgements and sensitivities have been assessed by management by considering movements in the discount rate applied and movements in revenue forecasts. The chance of success of product development is based on published market data. See Note 24, Fair value measurement and financial risk management, for quantification of these sensitivities.

Research and development expenses

Development costs are capitalized as an intangible asset if all of the following criteria are met:

• completing the asset is technically feasible so that the asset will be available for use or sale;

• there is an intention to complete the asset and use or sell it;

• there is an ability to use or sell the asset;

• the asset will generate probable future economic benefits and demonstrate the existence of a market or the usefulness of the asset if it is to be used internally;

• adequate technical, financial and other resources are available to complete the development of the asset and to use or sell it; and

• there is an ability to measure reliably the expenditure attributable to the intangible asset.

In process R&D acquired as part of a business combination is capitalized at the date of acquisition. Research costs are expensed when they are incurred.

Factors which impact our judgement to capitalize certain research and development expenditures include the degree of regulatory approval for products and the results of any market research to determine the likely future commercial success of products being developed. Management reviews these factors each year to determine whether previous estimates as to feasibility, viability and recovery should be changed.

The assessment whether development costs can be capitalized requires management to make significant judgements. Management has reviewed the facts and circumstances of each project in relation to the above criteria and in management’s opinion, the criteria prescribed for capitalizing development costs as assets have not yet been met by the Group in relation to AP101 or AP103. Refer to Note 12, Intangible assets and goodwill, for further discussion on the impairment of AP102. Accordingly, all of the Group’s costs related to research and development projects are recognized as expenses in the Consolidated Statement of Comprehensive Loss in the period in which they are incurred. Management expects that the above criteria will be met on filing of a submission to the regulatory authority for final drug approval or potentially in advance of that on the receipt of information that strongly indicates that the development will be successful.

Business combination

On September 24, 2019, the Group acquired Aegerion. In accounting for this transaction, the Board of Directors considered the date of when control of Aegerion passed to the Group, the fair value of the consideration settled and the fair value of the assets and liabilities acquired. See Note 6, Business combinations and asset acquisitions, for further information on the determination of the fair value of the assets acquired.

Recognition of deferred tax assets

Deferred tax assets are determined using enacted tax rates for the effects of net operating losses and temporary differences between the book and tax bases of assets and liabilities. In assessing the realizability of deferred tax assets, management considers whether it is more likely than not that some portion or all of the deferred tax assets will not be realized. The ultimate realization of deferred tax assets is dependent upon the generation of future taxable income during the periods in which those temporary differences become deductible. While management considers the scheduled reversal of deferred tax liabilities, and projected future taxable income in

F-12

making this assessment, there can be no assurance that these deferred tax assets may be realizable. As at December 31, 2019, the Group did not recognize a deferred tax asset in respect of unused tax losses as described in Note 10, Tax on ordinary activities.

Principal accounting policies

Principal accounting policies are summarized below. They have been consistently applied throughout the period covered by the financial statements.

Revenue recognition

Revenue arises from the sale of metreleptin, lomitapide and Imlan. The Group sells direct to customers and also uses third parties in the distribution of products to customers.

To determine whether to recognize revenue, the Group follows a five-step process, as required by IFRS 15:

• identifying the contract with a customer;

• identifying the performance obligations;

• determining the transaction price;

• allocating the transaction price to the performance obligations; and

• recognizing revenue when/as performance obligation(s) are satisfied.

Revenue from contracts with customers is recognized when control of the goods or services are transferred to the customer at an amount that reflects the consideration to which the Group expects to be entitled to in exchange for those goods. The Group recognizes contract liabilities for consideration received in respect of unsatisfied performance obligations and reports these amounts as liabilities in the Consolidated Statement of Financial Position. Similarly, if the Group satisfies a performance obligation before it receives the consideration, the Group recognizes either a contract asset or a receivable in its Consolidated Statement of Financial Position, depending on whether something other than the passage of time is required before the consideration is due.

Revenue from sale of goods

Imlan revenue is generally recognized at a point in time when control of the inventory is transferred, generally the date of shipment, consistent with typical ex-works shipment terms.

Revenue is generally recognized at a point in time when control of the inventory is transferred to the end customer, generally on delivery of the goods.

Principal versus agent considerations

The Group enters into certain contracts for the sale of its products. This includes agreements with third parties to provide logistics, customer and commercial services, i.e. supply chain function and agreements with distributors. The Group determined that it has control over the goods before they are transferred to the customers and has the ability to direct the use or obtain benefits, hence the Group is the principal on the contracts due to the following factors:

• the Group is primarily responsible for fulfilling the promise to provide the promised goods;

• the Group bears the inventory risk before or after the goods have been ordered by the customer, during shipping or on return;

• the Group has the discretion in establishing the selling price of the goods to customers. The distributors’ consideration in these contracts is either the margin fee or commission; and

• the Group is exposed to the credit risk for the amounts receivable from the customers.

Where the above criteria are met, the Group recognizes revenue on a gross basis. The costs associated with the delivery of such goods to customers i.e. the costs associated with the services provided by the distributors to import and deliver the goods are recognized in the cost of sales.

F-13

Financial instruments

Recognition and derecognition

Financial instruments are classified on initial recognition as financial assets, financial liabilities or equity instruments in accordance with the substance of the contractual arrangement. Financial instruments are initially recognized when the Group becomes party to the contractual provisions of the instrument. Financial assets are de-recognized when the contractual rights to the cash flows from the financial asset expire or when the contractual rights to those assets are transferred. Financial liabilities are de-recognized when the obligation specified in the contract is discharged, cancelled or expired.

Classification and initial measurement of financial assets

Trade receivables are measured at the transaction price in accordance with IFRS 15. All financial assets are initially measured at fair value adjusted for transaction costs, if any.

Financial assets, other than those designated and effective as hedging instruments, are classified into the following categories:

• amortized cost;

• fair value through profit or loss (“FVTPL”); and

• fair value through other comprehensive income (“FVOCI”).

The Group did not have any financial assets categorized as FVTPL or FVOCI as at December 31, 2019 and 2018. The classification is determined by both:

• the Group’s business model for managing the financial asset; and

• the contractual cash flow characteristic of the financial asset.

Subsequent measurement of financial assets

Financial assets at amortized cost

Financial assets are measured at amortized cost if the assets meet the following conditions (and are not designated as FVTPL):

• they are held within a business model whose objective is to hold the financial assets and collect its contractual cash flows; and

• the contractual terms of the financial assets give rise to cash flows that are solely payments of principal and interest on the principal amount outstanding.

After initial recognition, these are measured at amortized cost using the effective interest method. Discounting is omitted where the effect of discounting is immaterial. The Group’s cash and cash equivalents and trade receivables fall into this category of financial instruments.

Cash and cash equivalents

Cash comprises cash on hand and bank balances. Cash equivalents are short-term, highly liquid investments that are readily convertible to known amounts of cash, which are subject to an insignificant risk of changes in value and have a maturity of three months or less at the date of acquisition.

Restricted cash

Restricted cash comprises current cash and cash equivalents that are restricted as to withdrawal or usage. Cash held by the Group’s distribution partner for LOJUXTA on behalf of the Group is treated as restricted cash in the financial statements. Aegerion also has restricted cash in an escrow account set-up in accordance with Aegerion’s bankruptcy plan as approved by the U.S. Bankruptcy Court.

Trade and other receivables

Trade and other receivables represent the Group’s right to an amount of consideration that is unconditional (i.e. only the passage of time is required before payment of the consideration is due).

F-14

Impairment of financial assets

The Group recognizes an allowance for expected credit losses (“ECLs”) for all debt instruments not held at FVTPL. ECLs are based on the difference between the contractual cash flows due in accordance with the contract and all the cash flows that the Group expects to receive, discounted at an approximation of the original effective interest rate. The expected cash flows will include cash flows from the sale of collateral held or other credit enhancements that are integral to the contractual terms.

For trade receivables, the Group applies a simplified approach in calculating ECLs. Therefore, the Group does not track changes in credit risk, but instead recognizes a loss allowance based on lifetime ECLs at each reporting date. The Group assesses ECL based on its historical credit loss experience, adjusted for forward-looking factors specific to the debtors and the economic environment.

Financial liabilities

Financial liabilities are categorized as “fair value through profit or loss” or “other financial liabilities measured at amortized costs using the effective interest method”.

Trade and other payables

Trade and other payables are initially measured at their fair value and are subsequently measured at their amortized cost using the effective interest rate method except for short-term payables when the recognition of interest would be immaterial.

Provisions

Provisions are recognized when the Group has a present legal or constructive obligation as a result of past events, it is probable that an outflow of resources will be required to settle the obligation and the amount can be reliably estimated.

The amount recognized as a provision is the best estimate of the consideration required to settle the present obligation at the reporting date, taking into account the risks and uncertainties surrounding the obligation. Where a provision is measured using the cash flows estimated to settle the present obligation, its carrying amount is the present value of those cash flows (when the effect of the time value of money is material).

When some or all of the economic benefits required to settle a provision are expected to be recovered from a third party, a receivable is recognized as an asset if it is virtually certain that reimbursement will be received and the amount of the receivable can be measured reliably.

Interest bearing loans and borrowings

Interest-bearing loans and borrowings are recognized initially at fair value less attributable transaction costs. Loans and borrowings are subsequently carried at amortized cost using the effective interest method. Interest is charged to the Consolidated Statement of Comprehensive Loss.

Convertible notes

Convertible notes are first assessed to determine classification as a financial liability or equity instrument for the financial instrument as a whole and components thereof. The initial carrying amount of a compound financial instrument is allocated to its equity and liability components.

The two components are evaluated first by measuring the fair value of the liability component. The fair value of the liability component is assessed using a discounted cash flow calculation based on the future contractual cash flows in the contract which are discounted at an estimated market prevailing rate of interest an identical financial instrument without a conversion feature would be subject to. The equity component is measured by determining the residual of the fair value of the instrument less the estimated fair value of the liability component.

The liability component is carried at amortized cost. Interest is calculated by applying the estimated prevailing market interest rate at the time of issue. The equity component is recognized in equity and is not subsequently remeasured.

F-15

Contingent consideration

Contingent consideration arising as a result of business combinations is initially recognized at fair value using a probability adjusted present value model. Key inputs in the model include the probability of success and the expected timing of potential revenues. The fair value of the contingent consideration will be updated at each reporting date. Adjustments to contingent consideration are recognized in the Consolidated Statement of Comprehensive Loss.

Offsetting financial instruments

Financial assets and financial liabilities are offset and the net amount is reported in the Consolidated Statement of Financial Position if there is a currently enforceable legal right to offset the recognized amounts and there is an intention to settle on a net basis, or to realize the asset and settle the liability simultaneously.

Inventories

Inventories are valued at the lower of cost or net realizable value. The costs are calculated according to the first in-first out method (“FIFO”). Cost includes materials, direct labor and an attributable proportion of manufacturing overhead based on normal levels of activity. Work in progress valuation is based on the stage of quality checks successfully performed during the production process. An inventory valuation adjustment is made if the net realizable value is lower than the book value. Net realizable value is determined as estimated selling prices less all costs of completion and costs incurred in selling and distribution.

Inventories held by third-party supply chain partners are included in inventory totals when control has deemed to be transferred to the Group under the contract terms of the distribution agreement. The cost to acquire the inventory held by the supply chain partners is recognized as a liability of the Group.

Leases

A lease is defined as a contract that conveys the right to use an underlying asset for a period of time in exchange for consideration. A contract is or contains a lease if:

• the underlying asset is identified in the contract; and

• the customer has both the right to direct the identified asset’s use and to obtain substantially all the economic benefits from that use.

Under IFRS 16, the Group is required to recognize a right-of-use asset representing its right to use the underlying asset and a lease liability representing its obligation to make lease payments for almost all leases.

Lease liabilities

Lease liabilities are initially recognized at the present value of the following payments, when applicable:

• fixed lease payments (including in-substance fixed payments), less any lease incentives receivable;

• variable lease payments (linked to an index or interest rate);

• expected payments under residual value guarantees;

• the exercise price of purchase options, where exercise is reasonably certain;

• lease payments in optional renewal periods, where exercise of extension options is reasonably certain; and

• penalty payments for the termination of a lease, if the lease term reflects the exercise of the respective termination option.

Lease payments are discounted using the implicit interest rate underlying the lease if this rate can be readily determined. Otherwise, the incremental borrowing rate is used as the discount rate.

Lease liabilities are subsequently measured at amortized cost using the effective interest method. Furthermore, lease liabilities may be remeasured due to lease modifications or reassessments of the lease. A lease modification is any change in lease terms that was not part of the initial terms and conditions of the lease, including increases

F-16

of the scope of the lease by adding the right to use one or more underlying assets or extending the contractual lease term, decreases of the scope of the lease by removing the right to use one or more underlying assets or shortening the contractual lease term or changes in the consideration. Reassessments are changes in estimates or changes triggered by a clause that was part of the initial lease contract, including changes in future lease payments arising from a change in an index or rate, change in the Group’s estimate of the amount expected to be payable under residual value guarantees or change in the Group’s assessment of whether it will exercise purchase, extension or termination options.

Right-of-use assets

The Group recognizes right-of-use assets at the commencement date of the respective lease. Right-of-use assets are stated at cost less accumulated depreciation. Upon initial recognition, cost comprises:

• the initial lease liability amount;

• initial direct costs incurred when entering into the lease;

• (lease) payments before commencement date of the respective lease;

• an estimate of costs to dismantle and remove the underlying asset; and

• less any lease incentives received.

Right-of-use assets are depreciated over the shorter of the lease term or the useful life of the underlying asset using the straight-line method. In addition, right-of-use assets are reduced by impairment losses, if any, and adjusted for certain remeasurements.

Foreign currency translation

Presentation currency

The Group translates foreign currency transactions into its presentational currency, US$, as described in “Presentation of balances” above.

Functional currency

The Company’s functional currency is US$.

Transactions in currencies other than the functional currency of the Group entities are recorded at the exchange rates prevailing at the dates of the related transactions. Foreign exchange gains and losses resulting from the settlement of such transactions, as well as from the translation at year-end exchange rates of monetary assets and liabilities denominated in foreign currencies, are recognized in the Consolidated Statement of Comprehensive Loss. At each balance sheet date, monetary assets and liabilities that are denominated in foreign currencies are translated to the respective functional currencies of the Group’s entities at the rates prevailing on the relevant balance sheet date. Non-monetary items that are measured in terms of historical cost in a foreign currency are translated using exchange rates at the dates of the initial transactions.

The financial statements of the Group’s foreign subsidiaries, where the local currency is the functional currency, are translated using exchange rates in effect at the end of the year for assets and liabilities and average exchange rates during the year for results of operations. The resulting foreign currency translation adjustment is recognized in other comprehensive income.

Property, plant and equipment

Property, plant and equipment is comprised of property and office equipment. Items of property, plant and equipment are stated at cost less any accumulated depreciation and any impairment losses. It is not Group policy to revalue any items of property, plant and equipment.

Depreciation is charged to the Consolidated Statement of Comprehensive Loss on a straight-line basis to write-off the cost of the assets over their expected useful lives as follows:

•	Property, plant and machinery	5 to 15 years
•	Office equipment	3 to 10 years

F-17

Business combinations

Business combinations, including the Aegerion acquisition, are accounted for using the acquisition method. The cost of an acquisition is measured as the aggregate of the consideration transferred, measured at acquisition date fair value and the amount of any non-controlling interest in the acquiree. Fair values are attributed to the identifiable assets and liabilities unless the fair value cannot be measured reliably, in which case the value is subsumed into goodwill. In the consolidated financial statements, acquisition costs incurred are expensed and included in general and administrative expenses.

To the extent that settlement of all or any part of the consideration for a business combination is deferred, the fair value of the deferred component is determined through discounting the amounts payable to their present value at the date of the exchange. The discount component is unwound as an interest charge in the Consolidated Statement of Comprehensive Income over the life of the obligation. Any contingent consideration is recognized at fair value at the acquisition date and included in the cost of the acquisition. The fair value of contingent consideration at acquisition date is arrived at through discounting the expected payment (based on scenario modelling) to present value. In general, in order for contingent consideration to become payable, pre-defined revenues and/or milestone dates must be exceeded. Subsequent changes to the fair value of the contingent consideration will be recognized in profit or loss unless the contingent consideration is classified as equity, in which case it is not remeasured and settlement is accounted for within equity.

When the initial accounting for a business combination is determined provisionally, any adjustments to the provisional values allocated to the consideration, identifiable assets or liabilities (and contingent liabilities, if relevant) are made within the measurement period, a period of no more than one year from the acquisition date.

Frequently, the acquisition of pharmaceutical patents and licenses is effected through a non-operating corporate structure. As these structures do not represent a business, it is considered that the transactions do not meet the definition of a business combination. Accordingly, the transactions are accounted for as the acquisition of an asset. The net assets acquired are recognized at cost.

Intangible assets

Intangible assets primarily relate to developed technology on the Company’s commercially marketed products and IPR&D. Intangible assets are recorded at fair value at the time of their acquisition and are stated in the Consolidated Statement of Financial Position, net of accumulated amortization and impairments, if applicable.

Acquired intangible assets outside business combinations are stated at the lower of cost less provision for amortization and impairment or the recoverable amount. Acquired intangible assets are amortized over their expected useful economic life on a straight-line basis. In determining the useful economic life, each acquisition is reviewed separately and consideration is given to the period over which the Group expects to derive economic benefit.

In connection with the acquisition of Aegerion, the Group acquired developed technology on metreleptin and lomitapide, which are amortized over the remaining patent lives through February 2026 and August 2027, respectively.

The useful life of other acquired intangible assets is as follows:

•	Software and hardware	3-10 years
•	Website development	5-10 years

Intangible assets acquired in 2016 as part of the acquisitions of Amryt AG and SomPharmaceuticals are currently not being amortized as the assets are still under development.

Factors which impact our judgement to capitalize certain research and development expenditures include the degree of regulatory approval for products and the results of any market research to determine the likely future commercial success of products being developed. Management reviews these factors each year to determine whether previous estimates as to feasibility, viability and recovery should be changed.

Impairment of non-financial assets

At each reporting date, the Group reviews the carrying amounts of its property, plant and equipment and acquired intangible assets to determine whether there is any indication that those assets have suffered an

F-18

impairment loss. If any such indication exists, the recoverable amount of the asset is estimated in order to determine the extent of the impairment loss. Any impairment loss arising from the review is charged to the Consolidated Statement of Comprehensive Loss.

The Group assesses each asset or cash-generating unit annually to determine whether any indication of impairment exists. Where an indicator of impairment exists, a formal estimate of the recoverable amount is made, which is considered to be the higher of the carrying value and value in use. These assessments require the use of estimates and assumptions such as discount rates, future capital requirements, general risks affecting the pharmaceutical industry and other risks specific to the individual asset. Fair value is determined as the amount that would be obtained from the sale of the asset in an arm’s length transaction between knowledgeable and willing parties. Fair value is generally determined as the present value of estimated future cash flows arising from the continued use of the asset, using assumptions that an independent market participant may take into account. Cash flows are discounted to their present value using a pre-tax discount rate that reflects current market assessments of the time value of money and the risks specific to the asset. Assets are grouped into the smallest group that generates cash inflows which are independent of other assets.

Taxes

Tax comprises current and deferred tax. Current tax is the expected tax payable on the taxable income for the year, using tax rates enacted or substantively enacted at the reporting date and taking into account any adjustments stemming from prior years. Deferred tax assets or liabilities are recognized where the carrying value of an asset or liability in the Consolidated Statement of Financial Position differs to its tax base and is accounted for using the statement of financial position liability method. Recognition of deferred tax assets is restricted to those instances where it is probable that taxable profit will be available against which the difference can be utilized.

In connection with business combinations, deferred tax balances are recognized if related to temporary differences and loss carry-forwards at the acquisition date or if they arise as a result of the acquisition and are measured in accordance with IAS 12 Income Taxes.

Share-based payments

The Group issues share options as an incentive to certain senior management and staff. The fair value of options granted is recognized as an expense with a corresponding credit to the share-based payment reserve. The fair value is measured at grant date and spread over the period during which the awards vest.

For equity-settled share-based payment transactions, the goods or services received and the corresponding increase in equity are measured directly at the fair value of the goods or services received, unless that fair value cannot be estimated reliably. If it is not possible to estimate reliably the fair value of the goods or services received, the fair value of the equity instruments granted as calculated using the Black-Scholes model is used as a proxy.

The Group may issue warrants to key consultants, advisers and suppliers in payment or part payment for services or supplies provided to the Group. The fair value of warrants granted is recognized as an expense. The corresponding credits are charged to the share-based payment reserve. The fair value is measured at grant date and spread over the period during which the warrants vest. The fair value is measured using the Black-Scholes model if the fair value of the services received cannot be measured reliably.

The estimate of the fair value of services received is measured based on the Black-Scholes model using input assumptions, including weighted average share price, expected volatility, weighted average expected life and expected yield. The expected life of the options is based on historical data and is not necessarily indicative of exercise patterns that may occur. The expected volatility is based on the historical volatility (calculated based on the expected life of the options). The Group has considered how future experience may affect historical volatility.

Employee Benefits

Defined contribution plans

The Group operates defined contribution schemes in various locations where employees are based. Contributions to the defined contribution schemes are recognized in the Consolidated Statement of Comprehensive Loss in the

F-19

period in which the related services are received from the employee. Under these schemes, the Group has no obligation, either legal or constructive, to pay further contributions in the event that the fund does not hold sufficient assets to meet its benefit commitments.

3. Segment information

The Group is a global, commercial-stage biopharmaceutical company dedicated to commercializing and developing novel therapeutics to treat patients suffering from serious and life-threatening rare diseases.

In 2018, the Group reported two operating segments: commercial and research and development. As a result of an internal reorganization, the Group now identifies one business segment. Corresponding items of the earliest period presented have been restated to reflect this change.

The Group currently operates as one business segment, pharmaceuticals, and is focused on the development and commercialization of two commercial products and two development products. The Group derives its revenues primarily from one source, being the pharmaceutical sector with high unmet medical need.

The Group’s Chief Executive Officer, Joseph Wiley, is currently the Company’s chief operating decision maker (“CODM”). The Group does not operate any separate lines of business or separate business entities with respect to its products. Accordingly, the Group does not accumulate discrete financial information with respect to separate service lines and does not have separate reportable segments.

The following table summarizes total revenues from external customers by product and by geographic region, based on the location of the customer. Revenues represent the revenue from the Group for the full year (which includes revenue from Aegerion, with acquired products and additional regions, from September 24, 2019 onward).

	December 31, 2019
	U.S.	EMEA	Other	Total
	US$'000
Metreleptin	$14,944	$8,048	$2,096	$25,088
Lomitapide	10,616	18,985	2,659	32,260
Other	—	671	105	776
Total revenue	$25,560	$27,704	$4,860	$58,124

	December 31, 2018
	U.S.	EMEA	Other	Total
	US$'000
Metreleptin	$—	$—	$—	$—
Lomitapide	—	15,132	978	16,110
Other	—	928	57	985
Total revenue	$—	$16,060	$1,035	$17,095

Major Customers

For the year ended December 31, 2019, one customer accounted for 44% of the Group’s net revenues and accounted for 44% of the Group’s December 31, 2019 accounts receivable balance. For the year ended December 31, 2018, the Group generated over 76% of its lomitapide revenue in Italy, the Netherlands and Greece. The largest customer in 2018 was a hospital in Greece.

4. Cost of sales

	December 31,
	2019	2018
	US$'000
Cost of product sales	$11,384	$3,588
Amortization of acquired intangibles (see Note 12)	11,831	—
Amortization of inventory fair value step-up (see Note 15)	10,367	—
Royalty expenses	8,419	2,678
Total cost of sales	$42,001	$6,266

F-20

As a result of the acquisition of Aegerion in September 2019, the Group acquired certain inventory, which were measured at fair value on the acquisition date. Refer to Note 2, Accounting policies, for further discussion on the key assumptions utilized to estimate the fair value. The difference between the estimated fair value and the book value of the acquired inventory was amortized, using the straight-line method, over the estimated period that the Group intends to sell this inventory.

5. Share based payments

On July 10, 2019, the shareholders of the Company approved a resolution to give authority to the Company to undertake a consolidation of the existing ordinary shares in the capital of the Company under which every 6 existing ordinary shares were consolidated into one ordinary share.

In the table below, for presentational purposes, the number of share options and warrants outstanding at January 1, 2019 and 2018 and the share options and warrants granted and lapsing during the years ended December 31, 2019 and 2018 have been restated to reflect the 2019 6-for-1 share consolidation.

Under the terms of the Company’s Employee Share Option Plan, options to purchase 14,481,720 shares were outstanding at December 31, 2019. Under the terms of this plan, options are granted to officers, consultants and employees of the Group at the discretion of the Remuneration Committee. A total of 11,330,641 share options were granted to directors and employees in 2019. There were no new share options granted during the year ended December 31, 2018.

The Company has issued warrants pre-2018 to key consultants, advisers and suppliers in payment or part payment for services or supplies provided to the Group.

There were no similar warrants granted during either of the years ended December 31, 2019 and December 31, 2018.

The terms and conditions of the grants are as follows, whereby all options are settled by physical delivery of shares:

Vesting conditions

The employee share options vest following a period of service by the officer or employee. The required period of service is determined by the Remuneration Committee at the date of grant of the options (usually the date of approval by the Remuneration Committee) and it is generally over a three-year period. There are no market conditions associated with the share option vesting periods.

Contractual life

The term of an option is determined by the Remuneration Committee provided that the term may not exceed a period of seven to ten years from the date of grant. All options will terminate 90 days after termination of the option holder’s employment, service or consultancy with the Group except where a longer period is approved by the Board of Directors. Under certain circumstances involving a change in control of the Group, each option will automatically accelerate and become exercisable in full as of a date specified by the Board of Directors.

The number and weighted average exercise price (in Sterling pence) of share options and warrants per ordinary share is as follows:

	Share Options		Warrants
	Units	Weighted average exercise price (Sterling pence)	Units	Weighted average exercise price (Sterling pence)
Balance at January 1, 2018 (pre share consolidation)	19,696,586	19.16p	23,103,481	24.74p
Balance at January 1, 2018 (restated for 6:1 share consolidation)	3,282,764	114.96p	3,850,580	148.44p
Lapsed	(31,909)	142.50p	(32,255)	672.00p
Outstanding at December 31, 2018	3,250,855	115.20p	3,818,325	144.00p
Exercisable at December 31, 2018	1,327,406	116.83p	3,818,325	144.00p

F-21

	Share Options		Warrants
	Units	Weighted average exercise price (Sterling pence)	Units	Weighted average exercise price (Sterling pence)
Balance at January 1, 2019 (pre share consolidation)	3,250,855	115.20p	3,818,325	144.00p
Granted	11,330,641	117.01p	18,841,378	—
Lapsed	(99,776)	197.66p	(3,472,783)	144.00p
Exercised	—	—	(1,645,105)	—
Outstanding at December 31, 2019	14,481,720	116.00p	17,541,815	0.03p
Exercisable at December 31, 2019	2,468,310	109.08p	17,541,815	0.03p

The 18,841,378 warrants granted during the year ended December 31, 2019 consist of 8,065,000 zero cost warrants issued to acquire Aegerion, 5,911,722 warrants issued to investors in connection with the US$60,000,000 equity raise and 4,864,656 warrants that were issued in connection with the repurchase of ordinary shares from certain shareholders. Refer to Note 17, Share capital and reserves for further details on the warrants exercised during the year ended December 31, 2019.

Outstanding warrants at December 31, 2019 consisted of 17,196,273 zero cost warrants with no expiration date that were issued to Aegerion creditors in connection with the acquisition of Aegerion (see Note 6, Business combinations and asset acquisitions) and investors in connection with the US$60,000,000 equity raise (see Note 17, Share capital and reserves). The remaining warrants consisting of 345,542 warrants were issued in connection with the admission to the AIM in 2016 and any subsequent reference to warrants in this note relate to the warrants issued in 2016.

Fair value is estimated at the date of grant using the Black-Scholes pricing model, taking into account the terms and conditions attached to the grant. The following are the inputs to the model for the equity instruments granted during the year:

	2019 Options Inputs	2019 Warrant Inputs	2018 Options Inputs	2018 Warrant Inputs
Days to Expiration	2,555	—	—	—
Volatility	27% - 48%	—	—	—
Risk free interest rate	0.38% - 0.83%	—	—	—
Share price at grant	75.84p – 121.5p	—	—	—

In 2019, a total of 11,330,641 share options exercisable at a weighted average price of £1.17 were granted. The fair value of share options granted in 2019 was £13,258,000/US$16,919,000. There were no new share options granted in 2018.

The share options outstanding as at December 31, 2019 have a weighted remaining contractual life of 6.19 years with exercise prices ranging from £0.76 to £1.50. The share options outstanding as at December 31, 2018 had a weighted remaining contractual life of 4.94 years with exercise prices ranging from £0.93 to £2.88.

The warrants outstanding as at December 31, 2019 have a weighted remaining contractual life of 1.3 years with an exercise price of £1.44. The remaining warrants outstanding as at December 31, 2018 had a weighted remaining contractual life of 0.25 years with an exercise price of £1.44.

The value of share options charged to the Consolidated Statement of Comprehensive Loss during the year is as follows:

	December 31,
	2019	2018
	US$’000
Share option expense	$841	$821
Total share option expense	$841	$821

F-22

6. Business combinations and asset acquisitions

Acquisition of Aegerion Pharmaceuticals

On May 20, 2019, Amryt entered into a Restructuring Support Agreement (as subsequently amended on June 12, 2019) and Plan Funding Agreement pursuant to which, among other matters, Amryt agreed to the acquisition of Aegerion Pharmaceuticals, Inc. (“Aegerion”), a former wholly-owned subsidiary of Novelion Therapeutics Inc. (“Novelion”). On May 20, 2019, Aegerion and its U.S. subsidiary, Aegerion Pharmaceuticals Holdings, Inc., filed voluntary petitions under Chapter 11 of Title 11 of the U.S. Code in the Bankruptcy Court. On September 24, 2019, Amryt completed the acquisition of Aegerion. Amryt acquired Aegerion upon its emergence from bankruptcy in an exchange for ordinary shares and zero cost warrants in Amryt. Amryt issued 85,092,423 effective shares at US$1.793 per share, which is made up of 77,027,423 ordinary shares and 8,065,000 zero cost warrants, to acquire Aegerion for a value of US$152,615,000.

The Company believes that the acquisition of Aegerion will enable the Group to advance the Group’s ambition to create a global leader in rare and orphan diseases with a diversified offering of multiple development-stage and commercial assets and provides it with scale to support further growth.

As part of the acquisition of Aegerion, it was agreed, for certain Aegerion creditors who wished to restrict their percentage share interest in Amryt’s issued share capital, to issue to the relevant Aegerion creditor, as an alternative to Amryt’s ordinary shares, an equivalent number of new zero cost warrants to subscribe for Amryt’s ordinary shares to be constituted on the terms of the zero cost warrant. Refer to Note 23, Related party transactions, for further discussion.

Relevant Aegerion creditors are entitled at any time to exercise the zero cost warrants, at which point in time, the Company would issue to that Aegerion creditor the relevant number of fully paid ordinary shares in return for the exercise of the zero cost warrants. Each zero cost warrant entitles the holder thereof to subscribe for one ordinary share. The zero cost warrants constitute the Company’s direct and unsecured obligations and rank pari passu and without any preference among themselves (save for any obligations to be preferred by law) at least equally with the Company’s other present and future unsecured and unsubordinated obligations. The zero cost warrants are not transferable except with the Company’s prior written consent.

On November 14, 2019, the Company repurchased a combined 4,864,656 ordinary shares from Highbridge Tactical Master Fund L.P., Highbridge SCF Special Situations SPV, L.P. and Nineteen77 Global Multi Strategy Alpha Master Limited. In exchange for the ordinary shares, these institutions were issued an equivalent number of zero cost warrants.

The table below reflects the fair value of the identifiable net assets acquired in respect of the acquisition completed during the year. Any amendments to fair values will be made within the twelve-month period from the date of acquisition, as permitted by IFRS 3 Business Combinations.

	Provisional Fair Value at date of acquisition
	US$’000
Assets
Non-current assets
Property, plant and equipment	$276
Right of use assets	924
Intangible Assets	308,374
Other assets	2,334
Total non-current assets	311,908
Current assets
Cash and cash equivalents	24,985
Trade and other receivables	23,259
Inventory	45,959
Prepaid expenses and other assets	2,469
Total current assets	96,672
Total assets	408,580

F-23

	Provisional Fair Value at date of acquisition
	US$’000
Current liabilities
Accounts payable	5,137
Accrued liabilities	64,088
Lease liabilities – current	384
Provision for legal settlements – current	14,916
Total current liabilities	84,525
Non-current liabilities
Lease liabilities - long term	538
Long term debt	54,469
Convertible notes debt and equity components - long term	125,000
Provision for legal settlements - long term	7,821
Deferred tax liability	14,425
Total non-current liabilities	202,253
Total liabilities	286,778
Total identifiable net assets at fair value	121,802
Goodwill arising on acquisition	30,813
Consideration	152,615
Consideration
Issue of fully paid up ordinary shares and zero cost warrants	152,615
Total consideration	$ 152,615

The acquired goodwill is attributable principally to the profit generating potential of the businesses, the assembled workforce and benefits arising from embedded infrastructure, that are expected to be achieved from integrating the acquired businesses into the Group’s existing business. No amount of goodwill is expected to be deductible for tax purposes.

In the post-acquisition period to December 31, 2019, the business acquired during the current year contributed revenue of US$38,392,000 and a trading loss of US$17,239,000 to the Group’s results.

The full year unaudited revenue and trading loss had the acquisitions taken place at the start of the year, would have been US$185,260,000 and US$53,057,000 respectively. In February 2019, the Aegerion Group out licensed the rights to JUXTAPID for distribution in Japan to Recordati Rare Diseases Inc. (“Recordati”). Included in the full year revenue total for 2019 is $28,495,000 relating to an upfront payment for the license and the transfer of the JUXTAPID marketing authorization to Recordati. The 2019 revenue total also includes a mix of product revenues to Japan from January 2019 until the end of the transition period in May 2019 and then royalty income from Recordati to Aegerion at a rate of 22.5% on net sales of JUXTAPID in Japan for the remaining part of the year.

The gross contractual value of trade and other receivables as at the dates of acquisition amounted to US$23,259,000, which approximated the fair value of these accounts as the amount not expected to be collected was insignificant.

The Group incurred acquisition and restructuring related costs of US$13,038,000 relating to external legal fees, advisory fees, due diligence costs and severance costs. These costs have been included in operating costs in the Consolidated Statement of Comprehensive Income.

The initial assignment of fair values to identifiable net assets acquired has been performed on a provisional basis due to the relative size of the acquisition and the timing of the transaction. Any amendments to these fair values within the twelve-month timeframe from the date of acquisition will be disclosed in the 2020 consolidated financial statements, as stipulated by IFRS3.

F-24

Contingent Value Rights

Related to the transaction, Amryt issued Contingent Value Rights (“CVRs”) pursuant to which up to US$85,000,000 may become payable to Amryt’s shareholders and option holders, who were on the register prior to the completion of the acquisition on September 20, 2019, if certain approval and revenue milestones are met in relation AP101, Amryt’s lead product candidate. If any such milestone is achieved, Amryt may elect to pay the holders of CVRs by the issue of Amryt shares or loan notes. If Amryt elects to issue Loan Notes to holders of CVRs, it will settle such loan notes in cash 120 days after their issue. If none of the milestones are achieved, scheme shareholders and option holders will not receive any additional consideration under the terms of the CVRs. In these circumstances, the value of each CVR would be zero.

The terms of the CVRs are as follows:

• The total CVR payable is up to US$85,000,000

• This is divided into three milestones which are related to the success of AP101 (the Group’s lead development asset, currently in Phase 3 clinical trials)

• FDA approval

○ US$35,000,000 upon FDA approval

○ 100% of the amount due if approval is obtained before December 31, 2021, with a sliding scale on a linear basis to zero if before July 1, 2022

• EMA approval

○ US$15,000,000 upon EMA approval

○ 100% of the amount due if approval is obtained before December 31, 2021, with a sliding scale on a linear basis to zero if before July 1, 2022

• Revenue targets

○ US$35,000,000 upon AP101 revenues exceeding US$75,000,000 in any 12-month period prior to June 30, 2024

• Payment can at the Board’s discretion be in the form of either:

○ 120-day loan notes (effectively cash), or

○ Shares valued using the 30 day / 45-day VWAP.

The CVRs were contingent on the successful completion of the acquisition and, accordingly, have been based on fair value as at September 24, 2019. In the Company-only accounts, the CVRs have been classified as a financial liability in the Consolidated Statement of Financial Position and debited to cost of investment in subsidiary. On consolidation, given that CVRs were issued to legacy Amryt shareholders in their capacity as owners of the identified acquirer as opposed to the seller in the transaction, management concluded that the most appropriate classification would be to recognize the CVR as a distribution on consolidation instead of goodwill.

Measurement of CVRs

As at December 31, 2019, the carrying value of the CVRs was US$49,413,000. The value of the potential payout was calculated using the probability-weighted expected returns method. Using this method, the potential payment amounts were multiplied by the probability of achievement and discounted to present value The probability adjusted present values took into account published orphan drug research data and statistics which were adjusted by management to reflect the specific circumstances applicable to the type of product acquired in the Amryt GmbH transaction. Discount rates of 10% and 16.5%, as applicable, were used in the calculation of the present value of the estimated contractual cash flows for the year ended December 31, 2019. Management was required to make certain estimates and assumptions in relation to revenue forecasts, timing of revenues and probability of achievement of commercialization of AP101. However, management notes that, due to issues outside their control (i.e. regulatory requirements and the commercial success of the product), the timing of when such revenue targets may occur may change. Such changes may have a material impact on the assessment of the expected cash flows of the CVRs.

F-25

Amryt reviews the expected cash flows on a regular basis as the discount on initial recognition is being unwound as financing expenses in the Consolidated Statement of Comprehensive Loss over the life of the obligation. It is reviewed on a quarterly basis and the appropriate finance charge is booked in the consolidated statement of income on a quarterly basis. The Group expects to read out top-line data from the Phase 3 trial of AP101 in Epidermolysis Bullosa (“EB”) in the second half of 2020, followed by applications for approval from the FDA and the EMA, if top-line data is positive. Coupled with this, management has completed its annual forecast and revenues and costs reflect these current expectations.

The total non-cash finance charge recognized in the Consolidated Statement of Comprehensive Loss for the year ended December 31, 2019 is US$1,511,000.

Acquisition of Amryt AG (previously “Birken”)

Amryt DAC signed a conditional share purchase agreement to acquire Amryt AG on October 16, 2015 (“Amryt AG SPA”). The Amryt AG SPA was completed on April 18, 2016 with Amryt DAC acquiring the entire issued share capital of Amryt GmbH. The consideration included contingent consideration comprising milestone payments and sales royalties as follows:

• Milestone payments of:

○ €10,000,000 on receipt of first marketing approval by the EMA of Episalvan, paid on the completion date (April 18, 2016);

○ Either (i) €5,000,000 once net ex-factory sales of Episalvan have been at least €100,000 or (ii) if no commercial sales are made within 24 months of EMA first marketing approval (being January 14, 2016), €2,000,000 24 months after receipt of such approval, which was paid in January 2018, and €3,000,000 following the first commercial sale;

○ €10,000,000 on receipt of marketing approval by the EMA or the FDA of a pharmaceutical product containing Betulin as its API for the treatment of EB;

○ €10,000,000 once net ex-factory sales/net revenue in any calendar year exceed €50,000,000;

○ €15,000,000 once net ex-factory sales/ net revenue in any calendar year exceed €100,000,000;

• Cash consideration of €150,000, due and paid on the completion date (April 18, 2016); and

• Royalties of 9% on sales of Episalvan products for 10 years from first commercial sale.

Fair Value Measurement of Contingent Consideration

As of December 31, 2019, the fair value of the contingent consideration was estimated to be US$53,048,000 (2018: US$47,316,000). The fair value of the royalty payments was determined using probability weighted revenue forecasts and the fair value of the milestone payments was determined using probability adjusted present values (see Note 24, Fair value measurement and financial risk management, for fair value hierarchy applied and impact of key unobservable impact data). The probability adjusted present values took into account published orphan drug research data and statistics which were adjusted by management to reflect the specific circumstances applicable to the type of product acquired in the Amryt GmbH transaction. A discount rate of 24.4% (2018: 28.5%) was used in the calculation of the fair value of the contingent consideration for the year ended December 31, 2019. At that time management anticipated that AP101 for EB would be ready to launch in 2019. However, management noted that due to issues outside their control, the timing of when such revenue targets may occur may change. Such changes may have a material impact on the assessment of the fair value of the contingent consideration.

Amryt reviews the contingent consideration on a regular basis as the probability adjusted fair values are being unwound as financing expenses in the Consolidated Statement of Comprehensive Loss over the life of the obligation. The finance charge is being unwound as a financing expense in the Consolidated Statement of Comprehensive Loss on a quarterly basis.

The total non-cash finance charge recognized in the Consolidated Statement of Comprehensive Income for the year ended December 31, 2019 is US$6,740,000 (2018: US$10,566,000).

In January 2019, the Group received the results of an unblinded interim efficacy analysis for the Phase 3 trial of AP101 in EB. This analysis was conducted by an independent data safety monitoring committee and

F-26

recommended that the trial should continue with an increase of 48 patients in the study to a total of 230 evaluable patients in order to be able to achieve 80% conditional statistical power. The Group expects to read out top-line data from this trial in the second half of 2020, followed by applications for approval from the FDA and the EMA, if top-line data is positive. Coupled with this, management has completed its annual forecast and revenues and costs have been amended to reflect current expectations. These factors have resulted in a change to the probability weighted revenue forecasts and the probability of the adjusted present values which are used in the calculation of the contingent consideration balance and impact the amount being unwound to the consolidated statement of comprehensive income.

7. Operating loss for the year

Operating loss for the year is stated after charging (crediting):

	December 31,
	2019	2018
	US$'000
Fees payable to the Group’s auditor and their associates	$611	$106
Changes in inventory expensed (excluding fair value step-up)	11,335	1,700
Amortization of inventory fair value step-up	10,367	—
Research and development expenses	15,827	10,703
Share based payments	841	821
Pension costs	769	583
Depreciation of property, plant and equipment	698	317
Amortization of intangible assets	11,957	50
Operating lease rentals	170	300
Foreign exchange (gains) losses	$(3,750)	$223

8. Employees

Including the directors, the Group’s average number of employees during the year was 99 (2018: 61).

Aggregate remuneration comprised:

	December 31,
	2019	2018
	US$'000
Wages and salaries	$17,268	$7,249
Social security costs	2,037	1,005
Pension costs - employees	769	583
Directors' remuneration	2,555	1,565
Shared based payments - directors	510	175
Shared based payments - employees/consultants	331	646
Total employee costs	$23,470	$11,223

The directors of the Company held the following share options over shares of Amryt Pharma plc at December 31, 2019:

	December 31, 2019
Director	Number	Exercise price (Sterling pence)	Expiration Date
Joseph Wiley	6,437,460	0.76p - 121.50p	November 27, 2024 - November 4, 2026

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Rory Nealon was a director of the Company throughout 2018 and resigned as a director of the Company on September 24, 2019.

The options held by the directors of the Company at December 31, 2018 have been restated to reflect the 6:1 share consolidation in 2019.

	December 31, 2018
Director	Number	Exercise price (Sterling pence)	Expiration Date
Joseph Wiley	343,521	120.72p	November 27, 2024
Rory Nealon	137,409	120.72p	November 27, 2024

No share options were granted to any of the directors in 2018.

Further information on the compensation of key management personnel is included in Note 23, Related party transactions, of these financial statements.

9. Net finance expense — other

	December 31,
	2019	2018
	US$'000
Interest on loans	$8,481	$1,603
Charges and fees paid	120	20
Interest received	(92)	(5)
Foreign exchange losses (gains)	(3,750)	223
Total	$4,759	$1,841

10. Tax on ordinary activities

A corporation tax credit of US$1,226,000 arises in the year ended December 31, 2019 (2018: charge of US$43,000). A reconciliation of the expected tax benefit computed by applying the tax rate applicable in the primary jurisdiction, the Republic of Ireland, to the loss before tax to the actual tax credit is as follows:

	December 31,
	2019	2018
	US$’000
Loss before tax	$(66,760)	$(30,444)
Tax credit at Irish corporation tax rate of 12.5%	(8,345)	(3,806)
Effect of:
Movement in unrecognized deferred tax assets	3,508	4,182
Permanent differences	6,474	43
Differences in overseas taxation rates	(2,863)	(376)
Total tax (credit)/charge on loss on ordinary activities	$(1,226)	$43

At December 31, 2019 and 2018, the Group had unutilized net operating losses in the following jurisdictions as follows:

	December 31,
	2019	2018
	US$’000
Ireland	$53,266	$36,428
United States	36,334	—
Germany	26,228	27,236
United Kingdom	16,828	7,812
ROW	—	315
Total	$132,656	$71,791

F-28

The deferred tax asset on tax losses of US$25,858,892 (2018: US$14,503,000), which was calculated at corporation tax rates ranging from 12.5% to 32%, has not been recognized due to the uncertainty of the recovery. Tax losses in Ireland, Germany and the UK can be carried forward indefinitely. U.S. losses related to tax periods prior to 2018 can be carried forward for 20 years while losses from 2018 onwards can be carried forward indefinitely.

Due to historical changes in ownership of the U.S. business, the U.S. tax losses carried forward are restricted in how they can be used against future profits of the Group.

All current and deferred tax related charges are recognized in the Consolidated Statement of Comprehensive Loss.

11. Loss per share - basic and diluted

The weighted average number of shares in the loss per share (“LPS”) calculation, reflects the weighted average total actual shares of Amryt Pharma plc in issue at December 31, 2019, as adjusted (see below).

Issued share capital - ordinary shares of £0.06 each

	Number of shares	Weighted average shares
December 31, 2019	154,498,887	75,871,562
December 31, 2018	274,817,283	274,817,283
December 31, 2018, as adjusted	45,802,880	45,802,880

The number of shares in issue at December 31, 2018 has been adjusted to reflect the share consolidation on July 10, 2019, whereby each ordinary shareholder received one ordinary share for every six shares held at that date.

The calculation of loss per share is based on the following:

	December 31,
	2019	2018
Loss after tax attributable to equity holders of the Company (US$'000)	$(65,535)	$(30,487)
Weighted average number of ordinary shares in issue	75,871,562	45,802,880
Fully diluted average number of ordinary shares in issue	75,871,562	45,802,880
Basic and diluted loss per share (US$)	$(0.86)	$(0.67)

The basic and diluted loss per share for 2019 of US$0.86 (2018: US$0.67) was calculated using the post consolidation number of ordinary shares in issue.

Where a loss has occurred, basic and diluted LPS are the same because the outstanding share options and warrants are anti-dilutive. Accordingly, diluted LPS equals the basic LPS. The share options and warrants outstanding as at December 31, 2019 totaled 32,023,535 (2018: 7,069,180 as restated) and are potentially dilutive.

F-29

12. Intangible assets and goodwill

The following table summarizes the Group’s intangible assets and goodwill:

	Developed technology - metreleptin	Developed technology - lomitapide	In process R&D	Other intangible assets	Total intangible assets	Goodwill
	US$'000
Cost
At January 1, 2018	$—	$—	$62,498	$114	$62,612	$—
Additions	—	—	—	155	155	—
Disposals	—	—	—	(1)	(1)	—
Foreign exchange movement	—	—	(2,407)	(10)	(2,417)	—
At December 31, 2018	—	—	60,091	258	60,349	—
Additions	—	—	—	74	74	—
Acquired assets	185,000	123,000	—	374	308,374	30,813
Impairment charge	—	—	(4,670)	—	(4,670)	—
Foreign exchange movement	—	—	(1,160)	(5)	(1,165)	—
At December 31, 2019	$185,000	$123,000	$54,261	$701	$362,962	$30,813
Accumulated amortization
At January 1, 2018	—	—	—	5	5	—
Amortization charge	—	—	—	50	50	—
Amortization charge on disposals	—	—	—	(1)	(1)	—
Foreign exchange movement	—	—	—	(2)	(2)	—
At December 31, 2018	—	—	—	52	52	—
Amortization charge	7,688	4,143	—	126	11,957	—
Foreign exchange movement	—	—	—	—	—	—
At December 31, 2019	$7,688	$4,143	$—	$178	$12,009	$—
Net book value
At December 31, 2018	$—	$—	$60,091	$206	$60,297	$—
At December 31, 2019	$177,312	$118,857	$54,261	$523	$350,953	$30,813

Developed technology on commercially marketed products

In connection with the acquisition of Aegerion in September 2019, the Group acquired developed technology, metreleptin and lomitapide. Refer to Note 2, Accounting policies - critical accounting judgements and key sources of estimation uncertainty, for further discussion on the valuation related to the developed technology, including the key assumptions utilized. These intangible assets are amortized over their estimated useful lives and the remaining useful lives for metreleptin and lomitapide are approximately 6.2 and 7.7 years, respectively, as of December 31, 2019. At the reporting date, the Group reviews its intangible assets for impairment when events or changes in circumstances indicate that their carrying value may not be recoverable. At December 31, 2019, there were no events or changes in circumstances that indicated the carrying value of metreleptin and lomitapide may not be recoverable, as such there was no impairment charge recorded during the year ended December 31, 2019.

The amortization associated with metreleptin and lomitapide is recorded as part of cost of sales. As of December 31, 2019, the estimated amortization expense related to these intangibles for future periods is as follows:

	Metreleptin	Lomitapide
Years Ending	US$'000
2020	$28,831	$15,537
2021	28,831	15,537
2022	28,831	15,537
2023	28,831	15,537
2024	28,831	15,537
Thereafter	33,157	41,172
Total intangible assets subject to amortization	$177,312	$118,857

F-30

In-process R&D

As a result of the acquisition of Amryt GmbH, in 2016, the Group recognized in-process R&D costs of US$54,268,000 which is related to the Group’s lead development asset, AP101. The Group reviews the carrying amount of AP101 on an annual basis to determine whether there are any indications that the asset has suffered an impairment loss. If any such indications exist, the recoverable amount of the asset is estimated in order to determine the extent of the impairment loss. Impairment indications include events causing significant changes in any of the underlying assumptions used in the income approach utilized in valuing in process R&D. These key assumptions are: the probability of success; the discount factor; the timing of future revenue flows; market penetration and peak sales assumptions; and expenditures required to complete development.

These cash flows are projected forward for a further 10 years to 2032 using projected revenue and cost growth to determine the basis for an annuity-based terminal values. The terminal values are used in the value in use calculation. The value in use represents the present value of the future cash flows, including the terminal value, discounted at a rate that is considered appropriate for the Group’s size and structure.

The key assumptions employed in arriving at the estimates of future cash flows are subjective and include projected EBITDA, an orphan drug market-based probability chance of success, net cash flows, discount rates and the duration of the discounted cash flow model. The assumptions and estimates used were derived from a combination of internal and external factors based on historical experience. The pre-tax discount rate used in 2019 and 2018 was 24.4% and 28.5%, respectively. The market-based probability chance of success is based on market benchmarks for orphan drugs, which is approximately 72% (same as 2018).

The value-in-use calculation is subject to significant estimation, uncertainty and accounting judgements and key sensitivities arise in the following areas:

• In the event that there was a variation of 10% in the assumed level of future growth in revenues, which would, in management’s view, represent a reasonably likely range of outcomes, this variation would not result in an impairment loss at December 31, 2019.

• In the event there was a 10% increase in the discount rate used in the value in use model which would in management’s view represent a reasonably likely range of outcomes, this variation would not result in an impairment loss at December 31, 2019.

The Group made changes in the assumptions used in the assessment of these carrying value of the AP101 asset in 2019. In January 2019, the Group received the results of an unblinded interim efficacy analysis from the Phase 3 trial of AP101 in EB. This analysis was conducted by an independent data safety monitoring committee and recommended that the trial should continue with an increase of 48 patients in the study to a total of 230 evaluable patients in order to be able to achieve 80% statistical power. The Group expects to read out top-line data in the second half of 2020, followed by applications for approval from the FDA and the EMA, if top-line data is positive. Coupled with this, management has completed its annual forecast and revenues and costs have been amended to reflect current expectations. The Group also adjusted the discount rate used in the discounted cash flow model, reducing the rate from 28.5% in 2018 to 24.4%. The acquisition of Aegerion in 2019 has significantly increased the size of the Group and also changed the debt and equity structure of the Group. As a result, management believed it was appropriate to update the discount rate to reflect the new structure of the Group. These factors have resulted in a change to the probability weighted revenue forecasts and the probability of the adjusted present values used in 2019.

Additionally, as a result of the acquisition of Som Therapeutics Corp., in 2016, the Group recognized in-process R&D costs of US$4,522,000 as an intangible. This is related to the Group’s development project AP102, which is an early stage drug asset. AP102 may represent a novel, next generation somatostatin analogue (“SSA”) peptide medicine for patients with rare neuroendocrine diseases, where there is a high unmet medical need, including acromegaly. Acromegaly is a rare endocrine disorder in which the body produces excessive growth hormone, leading to abnormal growth throughout the body over time. The Group also reviews the carrying amounts of AP102 on an annual basis to determine whether there are any indications that those assets have suffered an impairment loss.

In 2019, following the acquisition of Aegerion by the Group, a decision was made not to pursue the development of AP102 and therefore, the Group has written off this asset, resulting in an impairment charge of US$4,670,000 recognized as other expense during the year ended December 31, 2019. The decision to impair this intangible

F-31

asset is primarily based on the grounds that the acquisition of Aegerion has been transformational for the Group, as it has now become a global, commercial-stage biopharmaceutical company dedicated to commercializing and developing novel therapeutics to treat patients suffering from serious and life-threatening rare diseases. The Group’s diversified portfolio is comprised of two commercial rare disease products, as well as a development-stage pipeline focused on rare skin diseases. Since the commercial products, lomitapide for the treatment of homozygous familial hypercholesterolemia (“HoFH”), and metreleptin for the treatment of generalized lipodystrophy (“GL”) and partial lipodystrophy (“PL”), have each been sold globally through the Group’s commercial infrastructure for over six years, management believes it is in the best interest of the Group to concentrate resources on these new development pipeline activities which will better complement the existing commercial products. The Group may look to partner AP102 in the long-term future but in the short and medium term, the Group will continue to concentrate on AP101, AP103 and expansion opportunities for the existing commercial products.

Other intangible assets

Other intangible assets include website costs and the Group’s computer software and hardware. The amortization associated with computer software, hardware and website costs is recorded in both SG&A and R&D expenses. These assets are stated at cost and amortized using straight-line method based on the estimated economic lives, ranging from 3 - 10 years.

Goodwill

During 2019, the Group completed the acquisition of Aegerion, which resulted in aggregate goodwill of US$30,813,000. Refer to Note 6, Business combinations and asset acquisitions, for further details. The Group believes that the business, as a whole, represents a single CGU, as it is the smallest identifiable group of assets that generates cash inflows that are largely independent of the cash inflows from other assets or groups of assets. Additionally, the Group only operates in one business segment and does not operate any separate lines of business or separate business entities with respect to its products. Accordingly, the Group does not accumulate discrete financial information with respect to separate service lines and does not have separate reportable segments.

Goodwill is subject to impairment testing on an annual basis. The recoverable amount of the Group’s CGU is determined based on a value-in-use computation. The Group’s value-in-use calculations included the cash flow projections based on the 2020 budget which has been approved by the Board of Directors and the Group’s strategic plan for a further three years using projected revenue and cost growth rates of between 0% and 9%. At the end of the four-year forecast period, the terminal value, based on a long-term growth rate of 2%, was used in the value-in-use calculations. The value-in-use represents the present value of the future cash flows, including the terminal value, discounted at a rate appropriate to the Group. The key assumptions employed in arriving at the estimates of future cash flows are subjective and include projected EBITDA, net cash flows, discount rates and the duration of the discounted cash flow model. The Group have used a discount rate of 16.5% which is a conservative estimate for the Group as well as the Group’s risk profile.

The 2019 annual goodwill impairment testing process resulted in no impairment for the year ended December 31, 2019.

13. Property, plant and equipment

	Property	Plant and Machinery	Office Equipment	Right-of-use Asset	Total
	US$'000
Cost
At January 1, 2018	$401	$1,077	$389	$—	$1,867
Additions	—	11	69	—	80
Disposals	—	(7)	(21)	—	(28)
Foreign exchange movement	(15)	(42)	(16)	—	(73)
At December 31, 2018	$386	$1,039	$421	$—	$1,846

F-32

	Property	Plant and Machinery	Office Equipment	Right-of-use Asset	Total
	US$'000
Additions	6	253	167	152	578
Impact of IFRS 16 adoption	—	—	—	874	874
Acquired assets	—	276	—	924	1,200
Disposals	—	(114)	(32)	—	(146)
Foreign exchange movement	(9)	(22)	(9)	50	10
At December 31, 2019	$383	$1,432	$547	$2,000	$4,362

	Property	Plant and Machinery	Office Equipment	Right-of-use Asset	Total
	US$'000
Accumulated Depreciation
At January 1, 2018	$176	$201	$109	$—	$486
Depreciation charge	103	137	77	—	317
Depreciation charged on disposals	—	(7)	(21)	—	(28)
Foreign exchange movement	(10)	(12)	(5)	—	(27)
At December 31, 2018	269	319	160	—	748
Depreciation charge	90	162	64	382	698
Depreciation charged on disposals	—	(71)	(32)	—	(103)
Foreign exchange movement	(6)	(6)	(5)	—	(17)
At December 31, 2019	$353	$404	$187	$382	$1,326
Net book value
At December 31, 2018	117	720	261	—	1,098
At December 31, 2019	$30	$1,028	$360	$1,618	$3,036

14. Trade and other receivables

	December 31,
	2019	2018
	US$'000
Trade receivables	$28,607	$3,572
Accrued income and other debtors	5,934	2,326
VAT recoverable	1,846	29
Trade and other receivables	$36,387	$5,927

Trade receivables at December 31, 2019 includes US$752,000 (2018: US$338,000) which is due greater than 120 days. No impairment is considered necessary.

The December 31, 2019 accrued income and other debtors balance includes US$857,000 (2018: US$1,546,000) in relation to prepaid Phase 3 clinical trial costs.

15. Inventory

	December 31,
	2019	2018
	US$'000
Raw materials	$17,689	$303
Work in progress	2,488	782
Finished goods	23,446	1,052
Inventories	$43,623	$2,137

In 2019, a total of US$11,335,000 (2018: US$1,700,000) of inventories was included in the profit or loss as an expense (excluding the fair value step-up).

F-33

The fair value of net inventory acquired as part of the acquisition of Aegerion on September 24, 2019 amounted to US$45,959,000, net of US$61,842,000 of non-saleable inventory acquired in connection with the acquisition of Aegerion. The non-saleable inventories were determined based on the expiration dates and future manufacturing commitments which could result in inventory levels in excess of forecast demand. Under IFRS 3, the finished goods inventory on hand at the date of acquisition was valued at the expected selling price less the sum of (a) remaining costs of disposal and (b) a reasonable profit margin for the selling effort of the acquiring entity based on the EBITDA margin as a percentage of sales. The costs to dispose were calculated based on the average costs as a percentage of revenue through the period in which the current finished goods inventory is expected to be sold. This resulted in a non-cash step up at the valuation of finished goods inventory at September 24, 2019 of US$28,068,000. The non-cash step up in inventory is being unwound to the Consolidated Statement of Comprehensive Loss over the period in which this saleable inventory is expected to be sold which is less than one year. At December 31, 2019, US$17,701,000 of this non-cash inventory step up is included in finished good inventory.

All inventory was reviewed at year end and no impairment was deemed necessary.

16. Cash and cash equivalents

	December 31,
	2019	2018
	US$'000
Cash at bank available on demand	$65,197	$9,864
Restricted cash	2,032	1,362
Total cash and cash equivalents	$67,229	$11,226

Cash and cash equivalents include cash at bank available on demand and restricted cash.

Of the US$2,032,000 held in restricted cash, US$1,219,000 was held in an escrow account set-up in accordance with Aegerion’s bankruptcy plan as approved by the U.S. Bankruptcy Court to meet the costs associated with the bankruptcy process. Additionally, US$813,000 is cash held by a third-party distributor at year end; the funds from the third-party distributor were transferred to Amryt in January 2020.

17. Share capital and reserves

Details of issued ordinary shares with a nominal value of Sterling 6 pence (2018: 1 pence) each are in the table below. The ordinary shares and share price in 2018 were adjusted for the share consolidation completed in 2019.

Date	Number of ordinary shares	Number of deferred shares	Total Share Capital US$'000	Total Share Premium US$'000
At December 31, 2019	159,363,543	—	$11,918	$2,422
At December 31, 2018	274,817,283	43,171,134	$25,198	$68,233

The number of ordinary shares issued at December 31, 2019 includes treasury shares of 4,864,656.

The Company repurchased all of the 43,171,134 deferred ordinary shares in July 2019 for an aggregate consideration of £0.01 and the Deferred Shares were immediately cancelled. Simultaneously the Company allotted four additional ordinary shares of par value £0.01 each in the capital of the Company, in connection with a 6 to 1 consolidation of the Company’s share capital.

In an US$8,000,000 equity raise, the company issued 7,346,189 ordinary shares, 4,580,288 shares in August 2019 and 2,765,901 shares in September 2019.

On September 24, 2019, the following equity issuances were conducted:

• 77,027,423 ordinary shares and 8,065,000 warrants for a consideration of US$152,615,000 were issued as part of the Aegerion acquisition whereby the company acquired the entire share capital of Aegerion.

• 27,541,944 ordinary shares and 5,911,722 warrants were issued as part of a US$60,000,000 fund raising.

On December 19, 2019, the Company issued 1,645,105 shares to certain shareholders in consideration of warrants.

F-34

Share Capital

Share capital represents the cumulative par value arising upon issue of ordinary shares of Sterling 6 pence each.

The ordinary shares have the right to receive notice of, attend and vote at general meetings and participate in the profits of the Company.

Share Premium

Share premium represents the consideration that has been received in excess of the nominal value on issue of share capital net of issue costs and transfers to distributable reserves. By special resolution of the Company duly passed on September 23, 2019, in accordance with section 283 of the UK Companies Act 2006, it was resolved that the entire amount outstanding to the credit of the share premium account and capital redemption reserve of the Company be cancelled. The reduction in capital, amounting to US$268,505,000, representing the entire amount of share premium at that time, was approved by the High Court of Justice of England and Wales on November 5, 2019.

Warrant reserve

The warrant reserve represents zero cost warrants issued as part of the equity raise on September 24, 2019 net of issue costs apportioned to warrants issued and additional warrants issued to certain shareholders on November 14, 2019. Each warrant entitles the holder to subscribe for one ordinary share at zero cost. On December 19, 2019, the company issued 1,645,105 ordinary shares in consideration for certain warrants.

Treasury Shares

On November 14, 2019, the Company repurchased a combined 4,864,656 ordinary shares from certain shareholders. In exchange for the ordinary shares, these shareholders were issued an equivalent number of zero cost warrants. These ordinary shares are now held as treasury shares.

Share based payment reserve

Share based payment reserve relates to the charge for share based payments in accordance with IFRS 2.

Merger reserve

The merger reserve was created on the acquisition of Amryt DAC by Amryt Pharma plc in April 2016. Ordinary shares in Amryt Pharma plc were issued to acquire the entire issued share capital of Amryt DAC. Under section 612 of the UK Companies Act 2006, the premium on these shares has been included in a merger reserve.

Reverse acquisition reserve

The reverse acquisition reserve arose during the period ended December 31, 2016 in respect of the reverse acquisition of Amryt Pharma plc by Amryt DAC. Since the shareholders of Amryt DAC became the majority shareholders of the enlarged Group, the acquisition is accounted for as though there is a continuation of Amryt DAC’s financial statements. The reverse acquisition reserve is created to maintain the equity structure of Amryt Pharma plc in compliance with UK company law.

Equity component of convertible notes

The equity component of convertible notes represents the equity component of the US$125,000,000 convertible debt and is measured by determining the residual of the fair value of the instrument less the estimated fair value of the liability component. The equity component is recognized in equity and is not subsequently remeasured.

Other distributable reserves

Other distributable reserves comprise the following:

• Distribution of the share premium amount on November 6, 2019 of US$268,505,000.

• A deemed distribution of US$47,902,000 arising from the issuance of CVRs.

F-35

• A deemed distribution of US$2,969,000 arising from the scheme of arrangement in September 2019 whereby Amryt Pharma plc, which was incorporated in July 2019, became a 100% shareholder of Amryt Pharma Holdings Limited (formerly named Amryt Pharma plc) (the “Acquisition of subsidiary without a change of control”).

Currency translation reserve

The currency translation reserve arises on the retranslation of non-U.S, dollar denominated foreign subsidiaries.

Accumulated deficit

Accumulated deficit represents losses accumulated in previous periods and the current year.

18. Deferred tax liability

	Total
	US$'000
At January 1, 2018	$6,161
Movement during the year	—
At December 31, 2018	6,161
Net movement during the year	12,760
At December 31, 2019	$18,921

A deferred tax liability arose in 2016 on the acquisition of Amryt GmbH. An intangible asset was recognized in relation to in process R&D. As the intangible asset only arises on consolidation and there may not be tax deductions available on sale, its tax base is nil.

When the intangible asset is amortized the tax difference will be reduced and the movement in the deferred tax liability will be recognized in profit or loss. The in-process R&D is currently not being amortized and as a result the deferred tax liability in relation to the Birken acquisition continues to be in place.

A deferred tax liability, in the amount of US$14,425,000, also arose in 2019 in connection with the acquisition of Aegerion Pharmaceuticals, Inc. (see Note 6, Business combinations and asset acquisitions). The intangible assets have been recognized at their fair value. As the transaction was completed as a share acquisition, the intangible assets were not re-based to fair value from a tax perspective with a deferred tax liability being recognized on acquisition. These intangibles are being amortized and the resulting reduction in the deferred tax liability will be recognized in profit or loss.

19. Long term loan

	December 31,
	2019	2018
	US$'000
Long term loan	$81,610	$17,164
Long term loan interest	—	1,847
Long term loan and interest	$81,610	$19,011

In December 2016, Amryt DAC entered into a euro denominated €20,000,000 facility agreement (“facility”) with the European Investment Bank (“EIB”) on attractive terms for the Group. The facility was significant because it provided non-dilutive funding that secured the Group’s near and mid-term funding needs for its lead development candidate, AP101.

The facility was split into three tranches, with €10,000,000 available immediately and two further tranches of €5,000,000 available upon the achievement of certain milestones. In April 2017, the Group drew down the first tranche of €10,000,000. In October 2017, the terms of the second tranche of €5,000,000 were amended by the EIB resulting in the Group being given option to draw this amount down on demand. The Group drew down this second tranche of €5,000,000 in September 2018. In December 2018, the terms of the third tranche were amended by the EIB to give the Group the option to draw down this final tranche on demand on the condition that the EASE Phase 3 trial interim efficacy results were positive. In January 2019, the Group received the

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results of this unblinded interim efficacy analysis. The Independent Monitoring Committee recommended that the trial should continue with an increase in patients. Following this positive result, the original conditions of the final tranche were waived and the final tranche of €5,000,000 was drawn down in February 2019. The facility was secured over the Intellectual Property assets of the Group and there was also a negative pledge whereby Amryt cannot permit any security to be granted over any of its assets over the course of the loan period.

The facility had a five-year term from the date of drawdown for each tranche. The facility had an interest rate of 3% to be paid on an annual basis, the first instalment of short-term interest on the €10,000,000 tranche 1 was paid in April 2018. A further annual fixed rate of 10% was payable together with the outstanding principal amount on expiry of the facility. At December 31, 2018, the Group had short term interest payable accrued amounting US$319,000 which was repayable in April 2019 and long-term interest payable of US$1,847,000 which represents the present value of the long-term interest accrued but not payable until each tranche matured.

On September 24, 2019, the EIB loan was repaid in full.

As part of the acquisition of Aegerion on September 24, 2019, Aegerion entered into a new U.S. dollar denominated US$81,021,000 secured term loan debt facility (“Term Loan”) with various lenders. The Term Loan is made up of a US$54,469,000 loan that was in place prior to the acquisition which was refinanced as part of the acquisition and a US$26,552,000 additional loan that was drawn down on September 24, 2019 and was used to repay the EIB secured loan facility. The Term Loan has a five-year term from the date of the draw down, September 24, 2019 and matures on September 24, 2024. Under the Term Loan, interest will be payable at the option of the Group at the rate of 11% per annum paid in cash on a quarterly basis or at a rate of 6.5% paid in cash plus 6.5% paid in kind that will be paid when the principal is repaid, which rolls up and is included in the principal balance outstanding, on a quarterly basis. The Term Loan may be prepaid, in whole or in part, by Aegerion at any time subject to payment of an exit fee, which depending on the stage of the loan term, ranges from 5.00% to 0.00% of the principal then outstanding on the Term Loan.

In connection with the Term Loan, the Group incurred approximately US$870,000 of debt issuance costs, which primarily consisted of underwriting, legal and other professional fees. These costs are being amortized over the expected life of the loan using the effective interest method.

The Term Loan is guaranteed by Amryt and certain subsidiaries of the Group. In connection with the loan agreement, fixed and floating charges have been placed on property and undertakings of Amryt and certain subsidiaries of the Group.

The Term Loan agreement includes affirmative and negative covenants, including prohibitions on the incurrence of additional indebtedness, granting of liens, certain asset dispositions, investments and restricted payments, in each case, subject to certain exceptions set forth in the Loan Agreement. The Term Loan agreement also includes customary events of default for a transaction of this type, and includes (i) a cross-default to the occurrence of any event of default under material indebtedness of Aegerion and certain subsidiaries of the Group and Amryt, including the convertible notes, and (ii) Amryt or any of its subsidiaries being subject to bankruptcy or other insolvency proceedings. Upon the occurrence of an event of default, the lenders may declare all of the outstanding Term Loan and other obligations under the Term Loan agreement to be immediately due and payable and exercise all rights and remedies available to the lenders under the Term Loan agreement and related documentation. There have been no events of default or breaches of the covenants occurring for the year ended December 31, 2019.

	Total
Changes in long term loans from financing activities:	US$'000
At January 1, 2019	$19,011
Cash-flows
Proceeds from loans and borrowings	31,176
Repayment of loans and borrowings	(21,990)
Liability related
Effect of changes in foreign exchange rates	797
Acquired loans and borrowings	54,469
Interest accrual	(1,853)
At December 31, 2019	$81,610

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20. Convertible notes

	December 31, 2019
	US$'000
Issuance of convertible notes	$125,000
Amount classified as equity	(29,210)
Accreted interest	1,066
Total convertible notes	$96,856

As part of the acquisition, Aegerion issued convertible notes with an aggregate principal amount of US$125,000,000 to Aegerion creditors. Refer to Note 23, Related party transactions, for further details.

The convertible notes are senior unsecured obligations and bear interest at a rate of 5.0% per year, payable semi-annually in arrears on April 1 and October 1 of each year, beginning on April 1, 2020. The convertible notes will mature on April 1, 2025, unless earlier repurchased or converted.

The convertible notes are convertible into Amryt’s ordinary shares at a conversion rate of 386.75 ordinary shares per US$1,000 principal amount of the convertible notes. If the holders elect to convert the convertible notes, Aegerion can settle the conversion of the convertible notes through payment or delivery of cash, common shares, or a combination of cash and common shares, at its discretion. As a result of the conversion feature in the convertible notes, the convertible notes were assessed to have both a debt and an equity component. The two components were assessed separately and classified as a financial liability and equity instrument. The financial liability component was measured at fair value based on the discounted cash flows expected over the expected term of the notes using a discount rate based on a market interest rate that a similar debt instrument without a conversion feature would be subject to. Refer to Note 17, Share capital and reserves, for further details on the equity component of the convertible notes.

From September 24, 2019 until the close of business on the second scheduled trading day immediately preceding the maturity date, holders may convert all or any portion of their convertible notes, in multiples of US$1,000 principal amount, at the option of the holder.

The indenture does not contain any financial covenants or restrict the Group’s ability to repurchase securities, pay dividends or make restricted payments in the event of a transaction that substantially increases the Group’s level of indebtedness in certain circumstances.

The indenture contains customary terms and covenants and events of default. If an event of default (other than certain events of bankruptcy, insolvency or reorganization involving Aegerion, Amryt and certain subsidiaries of the Group) occurs and is continuing, the trustee by notice to Aegerion, or the holders of at least 25% in principal amount of the outstanding convertible notes by written notice to Aegerion and the trustee, may declare 100% of the principal of and accrued and unpaid interest, if any, on all of the convertible notes to be due and payable. Upon such a declaration of acceleration, such principal and accrued and unpaid interest, if any, will be due and payable immediately. Upon the occurrence of certain events of bankruptcy, insolvency or reorganization involving Aegerion, 100% of the principal and accrued and unpaid interest, if any, on the convertible notes will become due and payable automatically. Notwithstanding the foregoing, the indenture provides that, upon Aegerion’s election, and for up to 180 days, the sole remedy for an event of default relating to certain failures by Aegerion to comply with certain reporting covenants in the indenture consists exclusively of the right to receive additional interest on the convertible notes. There have been no events of default or breaches of the covenants occurring for the year ended December 31, 2019.

21. Trade and other payables

	December 31,
	2019	2018
	US$'000
Trade payables	$23,418	$5,339
Accrued expenses	52,382	6,204
Social security costs and other taxes	796	500
Trade and other payables	$76,596	$12,043

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The accruals mainly consist of costs related to government revenue rebates, convertible note interest, royalty expenses, restructuring costs, clinical and R&D activities.

22. Provisions and other liabilities

	December 31,
	2019	2018
	US$'000
Non-current liabilities
Provisions and other liabilities	$3,910	$—
Leases due greater than 1 year	1,053	—
	4,963	—
Current liabilities
Provisions and other liabilities	23,047	—
Leases due less than 1 year	571	—
	23,618	—
Total provisions and other liabilities	$28,581	$—

Refer to Note 25, Commitments and contingencies for further details on provisions.

23. Related party transactions

Compensation of key management personnel of the Group

At December 31, 2019 the key management personnel of the Group were made up of two key personnel, the executive director, Joe Wiley and the Chief Financial Officer and Chief Operating Officer, Rory Nealon. Rory Nealon was an executive director of the Company in 2018 and resigned from this position on September 24, 2019.

Compensation for the year ended December 31, 2019 of these personnel is detailed below:

	December 31,
	2019	2018
	US$'000
Short-term employee benefits	$1,049	$803
Performance related bonus	1,286	420
Post-employment benefits	86	76
Share-based compensation benefits	510	175
Total compensation	$2,931	$1,474

Shares purchased by directors

The directors of the Company did not purchase any shares in the Company in 2018.

The Chairman, Ray Stafford, purchased 918,273 Amryt ordinary shares as part of the interim fundraise in August 2019. The executive director, Joe Wiley purchased 7,999 shares on the open market in January 2020.

Agreements with principal shareholders

Long term loan

On September 24, 2019, the Group entered into a long term loan. Proceeds from the long term loan were used to refinance Aegerion’s existing secured bridge loan in the principal amount of approximately US$50,000,000 (in principal) held by certain funds managed by Athyrium Capital Management, LP and Highbridge Capital Management, LLC, respectively, and Amryt’s existing €20,000,000 (in principal) secured loan facility with EIB. Further information on the terms of the long term loan is included in Note 19, Long term loan, of these financial statements.

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Convertible notes

On September 24, 2019, the Company issued US$125,000,000 aggregate principal amount of convertible notes due 2025 to certain creditors of Aegerion. The convertible notes bear interest at a rate of 5% per annum, payable in cash semi-annually. The convertible notes will mature approximately five and a half years after issuance, unless earlier repurchased, redeemed or converted. Further information on the terms of the convertible notes is included in Note 20, Convertible notes, of these financial statements.

Zero Cost Warrants

The Company agreed, for certain Aegerion creditors who wished to restrict their percentage share interest in Amryt’s issued share capital, to issue to the relevant Aegerion creditor, as an alternative to Amryt ordinary shares, an equivalent number of new zero cost warrants to subscribe for Amryt ordinary shares to be constituted on the terms of the zero cost warrant. The relevant Aegerion creditors are entitled at any time to exercise the zero cost warrants, at which point in time the Company would issue to that Aegerion creditor the relevant number of fully paid ordinary shares in return for the exercise of the zero cost warrants.

On September 24, 2019, certain of Aegerion’s creditors elected to receive 8,065,000 zero cost warrants to subscribe for Amryt ordinary shares as consideration for the acquisition. Separately 5,911,722 warrants were issued to investors in connection with the US$60,000,000 equity raise.

On November 14, 2019, the Company repurchased a combined 4,864,656 ordinary shares from Highbridge Tactical Master Fund L.P., Highbridge SCF Special Situations SPV, L.P. and Nineteen77 Global Multi Strategy Alpha Master Limited. In exchange for the ordinary shares, these institutions were issued an equivalent number of zero cost warrants. Each warrant entitles the holder to subscribe for one ordinary share at zero cost. These ordinary shares are now held as treasury shares. On December 19, 2019, Highbridge MSF International Ltd exercised 1,645,105 zero cost warrants in exchange for 1,645,105 ordinary shares.

24. Fair value measurement and financial risk management

Categories of financial instruments

	December 31,
	2019	2018
	US$'000
Financial assets (all at amortized cost):
Cash and cash equivalents	$67,229	$11,226
Trade receivables	28,607	3,572
Total financial assets	95,836	14,798
Financial liabilities:
At amortized cost
Trade payables and accrued expenses	75,800	11,543
Lease liabilities	1,624‬	—
Other liabilities	19,457	—
Convertible notes	96,856	—
Long term loan	81,610	19,011
Contingent value rights	49,413	—
At fair value
Contingent consideration	53,048	47,316
Total financial liabilities	377,808	77,870
Net	$(281,972)	$(63,072)

Financial instruments evaluated at fair value can be classified according to the following valuation hierarchy, which reflects the extent to which the fair value is observable:

• Level 1: fair value evaluations using prices listed on active markets (not adjusted) of identical assets or liabilities.

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• Level 2: fair value evaluations using input data for the asset or liability that are either directly observable (as prices) or indirectly observable (derived from prices), but which do not constitute listed prices pursuant to Level 1.

• Level 3: fair value evaluations using input data for the asset or liability that are not based on observable market data (unobservable input data).

The contingent consideration has been valued using Level 3. The contingent consideration comprises:

• Contingent consideration relating to the acquisition of Amryt GmbH (see Note 6, Business combinations and asset acquisitions) that was measured at US$53,048,000 as at December 31, 2019 (2018: US$47,316,000). The fair value comprises royalty payments which was determined using probability weighted revenue forecasts and the fair value of the milestones payments which was determined using probability adjusted present values. It also included a revision to the discount rate used, and revenue and costs forecasts have been amended to reflect management’s current expectations.

Impact of key unobservable input data

• An increase of 10% in estimated revenue forecasts would result in an increase to the fair value of US$3,710,000. A decrease would have the opposite effect.

• A 5% increase in the discount factor used would result in a decrease to the fair value of US$9,761,000. A decrease of 5% would result in an increase to the fair value of US$13,312,000.

• A six-month delay in the launch date for AP101 for EB would result in a decrease to the fair value of US$4,313,000.

Policies and Objectives

The Group’s operations expose it to some financial risks arising from its use of financial instruments, the most significant ones being liquidity, market risk and credit risk. The Board of Directors is responsible for the Group and Company’s risk management policies and whilst retaining responsibility for them it has delegated the authority for designing and operating processes that ensure the effective implementation of the objectives and policies to the Group’s finance function. The main policies for managing these risks are as follows:

Liquidity risk

The Group is not subject to any externally imposed capital requirement. Accordingly, the Group’s objectives are to safeguard the ability to continue as a going concern in order to provide returns for shareholders and benefits to other stakeholders and to maintain an optimal capital structure to reduce the cost of capital. Working capital forecasts are prepared to ensure the Group has sufficient funds to complete contracted work commitments.

The following table shows the maturity profile of trade payables of the Group:

	Less than 1 month	Between 1 and 3 months	Between 3 and 6 months	Total
December 31, 2019	US$'000
Trade payables	$17,995	$3,272	$2,151	$23,418

	Less than 1 month	Between 1 and 3 months	Between 3 and 6 months	Total
December 31, 2018	US$'000
Trade payables	$4,344	$—	$995	$5,339

The following table shows the maturity profile of lease liabilities and other liabilities of the Group:

	Less than 1 year	Between 1 and 3 years	Between 3 and 5 years	Greater than 5 years	Total
December 31, 2019	US$'000
Lease liabilities	$969	$916	$143	$20	$2,048
Other liabilities	15,722	3,928	—	—	19,650
	$16,691	$4,844	$143	$20	$21,698

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The following table shows the undiscounted maturity profile of long-term loans of the Group, including principal and interest:

	Less than 1 year	Between 1 and 3 years	Between 3 and 5 years	Greater than 5 years	Total
December 31, 2019	US$'000
Long term loan	$5,585	$12,296	$124,427	$—	$142,308
Convertible notes	6,372	12,500	12,500	128,125	159,497
	$11,957	$24,796	$136,927	$128,125	$301,805

	Less than 1 year	Between 1 and 3 years	Between 3 and 5 years	Greater than 5 years	Total
December 31, 2018	US$'000
Long term loan	$—	$—	$19,358	$—	$19,358

The following table shows the undiscounted maturity profile of the contingent consideration and contingent value rights of the Group:

	Less than 1 year	Between 1 and 3 years	Between 3 and 5 years	Greater than 5 years	Total
December 31, 2019	US$'000
Contingent consideration and contingent value rights	$—	$99,559	$27,998	$—	$127,557

	Less than 1 year	Between 1 and 3 years	Between 3 and 5 years	Greater than 5 years	Total
December 31, 2018	US$'000
Contingent consideration and contingent value rights	$—	$14,875	$28,607	$—	$43,482

Capital management

The Group considers its capital to be its ordinary share capital, share premium, other reserves and accumulated deficit. The Group manages its capital to ensure that entities within the Group will be able to continue individually as going concerns, while maximizing the return to shareholders through the optimization of debt and equity balances. The Group manages its capital structure and makes adjustments to it, in the light of changes in economic conditions. To maintain or adjust its capital structure, the Group may adjust or issue new shares or raise debt. On a regular basis, management receives financial and operational performance reports that enable continuous management of assets, liabilities and liquidity. No changes were made in the objectives, policies or processes during the years ended December 31, 2019 and December 31, 2018.

Market risk

Market risk arises from the use of interest-bearing financial instruments and represents the risk that future cash flows of a financial instrument will fluctuate as a result of changes in interest rates. It is the Group’s policy to ensure that significant contracts are entered into in its functional currency whenever possible and to maintain the majority of cash balances in the functional currency of the Company. The Group considers this policy minimizes any unnecessary foreign exchange exposure. In order to monitor the continuing effectiveness of this policy, the Board of Directors reviews the currency profile of cash balances and managements accounts.

It is the Group’s policy to enter into long term borrowings at fixed rates of interest where possible to reduce the Group’s exposure to cash flow interest rate risk. During the years ended December 31, 2019 and December 31, 2018, the long term borrowings of the Group were subject to fixed rates of interest.

During the year 2019, the Group earned interest on its interest-bearing financial assets at rates between 0% and 2%. The effect of a 1% change in interest rates obtainable during the year on cash and on short-term deposits would be to increase or decrease the Group loss before tax by US$71,000 (2018: US$64,000).

In addition to cash balances maintained in US$, the Group had balances in £ and € amongst others at year-end. A theoretical 10% adverse movement in the year end €:US$ exchange rate would lead to an increase in the Group loss before tax by US$573,000 with a corresponding reduction in the Group loss before tax with a 10%

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favorable movement. A theoretical 10% adverse movement in £:US$ exchange rates would lead to an increase in the Group loss before tax by US$438,000 with a corresponding reduction in the Group loss before tax with a 10% favorable movement.

Credit risk

The Group has no significant concentrations of credit risk. Exposure to credit risk is monitored on an ongoing basis. If necessary, the Group maintains specific provisions for potential credit losses. To date there has been no requirement for such provisions. The Group maintains cash and cash equivalents with various financial institutions. The Group performs regular and detailed evaluations of these financial institutions to assess their relative credit standing. The carrying amount reported in the balance sheet for cash and cash equivalents approximate their fair value. Credit risk is the risk that the counterparty will default on its contractual obligations resulting in financial loss. Credit risk arises from cash and cash equivalents and from exposure via deposits with the Group’s bankers. For cash and cash equivalents, the Group only uses recognized banks with high credit ratings.

Credit risk related to customers is managed through risk assessment procedures, through assessment of credit quality, taking into account the financial position of the customer, past experience and other factors. The compliance with credit terms is monitored on a regular basis by management. Credit terms may vary from one month to several months depending on the region and customer. The major customers contribute to 58% of the total trade receivables of the group outstanding as at December 31, 2019 (2018: 92%).

For trade receivables, the Group applies a simplified approach in calculating ECLs. Therefore, the Group does not track changes in credit risk, but instead recognizes a loss allowance based on lifetime ECLs at each reporting date. The Group assesses ECL based on its historical credit loss experience, adjusted for forward-looking factors specific to the debtors and the economic environment.

25. Commitments and contingencies

Contingent consideration and contingent value rights

See Note 6, Business combinations and asset acquisitions, in relation to contingent consideration and contingent value rights as a result of the acquisition of Amryt GmbH and Aegerion.

License Agreements

In connection with metreleptin, the Group has license agreements for the exclusive license and patents for the use of metreleptin to develop, manufacture and commercialize a preparation containing metreleptin. Under the license agreements the Group is required to make royalty payments on net sales on a country-by-country basis. During the year ended December 31, 2019, following the Aegerion acquisition on September 24, 2019, the Group made aggregate royalty payments of US$5,104,000 (2018: US$nil).

The Group holds a license agreement for the exclusive, worldwide license of certain know-how and a range of patent rights applicable to lomitapide. The Group is obligated to use commercially reasonable efforts to develop, commercialize, market and sell at least one product covered by the licensed patent right, such as lomitapide. Additionally, the Group is required to make royalty payments on net sales of products. During the year ended December 31, 2019, following the Aegerion acquisition on September 24, 2019, the Group recorded aggregate royalty expenses to third parties of US$803,000 (2018: US$nil).

Prior to the Aegerion acquisition, Amryt had the exclusive right to sell LOJUXTA across the licensed territories pursuant to a license agreement with Aegerion. During the year ended December 31, 2019, Amryt recorded aggregate royalty expenses to Aegerion of US$2,512,000 (2018: US$2,678,000).

The Group entered into a license agreement for the exclusive, worldwide license to the patent rights for a novel polymer-based topical gene therapy delivery platform for potential use in the treatment of rare genetic diseases. The first product candidate utilizing this platform, AP103, is currently in preclinical development for the treatment of recessive dystrophic EB, a subset of severe EB. Under the license agreement Amryt is required to pay milestone payments and, upon the sale of product, royalty payments on net sales of products.

The Group entered into a license agreement for the non-exclusive, worldwide license to the patent rights for the design and development of gene coded therapy vectors and methods for making such vectors, in order for Amryt

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to develop and commercialize its genetic encoded therapies relating to AP103. Under this agreement Amryt is required to make milestone payments and royalty payments on net sales of products.

Legal matters

Prior to the acquisition of Aegerion by Amryt, Aegerion entered into settlement agreements with governmental entities including the Department of Justice (“DOJ”) and the FDA in connection with JUXTAPID investigations. The settlement agreements require Aegerion to pay specified fines and engage in regulatory compliance efforts. Subsequent to the acquisition, Aegerion made US$3,387,000 of settlement payments, including interest, and the total amount of the settlements that remains due as a current liability and a non-current liability is $15,547,000 and $3,910,000, respectively, as of December 31, 2019.

Other legal matters

The Group recognizes a liability for legal contingencies when it believes that it is both probable that a liability has been incurred and that it can reasonably estimate the amount of the loss. The Group reviews these accruals and adjusts them to reflect ongoing negotiations, settlements, rulings, advice of legal counsel and other relevant information. To the extent new information is obtained and the Group’s views on the probable outcomes of claims, suits, assessments, investigations or legal proceedings change, changes in the Group’s liability accrual would be recorded in the period in which such determination is made. At December 31, 2019 the Group had recognized liabilities of US$7,500,000 in relation to ongoing legal matters.

Lease commitments

The Group had no finance lease commitments in 2019 (2018: nil). In February 2020, the Group entered an 8-year term lease for its U.S. operational office, located in Boston, Massachusetts. The lease will commence in April 2020, and the aggregate lease payment amounts over the lease term is approximately US$2,400,000.

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26. Investment in subsidiaries

List of subsidiary companies:

Subsidiary	Ownership	Activities	Company Number	Incorporation	2019 % Holding	2018 % Holding
Amryt Pharma Holdings Ltd.	Direct	Holding company and management services	5316808	UK	100	100
Amryt Pharmaceuticals DAC	Indirect	Holding company and management services	566448	Ireland	100	100
Amryt Research Limited	Indirect	Pharmaceuticals R&D	571411	Ireland	100	100
Amryt Endocrinology Limited	Indirect	Pharmaceuticals R&D	572984	Ireland	100	100
Amryt Lipidology Limited	Indirect	Licensee for Lojuxta	593833	Ireland	100	100
Amryt Genetics Limited	Indirect	Pharmaceutical R&D	622577	Ireland	100	100
Amryt Pharma (UK) Limited	Indirect	Management services	10463152	UK	100	100
Amryt Pharma France	Indirect	Dormant	824 418 156 00017	France	100	100
Amryt Pharma Italy SRL	Indirect	Management services	2109476	Italy	100	100
Amryt Pharma Spain SL	Indirect	Management services	B67130567	Spain	100	100
Amryt GmbH (previously Amryt AG)	Indirect	Product Sales and Pharmaceuticals R&D	HRB 711487	Germany	100	100
SomPharmaceuticals SA	Indirect	Pharmaceuticals R&D and management services	CHE-435.396.568	Switzerland	100	100
SomTherapeutics, Corp	Indirect	License holder	P14000071235	USA	100	100
Aegerion Pharmaceuticals, Inc.	Indirect	Holding company and management services	3922075	USA	100	Not applicable
Aegerion International Ltd.	Indirect	Management services	52048	Bermuda	100	Not applicable
Aegerion Securities Corporation	Indirect	Management services	464215084	USA	100	Not applicable
Aegerion Pharmaceuticals Holdings, Inc.	Indirect	Management services	5213687	USA	100	Not applicable
Aegerion Argentina S.R.L.	Indirect	Management services	901-709682-0	Argentina	100	Not applicable
Aegerion Pharmaceuticals (Canada) Ltd.	Indirect	Management services	85134 5132 RT0001	Canada	100	Not applicable
Aegerion Colombia S.A.S.	Indirect	Management services	R048196625	Colombia	100	Not applicable
Aegerion Pharmaceuticals K.K.	Indirect	Management services	0104-01-107816	Japan	100	Not applicable
Aegerion Brasil Comercio E Importacao De Medicamentos LTDA	Indirect	Management services	3522602510-1	Brazil	100	Not applicable
Aegerion Pharmaceuticals Ltd.	Indirect	Management services	46134	Bermuda	100	Not applicable
Aegerion Pharmaceuticals Limited	Indirect	Management services	8114919	UK	100	Not applicable
Amryt Pharmaceuticals, SAS	Indirect	Management services	534 195 59900012	France	100	Not applicable
Aegerion Pharmaceuticals S.r.l.	Indirect	Management services	1166250	Italy	100	Not applicable
Aegerion Pharmaceuticals GmbH	Indirect	Management services	HRB 95895	Germany	100	Not applicable
Aegerion İlaç Ticaret Limited Şirketi	Indirect	Management services	907292	Turkey	100	Not applicable
Aegerion Pharmaceuticals SARL	Indirect	Management services	CHE-497.494.599	Switzerland	100	Not applicable
Aegerion Pharmaceuticals B.V.	Indirect	Management services	69859647	Netherlands	100	Not applicable
Aegerion Pharmaceuticals Spain, S.L.	Indirect	Management services	B88019161	Spain	100	Not applicable

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List of registered offices:

Company	Registered Office Address
Amryt Pharma Holdings Ltd	Dept 920a 196 High Road, Wood Green, London, United Kingdom, N22 8HH
Amryt Pharmaceuticals DAC	90 Harcourt Street, Dublin 2
Amryt Research Limited	90 Harcourt Street, Dublin 2
Amryt Endocrinology Limited	90 Harcourt Street, Dublin 2
Amryt Lipidology Limited	90 Harcourt Street, Dublin 2
Amryt Genetics Limited	90 Harcourt Street, Dublin 2
Amryt Pharma (UK) Limited	3rd Floor 1 Ashley Road, Altrincham, Cheshire, United Kingdom, WA14 2DT
Amryt Pharma France	17 Avenue George V, 75008 Paris
Amryt Pharma Italy SRL	Milano (MI)-Via Dell'Annunciata 23/4
Amryt Spain SL	Barcelona, calle Diputacio, number 260
Amryt GmbH (previously Amryt AG)	Streiflingsweg 11, 75223 Niefern-Öschelbronn
SomPharmaceuticals SA	Bahnofstrasse 21, 6300 Zug
SomTherapeutics, Corp	3795 Coventry Lane, Boca Raton, FL 33496
Aegerion Pharmaceuticals Inc.	245 First Street, Riverview II, 18th Floor, Cambridge, MA 02142
Aegerion International Ltd.	Clarendon House, 2 Church Street, Hamilton, HM11
Aegerion Securities Corporation	245 First Street, Riverview II, 18th Floor, Cambridge, MA 02142
Aegerion Pharmaceuticals Holdings, Inc.	245 First Street, Riverview II, 18th Floor, Cambridge, MA 02142
Aegerion Argentina S.R.L.	Avda. Camacua 421, Suite 102, Olivos, Vicente Lopez, 1636
Aegerion Pharmaceuticals Canada (Ltd).	5300 Commerce Court West, 199 Bay Street, Toronto, ON M5L 1B9
Aegerion Colombia S.A.S.	CR 12 89 33 P 5, Bogota DC, Bogota 110111
Aegerion Pharmaceuticals K.K.	12F, Ark Mori Building, 1-12-32 Akasaka, Minato-ku, Tokyo
Aegerion Brazil Comercio E Importacao De Medicamentos. LTDA	Rua Joseefina, 200-Guarulhos City, Sao Paulo
Aegerion Pharmaceuticals Ltd.	Clarendon House, 2 Church Street, Hamilton, HM11
Aegerion Pharmaceuticals Limited	Royal Albert House, Sheet Street, Windsor, UK SL4 1BE
Amryt Pharmaceuticals, SAS	235, Avenue Le Jour se Leve, Boulogne-Billancourt, 92 100
Aegerion Pharmaceuticals, S.r.l.	Viale Abruzzi n. 94, Milano, 20131
Aegerion Pharmaceuticals GmbH	Maximilianstrasse 35A, Munich, Germany, 80539
Aegerion ILac Ticaret Limited Sirketi	Orjin Maslak, Eski Buyukdere Caddesi No: 27 K:11, Maslak, Istanbul, 34485
Aegerion Pharmaceuticals SARL	Rue de Rive 5, Nyon, Switzerland 1260
Aegerion Pharmaceuticals B.V.	Atrium Building, 8th Floor, Strawinskylaan 3127, 8e verdieping, Amsterdam
Aegerion Pharmaceuticals Spain, S.L.	Calle Josep Coroleu, 83 2-2, Vilanova I la Geltru, Barcelona 08800

27. Events after the reporting period

On February 18, 2020, Amryt confidentially submitted a draft registration statement on Form F-1 to the U.S. Securities and Exchange Commission (“SEC”) relating to the American Depositary Shares (“ADSs”), each representing five Amryt ordinary shares, proposed listing of the ADSs on the Nasdaq Global Select Market (“Nasdaq”).

Since a novel strain of coronavirus (SARS-CoV-2) causing a disease referred to as COVID-19 was first reported in December 2019, the disease has spread across the world, including countries in which we have patients and in which we have planned or active clinical trial sites. The outbreak and government measures taken in response have had a significant impact, both direct and indirect, on all businesses and commerce as supply chains have

F-46

been disrupted, facilities and production have been suspended and demand for certain goods and services has spiked while demand for other goods and services has fallen. As COVID-19 continues to spread around the globe, Amryt may experience disruptions that could affect its business, preclinical studies and clinical trials.

In response to the spread of COVID-19, Amryt has closed its executive offices with its administrative employees continuing their work outside of our offices and limited the number of staff in Amryt’s manufacturing facility in Germany. Amryt provides therapeutic products to HoFH and lipodystrophy patients globally on a recurring basis. Once lomitapide (for the treatment of HoFH) or metreleptin (for the treatment of lipodystrophy) is prescribed by physicians, patients are typically on treatment over a long period of time with repeat prescriptions for each patient.

F-47

Amryt Pharma plc

Condensed Consolidated Statement of Financial Position

		As at,
		March 31, 2020 (unaudited)	December 31, 2019 (audited)
	Note	US$’000
Assets
Non-current assets
Goodwill	7	$30,813	$30,813
Intangible assets	7	339,094	350,953
Property, plant and equipment		2,862	3,036
Other non-current assets		2,310	2,306
Total non-current assets		375,079	387,108
Current assets
Trade and other receivables	8	41,179	36,387
Inventories		33,904	43,623
Cash and cash equivalents, including restricted cash	9	68,067	67,229
Total current assets		143,150	147,239
Total assets		518,229	534,347
Equity and liabilities
Equity attributable to owners of the parent
Share capital	10	11,918	11,918
Share premium	10	2,422	2,422
Other reserves		249,386	248,656
Accumulated deficit		(162,569)	(133,674)
Total equity		101,157	129,322
Non-current liabilities
Contingent consideration and contingent value rights	5	106,145	102,461
Deferred tax liability		17,345	18,921
Long term loan	11	82,989	81,610
Convertible notes	12	97,872	96,856
Provisions and other liabilities	13	1,014	4,963
Total non-current liabilities		305,365	304,811
Current liabilities
Trade and other payables		87,575	76,596
Provisions and other liabilities	13	24,132	23,618
Total current liabilities		111,707	100,214
Total liabilities		417,072	405,025
Total equity and liabilities		$518,229	$534,347

F-48

Amryt Pharma plc

Condensed Consolidated Statement of Comprehensive Loss

		Three months ended March 31,
		2020 (unaudited)	2019 (unaudited)
	Note	US$’000
Revenue	3	$44,574	$4,542
Cost of sales		(32,620)	(1,830)
Gross profit		11,954	2,712
Research and development expenses		(8,934)	(1,505)
Selling, general and administrative expenses		(18,406)	(3,896)
Acquisition and severance related costs		(853)	—
Share based payment expenses	4	(745)	(91)
Operating loss before finance expense		(16,984)	(2,780)
Non-cash change in fair value of contingent consideration	5	(2,906)	(1,938)
Non-cash contingent value rights finance expense	5	(1,448)	—
Net finance expense - other		(9,416)	(661)
Loss on ordinary activities before taxation		(30,754)	(5,379)
Tax credit/(charge) on loss on ordinary activities		1,857	(6)
Loss for the period attributable to the equity holders of the Company		(28,897)	(5,385)
Exchange translation differences which may be reclassified through profit or loss		(13)	80
Total other comprehensive (loss)/income		(13)	80
Total comprehensive loss for the period attributable to the equity holders of the Company		$(28,910)	$(5,305)
Loss per share
Loss per share - basic and diluted, attributable to ordinary equity holders of the parent (US$)	6	$(0.19)	$(0.12)

F-49

Amryt Pharma plc

Condensed Consolidated Statement of Cash Flows

		Three months ended March 31,
		2020 (unaudited)	2019 (unaudited)
	Note	US$’000
Cash flows from operating activities
Loss on ordinary activities after taxation		$(28,897)	$(5,385)
Net finance expense - other		9,416	661
Depreciation and amortization		11,241	91
Amortization of inventory fair value step-up		9,503	—
Share based payment expenses	4	745	91
Non-cash change in fair value of contingent consideration	5	2,906	1,938
Non-cash contingent value rights finance expense	5	1,148
Deferred taxation credit		(1,576)	—
Movements in working capital and other adjustments:
Change in trade and other receivables		(4,792)	(754)
Change in trade and other payables		9,416	(1,902)
Change in provision and other liabilities	13	(3,435)	—
Change in inventories		216	(255)
Change in non-current assets		(4)	74
Net cash flow from (used in) operating activities		6,187	(5,441)
Cash flow from investing activities
Payments for property, plant and equipment		(79)	(4)
Deposit interest received		66	—
Net cash used in investing activities		(13)	(4)
Cash flow from financing activities
Increase in long term debt		—	5,679
Interest paid		(1,506)	(4)
Net cash (used in) flow from financing activities		(1,506)	5,675
Exchange and other movements		(3,830)	(74)
Net change in cash and cash equivalents		838	156
Cash and cash equivalents at beginning of the period		67,229	11,226
Restricted cash at end of the period		1,093	—
Cash at bank available on demand at end of the period		66,974	11,382
Total cash and cash equivalents at end of the period		$68,067	$11,382

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Amryt Pharma plc
Condensed Consolidated Statement of Changes in Equity
For the period ended March 31, 2020

		Share capital	Share premium	Warrant reserve	Treasury shares	Share based payment reserve	Merger reserve	Reverse acquisition reserve	Equity component of convertible notes	Other distributable reserves	Currency translation reserve	Accumulated deficit	Total
	Note	US$’000
Balance at January 1, 2020 (audited)		$11,918	$2,422	$29,523	$(7,534)	$3,190	$42,627	$(73,914)	$29,210	$217,634	$7,920	$(133,674)	$129,322
Loss for the period		—	—	—	—	—	—	—	—	—	—	(28,897)	(28,897)
Foreign exchange translation reserve		—	—	—	—	—	—	—	—	—	(13)	—	(13)
Total comprehensive loss		—	—	—	—	—	—	—	—	—	(13)	(28,897)	(28,910)
Transactions with owners
Share based payment expense	4	—	—	—	—	745	—	—	—	—	—	—	745
Share based payment expense – Lapsed		—	—	—	—	(2)	—	—	—	—	—	2	—
Total transactions with owners		—	—	—	—	743	—	—	—	—	—	2	745
Balance at March 31, 2020 (unaudited)		$11,918	$2,422	$29,523	$(7,534)	$3,933	$42,627	$(73,914)	$29,210	$217,634	$7,907	$(162,569)	$101,157

F-51

Amryt Pharma plc
Condensed Consolidated Statement of Changes in Equity
For the period ended March 31, 2019

		Share capital	Share premium	Share based payment reserve	Merger reserve	Reverse acquisition reserve	Currency translation reserve	Accumulated deficit	Total
	Note	US$’000
Balance at January 1, 2019 (audited)		$25,198	$68,233	$6,473	$42,627	$(73,914)	$(51)	$(72,263)	$(3,697)
Loss for the period		—	—	—	—	—	—	(5,385)	(5,385)
Foreign exchange translation reserve		—	—	—	—	—	80	—	80
Total comprehensive loss		—	—	—	—	—	80	(5,385)	(5,305)
Transactions with owners
Share based payment expense	4	—	—	91	—	—	—	—	91
Total transactions with owners		—	—	91	—	—	—	—	91
Balance at March 31, 2019 (unaudited)		$25,198	$68,233	$6,564	$42,627	$(73,914)	$29	$(77,648)	$8,911

F-52

1. General information

We are a global, commercial-stage biopharmaceutical company dedicated to commercializing and developing novel therapeutics to treat patients suffering from serious and life-threatening rare diseases.

As used herein, references to “we,” “us,” “Amryt” or the “Group” in these condensed consolidated interim financial statements shall mean Amryt Pharma plc and its global subsidiaries, collectively. References to the “Company” in these condensed consolidated interim financial statements shall mean Amryt Pharma plc.

Amryt Pharma plc is a company incorporated in England and Wales. The Company is listed on the AIM market of the London Stock Exchange (ticker: AMYT) and the Euronext Growth Exchange of the Irish Stock Exchange (ticker: AYP).

Aegerion Pharmaceuticals, Inc. (“Aegerion”), a former subsidiary of Novelion Therapeutics Inc. (“Novelion”), is a rare and orphan disease company with a diversified offering of multiple commercial and development stage assets. The acquisition of Aegerion by Amryt in September 2019 has given Amryt an expanded commercial footprint to market two U.S. and EU approved products, lomitapide (JUXTAPID (U.S.) / LOJUXTA (EU)) and metreleptin (MYALEPT (U.S.) / MYALEPTA (EU)).

On July 10, 2019, the shareholders of the Company approved a resolution to give authority to the Company to undertake a consolidation of the existing ordinary shares in the capital of the Company under which every six existing ordinary shares were consolidated into one ordinary share. The number of shares in issue at March 31, 2019 has been adjusted to reflect this share consolidation on July 10, 2019 for the purposes of the loss per share calculation. The number of share options outstanding at January 1, 2019 and the share options granted and lapsing during the three months ended March 31, 2019 have been restated to reflect the 2019 share consolidation.

On September 20, 2019, Amryt registered FILSUVEZ as the trademark name for the Group’s lead development asset, AP101, in the European Union. On February 18, 2020, Amryt also registered this trademark name in the United States and is in the process of registering the FILSUVEZ trademark in other key jurisdictions.

2. Accounting policies

Basis of preparation

The condensed consolidated interim financial statements of the Group have been prepared in accordance with IAS 34 Interim Financial Reporting. They do not include all of the information required in annual financial statements in accordance with International Financial Reporting Standards (“IFRS”) and should be read in conjunction with the annual consolidated financial statements for the year ended December 31, 2019. Selected explanatory notes are included to explain events and transactions that are significant to an understanding of the Group’s financial position and performance since the last annual financial statements. The accounting policies used in the preparation of the interim financial information are the same as those used in the Group’s audited financial statements for the year ended December 31, 2019 and those which are expected to be used in the financial statements for the year ending December 31, 2020.

Results for the three-month period ended March 31, 2020 are not necessarily indicative of the results that may be expected for the financial year ending December 31, 2020.

Basis of going concern

Having considered the Group’s current financial position and cash flow projections, the Board of Directors believes that the Group will be able to continue in operational existence for at least the next 12 months from the date of approval of these condensed consolidated interim financial statements and that it is appropriate to continue to prepare the condensed consolidated interim financial statements on a going concern basis.

A key consideration for the impact on going concern is the acquisition of Aegerion, which was completed in September 2019. This acquisition represents a significant step forward for Amryt and has created value for Amryt with immediate effect post-deal close through enhanced scale of the combined Group, which Amryt believes has the potential to drive revenues and deliver operational synergies through a combination of medical, commercial, clinical, development and regulatory infrastructure. Additionally, Amryt completed a US$60,000,000 fundraising as part of the acquisition of Aegerion.

Since a novel strain of coronavirus (SARS-CoV-2) causing a disease referred to as COVID-19 was first reported in December 2019, the disease has spread across the world, including countries in which we have patients and in

F-53

which we have planned or active clinical trial sites. The outbreak and government measures taken in response have had a significant impact, both direct and indirect, on all businesses and commerce as supply chains have been disrupted, facilities and production have been suspended and demand for certain goods and services has spiked while demand for other goods and services has fallen. As COVID-19 continues to spread around the globe, Amryt may experience disruptions that could affect its business, preclinical studies and clinical trials.

Amryt provides therapeutic products to Homozygous Familial Hypercholesterolemia (“HoFH”) and lipodystrophy patients globally on a recurring basis. Once lomitapide (for the treatment of HoFH) or metreleptin (for the treatment of lipodystrophy) is prescribed by physicians, patients are typically on treatment over a long period of time with repeat prescriptions for each patient. To date the Company has seen minimal impact of the COVID-19 pandemic on the business given the majority of revenues are recurring in nature and the Company has a strong cash position and resources to support the Company’s ability to continue as a going concern.

Basis of consolidation

The condensed consolidated interim financial statements comprise the financial statements of the Group for the three months ended March 31, 2020. Subsidiaries are entities controlled by the Company. Where the Company has control over an investee, it is classified as a subsidiary. The Company controls an investee if all three of the following elements are present: power over an investee, exposure or rights to variable returns from its involvement with the investee and the ability to use its power to affect those variable returns. Control is reassessed whenever facts and circumstances indicate that there may be a change in any of these elements of control.

Subsidiaries are fully consolidated from the date that control commences until the date that control ceases. Accounting policies of subsidiaries have been changed where necessary to ensure consistency with the policies adopted by the Group. Intergroup balances and any unrealized gains or losses, income or expenses arising from intergroup transactions are eliminated in preparing the consolidated financial statements.

Presentation of balances

The condensed consolidated interim financial statements are presented in U.S. dollars (“US$”) which is the functional currency and presentation currency of the Group.

The following table discloses the major exchange rates of those currencies other than the functional currency of US$ that are utilized by the Group:

Foreign currency units to 1 US$	€	£	CHF	SEK	NOK	DKK
Average period to March 31, 2019 (unaudited)	0.8804	0.7683	0.9967	9.1704	8.5802	6.5711
At March 31, 2019 (unaudited)	0.8915	0.7673	0.9953	9.2979	8.6271	6.6550

Foreign currency units to 1 US$	€	£	CHF	SEK	NOK	DKK
Average period to December 31, 2019 (audited)	0.8932	0.7836	0.9938	9.4533	8.7976	6.6690
At December 31, 2019 (audited)	0.8929	0.7624	0.971	9.3282	8.8046	6.6698

Foreign currency units to 1 US$	€	£	CHF	SEK	NOK	DKK
Average period to March 31, 2020 (unaudited)	0.9068	0.7809	0.9679	9.6618	9.4731	6.7750
At March 31, 2020 (unaudited)	0.9043	0.8068	0.9570	9.9977	10.5721	6.7517

(€ = Euro; £ = Pounds Sterling, CHF = Swiss Franc, SEK = Swedish Kroner, NOK = Norwegian Kroner, DKK = Danish Kroner)

Changes in accounting policies and disclosures

There are no new standards and amendments to IFRS effective as of January 1, 2020 that are relevant to the Group.

Critical accounting judgements and key sources of estimation uncertainty

The preparation of financial statements in conformity with IFRS requires management to make judgements, estimates and assumptions that affect the application of policies and amounts reported in the financial statements and accompanying notes. The estimates and associated assumptions are based on historical experience and

F-54

various other factors that are believed to be reasonable under the circumstances, the results of which form the basis of making the judgements about the carrying value of assets and liabilities that are not readily apparent from other sources. Actual results may differ from these estimates.

The estimates and underlying assumptions are reviewed on an ongoing basis. Revisions to accounting estimates are recognized in the period in which the estimate is revised if the revision affects only that period or in the period of the revision and future periods if the revision affects both current and future periods.

The significant estimates, assumptions or judgements, applied in the condensed consolidated interim financial statements were the same as those applied in the Group’s audited financial statements for the year ended December 31, 2019.

Principal accounting policies

The condensed consolidated interim financial statements have been prepared in accordance with the accounting policies adopted in the Group’s audited financial statements for the year ended December 31, 2019.

3. Segment information

The Group is a global, commercial-stage biopharmaceutical company dedicated to commercializing and developing novel therapeutics to treat patients suffering from serious and life-threatening rare diseases.

The Group currently operates as one business segment, pharmaceuticals, and is focused on the development and commercialization of two commercial products and two development products. The Group derives its revenues primarily from one source, the pharmaceutical sector with high unmet medical need.

The Group’s Chief Executive Officer, Joseph Wiley, is currently the Company’s chief operating decision maker (“CODM”). The Group does not operate any separate lines of business or separate business entities with respect to its products. Accordingly, the Group does not accumulate discrete financial information with respect to separate service lines and does not have separate reportable segments.

The following table summarizes total revenues from external customers by product and by geographic region, based on the location of the customer. Revenues represent the revenue from the Group for the three months ended March 31, 2020 and 2019. Revenue in the three months ended March 31, 2020 include revenues from the acquired Aegerion Group and associated products and regions.

	Three months ended March 31, 2020 (unaudited)
	U.S.	EMEA	Other	Total
	US$’000
Metreleptin	$14,914	$8,628	$3,385	$26,927
Lomitapide	9,470	5,233	2,718	17,421
Other	—	226	—	226
Total revenue	$24,384	$14,087	$6,103	$44,574

	Three months ended March 31, 2019 (unaudited)
	U.S.	EMEA	Other	Total
	US$’000
Metreleptin	$—	$—	$—	$—
Lomitapide	—	4,419	—	4,419
Other	—	123	—	123
Total revenue	$—	$4,542	$—	$4,542

Major Customers

For the three months ended March 31, 2020, one customer accounted for 55% of the Group’s net revenues and accounted for 39% of the Group’s March 31, 2020 accounts receivable balance. For the three months ended March 31, 2019, the Group generated over 72% of its lomitapide revenue in Italy, the Netherlands and Greece. The largest lomitapide customer in the three months ended March 31, 2019 was a distributor in Italy.

F-55

4. Share based payments

On July 10, 2019, the shareholders of the Company approved a resolution to give authority to the Company to undertake a consolidation of the existing ordinary shares in the capital of the Company under which every 6 existing ordinary shares were consolidated into one ordinary share.

Under the terms of the Company’s Employee Share Option Plan, options to purchase 17,154,554 shares were outstanding at March 31, 2020. Under the terms of this plan, options are granted to officers, consultants and employees of the Group at the discretion of the Remuneration Committee. A total of 2,687,000 share options were granted to employees in the three-month period ended March 31, 2020. For the year ended December 31, 2019, a total of 11,330,641 share options were granted to directors and employees.

Outstanding warrants at March 31, 2020 and December 31, 2019 consisted of 17,196,273 zero cost warrants with no expiration date that were issued to Aegerion creditors in connection with the acquisition of Aegerion. The remaining warrants consisting of 345,542 warrants were issued in connection with the admission to the AIM in 2016.

The number and weighted average exercise price (in Sterling pence) of share options and warrants per ordinary share is as follows:

	Share Options		Warrants
	Units	Weighted average exercise price (Sterling pence)	Units	Weighted average exercise price (Sterling pence)
Balance at January 1, 2019 (restated for 6:1 share consolidation)	3,250,855	115.20p	3,818,325	144.00p
Granted	11,330,641	117.01p	18,841,378	—
Lapsed	(99,776)	197.66p	(3,472,783)	144.00p
Exercised	—	—	(1,645,105)	—
Outstanding at December 31, 2019 (audited) (unaudited)	14,481,720	116.00p	17,541,815	0.03p
Exercisable at December 31, 2019 (audited)	2,468,310	109.08p	17,541,815	0.03p
Balance at January 1, 2020	14,481,720	116.00p	17,541,815	0.03p
Granted	2,687,000	123.50p	—	—
Lapsed	(14,166)	75.84p	—	—
Exercised	—	—	—	—
Outstanding at March 31, 2020 (unaudited)	17,154,554	117.21p	17,541,815	0.03p
Exercisable at March 31, 2020 (unaudited)	2,712,679	109.25p	17,541,815	0.03p

Fair value is estimated at the date of grant using the Black-Scholes pricing model, taking into account the terms and conditions attached to the grant. The following are the inputs to the model for the equity instruments granted during the year:

	March 31, 2020 Options Inputs (unaudited)	March 31, 2020 Warrant Inputs (unaudited)	December 31, 2019 Options Inputs (audited)	December 31, 2019 Warrant Inputs (audited)
Days to Expiration	2,555	—	2,555	—
Volatility	33%	—	27% – 48%	—
Risk free interest rate	0.46%	—	0.38% – 0.83%	—
Share price at grant	123.5p	—	75.84p – 121.5p	—

In the three months ended March 31, 2020, a total of 2,687,000 share options exercisable at a weighted average price of £1.235 were granted. The fair value of share options granted in the three months ended March 31, 2020 was £3,318,445/US$4,249,000. The share options outstanding as at March 31, 2020 have a weighted remaining contractual life of 6.08 years with exercise prices ranging from £0.76 to £1.55.

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The 2016 warrants outstanding as at March 31, 2020 have a weighted remaining contractual life of 1.05 years with an exercise price of £1.44.

The value of share options charged to the Consolidated Statement of Comprehensive Loss during the three-month period is as follows:

	Three months ended March 31,
	2020 (unaudited)	2019 (unaudited)
	US$’000
Share option expense	$745	$91
Total share option expense	$745	$91

5. Business combinations and asset acquisitions

Acquisition of Aegerion Pharmaceuticals

On May 20, 2019, Amryt entered into a Restructuring Support Agreement (as subsequently amended on June 12, 2019) and Plan Funding Agreement pursuant to which, among other matters, Amryt agreed to the acquisition of Aegerion, a former wholly-owned subsidiary of Novelion. On May 20, 2019, Aegerion and its U.S. subsidiary, Aegerion Pharmaceuticals Holdings, Inc., filed voluntary petitions under Chapter 11 of Title 11 of the U.S. Code in the Bankruptcy Court. On September 24, 2019, Amryt completed the acquisition of Aegerion. Amryt acquired Aegerion upon its emergence from bankruptcy in an exchange for ordinary shares and zero cost warrants in Amryt. Amryt issued 85,092,423 effective shares at US$1.793 per share, which is made up of 77,027,423 ordinary shares and 8,065,000 zero cost warrants, to acquire Aegerion for a value of US$152,615,000.

The acquired goodwill is attributable principally to the profit generating potential of the businesses, the assembled workforce and benefits arising from embedded infrastructure, that are expected to be achieved from integrating the acquired businesses into the Group’s existing business. No amount of goodwill is expected to be deductible for tax purposes.

The initial assignment of fair values to identifiable net assets acquired has been performed on a provisional basis due to the relative size of the acquisition and the timing of the transaction. Any amendments to these fair values within the twelve-month timeframe from the date of acquisition will be disclosed in the consolidated financial statements for the year ending December 31, 2020, as stipulated by IFRS 3 Business combinations.

Contingent Value Rights

Related to the transaction, Amryt issued Contingent Value Rights (“CVRs”) pursuant to which up to US$85,000,000 may become payable to Amryt’s shareholders and option holders, who were on the register prior to the completion of the acquisition on September 20, 2019, if certain approval and revenue milestones are met in relation AP101, Amryt’s lead product candidate. If any such milestone is achieved, Amryt may elect to pay the holders of CVRs by the issue of Amryt shares or loan notes. If Amryt elects to issue Loan Notes to holders of CVRs, it will settle such loan notes in cash 120 days after their issue. If none of the milestones are achieved, scheme shareholders and option holders will not receive any additional consideration under the terms of the CVRs. In these circumstances, the value of each CVR would be zero.

The terms of the CVRs are as follows:

• The total CVR payable is up to US$85,000,000

• This is divided into three milestones which are related to the success of AP101 (the Group’s lead development asset, currently in Phase 3 clinical trials)

• FDA approval

○ US$35,000,000 upon FDA approval

○ 100% of the amount due if approval is obtained before December 31, 2021, with a sliding scale on a linear basis to zero if before July 1, 2022

• EMA approval

○ US$15,000,000 upon EMA approval

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○ 100% of the amount due if approval is obtained before December 31, 2021, with a sliding scale on a linear basis to zero if before July 1, 2022

• Revenue targets

○ US$35,000,000 upon AP101 revenues exceeding US$75,000,000 in any 12-month period prior to June 30, 2024

• Payment can, at the Board’s discretion, be in the form of either:

○ 120-day loan notes (effectively cash), or

○ Shares valued using the 30 day / 45-day VWAP.

The CVRs were contingent on the successful completion of the acquisition and, accordingly, have been based on fair value as at September 24, 2019. In the Company-only accounts, the CVRs have been classified as a financial liability in the Consolidated Statement of Financial Position and debited to cost of investment in subsidiary. On consolidation, given that CVRs were issued to legacy Amryt shareholders in their capacity as owners of the identified acquirer as opposed to the seller in the transaction, management concluded that the most appropriate classification would be to recognize the CVR as a distribution on consolidation instead of goodwill.

Measurement of CVRs

As at March 31, 2020, the carrying value of the CVRs was US$50,861,000 (December 31, 2019: US$49,413,000). The value of the potential payout was calculated using the probability-weighted expected returns method. Using this method, the potential payment amounts were multiplied by the probability of achievement and discounted to present value. The probability adjusted present values took into account published orphan drug research data and statistics which were adjusted by management to reflect the specific circumstances applicable to the type of product acquired in the Amryt GmbH transaction. Discount rates of 10% and 16.5%, as applicable, (December 31, 2019: 10% and 16.5%) were used in the calculation of the present value of the estimated contractual cash flows for the three months ended March 31, 2020. Management was required to make certain estimates and assumptions in relation to revenue forecasts, timing of revenues and probability of achievement of commercialization of AP101. However, management notes that, due to issues outside their control (i.e. regulatory requirements and the commercial success of the product), the timing of when such revenue targets may occur may change. Such changes may have a material impact on the expected cash flows of the CVRs.

Amryt reviews the expected cash flows on a regular basis as the discount on initial recognition is being unwound as financing expenses in the Consolidated Statement of Comprehensive Loss over the life of the obligation. It is reviewed on a quarterly basis and the appropriate finance charge is booked in the consolidated statement of income on a quarterly basis. The Group expects to read out top-line data from the Phase 3 trial of AP101 in Epidermolysis Bullosa (“EB”) in the second half of 2020, followed by applications for approval from the FDA and the EMA, if top-line data is positive. Coupled with this, management has completed its annual forecast and revenues and costs reflect these current expectations.

The total non-cash finance charge recognized in the Condensed Consolidated Statement of Comprehensive Loss for the year ended March 31, 2020 is US$1,448,000 (March 31, 2019: US$nil).

Acquisition of Amryt AG (previously “Birken”)

Amryt DAC signed a conditional share purchase agreement to acquire Amryt AG on October 16, 2015 (“Amryt AG SPA”). The Amryt AG SPA was completed on April 18, 2016 with Amryt DAC acquiring the entire issued share capital of Amryt GmbH. The consideration included contingent consideration comprising milestone payments and sales royalties as follows:

• Milestone payments of:

○ €10,000,000 on receipt of first marketing approval by the EMA of Episalvan, paid on the completion date (April 18, 2016);

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○ Either (i) €5,000,000 once net ex-factory sales of Episalvan have been at least €100,000 or (ii) if no commercial sales are made within 24 months of EMA first marketing approval (being January 14, 2016), €2,000,000 24 months after receipt of such approval, which was paid in January 2018, and €3,000,000 following the first commercial sale;

○ €10,000,000 on receipt of marketing approval by the EMA or FDA of a pharmaceutical product containing Betulin as its API for the treatment of EB;

○ €10,000,000 once net ex-factory sales/net revenue in any calendar year exceed €50,000,000;

○ €15,000,000 once net ex-factory sales/ net revenue in any calendar year exceed €100,000,000;

• Cash consideration of €150,000, due and paid on the completion date (April 18, 2016); and

• Royalties of 9% on sales of Episalvan products for 10 years from first commercial sale;

Fair Value Measurement of Contingent Consideration

As of March 31, 2020, the fair value of the contingent consideration was estimated to be US$55,284,000 (December 31, 2019: US$53,048,000). The fair value of the royalty payments was determined using probability weighted revenue forecasts and the fair value of the milestone payments was determined using probability adjusted present values (see Note 14, Fair value measurement and financial risk management, for fair value hierarchy applied). The probability adjusted present values took into account published orphan drug research data and statistics which were adjusted by management to reflect the specific circumstances applicable to the type of product acquired in the Amryt GmbH transaction. A discount rate of 24.4% (December 31, 2019: 24.4%) was used in the calculation of the fair value of the contingent consideration for the three months ended March 31, 2020. Management was required to make certain estimates and assumptions in relation to revenue forecasts, timing of revenues and probability of achievement of commercialization of AP101. However, management noted that due to issues outside their control, the timing of when such revenue targets may occur may change. Such changes may have a material impact on the assessment of the fair value of the contingent consideration.

In January 2019, the Group received the results of an unblinded interim efficacy analysis for the Phase 3 trial of AP101 in EB. This analysis was conducted by an independent data safety monitoring committee and recommended that the trial should continue with an increase of 48 patients in the study to a total of 230 evaluable patients in order to be able to achieve 80% statistical power. In April 2020, given that the EASE study was already close to full enrollment, the Group announced that it had taken advice from an independent expert and concluded that the statistical impact of further patient recruitment would most likely be negligible. Amryt therefore decided to close the EASE study to further enrollment. The Group expects to read out top-line data from this trial in the second half of 2020, followed by applications for approval from the FDA and the EMA, if top-line data is positive. Coupled with this, management has completed its annual forecast and revenues and costs have been amended to reflect current expectations. These factors have resulted in a change to the probability weighted revenue forecasts and the probability of the adjusted present values which are used in the calculation of the contingent consideration balance and impact the amount being unwound to the consolidated statement of comprehensive loss.

Amryt reviews the contingent consideration on a regular basis as the probability adjusted fair values are being unwound as financing expenses in the Consolidated Statement of Comprehensive Loss over the life of the obligation. The finance charge is being unwound as a financing expense in the Consolidated Statement of Comprehensive Loss on a quarterly basis.

The total non-cash finance charge recognized in the Consolidated Statement of Comprehensive Loss for the three months ended March 31, 2020 is US$2,906,000 (March 31, 2019: US$1,938,000).

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6. Loss per share - basic and diluted

The weighted average number of shares in the loss per share (“LPS”) calculation, reflects the weighted average total actual shares of Amryt Pharma plc in issue at March 31, 2020, as adjusted (see below).

Issued share capital - ordinary shares of £0.06 each

	Number of shares	Weighted average shares
March 31, 2020 (unaudited)	154,498,887	154,498,887
March 31, 2019 (unaudited)	274,817,283	274,817,283
March 31, 2019, as adjusted (unaudited)	45,802,880	45,802,880

The number of shares in issue at March 31, 2019 has been adjusted to reflect the share consolidation on July 10, 2019, whereby each ordinary shareholder received one ordinary share for every six shares held at that date.

The calculation of loss per share is based on the following:

	Three months ended March 31,
	2020 (unaudited)	2019 (unaudited)
Loss after tax attributable to equity holders of the Company (US$’000)	$(28,897)	$(5,385)
Weighted average number of ordinary shares in issue	154,498,887	45,802,880
Fully diluted average number of ordinary shares in issue	154,498,887	45,802,880
Basic and diluted loss per share (US$)	$(0.19)	$(0.12)

The basic and diluted loss per share as at March 31, 2020 of US$0.19 (March 31, 2019: US$0.12) was calculated using the post consolidation number of ordinary shares in issue.

Where a loss has occurred, basic and diluted LPS are the same because the outstanding share options and warrants are anti-dilutive. Accordingly, diluted LPS equals the basic LPS. The share options and warrants outstanding as at March 31, 2020 totalled 34,696,369 (March 31, 2019: 3,596,398 as adjusted) and are potentially dilutive.

7. Intangible assets and goodwill

The following table summarizes the Group’s intangible assets and goodwill:

	Developed technology - metreleptin	Developed technology - lomitapide	In process R&D	Other intangible assets	Total intangible assets	Goodwill
	US$’000
Cost
At January 1, 2019
(audited)	$—	$—	$60,091	$258	$60,349	$—
Additions	—	—	—	74	74	—
Acquired assets	185,000	123,000	—	374	308,374	30,813
Impairment charge	—	—	(4,670)	—	(4,670)	—
Foreign exchange movement	—	—	(1,160)	(5)	(1,165)	—
At December 31, 2019 (audited)	185,000	123,000	54,261	701	362,962	30,813
Foreign exchange movement	—	—	(685)	(11)	(696)	—
At March 31, 2020 (unaudited)	$185,000	$123,000	$53,576	$690	$362,266	$30,813
Accumulated amortization
At January 1, 2019 (audited)	—	—	—	52	52	—
Amortization charge	7,688	4,143	—	126	11,957	—
Foreign exchange movement	—	—	—	—	—	—

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	Developed technology - metreleptin	Developed technology - lomitapide	In process R&D	Other intangible assets	Total intangible assets	Goodwill
	US$’000
At December 31, 2019 (audited)	7,688	4,143	—	178	12,009	—
Amortization charge	7,208	3,884	—	68	11,160	—
Foreign exchange movement	—	—	—	3	3	—
At March 31, 2020 (unaudited)	$14,896	$8,027	$—	$249	$23,172	$—
Net book value
At December 31, 2019 (audited)	$177,312	$118,857	$54,261	$523	$350,953	$30,813
At March 31, 2020 (unaudited)	$170,104	$114,973	$53,576	$441	$339,094	$30,813

Developed technology on commercially marketed products

In connection with the acquisition of Aegerion in September 2019, the Group acquired developed technology, metreleptin and lomitapide. These intangible assets are amortized over their estimated useful lives and the remaining useful lives for metreleptin and lomitapide are approximately 5.9 and 7.4 years, respectively, as of March 31, 2020.

In-process R&D

As a result of the acquisition of Amryt GmbH, in 2016, the Group recognized in-process R&D costs of US$54,268,000 which is related to the Group’s lead development asset, AP101.

Goodwill

During 2019, the Group completed the acquisition of Aegerion, which resulted in aggregate goodwill of US$30,813,000.

The Group reviews events or changes in circumstances that may indicate a triggering event for impairment. Management applied its judgment in determining that there were no events or changes in circumstances causing any impairment triggers as of March 31, 2020. As such there was no impairment charge recorded during the three months ended March 31, 2020.

8. Trade and other receivables

	As at
	March 31, 2020 (unaudited)	December 31, 2019 (audited)
	US$’000
Trade receivables	$32,276	$28,607
Accrued income and other debtors	5,556	5,934
VAT recoverable	3,347	1,846
Trade and other receivables	$41,179	$36,387

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9. Cash and cash equivalents

	As at
	March 31, 2020 (unaudited)	December 31, 2019 (audited)
	US$’000
Cash at bank available on demand	$66,974	$65,197
Restricted cash	1,093	2,032
Total cash and cash equivalents	$68,067	$67,229

Cash and cash equivalents include cash at bank available on demand and restricted cash.

At March 31, 2020, there was US$653,000 (December 31, 2019: US$1,219,000) of restricted cash predominantly consisting of cash held in an escrow account set-up in accordance with Aegerion’s bankruptcy plan as approved by the U.S. Bankruptcy Court to meet the costs associated with the bankruptcy process. Additionally, US$440,000 is cash held by a third-party distributor at March 31, 2020 (December 31, 2019: US$813,000); the funds from the third-party distributor were transferred to Amryt in April 2020.

10. Share capital and reserves

Details of issued ordinary shares with a nominal value of Sterling 6 pence (2019: 1 pence) each are in the table below.

Date	Number of ordinary shares	Total Share Capital US$’000	Total Share Premium US$’000
At March 31, 2020 (unaudited)	159,363,543	$11,918	$2,422
At December 31, 2019 (audited)	159,363,543	$11,918	$2,422

The number of ordinary shares issued at March 31, 2020 and December 31, 2019 includes treasury shares of 4,864,656.

Share Capital

Share capital represents the cumulative par value arising upon issue of ordinary shares of Sterling 6 pence each.

The ordinary shares have the right to receive notice of, attend and vote at general meetings and participate in the profits of the Company.

Share Premium

Share premium represents the consideration that has been received in excess of the nominal value on issue of share capital net of issue costs and transfers to distributable reserves.

Warrant reserve

The warrant reserve represents zero cost warrants issued as part of the equity raise on September 24, 2019 net of issue costs apportioned to warrants issued and additional warrants issued to certain shareholders on November 14, 2019. Each warrant entitles the holder to subscribe for one ordinary share at zero cost.

Treasury Shares

On November 14, 2019, the Company repurchased a combined 4,864,656 ordinary shares from certain shareholders. In exchange for the ordinary shares, these shareholders were issued an equivalent number of zero cost warrants. These ordinary shares are now held as treasury shares.

Share based payment reserve

Share based payment reserve relates to the charge for share based payments in accordance with IFRS 2.

Merger reserve

The merger reserve was created on the acquisition of Amryt DAC by Amryt Pharma plc in April 2016. Ordinary shares in Amryt Pharma plc were issued to acquire the entire issued share capital of Amryt DAC. Under section 612 of the UK Companies Act 2006, the premium on these shares has been included in a merger reserve.

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Reverse acquisition reserve

The reverse acquisition reserve arose during the period ended December 31, 2016 in respect of the reverse acquisition of Amryt Pharma plc by Amryt DAC. Since the shareholders of Amryt DAC became the majority shareholders of the enlarged Group, the acquisition is accounted for as though there is a continuation of Amryt DAC’s financial statements. The reverse acquisition reserve is created to maintain the equity structure of Amryt Pharma plc in compliance with UK company law.

Equity component of convertible notes

The equity component of convertible notes represents the equity component of the US$125,000,000 convertible debt, that was issued on September 24, 2019, and is measured by determining the residual of the fair value of the instrument less the estimated fair value of the liability component. The equity component is recognized in equity and is not subsequently remeasured.

Other distributable reserves

Other distributable reserves comprise the following:

• Distribution of the share premium amount on November 6, 2019 of US$268,505,000.

• A deemed distribution of US$47,902,000 arising from the issuance of CVRs.

• A deemed distribution of US$2,969,000 arising from the scheme of arrangement in September 2019 whereby Amryt Pharma plc, which was incorporated in July 2019, became a 100% shareholder of Amryt Pharma Holdings Limited (formerly named Amryt Pharma plc) (the “Acquisition of subsidiary without a change of control”).

Currency translation reserve

The currency translation reserve arises on the retranslation of non-U.S, dollar denominated foreign subsidiaries.

Accumulated deficit

Accumulated deficit represents losses accumulated in previous periods and the current year.

11. Long term loan

	As at
	March 31, 2020 (unaudited)	December 31, 2019 (audited)
	US$’000
Long term loan principal	$81,021	81,021
Accrued unpaid interest	2,789	1,435
Unamortized debt issuance costs	(821)	(846)
Long term loan	$82,989	$81,610

As part of the acquisition of Aegerion on September 24, 2019, Aegerion entered into a new U.S. dollar denominated US$81,021,000 secured term loan debt facility (“Term Loan”) with various lenders. The Term Loan is made up of a US$54,469,000 loan that was in place prior to the acquisition which was refinanced as part of the acquisition and a US$26,552,000 additional loan that was drawn down on September 24, 2019. The Term Loan has a five-year term from the date of the draw down, September 24, 2019 and matures on September 24, 2024. Under the Term Loan, interest will be payable at the option of the Group at the rate of 11% per annum paid in cash on a quarterly basis or at a rate of 6.5% paid in cash plus 6.5% paid in kind that will be paid when the principal is repaid, which rolls up and is included in the principal balance outstanding, on a quarterly basis. The Term Loan may be prepaid, in whole or in part, by Aegerion at any time subject to payment of an exit fee, which depending on the stage of the loan term, ranges from 5.00% to 0.00% of the principal then outstanding on the Term Loan.

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The Term Loan is guaranteed by Amryt and certain subsidiaries of the Group. In connection with the loan agreement, fixed and floating charges have been placed on property and undertakings of Amryt and certain subsidiaries of the Group.

The Term Loan agreement includes affirmative and negative covenants, including prohibitions on the incurrence of additional indebtedness, granting of liens, certain asset dispositions, investments and restricted payments, in each case, subject to certain exceptions set forth in the Loan Agreement. The Term Loan agreement also includes customary events of default for a transaction of this type, and includes (i) a cross-default to the occurrence of any event of default under material indebtedness of Aegerion and certain subsidiaries of the Group and Amryt, including the convertible notes, and (ii) Amryt or any of its subsidiaries being subject to bankruptcy or other insolvency proceedings. Upon the occurrence of an event of default, the lenders may declare all of the outstanding Term Loan and other obligations under the Term Loan agreement to be immediately due and payable and exercise all rights and remedies available to the lenders under the Term Loan agreement and related documentation. There have been no events of default or breaches of the covenants occurring for the three months ended March 31, 2020 and for year ended December 31, 2019.

12. Convertible notes

	As at
	March 31, 2020 (unaudited)	December 31, 2019 (audited)
	US$’000
Issuance of convertible notes	$125,000	$125,000
Amount classified as equity	(29,210)	(29,210)
Accreted interest	2,082	1,066
Total convertible notes	$97,872	$96,856

As part of the acquisition, Aegerion issued convertible notes with an aggregate principal amount of US$125,000,000 to Aegerion creditors.

The convertible notes are senior unsecured obligations and bear interest at a rate of 5.0% per year, payable semi-annually in arrears on April 1 and October 1 of each year, beginning on April 1, 2020. The convertible notes will mature on April 1, 2025, unless earlier repurchased or converted.

The convertible notes are convertible into Amryt’s ordinary shares at a conversion rate of 386.75 ordinary shares per US$1,000 principal amount of the convertible notes. If the holders elect to convert the convertible notes, Aegerion can settle the conversion of the convertible notes through payment or delivery of cash, common shares, or a combination of cash and common shares, at its discretion. As a result of the conversion feature in the convertible notes, the convertible notes were assessed to have both a debt and an equity component. The two components were assessed separately and classified as a financial liability and equity instrument. The financial liability component was measured at fair value based on the discounted cash flows expected over the expected term of the notes using a discount rate based on a market interest rate that a similar debt instrument without a conversion feature would be subject to. Refer to Note 10, Share capital and reserves, for further details on the equity component of the convertible notes.

From September 24, 2019 until the close of business on the second scheduled trading day immediately preceding the maturity date, holders may convert all or any portion of their convertible notes, in multiples of US$1,000 principal amount, at the option of the holder.

The indenture does not contain any financial covenants and does not restrict the Group’s ability to repurchase securities, pay dividends or make restricted payments in the event of a transaction that substantially increases the Group’s level of indebtedness in certain circumstances.

The indenture contains customary terms and covenants and events of default. If an event of default (other than certain events of bankruptcy, insolvency or reorganization involving Aegerion, Amryt and certain subsidiaries of the Group) occurs and is continuing, the trustee by notice to Aegerion, or the holders of at least 25% in principal amount of the outstanding convertible notes by written notice to Aegerion and the trustee, may declare 100% of the principal of and accrued and unpaid interest, if any, on all of the convertible notes to be due and payable. Upon such a declaration of acceleration, such principal and accrued and unpaid interest, if any, will be due and

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payable immediately. Upon the occurrence of certain events of bankruptcy, insolvency or reorganization involving Aegerion, 100% of the principal and accrued and unpaid interest, if any, on the convertible notes will become due and payable automatically. Notwithstanding the foregoing, the indenture provides that, upon Aegerion’s election, and for up to 180 days, the sole remedy for an event of default relating to certain failures by Aegerion to comply with certain reporting covenants in the indenture consists exclusively of the right to receive additional interest on the convertible notes. There have been no events of default or breaches of the covenants occurring for the three months ended March 31, 2020 and for year ended December 31, 2019.

13. Provisions and other liabilities

	As at
	March 31, 2020 (unaudited)	December 31, 2019 (audited)
	US$’000
Non-current liabilities
Provisions and other liabilities	$—	$3,910
Leases due greater than 1 year	1,014	1,053
	1,014	4,963
Current liabilities
Provisions and other liabilities	23,670	23,047
Leases due less than 1 year	462	571
	24,132	23,618
Total provisions and other liabilities	$25,146	$28,581

Legal matters

Prior to the acquisition of Aegerion by Amryt, Aegerion entered into settlement agreements with governmental entities including the Department of Justice (“DOJ”) and the FDA in connection with JUXTAPID investigations. The settlement agreements require Aegerion to pay specified fines and engage in regulatory compliance efforts. The settlements that remain due as a current liability and a non-current liability is $16,169,000 and $nil, respectively, as of March 31, 2020 (December 31, 2019: $15,547,000 and $3,910,000, respectively).

Other legal matters

The Group recognizes a liability for legal contingencies when it believes that it is both probable that a liability has been incurred and that it can reasonably estimate the amount of the loss. The Group reviews these accruals and adjusts them to reflect ongoing negotiations, settlements, rulings, advice of legal counsel and other relevant information. To the extent new information is obtained and the Group’s views on the probable outcomes of claims, suits, assessments, investigations or legal proceedings change, changes in the Group’s liability accrual would be recorded in the period in which such determination is made. At March 31, 2020 and December 31, 2019, the Group had recognized liabilities of US$7,500,000 in relation to ongoing legal matters.

14. Fair value measurement and financial risk management

Categories of financial instruments

	As at
	March 31, 2020 (unaudited)	December 31, 2019 (audited)
	US$’000
Financial assets (all at amortized cost):
Cash and cash equivalents	$68,067	$67,229
Trade receivables	32,276	28,607
Total financial assets	100,343	95,836

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	As at
	March 31, 2020 (unaudited)	December 31, 2019 (audited)
	US$’000
Financial liabilities:
At amortized cost
Trade payables and accrued expenses	87,460	75,800
Lease liabilities	1,476‬	1,624‬
Other liabilities	16,169	19,457
Convertible notes	97,872	96,856
Long term loan	82,989	81,610
Contingent value rights	50,861	49,413
At fair value
Contingent consideration	55,284	53,048
Total financial liabilities	392,111	377,808
Net	$(291,768)	$(281,972)

Financial instruments evaluated at fair value can be classified according to the following valuation hierarchy, which reflects the extent to which the fair value is observable:

• Level 1: fair value evaluations using prices listed on active markets (not adjusted) of identical assets or liabilities.

• Level 2: fair value evaluations using input data for the asset or liability that are either directly observable (as prices) or indirectly observable (derived from prices), but which do not constitute listed prices pursuant to Level 1.

• Level 3: fair value evaluations using input data for the asset or liability that are not based on observable market data (unobservable input data).

The contingent consideration has been valued using Level 3. The contingent consideration comprises:

• Contingent consideration relating to the acquisition of Amryt GmbH (see Note 5, Business combinations and asset acquisitions) that was measured at US$55,284,000 as at March 31, 2020 (December 31, 2019: US$53,048,000). The fair value comprises royalty payments which was determined using probability weighted revenue forecasts and the fair value of the milestones payments which was determined using probability adjusted present values.

Impact of key unobservable input data

• An increase of 10% in estimated revenue forecasts would result in an increase to the fair value of US$3,900,000. A decrease would have the opposite effect.

• A 5% increase in the discount factor used would result in a decrease to the fair value of US$9,822,000. A decrease of 5% would result in an increase to the fair value of US$13,296,000.

• A six-month delay in the launch date for AP101 for EB would result in a decrease to the fair value of US$4,540,000.

15. Events after the reporting period

COVID-19

Since a novel strain of coronavirus (SARS-CoV-2) causing a disease referred to as COVID-19 was first reported in December 2019, the disease has spread across the world, including countries in which we have patients and in which we have planned or active clinical trial sites. The outbreak and government measures taken in response have had a significant impact, both direct and indirect, on all businesses and commerce as supply chains have been disrupted, facilities and production have been suspended and demand for certain goods and services has spiked while demand for other goods and services has fallen. As COVID-19 continues to spread around the globe, Amryt may experience disruptions that could affect its business, preclinical studies and clinical trials.

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In response to the spread of COVID-19, Amryt has closed its executive offices with its administrative employees continuing their work outside of our offices and limited the number of staff in Amryt’s manufacturing facility in Germany. Amryt provides therapeutic products to HoFH and lipodystrophy patients globally on a recurring basis. Once lomitapide (for the treatment of HoFH) or metreleptin (for the treatment of lipodystrophy) is prescribed by physicians, patients are typically on treatment over a long period of time with repeat prescriptions for each patient.

Other

In May 2020, the Group entered into a 20-year term lease for its European operational office, located in Dublin, Ireland. The lease will commence in 2020 and contains an option to terminate after 12 years.

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Aegerion Pharmaceuticals, Inc.

INDEPENDENT AUDITORS’ REPORT

To the Board of Directors of Aegerion Pharmaceuticals, Inc.

We have audited the accompanying consolidated financial statements of Aegerion Pharmaceuticals, Inc. and its subsidiaries (the “Company”), which comprise the consolidated balance sheets as of December 31, 2018 and 2017, and the related consolidated statements of operations, comprehensive loss, shareholders' equity (deficit) and cash flows for the years then ended, and the related notes to the consolidated financial statements.

Management's Responsibility for the Consolidated Financial Statements

Management is responsible for the preparation and fair presentation of these consolidated financial statements in accordance with accounting principles generally accepted in the United States of America; this includes the design, implementation and maintenance of internal control relevant to the preparation and fair presentation of consolidated financial statements that are free from material misstatement, whether due to fraud or error.

Auditors' Responsibility

Our responsibility is to express an opinion on these consolidated financial statements based on our audits. We conducted our audits in accordance with auditing standards generally accepted in the United States of America. Those standards require that we plan and perform the audit to obtain reasonable assurance about whether the consolidated financial statements are free from material misstatement.

An audit involves performing procedures to obtain audit evidence about the amounts and disclosures in the consolidated financial statements. The procedures selected depend on the auditor’s judgment, including the assessment of the risks of material misstatement of the consolidated financial statements, whether due to fraud or error. In making those risk assessments, the auditor considers internal control relevant to the Company's preparation and fair presentation of the consolidated financial statements in order to design audit procedures that are appropriate in the circumstances, but not for the purpose of expressing an opinion on the effectiveness of the Company's internal control. Accordingly, we express no such opinion. An audit also includes evaluating the appropriateness of accounting policies used and the reasonableness of significant accounting estimates made by management, as well as evaluating the overall presentation of the consolidated financial statements.

We believe that the audit evidence we have obtained is sufficient and appropriate to provide a basis for our audit opinion.

Opinion

In our opinion, the consolidated financial statements referred to above present fairly, in all material respects, the financial position of Aegerion Pharmaceuticals, Inc. and its subsidiaries as of December 31, 2018 and 2017, and the results of their operations and their cash flows for the years then ended in accordance with accounting principles generally accepted in the United States of America.

Emphasis of Matter Regarding Going Concern

The accompanying consolidated financial statements have been prepared assuming that the Company will continue as a going concern. As discussed in Note 1 to the consolidated financial statements, the Company’s level of indebtedness and Chapter 11 bankruptcy filing raise substantial doubt about its ability to continue as a going concern. Management's evaluation of the events and conditions and management’s plans regarding these matters are also described in Note 1. The consolidated financial statements do not include any adjustments that might result from the outcome of this uncertainty. Our opinion is not modified with respect to this matter.

Emphasis of Matter Regarding Transactions with Parent Company

As discussed in Note 2 to the consolidated financial statements, such financial statements include significant transactions with and allocations from its former parent company, Novelion Therapeutics Inc., and its affiliates. As a result, the accompanying consolidated financial statements have been prepared from the separate records maintained by the Company and may not necessarily be indicative of the conditions that would have existed or the results of operations if the Company had been operated as an unaffiliated company. Our opinion is not modified with respect to this matter.

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Emphasis of Matter Regarding Bankruptcy Proceedings

As discussed in Notes 1 and 16 to the consolidated financial statements, on May 20, 2019, the Company filed for reorganization under Chapter 11 of the U.S. Bankruptcy Code. The accompanying consolidated financial statements do not purport to reflect or provide for the consequences of the bankruptcy proceedings. In particular, such consolidated financial statements do not purport to show (1) as to assets, their realizable value on a liquidation basis or their availability to satisfy liabilities; (2) as to prepetition liabilities, the settlement amounts for allowed claims, or the status and priority thereof; (3) as to stockholder accounts, the effect of any changes that may be made in the capitalization of the Company; or (4) as to operations, the effect of any changes that may be made in its business. Our opinion is not modified with respect to this matter.

/s/ Deloitte & Touche LLP

Boston, Massachusetts
February 18, 2020

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Aegerion Pharmaceuticals, Inc.
Consolidated Balance Sheets
(in thousands)

	December 31,
	2018	2017
Assets
Current assets:
Cash and cash equivalents	$31,881	$14,307
Accounts receivable, net	28,912	22,191
Inventories - current	12,745	15,886
Prepaid expenses and other current assets	15,292	10,499
Total current assets	88,830	62,883
Inventories - non-current	36,202	33,940
Property and equipment, net	1,397	2,572
Intangible assets, net	200,176	225,272
Other non-current assets	1,209	2,247
Total assets	$327,814	$326,914
Liabilities and shareholders’ deficit
Current liabilities:
Accounts payable	$4,995	$13,182
Accrued liabilities	42,356	36,197
Payable due to Novelion	11,003	—
Short-term debt	73,677	—
Short-term debt due to Novelion	37,264	—
Convertible notes, net	274,815	—
Provision for legal settlements - current	11,689	8,596
Total current liabilities	455,799	57,975
Convertible notes, net	—	258,538
Long-term debt due to Novelion	—	23,500
Provision for legal settlements - non-current	19,391	31,016
Payable due to Novelion	—	4,760
Other non-current liabilities	795	595
Total liabilities	475,985	376,384
Commitments and contingencies (Note 15)
Shareholders’ equity:
Common shares, without par value, 30,301 shares issued and outstanding at December 31, 2018 and 2017, respectively	—	—
Additional paid-in-capital	59,381	59,381
Accumulated deficit	(206,217)	(109,679)
Accumulated other comprehensive (loss) income	(1,335)	828
Total shareholders’ deficit	(148,171)	(49,470)
Total liabilities and shareholders’ deficit	$327,814	$326,914

See accompanying Notes to Consolidated Financial Statements.

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Aegerion Pharmaceuticals, Inc.
Consolidated Statements of Operations
(in thousands)

	Year Ended December 31,
	2018	2017
Net revenues	$130,432	$138,438
Cost of product sales	59,697	77,220
Operating expenses:
Selling, general and administrative	64,437	77,793
Research and development	38,064	44,895
Restructuring charges	2,171	121
Related party expenses (income), net	942	(177)
Total operating expenses	105,614	122,632
Loss from operations	(34,879)	(61,414)
Interest expense, net	(50,746)	(39,467)
Interest expense due to Novelion	(2,987)	(1,089)
Loss on extinguishment of debt	(4,333)	—
Other expense, net	(1,888)	(836)
Loss before provision for income taxes	(94,833)	(102,806)
Provision for income taxes	(1,705)	(594)
Net loss	$(96,538)	$(103,400)

See accompanying Notes to Consolidated Financial Statements.

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Aegerion Pharmaceuticals, Inc.
Consolidated Statements of Comprehensive Loss
(in thousands)

	Year Ended December 31,
	2018	2017
Net loss	$(96,538)	$(103,400)
Other comprehensive (loss) income:
Foreign currency translation	(2,163)	1,209
Other comprehensive (loss) income	(2,163)	1,209
Comprehensive loss	$(98,701)	$(102,191)

See accompanying Notes to Consolidated Financial Statements.

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Aegerion Pharmaceuticals, Inc.
Consolidated Statements of Shareholders’ Equity (Deficit)
(in thousands, except share information)

	Common Shares		Additional Paid-In Capital	Accumulated Deficit	Accumulated Other Comprehensive (Loss) Income	Total Shareholders Equity (Deficit)
	Shares	Amount
Balance at January 1, 2017	30,301,444	$—	$59,381	$(6,279)	$(381)	$52,721
Net loss	—	—	—	(103,400)	—	(103,400)
Foreign currency translation adjustment	—	—	—	—	1,209	1,209
Balance at December 31, 2017	30,301,444	—	59,381	(109,679)	828	(49,470)
Net loss	—	—	—	(96,538)	—	(96,538)
Foreign currency translation adjustment	—	—	—	—	(2,163)	(2,163)
Balance at December 31, 2018	30,301,444	$—	$59,381	$(206,217)	$(1,335)	$(148,171)

See accompanying Notes to Consolidated Financial Statements.

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Aegerion Pharmaceuticals, Inc.
Consolidated Statements of Cash Flows
(in thousands)

	Year Ended December 31,
Cash used in operating activities	2018	2017
Net loss	$(96,538)	(103,400)
Adjustments to reconcile net loss to net cash used in operating activities:
Depreciation	1,577	1,865
Amortization of intangible assets	25,091	25,052
Stock-based compensation	1,465	2,145
Non-cash interest expense	39,670	32,954
Non-cash interest expense due to Novelion	2,987	1,089
Provision for inventory excess and obsolescence	2,062	18,814
Unrealized foreign exchange loss (gain)	2,908	(818)
Amortization of debt issuance costs and debt discount	3,251	—
Deferred income taxes	858	138
Other non-cash operating activities	(13)	24
Loss on extinguishment of Shareholder Term Loans	4,025	—
Changes in assets and liabilities:
Accounts receivable	(6,745)	(13,032)
Inventories	(1,700)	6,081
Prepaid expenses and other assets	(4,876)	19,282
Accounts payable	(10,517)	(207)
Payable due to Novelion	448	(406)
Accrued and other liabilities	(2,715)	(23,970)
Net cash used in operating activities	(38,762)	(34,389)
Cash used in investing activities
Purchase of property and equipment	(442)	(372)
Net cash used in investing activities	(442)	(372)
Cash provided by financing activities
Net proceeds from Bridge Loans	70,000	—
Net proceeds from Novelion Loan	15,000	22,000
Repayment of Shareholder Term Loans	(20,000)	—
Repayment of Novelion Loan	(3,500)	—
Payment of debt issuance costs	(2,966)	—
Net cash provided by financing activities	58,534	22,000
Exchange rate effect on cash	(1,756)	2,032
Net increase (decrease) in cash and cash equivalents	17,574	(10,729)
Cash and cash equivalents, beginning of period	14,307	25,036
Cash and cash equivalents, end of period	$31,881	14,307
Supplemental disclosures of cash flow information
Cash paid for interest	$8,182	$6,514
Cash paid for taxes, net	$42	$1,671
Non-cash investing activities
Purchases of property and equipment included in accounts payable	$10	$122
Non-cash financing activities
Refinance from Roll Up Loans	$22,500	$—
Retirement of Convertible Notes	$(22,500)	$—

See accompanying Notes to Consolidated Financial Statements.

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Aegerion Pharmaceuticals, Inc.
Notes to Consolidated Financial Statements

1. Description of Business

Organization

Aegerion Pharmaceuticals, Inc. (“Aegerion” or the “Company”) is a rare disease biopharmaceutical company dedicated to developing new standards of care for individuals living with rare diseases. The Company is a private company incorporated in the state of Delaware in the United States (“U.S.”) and has international operations and two commercial products, metreleptin and lomitapide. Metreleptin, a recombinant analog of human leptin, is currently marketed in the U.S. under the brand name MYALEPT (metreleptin for injection). MYALEPT is approved in the U.S. as an adjunct to diet as replacement therapy to treat the complications of leptin deficiency in patients with congenital or acquired generalized lipodystrophy (“GL”). In July 2018, metreleptin, under the brand name MYALEPTA, was approved in the European Union (“EU”) as a treatment for the complications of leptin deficiency in patients with congenital or acquired GL in adults and children two years of age and above and familial or acquired Partial Lipodystrophy (“PL”) in adults and children 12 years of age and above for whom standard treatments have failed to achieve adequate metabolic control. Lomitapide, which is marketed in the U.S. under the brand name JUXTAPID (lomitapide) capsules (“JUXTAPID”), is approved in the U.S. as an adjunct to a low-fat diet and other lipid-lowering treatments, including low-density lipoprotein (“LDL”) apheresis where available, to reduce low-density lipoprotein cholesterol (“LDL-C”), total cholesterol (“TC”), apolipoprotein B (“apo B”) and non-high-density lipoprotein cholesterol (“non-HDL-C”) in adult patients with homozygous familial hypercholesterolemia (“HoFH”). Lomitapide is approved in the EU, under the brand name LOJUXTA (lomitapide) hard capsules (“LOJUXTA”) for the treatment of adult patients with HoFH, where it is commercialized by Aegerion’s licensee, Amryt Pharma plc (“Amryt”). In December 2016, Aegerion launched JUXTAPID as a treatment for HoFH in Japan and on February 5, 2019, Aegerion entered into a license agreement with Recordati Rare Diseases Inc. (“Recordati”) for the commercialization of JUXTAPID in Japan. Additionally, both metreleptin and lomitapide are sold, on a named patient basis, in certain countries outside of the U.S., such as Brazil, where such sales are permitted based on the approval of metreleptin and lomitapide in the U.S. or EU.

Merger with Amryt

As discussed in Note 16, Subsequent Events, on May 20, 2019, Aegerion and its U.S. subsidiary, Aegerion Pharmaceuticals Holdings, Inc., each was previously a subsidiary of Novelion Therapeutics Inc. (“Novelion”), filed voluntary petitions under chapter 11 (“Chapter 11”) of Title 11 of the U.S. Code (“Bankruptcy Code”) in the U.S. Bankruptcy Court for the Southern District of New York (“Bankruptcy Court”). In addition, on May 20, 2019, Aegerion Pharmaceuticals, Inc. and Aegerion Pharmaceuticals Holdings, Inc. entered into a Plan Funding Agreement (“Plan Funding Agreement”) with Amryt to set forth the terms and conditions of the acquisition by Amryt of 100 percent of the outstanding equity interests of the reorganized Aegerion Pharmaceuticals, Inc. (“Recognized Debtors”) (“Amryt Transaction”).

On September 24, 2019, Aegerion completed the Amryt Transaction (“Closing”). The consideration for the Amryt Transaction has been satisfied through the issuance of ordinary Amryt shares (“Ordinary Shares”), or American depositary receipts representing Ordinary Shares, to stakeholders of Aegerion. Through the Amryt Transaction, Novelion has been divested of its operating subsidiary, Aegerion. Refer to Note 16, Subsequent Events, for further discussion related to the completion of the Amryt Transaction.

Going Concern

The accompanying Consolidated Financial Statements have been prepared assuming Aegerion will continue to operate as a going concern, which contemplates the realization of assets and the settlement of liabilities and commitments in the normal course of business. As of December 31, 2018, Aegerion has significant indebtedness of $475.9 million, and the level of indebtedness has adversely impacted Aegerion’s financial condition. In addition, the filing of the Chapter 11 cases in May 2019 constituted an event of default with respect to certain of Aegerion’s existing debt obligations. As a result, Aegerion’s financial condition and the risks and uncertainties surrounding its Chapter 11 proceedings raise substantial doubt as to Aegerion’s ability to continue as a going concern.

As discussed above and in Note 16, Subsequent Events, the Bankruptcy Court entered an order (“Confirmation Order”) confirming Aegerion’s First Amended Joint Chapter 11 Plan (“Plan”), as modified to reflect certain

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resolutions agreed to among various parties. As result of the Plan, a significant amount of the Company’s indebtedness was discharged. Additionally, as discussed above, Aegerion was acquired by Amryt. Refer to Note 16, Subsequent Events, for further discussion on the indebtedness discharge and the Amryt Transaction.

As of December 31, 2019, Aegerion’s unrestricted cash balance is approximately $17.4 million. The Company believes that its existing funds would not be sufficient to satisfy its operating needs and its working capital for at least the next twelve months from the issuance of the Consolidated Financial Statements. Aegerion may, from time to time, need to seek additional funding from Amryt, its parent company. Should the Company be unable to receive funding from its parent company, the Company’s business, results of operations, liquidity and financial condition would be materially and negatively affected, and the Company would be unable to continue as a going concern.

2. Summary of Significant Accounting Principles

Basis of Presentation and Principles of Consolidation

The accompanying Consolidated Financial Statements have been prepared in accordance with accounting principles generally accepted in the United States of America (“GAAP”). All amounts herein are expressed in U.S. dollars (“USD”) unless otherwise noted.

The accompanying Consolidated Financial Statements include operations of Aegerion and its wholly-owned subsidiaries. All intercompany transactions and balances have been eliminated. Additionally, the Company engages in various transactions with its former parent company, Novelion, and its affiliates, including the term loan agreement and the shared service agreement. Refer to Note 10, Related Party Transactions, for further discussions. Accordingly, the accompanying Consolidated Financial Statements have been prepared from the separate records maintained by the Company and may not necessarily be indicative of the conditions that would have existed or the results of operations if the Company had been operated as an unaffiliated company.

Use of Estimates

The preparation of Consolidated Financial Statements in accordance with GAAP requires management to make estimates and assumptions that affect the reported amounts of assets and liabilities, the disclosure of contingent assets and liabilities at the date of the Consolidated Financial Statements and the reported amounts of revenues and expenses during the reporting periods presented. The Company’s estimates often are based on complex judgments, probabilities and assumptions that the Company believes to be reasonable but that are inherently uncertain and unpredictable. For any given individual estimate or assumption made by management, there may also be other estimates or assumptions that are reasonable. Actual results may differ from estimates made by management. Changes in estimates are reflected in reported results in the period in which they become known.

Reporting and Functional Currency

The Company’s reporting currency is the USD and its operations utilize the USD or local currency as the functional currency, where applicable.

Transactions in other currencies are recorded in the functional currency at the rate of exchange prevailing when the transactions occur. Monetary assets and liabilities denominated in other currencies are re-measured into the functional currency at the rate of exchange in effect at the balance sheet date. Exchange gains and losses arising from re-measurement of foreign currency-denominated monetary assets and liabilities are included in income in the period in which they occur.

For foreign entities where the local currency is the functional currency, assets and liabilities denominated in local currencies are translated into USD at end-of-period exchange rates and the resulting translation adjustments are reported as a component of accumulated other comprehensive income (loss) within shareholders’ deficit.

Cash and Cash Equivalents

Cash and cash equivalents consist of highly liquid instruments purchased with an original maturity of three months or less at the date of purchase.

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Accounts Receivable, net

The majority of the Company’s accounts receivable arise from product sales and primarily represents amounts due from distributors, named patients and other entities. The Company monitors the financial performance and creditworthiness of its customers to properly assess and respond to changes in their credit profiles, and provides reserves against accounts receivable for estimated losses that may result from a customer’s inability to pay. Amounts determined to be uncollectible are charged or written-off against the reserve. To date, the Company’s historical reserves and write-offs of accounts receivable have not been material.

Inventories and Cost of Product Sales

Inventories are stated at the lower of cost or net realizable value with cost determined on a first-in, first-out basis.

Inventory is maintained on the Company’s Consolidated Balance Sheets until the inventory is sold, donated as part of the Company’s compassionate use program, or used for clinical development. Inventory that is sold is recognized as cost of product sales in the Consolidated Statements of Operations, inventory that is donated as part of the Company’s compassionate use program is recognized as a selling, general and administrative (“SG&A”) expense in the Consolidated Statements of Operations, expired inventory is disposed of and the related costs are recognized as cost of product sales in the Consolidated Statements of Operations and inventory used for clinical development is recognized as research and development (“R&D”) expense in the Consolidated Statements of Operations.

Inventories are reviewed periodically to identify slow-moving inventory based on sales activity, both projected and historical, as well as product shelf-life. The portion of the slow-moving inventory not expected to be sold within one year is classified as non-current inventory in the Company's accompanying Consolidated Balance Sheets. At each balance sheet date, the Company evaluates its ending inventories for excess quantities and obsolescence. This evaluation includes an analysis of sales levels by product type, the expiration date of the Company’s finished goods on-hand at each balance sheet date, as well as the anticipated unit forecast for demand for each product. Any changes to the demand of either product may result in a reserve for excess and obsolescence if the Company believes inventory will expire prior to being commercially sold, and such charge will be recorded to cost of product sales.

If the asset becomes impaired or is abandoned, the carrying value is written down to its net realizable value, and an impairment charge is recorded in the period in which the impairment occurs. In evaluating the recoverability of inventories produced, the Company considers the probability that revenue will be obtained from the future sale of the related inventory.

Cost of product sales includes the cost of inventory sold, manufacturing and supply chain costs, product shipping and handling costs, charges for excess and obsolete inventory, amortization of acquired intangibles, as well as royalties payable to Amgen Inc. (“Amgen”), Rockefeller University and Bristol-Myers Squibb (“BMS”) related to the sale of metreleptin and royalties payable to The Trustees of the University of Pennsylvania (“UPenn”) related to the sale of lomitapide.

Prepaid Manufacturing Costs

Cash advances paid by the Company prior to receipt of inventory are recorded as prepaid manufacturing costs and included in prepaid expenses and other current assets on the Consolidated Balance Sheets. The cash advances are subject to forfeiture if the Company terminates the scheduled production. The Company expects the carrying value of the prepaid manufacturing costs to be fully realized. As of December 31, 2018 and 2017, $3.8 million and $4.1 million was recorded as prepaid manufacturing costs, respectively.

Property and Equipment

Property and equipment are stated at cost and depreciated using the straight-line method based on estimated economic lives of three to five years for office furniture, fixtures, research equipment and other equipment, and three years for computer software and hardware. Leasehold improvements are amortized over the shorter of the estimated useful lives of the improvements or the remaining lease term, which include lease extensions when reasonably assured. Repair and maintenance costs are expensed as incurred.

Intangible Assets

Intangible assets with definite useful lives are amortized, on a straight-line basis, to their estimated residual values over their estimated useful lives and reviewed for impairment if certain triggering events occur.

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Impairment of Long-lived Assets

Impairment testing and assessments of remaining useful lives are performed when a triggering event occurs that could indicate a potential impairment or change in useful life. Such testing first entails comparison of the carrying value of the long-lived asset to the undiscounted cash flows expected from that asset. If impairment is indicated by this test, the long-lived assets are written down by the amount, if any, by which the carrying value of the long-lived asset exceeds its estimated discounted cash flows.

Contingencies

The Company records a liability in the Consolidated Financial Statements for litigation related matters when a loss is considered probable and the amount can be reasonably estimated. If the loss is not probable or a range cannot reasonably be estimated, no liability is recorded in the Consolidated Financial Statements. Legal fees are expensed as incurred. Insurance recoveries related to potential claims are recognized up to the amount of the recorded liability when coverage is confirmed and the estimated recoveries are probable of payment. These recoveries are not netted against the related liabilities for financial statement presentation.

Revenue Recognition

The Company adopted Accounting Standards Codification (“ASC”) Topic 606, Revenue from Contracts with Customers (“ASC Topic 606”), effective January 1, 2018. Prior to January 1, 2018, the Company applied the revenue recognition guidance in accordance with Financial Accounting Standards Board (“FASB”) ASC Subtopic No. 605-15, Revenue Recognition-Products (“ASC 605”). The adoption of ASC Topic 606 did not materially change the Company’s revenue recognition and recognition of cost of product sales. As the Company did not identify any accounting changes that impacted the amount of net revenues, no adjustment to retained earnings was required upon adoption.

Prior to the second quarter of 2017, due to insufficient historical data to reasonably estimate the gross-to-net adjustments for rebates related to payers and insurance providers at the time of receipt by the Company’s distributor for MYALEPT in the U.S., the Company accounted for MYALEPT shipments using a deferred revenue recognition model (sell-through method). Beginning in the second quarter of 2017, the Company determined that there was sufficient history to reasonably estimate expected rebates, and, to align its existing and anticipated revenue streams of products sold within the U.S., began recognizing sales of MYALEPT upon title transfer to distributors (sell-in method). Accordingly, the Company recognized a one-time increase in net revenue of $2.3 million resulting from this change in estimate in 2017, representing previously deferred product sales.

Additionally, in the second quarter of 2017, to improve distribution efficiency, the Company signed a letter of intent for the distribution of JUXTAPID with the same specialty pharmacy that distributes MYALEPT in the U.S. The agreement was finalized in October 2017, and the transition of this distribution model was completed in November 2017. Prior to the transition, the specialty pharmacy that distributed JUXTAPID did not take title to JUXTAPID; title was transferred upon delivery of JUXTAPID to the patients (sell-through model), and revenue was recognized upon the delivery to the patients, which is consistent with the accounting guidance under ASC Topic 606. Subsequent to completion of the transition, revenue from sales of JUXTAPID in the U.S. has been recognized upon title transfer to distributors (sell-in method) under ASC 605.

The Company’s net revenues have primarily been derived from product sales; the Company’s remaining revenues are derived from the royalties on product sales made by its sublicensees in the European Union and other territories. The following summarizes the revenue recognition for the respective revenue streams.

Product Sales Revenues

The Company recognizes revenue from sales of metreleptin and lomitapide at the point in time when control transfers, typically upon transfer of product to the carrier or delivery of product to customers. Revenue is recognized net of estimated discounts, rebates and any taxes collected from customers which are subsequently remitted to governmental authorities. Payment terms vary by contract, but it is typically due within 30 to 120 days of delivery to the customer. Generally, the period between when the Company transfers or delivers the products and when payments are received is in one year or less, as such, the Company deems it unnecessary to assess whether a significant financing component exists and thus does not adjust the transaction price for the time value of money.

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Variable Consideration

Product sales revenues are recognized at the net sales price (“transaction price”) which includes estimated reserves for variable consideration, upon the transfer of control of the Company’s products. Variable consideration primarily includes government rebates, prompt payment discounts and distribution service fees. Estimates of variable consideration are made at contract inception and based on historical experience, market trends, industry data and statutory requirements are considered when determining such estimates. Variable consideration is included in the transaction price to the extent it is probable that a significant reversal of revenue will not occur. The Company reassesses variable consideration at the end of each reporting period as additional information becomes available with the variance recorded to product sales revenue.

Government Rebates: The Company is subject to government mandated rebates for Medicare, Medicaid, Tricare and other government programs in the U.S. and other countries. These rebates are estimated based on actual payer information. The Company records an accrued liability for unpaid rebates related to products for which control has been transferred to distributors. Refer to Note 6, Accrued Liabilities, for further discussion of the change in the government rebates for metreleptin and lomitapide.

Prompt Payment Discounts: The Company provides discounts to certain distributors if they pay for product within a defined period of time after title transfers, which terms are explicitly stated in the contract. These discounts are recorded as a reduction of revenue upon receipt of full payment from such distributors.

Distributor Service Fees: Certain distributors provide distribution services to the Company for a fee, and the costs associated with these services are generally recorded as a reduction of revenue.

Other Incentives: The Company offers other incentives that vary by contract; these incentives take into account specific relevant factors and are analyzed for revenue recognition purposes on a case by case basis.

Other Revenues

The Company has entered into agreements where the Company licenses certain rights to its products to sublicensees and earns royalties from product sales made by the sublicensees and milestone payments upon the achievement of certain levels of sales. Under ASC Topic 606, the Company recognizes royalty revenue and sales-related milestone payments, when applicable, at the later of (1) the time that the subsequent sale or usage occurs, or (2) the performance obligation to which some or all of the sales-based or usage-based royalty has been allocated has been satisfied (or partially satisfied).

Research and Development Expenses

R&D expenses are charged to expense as incurred, and they comprise costs incurred in performing research and development activities, including personnel-related costs, stock-based compensation, facilities-related overhead, clinical trial costs, costs to support certain medical affairs activities, manufacturing costs for clinical and preclinical materials as well as other contracted services, license fees and other external costs. Nonrefundable advance payments for goods and services that will be used in future research and development activities are expensed when the activity has been performed or when the goods have been received rather than when the payment is made.

Income Taxes

Income taxes are recorded using an asset and liability approach. The Company recognizes deferred tax assets and liabilities for the expected future tax consequences of events that have been included in the financial statements or tax returns. Deferred tax assets and liabilities are determined based on the difference between the financial statement and tax bases of assets and liabilities using enacted tax rates in effect for the year in which the differences are expected to reverse. Valuation allowances are provided, if based upon the weight of available evidence, it is more likely than not that some or all of the deferred tax assets will not be realized.

When uncertain tax positions exist, the Company recognizes the tax benefit of tax positions to the extent that the benefit will more likely than not be realized. The determination as to whether the tax benefit will more likely than not be realized is based upon the technical merits of the tax position as well as consideration of the available facts and circumstances. The Company accrues for potential interest and penalties related to unrecognized tax benefits and includes such charges in income tax expense. Additionally, the Company calculated income tax amounts from separate records maintained by the Company as if the Company had not been included in a consolidated income tax return with its former parent.

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Stock-Based Compensation

For service-based awards, compensation expense is measured at the grant date based on the fair value of the award and is recognized on a straight-line basis over the requisite service period, which is typically the vesting period. For awards that vest upon the achievement of a market condition, the Company recognizes compensation expense over the derived service period. For equity awards that have been modified and result in the change of the fair value, the vesting conditions or the classification of the award, any incremental increase in the fair value over the original award has been recorded as compensation expense on the date of the modification for vested awards or over the remaining service period for unvested awards.

The restricted stock units (“RSUs”) are issued to employees as consideration for their provision of future services. Restricted stock-based compensation expense is measured based on the fair value market price of Novelion’s common shares on the grant date and is recognized over the requisite service period, which coincides with the vesting period. RSUs can only be exchanged and settled for Novelion’s common shares, on a one-to-one basis, upon vesting.

As the common shares issued to Aegerion’s employees are indexed to and will be settled in Novelion’s equity, and Aegerion will reimburse its parent for the equity awards in cash, Aegerion recognizes a liability to the parent for the related stock-based compensation expense.

Comprehensive Loss

Comprehensive loss combines net loss and other comprehensive items. Other comprehensive items represent certain amounts that are reported as components of shareholders’ deficit in the accompanying Consolidated Balance Sheets, including foreign currency translation adjustments.

Recently Adopted Accounting Standards

Effective January 1, 2018, the Company adopted Accounting Standards Update (“ASU”) No. 2014-09, Revenue from Contracts with Customers (“ASU 2014-09”) and related ASUs, using the modified retrospective method. ASU 2014-09 represents a comprehensive new revenue recognition model that requires a company to recognize revenue to depict the transfer of promised goods or services to customers in an amount that reflects the consideration to which a company expects to be entitled in exchange for those goods or services. This ASU sets forth a new five-step revenue recognition model which replaces most existing revenue recognition guidance including industry-specific guidance. The adoption of ASU 2014-09 and the related ASUs did not materially change the Company’s revenue recognition and recognition of cost of product sales. As the Company did not identify any accounting changes that impacted the amount of net revenues, no adjustment to retained earnings was required upon adoption. Refer to the “Revenue Recognition” section above for the required disclosures and a discussion of the Company’s policies related to revenue recognition.

Recent Accounting Pronouncements Not Yet Adopted

In 2016, the FASB issued ASU No. 2016-02, Leases (“ASU 2016-02”), which amends a number of aspects of lease accounting and requires entities to recognize right-of-use assets and liabilities on the balance sheet for leases. In July 2018, the FASB issued ASU No. 2018-11, Leases (Topic 842): Targeted Improvements (“ASU 2018-11”), which offers a transition option to entities adopting ASC 842. Under ASU 2018-11, entities can elect to apply ASC 842 using a modified-retrospective adoption approach resulting in a cumulative effect adjustment to retained earnings/(accumulated deficit) at the beginning of the year in which the new lease standard is adopted, rather than adjustments to the earliest comparative period presented in their financial statements.

The Company elected to early adopt ASU 2016-02 effective January 1, 2019, using the modified retrospective method.

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3. Inventories

The components of inventories are as follows:

	December 31,
	2018	2017
	(in thousands)
Work-in-process	$26,676	$22,579
Finished goods	22,271	27,247
Total	48,947	49,826
Less: Inventories - current	(12,745)	(15,886)
Inventories - non-current	$36,202	$33,940

Non-current inventories primarily consist of the active pharmaceutical ingredients which do not expire. Additionally, a portion of finished goods is classified as non-current as of December 31, 2018 and 2017 based on forecasted consumption exceeding one year. During the years ended December 31, 2018 and 2017, the Company recorded charges to cost of product sales in the amounts of $2.1 million and $18.8 million, respectively, for excess and obsolete inventory in its Consolidated Statements of Operations. The amounts of the charges recorded were based on a review of forecasted sales activity, remaining product shelf-life and estimated fair value of the inventory.

4. Property and Equipment, net

Property and equipment, net consist of the following:

	December 31,
	2018	2017
	(in thousands)
Leasehold improvements	$1,730	$1,686
Office furniture and equipment	798	810
Computer and office equipment	2,482	1,975
Construction in progress	—	123
Property and equipment, at cost	5,010	4,594
Less accumulated depreciation	(3,613)	(2,022)
Property and equipment, net	$1,397	$2,572

Depreciation expense was $1.6 million and $1.9 million for the years ended December 31, 2018 and 2017, respectively.

5. Intangible Assets

The intangible assets are amortized over their estimated useful lives, which are the remaining patent lives of approximately 8 - 9 years, and reviewed for impairment when events and changes in circumstances indicate that the carrying amount may not be recoverable. In the fourth quarter of 2018, as a result of the decline in the stock price of Novelion, Aegerion’s then-parent company, it was determined that a recoverability test was required. Based on the sum of the undiscounted cash flows of the related intangible asset groups, the Company concluded that the carrying amount of the intangible assets was recoverable, and there was no impairment charge recorded during the year ended December 31, 2018. There was also no impairment charge recorded during the year ended December 31, 2017. Additionally, the Company reviewed the useful lives of the intangibles as of December 31, 2018 and believes the useful lives are still reasonable.

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Intangible asset balances as of December 31, 2018 and 2017 are as follows:

	December 31, 2018
	Gross Carrying Value	Accumulated Amortization	Net Carrying Value
	(in thousands)
Developed technology - metreleptin	$210,158	$(44,084)	$166,074
Developed technology - lomitapide	42,300	(8,198)	34,102
Total intangible assets	$252,458	$(52,282)	$200,176

	December 31, 2017
	Gross Carrying Value	Accumulated Amortization	Net Carrying Value
	(in thousands)
Developed technology - metreleptin	$210,158	$(22,924)	$187,234
Developed technology - lomitapide	42,300	(4,262)	38,038
Total intangible assets	$252,458	$(27,186)	$225,272

Amortization expense was $25.1 million for each of the years ended December 31, 2018 and 2017.

As of December 31, 2018, the estimated amortization expense related to these intangibles for future periods is as follows:

	Amount
Years Ending December 31,	(in thousands)
2019	$25,095
2020	25,095
2021	25,095
2022	25,095
2023	25,095
Thereafter	74,701
Total intangible assets subject to amortization	$200,176

6. Accrued Liabilities

Accrued liabilities as of December 31, 2018 and 2017 consist of the following:

	December 31,
	2018	2017
	(in thousands)
Accrued employee compensation and related costs	$2,108	$5,182
Accrued professional fees	592	1,952
Accrued allowances: government rebates	19,637	13,471
Accrued royalties	5,112	3,588
Other accrued liabilities	14,907	12,004
Total	$42,356	$36,197

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The following table summarizes combined activity for the allowances for the government rebates incurred in connection with the product sales of MYALEPT and JUXTAPID for the years ended December 31, 2018 and 2017.

	December 31,
	2018	2017
	(in thousands)
Beginning balance	$13,471	$7,849
Provision	24,646	23,087
Payments	(18,375)	(17,465)
Other adjustments	(105)	—
Ending balance	$19,637	$13,471

7. Restructuring

In August 2018, Novelion’s Board of Directors approved a cost-reduction plan which included a workforce reduction. The reduction in workforce impacted 35 employees, or approximately 22% of the Company’s global active employees, across nearly all functions, and was substantially completed in the third quarter of 2018. As a result of the workforce reduction, the Company incurred restructuring charges of approximately $2.2 million in the year ended December 31, 2018, which was fully paid by the third quarter of 2019. The restructuring charges include consulting fees related to the restructuring event and termination benefits, principally comprised of severance payments.

During the year ended December 31, 2017, the Company incurred approximately $0.1 million in restructuring charges related to the consolidation of similar positions during the integration of the business subsequent to the merger with Novelion. The restructuring charges consisted primarily of severance and benefits costs.

The following table sets forth the components of the restructuring charges and payments made related to restructuring activities.

	December 31,
	2018	2017
	(in thousands)
Beginning balance	$10	$118
Costs incurred	2,171	121
Payments	(1,813)	(229)
Other adjustments	(9)	—
Ending balance	$359	$10

8. Loan Facilities

Short-term debt, exclusive of convertible notes consists of the following as of December 31, 2018:

	December 31, 2018
	New Money Loans	Roll Up Loans	Novelion Loan	Total
	(in thousands)
Short-term principal and commitment fee	$51,000	$22,500	$37,777	$111,277
Exit fee payable	1,500	—	—	1,500
Accrued unpaid interest	826	66	2,987	3,879
Unamortized debt issuance costs	(1,054)	—	—	(1,054)
Debt discount	(1,161)	—	—	(1,161)
Repayment	—	—	(3,500)	(3,500)
Total short-term debt	$51,111	$22,566	$37,264	$110,941

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Long-term debt, exclusive of convertible notes consists of the following as of December 31, 2017:

	December 31,
	Novelion Loan
	(in thousands)
Principal	$22,411
Accrued unpaid interest	1,089
Total short-term debt	$23,500

Bridge Loans

On November 8, 2018, Aegerion entered into a bridge credit agreement (“Bridge Credit Agreement”) with certain funds managed by Highbridge Capital Management, who are investors in Novelion’s common shares, and Athyrium Capital Management, as lenders (“Bridge Lenders”), and Cantor Fitzgerald Securities, as agent (“Bridge Agent”), under which Aegerion borrowed from the Bridge Lenders new secured first lien term loans in cash in an original aggregate principal amount of $50.0 million (“New Money Loans”) and $22.5 million of new secured term loans that were funded, on behalf of Aegerion, to repurchase and retire an equal amount of Convertible Notes, at par, held by certain funds managed by the Bridge Lenders (“Roll Up Loans”). The Roll Up Loans and the New Money Loans comprise the Bridge Loans referenced above.

The Bridge Loans mature on the earliest to occur of (i) certain restructuring or bankruptcy events, (ii) June 30, 2019, and (iii) the acceleration after occurrence of an event of default. Refer to Note 16, Subsequent Events, for further discussion related to Aegerion’s bankruptcy filing in May 2019 and the related impact to the Bridge Loans.

In January 2019, the maturity date of the Bridge Loans was extended to June 30, 2019, upon the exercise of Aegerion’s option and the satisfaction by Aegerion of the conditions stated in the Bridge Credit Agreement. In connection with the extension of the maturity date, Aegerion repaid $3.0 million of New Money Loans principal, including exit fee, and paid an extension fee in the amount of $1.5 million to the Bridge Lenders.

The New Money Loans accrue interest at the rate of 11.00% per annum and the Roll Up Loans accrue interest at the rate of 2.00% per annum. Following an event of default and so long as an event of default is continuing, the interest rate on each of the New Money Loans and the Roll Up Loans would increase by 2.00% per annum. Interest on the New Money Loans and the Roll Up Loans accrue and compound quarterly in arrears and will not be payable in cash until the maturity date or any earlier time that the Bridge Loans become due and payable under the Bridge Credit Agreement. Aegerion incurred a commitment fee equal to 2.00% of the New Money Loans, which will be paid in kind and is included in the outstanding principal amount of the New Money Loans. The New Money Loans may be prepaid, in whole or in part, by Aegerion at any time subject to payment of an exit fee (including at maturity) equal to 3.00% of the commitments with respect to New Money Loans.

Aegerion’s obligations under the Bridge Credit Agreement are guaranteed by each domestic subsidiary of Aegerion other than Aegerion Securities Corporation, a Massachusetts corporation (“Guarantors”), and secured by a lien on substantially all of the assets of Aegerion and the Guarantors, including a pledge of 65% of Aegerion’s and the Guarantors’ first-tier foreign subsidiaries’ equity interests and substantially all of the intellectual property and related rights in respect of MYALEPT and JUXTAPID, subject to certain contractual limitations and exclusions set forth in the Bridge Credit Agreement and related documentation. The liens on the assets of Aegerion and the Guarantors granted to secure Aegerion’s obligations to the Bridge Lenders with respect to New Money Loans are senior to the liens granted to secure Aegerion’s obligations to Novelion with respect to the Novelion Loan. The liens on the assets of Aegerion and the Guarantors granted to secure Aegerion’s obligations to the Bridge Lenders with respect to Roll Up Loans are junior to the liens granted to secure Aegerion’s obligations to Novelion with respect to the Novelion Loan. Upon consummation of certain restructuring transactions consented to by the Bridge Lenders in their discretion, the liens securing the Roll Up Loans will be terminated and released, and the Roll Up Loans will be treated as unsecured obligations of Aegerion, pari passu with the other obligations of Aegerion with respect to the Convertible Notes.

The Bridge Credit Agreement includes affirmative and negative covenants binding on Aegerion and its subsidiaries, including prohibitions on the incurrence of additional indebtedness, granting of liens, certain asset dispositions, investments and restricted payments, in each case, subject to certain exceptions set forth in the

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Bridge Credit Agreement. The Bridge Credit Agreement also includes customary events of default for a transaction of this type, and includes (i) a cross-default to the occurrence of any event of default under material indebtedness of Aegerion, including the Convertible Notes or the Novelion Loan, and (ii) Novelion or any of its subsidiaries being subject to bankruptcy or other insolvency proceedings. Upon the occurrence of an event of default, the Bridge Lenders may declare all of the outstanding Bridge Loans and other obligations under the Bridge Credit Agreement to be immediately due and payable and exercise all rights and remedies available to the Bridge Lenders under the Bridge Credit Agreement and related documentation.

Pursuant to the terms of the Bridge Credit Agreement and after taking into account amounts payable to UPenn, as discussed in Note 16, Subsequent Events, Aegerion retained $15.0 million of the remaining upfront payment, of which $12.0 million is to be used in accordance with a proceeds reinvestment budget (which primarily relates to the development activities related to MYALEPT as a potential treatment for PL in the U.S.), and the balance is to be used in accordance with a general budget of Aegerion, in each case, subject to certain restrictions. Forty-two percent of the remaining net cash proceeds (less amounts owed to UPenn and amounts paid as transaction costs) was paid to Novelion to repay a portion of the outstanding Novelion Loan comprising the portion of the loan that the Bridge Lenders agreed would not be contested and 58% was paid to the Bridge Lenders to repay a portion of the outstanding Bridge Loans.

In connection with the Amended and Restated Novelion Loan Agreement (defined below) and the Bridge Credit Agreement, Aegerion was required to enter into separate deposit account control agreements with each of the lenders in order to perfect each lender’s security interest in the cash collateral in Aegerion’s operating account. In the event of a default under either loan agreement, subject to the terms of the subordination agreement with the Bridge Lenders and Bridge Agent (“Bridge Intercreditor Agreement”), the respective lender would have the right to take control of the operating account and restrict Aegerion’s access to the operating account and the funds therein.

In addition to the repurchase and cancellation of certain Convertible Notes with the proceeds of the Roll Up Loans, Aegerion used proceeds of the New Money Loans to repay, at par, (a) the amounts outstanding under the Shareholder Term Loan Agreement described below, in an aggregate principal amount of approximately $21.2 million, and (b) principal prepayments of the Novelion Loan in an amount of $3.5 million, in 2018.

Shareholder Term Loan Agreement

On March 15, 2018, Aegerion entered into a loan and security agreement with affiliates of Broadfin Capital, LLC and Sarissa Capital Management LP (“Shareholder Term Loan Agreement”), pursuant to which the lenders made a single-draw term loan to Aegerion in an aggregate amount of $20.0 million (“Shareholder Term Loans”), and secured by substantially all of Aegerion’s assets. The lenders or their affiliates were Novelion’s Shareholders given their investments in Novelion’s common shares, and two members of Novelion’s Board of Directors at that time were affiliates of the lenders.

The Company accrued unpaid interest and recorded amortization of debt issuance costs, which was recognized as interest expense, in the Consolidated Statement of Operations during the year ended December 31, 2018.

The Company determined that the acceleration of the maturity date upon the occurrence of a Convertible Notes restructuring was an embedded derivative, which required bifurcation and was separately ascribed with a fair value. The fair value of the embedded derivative liability on the Shareholder Term Loans issuance date was calculated by determining the fair value of the Shareholder Term Loans with and without the acceleration of the maturity date upon an occurrence of a Convertible Notes restructuring, using the same methodology and inputs in determining the fair value of the Shareholder Term Loans. The difference between the two fair values was determined to be the fair value of the embedded derivative liability. Accordingly, the Company initially recorded a derivative liability of $0.9 million as a reduction to debt payable, and the derivative liability was revalued on each reporting date, prior to the repayment of the Shareholder Term Loans in November 2018.

In connection with the entry into the Bridge Loans in November 2018, as discussed above, the Shareholder Term Loans were paid in full in November 2018. At the time of repayment, the outstanding principal of the Shareholder Term Loans totaled approximately $21.2 million, including paid in kind interest that had been added to the principal of the Shareholder Term Loans. As a result of the repayment of the Shareholder Term Loans, the Company recognized a loss on debt extinguishment of $4.3 million, which also included $0.6 million from the remeasurement and derecognition of the derivative liability and a write-off of $0.3 million of remaining unamortized debt issuance costs. There were no other penalties associated with the repayments.

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Novelion Loan

On June 14, 2016, Novelion and Aegerion entered into a term loan agreement, in which Novelion agreed to make term loans, from time to time, in an aggregate principal amount not to exceed (i) $3.0 million in any calendar month and (ii) $15.0 million in the aggregate, excluding any paid in kind interest that has been capitalized. Interest was accrued at 8% and would be paid in kind and added to the outstanding principal amount of the term loan.

In connection with the entry into the Shareholder Term Loan Agreement, on March 15, 2018 Aegerion and Novelion entered into an amended and restated senior secured term loan agreement, which had a maturity date of July 1, 2019 (“Amended and Restated Novelion Loan Agreement”), which amended and restated the secured loan facility between Aegerion and Novelion that was first entered into in connection with the Novelion Loan at the time of the acquisition of Aegerion. As of December 31, 2018 and 2017, the principal amount outstanding under the Novelion Loan was approximately $37.3 million and $23.5 million, respectively.

The Novelion Loan is not subordinated to the Roll Up Loans and the Bridge Lenders have agreed not to challenge $21.5 million of the Novelion Loan amount outstanding as of December 31, 2018 (which amount was subsequently reduced by a repayment in connection with the License Agreement entered with Recordati discussed in Note 16, Subsequent Events). Under the terms of the Bridge Intercreditor Agreement, the liens securing the Roll Up Loans rank junior to the liens securing the Novelion Loan.

9. Convertible Notes, net

In August 2014, Aegerion issued Convertible Notes with an aggregate principal amount of $325.0 million. The Convertible Notes are governed by the terms of an indenture and a supplemental indenture with The Bank of New York Mellon Trust Company, N.A., as the Trustee. The following are the key terms of the Convertible Notes:

• The Convertible Notes are senior unsecured obligations of Aegerion and bear interest at a rate of 2.0% per year, payable semi-annually in arrears on February 15 and August 15 of each year, beginning on February 15, 2015. The Convertible Notes matured on August 15, 2019, unless earlier repurchased or converted.

• After Novelion’s acquisition of Aegerion, the Convertible Notes became convertible into Novelion’s common shares at a conversion rate of 4.9817 common shares per $1,000 principal amount of the Convertible Notes. If the holders elect to convert the Convertible Notes, Aegerion can settle the conversion of the Convertible Notes through payment or delivery of cash, common shares, or a combination of cash and common shares, in its discretion.

• On or after February 15, 2019 until the close of business on the second scheduled trading day immediately preceding the maturity date, holders may convert all or any portion of their Convertible Notes, in multiples of $1,000 principal amount, at the option of the holder.

Refer to Note 16, Subsequent Events, for further discussion related to Aegerion’s bankruptcy filing in May 2019 and the related impact to the Convertible Notes.

The Company’s outstanding Convertible Note balances as of December 31, 2018 and 2017 consist of the following:

	December 31,
	2018	2017
	(in thousands)
Principal	$302,498	$324,998
Less: debt discount	(27,683)	(66,460)
Net carrying amount	$274,815	$258,538

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The expected life of the debt was equal to the five year term on the Convertible Notes. The effective interest rate was 16.42%, which was established as of the consummation of Novelion’s acquisition of Aegerion until the effective interest rate became 17.56% as a result of the retirement of a portion of the Convertible Notes in November 2018, as discussed in Note 8, Loan Facilities. The following table sets forth total interest expense recognized related to the Convertible Notes during the years ended December 31, 2018 and 2017:

	Year Ended December 31,
	2018	2017
	(in thousands)
Contractual interest expense	$6,435	$6,500
Amortization of debt discount	38,778	32,954
Total interest expense	$45,213	$39,454

Future minimum payments under the Convertible Notes as are as follows:

	Amount
	(in thousands)
Matured on August 15, 2019	$308,548
	308,548
Less amount representing interest	(6,050)
Less debt discount, net	(27,683)
Net carrying amount of Convertible Notes as of December 31, 2018	$274,815

On the Closing Date, under the terms of the Bridge Credit Agreement, the Roll Up Loans (consisting of new term loans under the Bridge Credit Agreement with an aggregate principal amount of $22.5 million) were deemed to have been funded and delivered, on behalf of Aegerion, to certain holders of Convertible Notes for the purchase of Convertible Notes in an equal principal amount outstanding, and Aegerion simultaneously directed the retirement of such Convertible Notes. Upon consummation of certain restructuring transactions consented to by the Bridge Lenders in their discretion, the liens securing the Roll Up Loans were terminated and released, and the Roll Up Loans will be treated as unsecured obligations of Aegerion, pari passu with the other obligations of Aegerion with respect to the Convertible Notes. The principal balance of Convertible Notes is approximately $302.5 million after the effect of this “roll up” of certain Convertible Notes with the proceeds of the Roll Up Loans under the Bridge Credit Agreement.

10. Related Party Transactions

During the years ended December 31, 2018 and 2017, the Company had the following related party transactions with Novelion:

Novelion Loan

As described in Note 8, Loan Facilities, in June 2016, the Company entered into a term loan agreement with Novelion, which was amended and restated from time to time. As of December 31, 2018 and 2017, the principal amount outstanding under the Novelion Loan was approximately $37.3 million and $23.5 million, respectively, which included accrued unpaid interest of $3.0 million and $1.1 million, respectively.

Shared Service Agreement

Aegerion entered into a shared service agreement with Novelion. As part of the agreement, Novelion provides executive, business development and administrative service to Aegerion. Aegerion provides back office type services such as finance and human resources to Novelion. Each party is compensated for their services rendered with a reimbursement of costs plus a margin on a quarterly basis. As part of the shared service agreement, Aegerion recognized $2.4 million and $5.9 million of related party income and incurred $3.3 million and $5.7 million of related party expense for the years ended December 31, 2018 and 2017, respectively. As of December 31, 2018 and 2017, the net payables due to Novelion was $11.0 million and $4.8 million, respectively.

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11. Stock-based Payments

Under the Amended and Restated Novelion 2017 Equity Incentive Plan (“NVLN Plan”), the Company may grant non-qualified stock options, incentive stock options and restricted stock units (“RSUs”) to employees and consultants. Common shares of Novelion will be issued upon exercise of stock options and the vesting of RSUs. Under the terms of the NVLN Plan, Novelion is entitled to grant awards in respect of its unissued common shares up to a maximum of 4,760,000 shares. As of December 31, 2018, Novelion has 2,250,137 shares of common shares available for issuance under the NVLN Plan.

The Company issues stock options and RSUs grants with service conditions (ESPP grants were also issued with service conditions), which are generally the vesting periods of the awards. Generally, the stock options, RSUs and ESPP grants expire within ten years of grant.

Determining the Fair Value of Stock Awards

(a) Stock Options

The fair value of stock options is measured with service-based vesting criteria to employees on the date of grant using the Black-Scholes option pricing model. In general, the stock options vest over three to four years. The Black-Scholes option pricing model was developed for use in estimating the value of traded options that have no vesting restrictions and are fully transferable. In addition, option pricing models require the input of highly subjective assumptions, including the expected stock price volatility. The expected volatility and expected life of Novelion’s stock options are projected based upon historical and other economic data trended into future years. The risk-free interest rate assumption is based upon observed interest rates appropriate for the expected life of Novelion’s stock options. The risk-free interest rate is determined by reference to implied yields available from U.S. Treasury securities with a remaining term equal to the expected life assumed at the date of grant.

The weighted-average grant date fair values of stock options granted during the years ended December 31, 2018 and 2017 were $1.54 and $3.87, respectively. The following weighted-average assumptions were used to value stock options granted in each of the years ended December 31, 2018 and 2017:

	Year Ended December 31,
	2018	2017
Expected stock price volatility	45.10%	38.38%
Risk-free interest rate	2.72%	1.99%
Expected life of options (years)	5.64	6.25
Expected dividend yield	—	—

The Company’s stock option activity for the year ended December 31, 2018 is as follows:

	Number of Stock Options	Weighted- Average Exercise Price Per Share	Weighted Average Remaining Contractual Life (years)	Aggregate Intrinsic Value
Outstanding at January 1, 2018	844,203	$9.40	7.84	$—
Granted	1,080,999	3.42
Exercised	—	—
Forfeited/cancelled	(613,484)	7.73
Outstanding at December 31, 2018	1,311,718	$5.25	9.00	$—
Vested and expected to vest at December 31, 2018	1,311,718	$5.25	9.00	$—
Exercisable at December 31, 2018	161,882	$9.27	7.54	$—

As of December 31, 2018, the total unrecognized compensation cost related to unvested stock options is $1.6 million and is expected to be recognized over a weighted average period of 1.43 years.

During the years ended December 31, 2018 and 2017, the weighted average exercise price of stock options granted was $3.42 and $9.49, respectively. There were no stock options exercised during the years ended December 31, 2018 and 2017.

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(b) RSUs

The Company issues RSUs to its employees as consideration for their provision of future services. The stock-based compensation expense related to RSUs is measured based on the quoted price of Novelion’s common shares on the grant date and is recognized on a straight-line basis over the requisite service period, which coincides with the vesting period. RSUs can only be exchanged and settled for Novelion’s common shares, on a one-to-one basis, upon vesting. RSUs are generally subject to forfeiture if employment terminates prior to the release of vesting restrictions.

The Company has outstanding time-vested and market-based RSUs. Time-vested RSUs are awarded to eligible employees and entitle the grantee to receive common shares at the end of a vesting period, subject solely to the employee’s continuing employment. The majority of time-vested RSUs vest over two to three years. All the market-based RSUs vest when the Company’s stock price is equal to or greater than the value of the original new hire strike price and expire on July 29, 2019.

The Company’s RSU activity for the year ended December 31, 2018 is as follows:

	Number of RSUs	Weighted- Average Grant Date Fair Value
Outstanding at January 1, 2018	219,752	$11.67
Granted	182,830	4.52
Vested	(122,014)	11.87
Forfeited/cancelled	(169,804)	7.85
Outstanding at December 31, 2018	110,764	$5.51

As of December 31, 2018, the total unrecognized compensation cost related to unvested RSUs is $0.4 million and is expected to be recognized over a weighted average period of 2.00 years.

(c) Stock-based Compensation Expense

The Company recorded stock-based compensation expense in the Consolidated Statements of Operations as follows:

	Year Ended December 31,
	2018	2017
	(in thousands)
Selling, general and administrative	$1,028	$1,591
Research and development	437	554
Total stock-based compensation expense	$1,465	$2,145

12. Employee Benefit Plan

The Company maintains a defined contribution 401(k) plan (“Plan”) in which substantially all of its and its subsidiaries' permanent U.S. employees are eligible to participate. Employee contributions are voluntary and are determined on an individual basis, limited by the maximum amounts allowable under the U.S. federal tax regulations. The Company makes matching contributions of 50% of the first 6% of employees’ contributions to the Plan up to the maximum allowed by the Internal Revenue Service. For both of the years ended December 31, 2018 and 2017, the Company recorded employer contribution expense of approximately $0.7 million. Additionally, for certain employees outside of the U.S., the Company contributes amounts for retirement benefits required by applicable local laws.

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13. Income Taxes

Loss before provision for income taxes is as follows:

	Year Ended December 31,
	2018	2017
	(in thousands)
U.S.	$(67,858)	$(69,842)
Other Foreign	(26,975)	(32,964)
Loss before provision for income taxes	$(94,833)	$(102,806)

Provision for income taxes for the years ended December 31, 2018 and 2017 is as follows:

	Year Ended December 31,
	2018	2017
	(in thousands)
Current benefit (provision):
U.S.	$52	$106
Other Foreign	(899)	(647)
	(847)	(541)
Deferred provision:
Other Foreign	(858)	(53)
Provision for income taxes	$(1,705)	$(594)

Differences between the Company’s statutory income tax rates and its effective income tax rates, as applied to the loss before income taxes for the years ended December 31, 2018 and 2017 are reconciled as follows:

	December 31,
	2018	2017
	(in thousands)
U.S. statutory tax rates	21%	35%
Loss before income taxes	$(94,833)	$(102,806)
Expected income tax benefit	19,915	35,982
Net increase in valuation allowance	(19,553)	(4,458)
Tax credits	891	(176)
Stock-based compensation	22	(11)
Foreign rate differential	(3,043)	(8,091)
Tax rate change	(680)	(22,654)
Non-taxable expenditures	(1,550)	(723)
Change in uncertain tax positions	(215)	(20)
Return to provisions	838	(2,185)
State tax	1,670	1,742
Provision for income taxes	$(1,705)	$(594)

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Deferred taxes are recognized for temporary differences between the basis of assets and liabilities for financial statement and income tax purposes. The primary components of the Company’s deferred tax assets and liabilities are comprised of the following:

	December 31,
	2018	2017
	(in thousands)
Deferred tax assets:
Net operating loss carryforwards	$24,664	$21,227
Research and development credits	1,836	187
Stock-based compensation	724	478
Capitalized research expenses	716	1,028
Depreciable and amortizable assets	10,811	9,727
Business interest expense limitation	11,609	—
Other temporary differences	13,093	11,689
Total gross deferred tax assets	63,453	44,336
Valuation allowance	(63,453)	(43,479)
Net deferred tax assets	$—	$857

As of December 31, 2018, the Company has evaluated the positive and negative evidence bearing upon the realizability of its deferred tax assets and concluded that based on the Company’s history of operating losses and operating as a going concern, that it is more likely than not that the benefit of its U.S. and all its foreign deferred tax assets will not be realized. Therefore, the Company has provided a valuation allowance against its U.S. and all its foreign deferred tax assets in 2018.

Additionally, as there have been conditions that raise substantial doubt about the Company’s ability to continue as a going concern, the presumption exists that unremitted foreign earnings will be required to meet the existing obligations of its U.S. operations. As such, as of December 31, 2018, the Company has not recognized deferred tax liabilities on earnings that are not considered indefinitely reinvested, as these amounts are deemed immaterial. The deferred tax liabilities associated with earnings not considered indefinitely reinvested are primarily associated with foreign withholding and foreign exchange gain or loss on distributions that would be incurred upon distributions.

As of December 31, 2018, the Company had approximately $107.1 million of U.S., Canadian and foreign net operating losses (“NOL”) of which $66.1 million relate to the Company’s U.S. subsidiaries, $30.8 million relate to the Company’s Swiss subsidiary and $10.1 million relate to the Company’s UK subsidiary. Of the $66.1 million U.S. federal NOL carryforwards, $59.2 million will expire at various dates from 2025 through 2037, if not utilized, and the remaining is not subject to expiration. As of December 31, 2018, the Company also had approximately $43.9 million of U.S. state NOL carryforwards that will expire at various dates from 2029 through 2038, if not utilized.

The following table summarizes the activity related to the Company’s provision for UTP:

	Year Ended December 31,
	2018	2017
	(in thousands)
Total provision for UTP as of January 1,	$1,029	$911
Increases related to current year tax positions	655	56
Changes in tax positions of prior periods	(1,044)	62
Total provision for UTP as of December 31,	640	1,029
Deferred tax assets available to offset provision for UTP	—	(612)
Total provision for UTP as of December 31,	$640	$417

As of December 31, 2018 and 2017, the Company has accrued an insignificant amount of interest as a result of the deferred assets available to offset its provision for UTP. The Swiss tax authorities have challenged the Company’s position as a principal company and are attempting to establish the Swiss subsidiary as a limited risk distributor at a formal transfer pricing arrangement. The UTP established in prior years for transfer pricing adjustments made at the Company and its Swiss subsidiary can be decreased as a result of the Swiss audit, which is expected to be completed in 2020.

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The Company and its subsidiaries file income tax returns in Canada, the U.S. and various U.S. states and in foreign jurisdictions. The Canadian income tax returns are generally subject to tax examination for the tax years ended December 31, 2012 through December 31, 2018. The U.S., U.S. state and foreign income tax returns are generally subject to tax examinations for the tax years ended December 31, 2015 through December 31, 2018. To the extent the Company has tax attribute carryforwards, the tax years in which the attribute was generated may still be adjusted upon examination by the tax authorities.

On December 22, 2017, the President of the United States signed into law the Tax Cuts and Jobs Act (“TCJA”). This TCJA includes significant changes in U.S. tax law, including a reduction in the corporate tax rates and creating a territorial tax system with a one-time mandatory tax on previously deferred foreign earnings of U.S. subsidiaries. The TCJA reduced the U.S. corporate tax rate from 35% to 21% for tax years beginning after December 31, 2017. As a result of the TCJA, the Company was required to revalue its existing U.S. deferred tax assets and liabilities as of December 31, 2017 from the 35% federal rate in effect through the end of 2017, to the new 21% rate. As a result of the change in law, the Company recorded a current period tax expense of $21.5 million and a corresponding reduction in the associated valuation allowance, for a net adjustment of zero, to its Consolidated Statement of Operations for the year ended December 31, 2017. The TCJA required the Company to pay tax on the unremitted earnings of its foreign subsidiaries through December 31, 2017. The Company has estimated that its foreign subsidiaries are in an overall net earnings deficit and as such would have no incremental U.S. tax and therefore has recorded no tax liability on its unremitted earnings at December 31, 2017.

14. Enterprise-wide Information

Net Revenues

The following table summarizes total net revenues from external customers by product and by geographic region, based on the location of the customer.

	Year Ended December 31, 2018
	U.S.	Japan	Brazil	Other Foreign Countries	Total
	(in thousands)
Metreleptin	$47,942	$945	$5,170	$17,303	$71,360
Lomitapide	35,461	10,822	388	12,401	59,072
Total net revenues	$83,403	$11,767	$5,558	$29,704	$130,432

	Year Ended December 31, 2017
	U.S.	Japan	Brazil	Other Foreign Countries	Total
	(in thousands)
Metreleptin	$50,972	$788	$6,837	$7,711	$66,308
Lomitapide	46,431	5,836	6,659	13,204	72,130
Total net revenues	$97,403	$6,624	$13,496	$20,915	$138,438

Net revenues generated from customers outside of the U.S., Japan and Brazil, as listed in the column “Other Foreign Countries,” were primarily derived from Canada, Colombia, France, Germany and Turkey for the year ended December 31, 2018 and from Canada, Argentina, Colombia and Greece during the year ended December 31, 2017.

Significant Customers

For the year ended December 31, 2018, one customer accounted for 64% of the Company’s net revenues and accounted for 35% of the Company’s December 31, 2018 accounts receivable balance. For the year ended December 31, 2017, two customers accounted for 70% of the Company’s net revenues; of these two customers, one customer accounted for 63% of the Company’s December 31, 2017 accounts receivable balance.

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Long-lived Assets

The Company’s long-lived assets are primarily comprised of intangible assets and property and equipment. As of December 31, 2018 and 2017, 65% of the intangible assets were attributable to Aegerion’s U.S. business, with the remaining 35% attributable to Aegerion’s European holding company, as of both December 31, 2018 and 2017.

As of December 31, 2018 and 2017, 75% and 86%, respectively, of the Company’s property and equipment resided in the Company’s U.S. subsidiaries, with the remaining assets residing in the Company’s entities located outside of the U.S.

15. Commitments and Contingencies

Leases

The Company leased certain office facilities and office equipment pursuant to operating leases during the year ended December 31, 2018. The future minimum payments for office space and office equipment over the next five years are summarized as follows:

	Lease Commitments
	(in thousands)
Years Ending December 31,:
2019	$1,246
2020	432
2021	190
2022	134
2023	97
Thereafter	41
Total	$2,140

Rent expense pursuant to operating leases was approximately $3.1 million and $3.2 million for the years ended December 31, 2018 and 2017, respectively.

Other Commitments

Amgen Licensing Agreements

Metreleptin. In connection with Aegerion’s acquisition of MYALEPT in January 2015, Aegerion acquired a license agreement between Amgen Inc. (“Amgen”) and Amylin Pharmaceuticals, Inc., dated February 7, 2006 (“Amgen License”) pursuant to which an exclusive worldwide license was obtained from Amgen to certain know-how and patents and patent applications covering the composition of matter and methods of use of metreleptin to develop, manufacture and commercialize a preparation containing metreleptin (“Amgen Licensed Products”).

As part of the Amgen License, an exclusive sublicense of Amgen’s exclusive rights to certain metreleptin-related patents and patent applications owned by the Rockefeller University and exclusively licensed to Amgen under a license agreement dated April 14, 1995, as amended (“Rockefeller License”) and an exclusive sublicense of Amgen’s non-exclusive rights to certain metreleptin-related patents and patent applications owned by The Regents of the University of California and non-exclusively licensed to Amgen under a license agreement dated July 13, 2005 (“UCSF License”) were obtained. Amgen retains rights to conduct research, development, manufacturing and commercialization activities with respect to products other than the Amgen Licensed Products.

Sublicenses under the licenses are permitted and are subject to certain limitations, including Amgen’s right of first offer for any out-license, partnership, co-development, commercialization, co-promotion or similar agreement related to metreleptin or the Amgen Licensed Products, which expires in February 2021. Under this license agreement, Amgen must notify Aegerion of any potential third-party partnership regarding any intellectual property rights controlled by Amgen in the neurology field and Aegerion will have a right of first negotiation for any license, partnership, co-development, commercialization, co-promotion or similar agreement, which expires in February 2021.

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Aegerion is required to make royalty payments to Amgen on net sales of each Amgen Licensed Product on a country-by-country basis (i) at a royalty rate in the low double digits where the Amgen Licensed Product has patent protection or market exclusivity granted by a regulatory authority at the time of regulatory approval in the applicable country during the applicable royalty term, which runs on a country-by-country basis until the later of (a) the expiration of the last-to-expire valid claim covering an Amgen Licensed Product in the applicable country, (b) expiration of any market exclusivity granted by a regulatory authority, and (c) ten years from the date on which an Amgen Licensed Product is first sold to a third party in a country after regulatory approval for the Amgen Licensed Product has been granted in such country (“Amgen Royalty Term”) or (ii) at a royalty rate in the mid-single digits to low double digits where the Amgen Licensed Product receives patent protection or market exclusivity following the time of regulatory approval in the applicable country, in either case subject to a variety of customary reductions.

Under the Amgen License, Aegerion is also required to directly meet certain payment obligations under the Rockefeller License and UCSF License. Aegerion is required to make royalty payments to Rockefeller University on net sales of each product with patent rights or know-how in the field of obesity genes, obesity gene products and molecules that modulate or mediate their action and/or regulation on a country-by-country basis at a range of royalty rates in the low single digits depending on whether the product has an orphan product designation or not until the later to occur of expiration of (i) patent protection, (ii) any market exclusivity period granted in the applicable country, or (iii) any data exclusivity period in the applicable country (with certain limitations related to the number of units sold). In February 2015, Aegerion paid a one-time $5.0 million milestone payment to Rockefeller University, which was due twelve months following the receipt of marketing approval for MYALEPT in the U.S. Aegerion is also required to pay to Rockefeller University a percentage in the low double digits of any upfront license fees or one-time fees Aegerion receives in consideration for any sublicense of the licensed rights. There are no material payment obligations outstanding under the UCSF License. Also, in connection with the acquisition of metreleptin, Aegerion entered into a letter agreement with AstraZeneca pursuant to which Aegerion agreed to make royalty payments payable by AstraZeneca and its affiliates to BMS with respect to net sales of metreleptin in the U.S. The time-based royalty rate ranges from mid-single digits to low double digits, increasing annually in years 2016 to 2019 from rates in the low single digits to low double digits, peaking in years 2019 to 2020 at a rate in the low double digits before decreasing in years 2022 through 2025 to rates in the high single digits to mid-single digits. The royalty obligation to BMS terminates in 2026.

The Amgen License will terminate upon the expiration of the last Amgen Royalty Term for any Amgen Licensed Product. Aegerion has the right to terminate the Amgen License for convenience upon 90 days prior written notice to Amgen or for Amgen’s uncured material breach of the Amgen License, or becoming subject to specified bankruptcy or liquidation events. Amgen may terminate the Amgen License for Aegerion’s uncured failure to make payments to Amgen or if Aegerion is the subject of specified bankruptcy or liquidation events.

During the years ended December 31, 2018 and 2017, Aegerion made aggregate royalty payments of $12.6 million and $9.7 million to Amgen, Rockefeller University and BMS, and had $4.5 million and $2.9 million in aggregate royalties payable as of December 31, 2018 and 2017, respectively.

University of Pennsylvania Licensing Agreement

Lomitapide. In May 2006, Aegerion entered into a license agreement with The Trustees of the University of Pennsylvania (“UPenn”) pursuant to which it obtained an exclusive, worldwide license from UPenn to certain know-how and a range of patent rights applicable to lomitapide. In particular, Aegerion obtained a license to certain patent and patent applications owned by UPenn relating to the dosing of microsomal triglyceride transfer protein inhibitors, including lomitapide, and certain patents and patent applications and know-how covering the composition of matter of lomitapide that were assigned to UPenn by BMS in the field of monotherapy or in combination with other dyslipidemic therapies, which are therapies for the treatment of patients, with abnormally high or low levels of plasma cholesterol or triglycerides.

Aegerion is obligated under this license agreement to use commercially reasonable efforts to develop, commercialize, market and sell at least one product covered by the licensed patent rights, such as lomitapide. In addition, Aegerion will be required to make specified royalty payments on net sales of products, at a range of royalty rates in the high single digits on net sales of lomitapide in countries where lomitapide has patent protection, and of any other products covered by the license (subject to a variety of customary reductions), and share with UPenn specified percentages of sublicensing royalties and certain other consideration that Aegerion

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receives under any sublicenses that Aegerion may grant. Pursuant to Aegerion’s current license agreement with UPenn, UPenn is entitled to receive 15% of the $25 million upfront payment, 15% of the marketing authorization transfer milestone and any subsequent sales milestone payments received from Recordati and 25% of royalty payments received by Aegerion from Recordati under the Japan License Agreement, as further discussed in Note 16, Subsequent Events. These same terms apply to other sublicenses under the UPenn agreement. During the years ended December 31, 2018 and 2017, Aegerion made royalty payments in the amounts of $2.6 million and $3.1 million, respectively, to UPenn. Additionally, Aegerion accrued an additional $0.7 million in royalties to UPenn as of both December 31, 2018 and 2017.

This license agreement will remain in effect on a country-by-country basis until the expiration of the last-to-expire licensed patent right in the applicable country. Aegerion has the right to terminate this license agreement for UPenn’s uncured material breach of the license agreement or for convenience upon 60 days’ prior written notice to UPenn, subject to certain specific conditions and consequences. UPenn may terminate this license agreement for Aegerion’s uncured material breach of the license agreement, its uncured failure to make payments to UPenn or if Aegerion is the subject of specified bankruptcy or liquidation events.

Indemnities

In connection with the sale of assets, the Company provided indemnities with respect to certain matters, including product liability, patent infringement, contractual breaches and misrepresentations, and the Company provides other indemnities to third parties under the clinical trial, license, service, supply and other agreements that it enters into in the normal course of its business. If the indemnified party were to make a successful claim pursuant to the terms of the indemnity, the Company would be required to reimburse the loss. These indemnities are generally subject to threshold amounts, specified claims periods and other restrictions and limitations. As of December 31, 2018 and 2017, no amounts have been accrued in connection with such indemnities.

Development and Post Marketing Regulatory Commitments

Aegerion has engaged Contract Research Organizations (“CROs”) to provide research, safety and project management services (“Services”) in connection with the execution of their potential clinical trials, post-marketing commitments and existing registries. Services would only give rise to liabilities to the extent that services are provided to Aegerion, as applicable, and pass through expenses are incurred. As of December 31, 2018, the Services have not yet been performed and the Company has potential commitments of approximately $29.9 million under these agreements. The amount reflected is based on the existing contracts and does not reflect any inflation, future modification to, or termination of, the existing contracts or anticipated or potential new contracts.

Legal Matters

The Company accrues a liability for legal contingencies when it believes that it is both probable that a liability has been incurred and that it can reasonably estimate the amount of the loss. The Company reviews these accruals and adjusts them to reflect ongoing negotiations, settlements, rulings, advice of legal counsel and other relevant information. To the extent new information is obtained and the Company’s views on the probable outcomes of claims, suits, assessments, investigations or legal proceedings change, changes in the Company’s loss contingency accrual would be recorded in the period in which such determination is made.

DOJ/SEC Investigations

In late 2013, Aegerion received a subpoena from the Department of Justice (“DOJ”), represented by the U.S. Attorney’s Office in Boston, requesting documents regarding its marketing and sale of JUXTAPID in the U.S., as well as related public disclosures (“DOJ investigation”). In late 2014, Aegerion received a subpoena from the Securities and Exchange Commission (“SEC”) requesting certain information related to Aegerion’s sales activities and disclosures related to JUXTAPID. The SEC also requested documents and information on a number of other topics, including documents related to the investigations by government authorities in Brazil into whether Aegerion’s activities in Brazil violated Brazilian anti-corruption laws, and whether Aegerion’s activities in Brazil violated the Foreign Corrupt Practices Act (“FCPA”). As a result of the SEC’s investigation, Aegerion consented to the entry of a final judgment, on September 25, 2017, in connection with a complaint filed by the

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SEC without admitting or denying the allegations set forth in the complaint (“SEC Judgment”). The complaint alleged negligent violations of Sections 17(a)(2) and (3) of the Securities Act of 1933, as amended, related to certain statements made by Aegerion in 2013 regarding the conversion rate for JUXTAPID prescriptions.

The SEC Judgment, which was approved by a U.S. District Court judge on September 25, 2017, provides that Aegerion must pay a civil penalty in the amount of $4.1 million, to be paid in installments over three years, plus interest on any unpaid balance at a rate of 1.75% per annum. As of December 31, 2018, $0.9 million remains due as a current liability, and $0.7 million remains due as a non-current liability. Aegerion’s payment of this civil penalty is subject to acceleration in the event of certain change of control transactions or certain transfers of Aegerion’s rights in MYALEPT or JUXTAPID. Aegerion’s payment schedule is also subject to acceleration in the event that Aegerion fails to satisfy its payment obligations under the SEC Judgment.

In connection with the DOJ investigation, Aegerion entered into a Plea Agreement, a Deferred Prosecution Agreement (“DPA”), a Civil Settlement, certain State Settlement Agreements and a Consent Decree of Permanent Injunction (“FDA Consent Decree”). Under the Court-approved DOJ Plea Agreement, Aegerion pled guilty to two misdemeanor misbranding violations of the Federal Food, Drug and Cosmetic Act (“FDCA”) and on January 30, 2018, a U.S. District Court Judge sentenced Aegerion.

The Court did not impose a criminal fine and instead ordered Aegerion to pay restitution, in the amount of $7.2 million payable over three years, plus interest on any unpaid balance at a rate of 1.75% per annum, into a fund managed by an independent claims administrator. As of December 31, 2018, $2.0 million remains due as a current liability, and $2.3 million remains due as a non-current liability. As contemplated by the Plea Agreement, Aegerion was further sentenced to a three-year term of probation. Among the terms of probation, Aegerion must (i) comply with federal, state and local laws, (ii) notify its probation officer of any prosecution, major civil litigation or administrative proceeding, (iii) seek permission of its probation officer prior to selling, assigning or transferring assets, (iv) notify its probation officer of any material change in its economic circumstances, (v) forbear from disparaging the factual basis of Aegerion’s plea or denying that Aegerion itself is guilty, and (vi) comply with the DPA and Corporate Integrity Agreement (“CIA”) (and submit certain reports prepared thereunder to its probation officer). Under the terms of the DPA, Aegerion admitted it engaged in conduct that constituted a conspiracy to violate the Health Insurance Portability and Accountability Act (“HIPAA”). The DPA provides that Aegerion must continue to cooperate fully with the DOJ concerning its investigation into other individuals or entities. The DPA provides that Aegerion must maintain a robust compliance and ethics program that includes significant certification, training, monitoring and other requirements. Aegerion, as well as Novelion’s Board of Directors (or a designated committee thereof), must also conduct regular reviews of its compliance and ethics program, provide certifications to the DOJ that the program is believed to be effective and notify the DOJ of any probable violations of HIPAA. In the event Aegerion breaches the DPA, there is a risk the government would seek to impose remedies provided for in the DPA, including instituting criminal prosecution against Aegerion and/or seeking to impose stipulated penalties against Aegerion. The DPA is subject to supervision by a U.S. District Court judge.

Aegerion also entered into the DOJ Civil Settlement Agreement to resolve allegations by the DOJ that false claims for JUXTAPID were submitted to governmental healthcare programs. The DOJ Civil Settlement Agreement requires Aegerion to pay a civil settlement in the amount of $28.8 million, which includes up to $2.7 million designated for certain U.S. states relating to Medicaid expenditures for JUXTAPID, to be paid in installments over three years. As of December 31, 2018, $8.8 million remains due as a current liability, and $16.4 million remains due as a non-current liability. Aegerion’s payment of this civil settlement amount is subject to acceleration in the event of certain change of control transactions or certain transfers of Aegerion’s rights in MYALEPT or JUXTAPID. In the event that Aegerion fails to satisfy its obligations under the DOJ Civil Settlement Agreement, Aegerion could be subject to additional penalties or litigation.

Aegerion also agreed to enter into the State Settlement Agreements to resolve claims under state law analogues to the federal False Claims Act. The terms of the State Settlement Agreements are substantially similar to those set forth in the DOJ Civil Settlement Agreement. As noted above, participating states will receive up to $2.7 million in the aggregate from the $28.8 million amount to be paid pursuant to the DOJ Civil Settlement Agreement.

Aegerion also agreed to the FDA Consent Decree with the DOJ and the FDA to resolve a separate civil complaint alleging that Aegerion violated the FDCA by failing to comply with the JUXTAPID REMS program

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and the requirement to provide adequate directions for all of the uses for which it distributed JUXTAPID. The FDA Consent Decree requires Aegerion, among other things, to comply with the JUXTAPID REMS program; retain a qualified independent auditor to conduct annual audits of its compliance with the JUXTAPID REMS program; and remediate any noncompliance identified by the auditor within specified timeframes. In the event Aegerion fails to comply with the JUXTAPID REMS program or any other provisions of the FDA Consent Decree, Aegerion could be subject to additional administrative remedies, civil or criminal penalties and/or stipulated damages. Aegerion is required to notify the FDA in advance of certain changes in control, or changes in its business that may affect its operations, assets, rights or liabilities in the United States. On March 20, 2019, the Court entered the FDA Consent Decree.

Separately, Aegerion entered into a CIA with the Department of Human Services Office of the Inspector General (“OIG”). The CIA requires Aegerion, among other things, to maintain a compliance program with significant requirements relating to, among other things, training, monitoring, annual risk assessment and mitigation processes, independent review of Aegerion’s compliance and other activities, a disclosure program and an executive financial recoupment program. Under the CIA, Aegerion, as well as the Board of Directors of the Company (or a designated committee thereof), must also conduct regular reviews of Aegerion’s compliance program and provide an annual resolution or certification to OIG that the program is believed to be effective. Additionally, Aegerion has certain certification and reporting obligations under the CIA. In the event Aegerion breaches the CIA, there is a risk the government would seek to impose remedies provided for in the CIA, including seeking to impose stipulated penalties against Aegerion and/or seeking to exclude Aegerion from participation in federal healthcare programs.

Investigations in Brazil

Federal prosecutors in Brazil are conducting an investigation to determine whether there have been violations of Brazilian laws related to the sales of JUXTAPID in Brazil. In July 2016, the Ethics Council of Interfarma fined Aegerion’s subsidiary in Brazil (“Aegerion Brazil”) approximately $0.5 million for violations to its Code of Conduct, to which Aegerion Brazil is bound due to its affiliation with Interfarma. Also, the Board of Directors of Interfarma imposed an additional penalty of suspension of Aegerion Brazil’s membership, without suspension of Aegerion Brazil’s membership contribution, for a period of 180 days for Aegerion Brazil to demonstrate the implementation of effective measures to cease alleged irregular conduct, or exclusion of the Company’s membership in Interfarma if such measures are not implemented. Aegerion Brazil paid the fine of approximately $0.5 million during the third quarter of 2016. In March 2017, after the suspension period ended, Interfarma’s Board of Directors decided to reintegrate Aegerion Brazil, enabling it to participate regularly in Interfarma activities, subject to meeting certain obligations. In April 2019, the Board of Directors of Interfarma agreed that Aegerion Brazil has successfully met all of the requirements imposed by the association, and the investigation was closed.

Also, in July 2016, Aegerion Brazil received an inquiry from a Public Prosecutor Office of the Brazilian State of Paraná asking it to respond to questions related to media coverage regarding JUXTAPID and its relationship with a patient association to which Aegerion made donations for patient support. This preliminary inquiry was later reclassified as a civil inquiry, which is a preliminary procedure by the Public Prosecutor’s Office that aims to verify if there are enough elements for it to file a formal lawsuit or to dismiss the inquiry. In March 2018, the Paraná State Public Prosecutor’s Office sent the civil inquiry to the Federal Public Prosecutor’s Office, after deciding that the potential case should be subject to federal jurisdiction. The Federal Public Prosecutor dismissed the case in January 2019.

In June 2017, the Federal Public Prosecutor of the City of São José dos Campos, State of São Paulo, in connection with its criminal investigation into former employees of Aegerion Brazil, requested that a Brazilian federal court provide federal investigators with access to the bank records of certain individuals and entities, including Aegerion Brazil, certain former Aegerion Brazil employees, a Brazilian patient association and certain Brazilian physicians. The Federal Trial Court Judge issued a decision on July 12, 2018 authorizing the access to the banking records on the terms that the Federal Public Prosecutor of the City of São José dos Campos had requested. On July 16, 2018, Aegerion Brazil filed an appeal of the decision that authorized the breach of the banking secrecy, which was denied by the Federal Court Judge. The Public Prosecutor in São José dos Campos continues to gather information in connection with this investigation. At this time, the Company does not know whether the inquiry of the Public Prosecutor in São José dos Campos will result in the commencement of any formal proceeding against Aegerion, but if Aegerion’s activities in Brazil are found to violate any laws or

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governmental regulations, Aegerion may be subject to significant civil lawsuits to be filed by the Public Prosecution office, and administrative penalties imposed by Brazilian regulatory authorities and additional damages and fines. Under certain circumstances, Aegerion could be barred from further sales to federal and/or state governments in Brazil, including sales of JUXTAPID and/or MYALEPT, due to penalties imposed by Brazilian regulatory authorities or through civil actions initiated by federal or state public prosecutors. Additionally, the SEC conducted inquiries with Aegerion concerning the investigations by Brazilian authorities, and in July 2019, the SEC concluded the investigation and no enforcement action was made against Aegerion. The Company cannot determine if a loss is probable as a result of the investigations and inquiry in Brazil and whether the outcome will have a material adverse effect on the Company’s business and, as a result, no amounts have been recorded for a loss contingency.

Qui Tam Litigation

In March 2014, an amended qui tam complaint was filed under seal in the District of Massachusetts against Aegerion, two former executive officers and a former employee. United States ex rel Clarke v. Aegerion Pharm. Inc., No. 13-cv-11785-IT. On September 22, 2017, the U.S. filed a notice of intervention as to Aegerion. On September 27, 2017, the qui tam relators filed a second amended complaint naming additional parties, including a former board member, former executives and former employees of Aegerion, as well as other third parties. The second amended complaint noted that the relators would file a joint stipulation of dismissal with respect to Aegerion upon the completion of certain conditions set forth in the Civil Settlement Agreement. On October 27, 2017, the court granted Aegerion and relators’ joint motion to stay proceedings until sentencing in the criminal matter is complete. On February 20, 2018, Aegerion was dismissed from the qui tam lawsuit. On June 5, 2018, two of the remaining defendants were dismissed from the lawsuit and on June 19, 2018, the remaining individual defendants filed a motion to dismiss the qui tam lawsuit. On March 31, 2019, the Court granted the Motion to Dismiss with respect to one defendant, and denied the motion with respect to the other remaining defendants. On April 17, 2019, the remaining defendants filed a motion to dismiss for lack of jurisdiction. Although Aegerion is not a party to the lawsuit, it could be liable for certain defense costs and damages for defendants remaining in the lawsuit. Although the Company does not believe the outcome of the lawsuit will have a material adverse effect on the Company, the Company cannot determine if a loss is probable as a result of the lawsuit and, as a result, no amounts have been recorded for a loss contingency.

16. Subsequent Events

The Company has evaluated subsequent events through February 18, 2020, which is the date that financial statements were available to be issued. Refer to the below for the material subsequent events occurred since December 31, 2018.

Loan Facilities

As described in Note 8, Loan Facilities, on January 31, 2019, Aegerion provided notice to the Bridge Agent electing to extend the initial maturity date of the Bridge Loans to June 30, 2019, as permitted under the Bridge Credit Agreement. Aegerion paid an extension fee in the amount of $1.5 million on February 15, 2019 and, accordingly, the maturity date of the Bridge Loans was extended to June 30, 2019. Refer to below for further discussion related to the treatment of the loan facilities upon Aegerion’s emergence from bankruptcy in September 2019.

Recordati License Agreement

On February 5, 2019 (“Transaction Effective Date”), Aegerion entered into a license agreement (“License Agreement”) with Recordati for the commercialization of JUXTAPID in Japan. Under the terms of the License Agreement, and subject to the conditions set forth therein, Aegerion granted to Recordati an exclusive license in Japan, for the current marketed indication for HoFH. During the term of the License Agreement, Recordati also has an exclusive right of first negotiation to any new indications for JUXTAPID in Japan that may be developed by Aegerion and the right to grant sub-licenses and to manufacture and commercialize JUXTAPID, under specific circumstances.

Pursuant to the terms of the License Agreement, and subject to the conditions set forth therein, Recordati is required to make the following payments to Aegerion: (i) $25.0 million as a one-time upfront payment on the

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Transaction Effective Date (which was paid to Aegerion in the first quarter of 2019), (ii) $5.0 million as a one-time payment within 45 days following the date on which the Japan marketing authorization for JUXTAPID was transferred to Recordati (“Completion Date”), which was paid to Aegerion in the second quarter of 2019, (iii) quarterly royalty payments, during the term of the License Agreement, equal to 22.5% of all net sales of JUXTAPID in Japan, and (iv) 20% of all other sublicense revenues received by Recordati or any of its affiliates.

In addition, pursuant to the terms of the License Agreement, Aegerion may receive from Recordati commercial milestone payments (up to a total of $80.0 million) for net sales in Japan, conditioned and based upon the achievement of certain net sales levels in Japan, the first $12.5 million installment of which becomes payable at the end of the first quarter in which cumulative net sales in Japan reach $70.0 million, and which are payable in incremental installments thereafter at the end of each quarter in which cumulative net sales in Japan increase by $70.0 million (in increments of $12.5 million until cumulative net sales reach $280.0 million and then in incremental installments of $5.0 million until cumulative net sales reach $700.0 million).

Further, pursuant to the Company’s current license agreement with The Trustees of the University of Pennsylvania (“UPenn”), UPenn received 15% of the $25 million upfront payment and 15% of the marketing authorization transfer milestone in the second half of 2019. Additionally, UPenn is entitled to receive 15% of any subsequent sales milestone payments received from Recordati and 25% of royalty payments received by the Company from Recordati under the License Agreement.

A portion of the upfront payment was used to repay the outstanding debt Aegerion has. Refer to Note 8, Loan Facilities, for further information.

Aegerion and Recordati have also entered into a customary supply agreement under which Aegerion will supply JUXTAPID to Recordati (or its affiliate) at cost plus an agreed upon markup for an initial term of two years with automatic renewal for successive two year terms, and a customary transitional services agreement under which Aegerion performed certain commercialization and administrative services on Recordati’s behalf until the Completion Date (during which time, in lieu of paying royalties and cost-plus supply and transitional services during this period, Aegerion retained 40% of the net sales of JUXTAPID in Japan and remitted the remaining 60% of net sales to Recordati) and certain other customary transitional services (if so requested by Recordati) at mutually agreed hourly rates for a term not to exceed six months from the Completion Date.

Chapter 11 Bankruptcy Filing and Amryt Acquisition

On May 20, 2019, Aegerion and its U.S. subsidiary, Aegerion Pharmaceuticals Holdings, Inc. filed voluntary petitions under chapter 11 of Title 11 of the U.S. Code in the Bankruptcy Court. On September 10, 2019, the Bankruptcy Court entered an order (“Confirmation Order”) confirming Aegerion’s First Amended Joint Chapter 11 Plan (“Plan”), as modified to reflect certain resolutions agreed to among various parties. The effective date of the Plan (“Effective Date”) is subject to the satisfaction of all conditions precedent to the Plan, as further described in the Plan.

In accordance with the Plan, the Debtors will continue to exist after the Effective Date as Reorganized Debtors. On and after the Effective Date, all property of the estates, wherever located, including all claims, rights and causes of action, as defined in the Plan, and any property, wherever located, acquired by the Debtors under or in connection with this Plan, shall revest in the Reorganized Debtors, as applicable, free and clear of all claims, liens, charges, other encumbrances and interests.

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As a result of the Plan, a significant amount of the Company’s indebtedness, as summarized in the following, was discharged upon Aegerion’s emergence from bankruptcy in September 2019.

• New Money Loans and Roll Up Loans − the full amount including the accrued unpaid interest, was discharged. Each holder of the New Money Loans received secured credit facility issued by Aegerion at the Closing on a dollar for dollar basis on account of its New Money Loan claim: each holder of the Roll Up Loans received new convertible notes issued by Aegerion at the Closing on a dollar for dollar basis on account of its Roll Up Loan claim.

• Novelion Loan – the full amount, including the accrued unpaid interest, was discharged. Novelion received American depositary receipts representing approximately 14.0 million Ordinary Shares, as discussed below.

• Convertible Notes – the outstanding unpaid principal and interest were discharged. Each Convertible Noteholders received its pro rata share of the net remaining new convertible notes and certain Amryt common stocks.

• Outstanding settlements due to DOJ and SEC – The Company will continue to pay the outstanding settlements to DOJ and SEC in accordance with payment terms as set forth in the original settlement agreements.

• Ongoing trade transactions – The Company will continue to follow the respective payment terms with each vendor. Any outstanding amount that was associated to the pre-Petition period but was put on hold for payment during the bankruptcy period was fully paid in cash in September 2019, plus post-petition interest at the applicable interest rate.

• Payables due to Novelion – the outstanding payables due to Novelion were discharged.

Additionally, as a result of confirmation of the Plan, the Bankruptcy Court has authorized Aegerion to consummate the transactions contemplated by the Plan, including the Amryt Transaction. Following the entry of the Confirmation Order, Amryt’s shareholders approved the Amryt Transaction at a meeting held on September 19, 2019.

On September 24, 2019, Aegerion completed the Amryt Transaction (“Closing”). The consideration for the Amryt Transaction has been satisfied through the issuance of ordinary Amryt shares (“Ordinary Shares”), or American depositary receipts representing Ordinary Shares, to stakeholders of Aegerion. Through the Amryt Transaction, Novelion has been divested of its operating subsidiary, Aegerion. In full satisfaction of its claims as creditor under Novelion Loan, Novelion received American depositary receipts representing approximately 14.0 million Ordinary Shares (“Novelion Consideration”). Novelion only received the Novelion Consideration and did not receive any other equity or property in connection with the Amryt Transaction.

*** ***

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Aegerion Pharmaceuticals, Inc.
(DEBTOR-IN-POSSESSION)
Consolidated Balance Sheets
(in thousands)
(UNAUDITED)

	June 30, 2019	December 31, 2018
Assets
Current assets:
Cash and cash equivalents	$36,080	$31,881
Accounts receivable, net	26,408	28,912
Inventories - current	13,486	12,745
Prepaid expenses and other current assets	17,153	15,292
Total current assets	93,127	88,830
Inventories - non-current	38,306	36,202
Property and equipment, net	754	1,397
Intangible assets, net	187,629	200,176
Other non-current assets	2,818	1,209
Total assets	$322,634	$327,814
Liabilities and shareholders’ deficit
Current liabilities:
Accounts payable	$3,615	$4,995
Accrued liabilities	49,749	42,356
Payable due to Novelion	—	11,003
Short-term debt	—	73,677
Short-term debt due to Novelion	—	37,264
Convertible notes, net	—	274,815
Provision for legal settlements - current	14,070	11,689
Total current liabilities	67,434	455,799
Provision for legal settlements - non-current	11,962	19,391
Other non-current liabilities	1,444	795
Total liabilities not subject to compromise	80,840	475,985
Liabilities subject to compromise	420,651	—
Total liabilities	501,491	475,985
Commitments and contingencies (Note 13)
Shareholders’ deficit:
Common shares, without par value, 30,301 shares issued and outstanding at June 30, 2019 and December 31, 2018, respectively	—	—
Additional paid-in-capital	59,381	59,381
Accumulated deficit	(237,092)	(206,217)
Accumulated other comprehensive loss	(1,146)	(1,335)
Total shareholders’ deficit	(178,857)	(148,171)
Total liabilities and shareholders’ deficit	$322,634	$327,814

See accompanying Notes to Consolidated Financial Statements.

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Aegerion Pharmaceuticals, Inc.
(DEBTOR-IN-POSSESSION)
Consolidated Statements of Operations
(in thousands)
(UNAUDITED)

	Six Months Ended June 30,
	2019	2018
Net revenues	$95,857	$59,388
Cost of product sales	35,364	29,208
Operating expenses:
Selling, general and administrative	43,424	38,568
Research and development	13,946	21,113
Related party expense, net	397	617
Total operating expenses	57,767	60,298
Income (loss) from operations	2,726	(30,118)
Reorganization items, net	(2,145)	—
Interest expense, net (contractual interest of $690)	(29,681)	(22,628)
Interest expense due to Novelion (contractual interest of $352)	(1,182)	(1,406)
Other expense, net	(224)	(1,054)
Loss before provision for income taxes	(30,506)	(55,206)
Provision for income taxes	(369)	(1,205)
Net loss	$(30,875)	$(56,411)

See accompanying Notes to Consolidated Financial Statements.

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Aegerion Pharmaceuticals, Inc.
(DEBTOR-IN-POSSESSION)
Consolidated Statements of Comprehensive Loss
(in thousands)
(UNAUDITED)

	Six Months Ended June 30,
	2019	2018
Net loss	$(30,875)	$(56,411)
Other comprehensive income (loss):
Foreign currency translation	189	(1,335)
Other comprehensive income (loss)	189	(1,335)
Comprehensive loss	$(30,686)	$(57,746)

See accompanying Notes to Consolidated Financial Statements.

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Aegerion Pharmaceuticals, Inc.
(DEBTOR-IN-POSSESSION)
Consolidated Statements of Changes in Shareholders’ Deficit
(in thousands, except share information)
(UNAUDITED)

	Six Months Ended June 30, 2019
	Common Shares		Additional Paid-In Capital	Accumulated Deficit	Accumulated Other Comprehensive Loss	Total Shareholders’ Deficit
	Shares	Amount
Balance at December 31, 2018	30,301,444	$—	$59,381	$(206,217)	$(1,335)	$(148,171)
Net loss	—	—	—	(30,875)	—	(30,875)
Foreign currency translation adjustment	—	—	—	—	189	189
Balance at June 30, 2019	30,301,444	$—	$59,381	$(237,092)	$(1,146)	$(178,857)

	Six Months Ended June 30, 2018
	Common Shares		Additional Paid-In Capital	Accumulated Deficit	Accumulated Other Comprehensive Income/(Loss)	Total Shareholders’ Deficit
	Shares	Amount
Balance at December 31, 2017	30,301,444	$—	$59,381	$(109,679)	$828	$(49,470)
Net loss	—	—	—	(56,411)	—	(56,411)
Foreign currency translation adjustment	—	—	—	—	(1,335)	(1,335)
Balance at June 30, 2018	30,301,444	$—	$59,381	$(166,090)	$(507)	$(107,216)

See accompanying Notes to Consolidated Financial Statements.

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Aegerion Pharmaceuticals, Inc.
(DEBTOR-IN-POSSESSION)
Consolidated Statements of Cash Flows
(in thousands)
(UNAUDITED)

	Six Months Ended June 30,
	2019	2018
Cash used in operating activities
Net loss	$(30,875)	$(56,411)
Adjustments to reconcile net loss to net cash provided by (used in) operating activities:
Depreciation	490	842
Non-cash lease expense	888	—
Amortization of intangible assets	12,548	12,548
Stock-based compensation	703	933
Non-cash interest expense	24,214	19,146
Non-cash interest expense due to Novelion	1,182	1,406
Unrealized foreign exchange (gain) loss	(31)	1,161
Amortization of debt issuance costs and debt discount	2,215	127
Deferred income taxes	—	919
Other non-cash operating activities	(8)	9
Loss on disposal of property and equipment	140	—
Changes in assets and liabilities:
Accounts receivable	2,565	3,811
Inventories	(2,355)	(1,433)
Prepaid expenses and other assets	(2,391)	(3,738)
Accounts payable	1,819	(3,958)
Accrued liabilities and other liabilities	1,093	(84)
Payable due to Novelion	(2,951)	235
Net cash provided by (used in) operating activities	9,246	(24,487)
Cash used in investing activities
Purchases of property and equipment	—	(384)
Net cash used in investing activities	—	(384)
Cash provided by financing activities
Net proceeds from Shareholder Term Loans, net of debt discount	—	19,977
Proceeds from Novelion Loan	—	15,000
Repayment of Bridge Loans	(2,996)	—
Repayment of Novelion Loan	(2,106)	—
Payment of issuance costs	—	(698)
Net cash (used in) provided by financing activities	(5,102)	34,279
Exchange rate effect on cash	55	(862)
Net increase in cash and cash equivalents	4,199	8,546
Cash and cash equivalents, beginning of period	31,881	14,307
Cash and cash equivalents, end of period	$36,080	$22,853
Supplemental disclosures of cash flow information
Cash paid for interest	$3,393	$3,422
Cash paid (received) for taxes, net	$352	$(71)
Non-cash investing activities and financing activities:
Purchases of property and equipment included in accounts payable	$—	$35
Right-of-use assets obtained in exchange for operating lease obligation	$1,945	$—
Lease liabilities arising in exchange for right of use assets	$1,950	$—

See accompanying Notes to Consolidated Financial Statements.

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Aegerion Pharmaceuticals, Inc.
(DEBTOR-IN-POSSESSION)
 
Notes to Consolidated Financial Statements
(UNAUDITED)

1. Description of Business and Basis of Presentation

Organization

Aegerion Pharmaceuticals, Inc. (“Aegerion” or the “Company”) is a rare disease biopharmaceutical company dedicated to developing new standards of care for individuals living with rare diseases. The Company is a private company incorporated in the state of Delaware in the United States (“U.S.”) and has international operations and two commercial products, metreleptin and lomitapide. Metreleptin, a recombinant analog of human leptin, is currently marketed in the U.S. under the brand name MYALEPT (metreleptin for injection). MYALEPT is approved in the U.S. as an adjunct to diet as replacement therapy to treat the complications of leptin deficiency in patients with congenital or acquired generalized lipodystrophy (“GL”). In July 2018, metreleptin, under the brand name MYALEPTA, was approved in the European Union (“EU”) as a treatment for the complications of leptin deficiency in patients with congenital or acquired GL in adults and children two years of age and above and familial or acquired Partial Lipodystrophy (“PL”) in adults and children 12 years of age and above for whom standard treatments have failed to achieve adequate metabolic control. Lomitapide, which is marketed in the U.S. under the brand name JUXTAPID (lomitapide) capsules (“JUXTAPID”), is approved in the U.S. as an adjunct to a low-fat diet and other lipid-lowering treatments, including low-density lipoprotein (“LDL”) apheresis where available, to reduce low-density lipoprotein cholesterol (“LDL-C”), total cholesterol (“TC”), apolipoprotein B (“apo B”) and non-high-density lipoprotein cholesterol (“non-HDL-C”) in adult patients with homozygous familial hypercholesterolemia (“HoFH”). Lomitapide is approved in the EU, under the brand name LOJUXTA (lomitapide) hard capsules (“LOJUXTA”) for the treatment of adult patients with HoFH, where it is commercialized by Aegerion’s licensee, Amryt Pharma plc (“Amryt”). In December 2016, Aegerion launched JUXTAPID as a treatment for HoFH in Japan and on February 5, 2019, Aegerion entered into a license agreement with Recordati Rare Diseases Inc. (“Recordati”) for the commercialization of JUXTAPID in Japan. Additionally, both metreleptin and lomitapide are sold, on a named patient basis, in certain countries outside of the U.S., such as Brazil, where such sales are permitted based on the approval of metreleptin and lomitapide in the U.S. or EU.

Merger with Amryt

As discussed in Note 15, Subsequent Events, on May 20, 2019 (“Petition Date”), Aegerion and its U.S. subsidiary, Aegerion Pharmaceuticals Holdings, Inc., each was previously a subsidiary of Novelion Therapeutics Inc. (“Novelion”), filed voluntary petitions (collectively, “Bankruptcy Petitions”) under chapter 11 (“Chapter 11”) of Title 11 of the United States Code (“Bankruptcy Code”) in the United States Bankruptcy Court for the Southern District of New York (“Bankruptcy Court”). In addition, on May 20, 2019, Aegerion Pharmaceuticals, Inc. and Aegerion Pharmaceuticals Holdings, Inc. entered into a Plan Funding Agreement (“Plan Funding Agreement”) with Amryt to set forth the terms and conditions of the acquisition by Amryt of 100 percent of the outstanding equity interests of the reorganized Aegerion Pharmaceuticals, Inc. (the “Reorganized Debtors”) (“Amryt Transaction”).

On September 24, 2019, the Amryt Transaction was completed (“Closing”). The consideration for the Amryt Transaction was satisfied through the issuance of ordinary Amryt shares (“Ordinary Shares”), or American depositary receipts representing Ordinary Shares, to the stakeholders of Aegerion. Through the Amryt Transaction, Novelion has been divested of Aegerion. Refer to Note 15, Subsequent Events for further discussion related to the completion of the Amryt Transaction.

Basis of Presentation and Principles of Consolidation

The accompanying Consolidated Financial Statements have been prepared in accordance with accounting principles generally accepted in the United States of America (“GAAP”). In the opinion of management, the accompanying Consolidated Financial Statements include all adjustments (including normal recurring accruals) considered necessary for fair presentation of Aegerion’s consolidated financial position, results of operations and cash flows for the periods presented. Operating results for the current interim period are not necessarily indicative of the results that may be expected for the fiscal year ending December 31, 2019. The accompanying

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Consolidated Financial Statements should be read in conjunction with the audited consolidated financial statements and accompanying notes in Aegerion’s consolidated financial statements for the year ended December 31, 2018.

The accompanying Consolidated Financial Statements include operations of Aegerion and its wholly-owned subsidiaries. All intercompany transactions and balances have been eliminated. Additionally, the Company engages in various transactions with its former parent company, Novelion, and its affiliates, including the term loan agreement and the shared service agreement. Refer to Note 12, Related Party Transactions, for further discussions. Accordingly, the accompanying Consolidated Financial Statements have been prepared from the separate records maintained by the Company and may not necessarily be indicative of the conditions that would have existed or the results of operations if the Company had been operated as an unaffiliated company.

As further discussed in Note 2, Bankruptcy Filing, Aegerion and Aegerion Pharmaceuticals Holdings, Inc. would continue to operate its business as a “debtor in possession” under the jurisdiction of the Bankruptcy Court and in accordance with the applicable provisions of the Bankruptcy Code and the orders of the Bankruptcy Court. For additional information related to the bankruptcy proceedings and the debtor-in-possession financial information, refer to Note 2, Bankruptcy Filing, and Note 14, Condensed Combined Debtor-in-Possession Financial Information.

The accompanying Consolidated Financial Statements have been prepared assuming Aegerion will continue to operate as a going concern, which contemplates the realization of assets and the settlement of liabilities and commitments in the normal course of business. As of June 30, 2019, Aegerion has significant indebtedness of $501.5 million, and the level of indebtedness has adversely impacted Aegerion’s financial condition. In addition, the filing of the Chapter 11 cases in May 2019 constituted an event of default with respect to certain of Aegerion’s existing debt obligations. As a result, Aegerion’s financial condition and the risks and uncertainties surrounding its Chapter 11 proceedings raise substantial doubt as to Aegerion’s ability to continue as a going concern.

As discussed above and in Note 15, Subsequent Events, the Bankruptcy Court entered an order (“Confirmation Order”) confirming Aegerion’s First Amended Joint Chapter 11 Plan (“Plan”), as modified to reflect certain resolutions agreed to among various parties. As result of the Plan, a significant amount of the Company’s indebtedness was discharged. Additionally, as discussed above, Aegerion was acquired by Amryt. Refer to Note 15, Subsequent Events, for further discussion on the indebtedness discharge and the Amryt Transaction.

As of December 31, 2019, Aegerion’s unrestricted cash balance is approximately $17.4 million. The Company believes that its existing funds would not be sufficient to satisfy its operating needs and its working capital for at least the next twelve months from the issuance of the Consolidated Financial Statements. Aegerion may, from time to time, need to seek additional funding from Amryt, its parent company. Should the Company be unable to receive funding from its parent company, the Company’s business, results of operations, liquidity and financial condition would be materially and negatively affected, and the Company would be unable to continue as a going concern.

Use of Estimates

The preparation of Consolidated Financial Statements in accordance with GAAP requires management to make estimates and assumptions that affect the reported amounts of assets and liabilities, the disclosure of liabilities subject to compromise as a result of the Company’s bankruptcy filing, the disclosure of contingent assets and liabilities at the date of the Consolidated Financial Statements, and the reported amounts of revenues and expenses during the reporting periods presented. The Company’s estimates often are based on complex judgments, probabilities and assumptions that we believe to be reasonable but that are inherently uncertain and unpredictable. For any given individual estimate or assumption made by us, there may also be other estimates or assumptions that are reasonable. Actual results may differ from estimates made by management. Changes in estimates are reflected in reported results in the period in which they become known.

Recently Adopted Accounting Standards

In 2016, the Financial Accounting Standards Board (“FASB”) issued Accounting Standards Update (“ASU”) No. 2016-02, Leases (“ASU 2016-02”), which amends a number of aspects of lease accounting and requires entities to recognize right-of-use assets and liabilities on the balance sheet for leases.

The Company adopted ASU 2016-02 effective January 1, 2019, using the modified retrospective method. The adoption of ASU 2016-02 did not change the classification of any of the existing leases as of the transition date,

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and the prior period results are not adjusted or restated and continue to be reported in accordance with ASC Topic 840: Leases (“Topic 840”). In addition, the Company elected the package of practical expedients permitted under the transition guidance, which, among other things, allowed the Company to combine lease and non-lease components for all of the leases. The Company also elected not to record leases with an initial term of 12 months or less on the balance sheet.

Upon the adoption of this standard, Aegerion recorded operating lease right-of-use assets and corresponding operating lease liabilities, each of approximately $1.8 million as of January 1, 2019, which are included in the Consolidated Balance Sheet. The adoption of this standard did not materially impact operations and cash flows. Refer to Note 8, Leases, for further discussion.

2. Bankruptcy Filing

Chapter 11 Proceedings

On the Petition Date, Aegerion and its U.S. subsidiary, Aegerion Pharmaceuticals Holdings, Inc. (together, “Debtors”), filed the Bankruptcy Petitions under Chapter 11 of the Bankruptcy Code in the Bankruptcy Court. Pursuant to a motion filed by the Debtors, the Chapter 11 cases (collectively, “Chapter 11 Cases”) were jointly administered under the caption In re Aegerion Pharmaceuticals, Inc., et al., Case No. 19-11632. Each Debtor would continue to operate its business as a “debtor in possession” under the jurisdiction of the Bankruptcy Court and in accordance with the applicable provisions of the Bankruptcy Code and the orders of the Bankruptcy Court. Documents filed on the docket of and other information related to the Chapter 11 Cases are available free of charge online at https://cases.primeclerk.com/aegerion/. Documents and other information available on such website are not part of this document and shall not be deemed incorporated by reference in this document.

Certain subsidiaries of Aegerion (collectively, “Non-Filing Entities”) were not part of the Chapter 11 Cases. The Non-Filing Entities include all the non-U.S. subsidiaries of Aegerion and they would continue to operate their businesses in the normal course; their results are included in the Consolidated Financial Statements. Refer to Note 14, Condensed Combined Debtor-in-Possession Financial Information, for the combined financial statements for the Debtors.

Significant Bankruptcy Court Actions

On the Petition Date, the Debtors filed a number of motions (“First Day Motions”) with the Bankruptcy Court, in an effort to stabilize the operations and facilitate the Debtors’ transition into Chapter 11. Certain of these motions sought authority from the Bankruptcy Court for the Debtors to make payments upon, or otherwise honor, certain obligations that arose prior to the Petition Date, including obligations related to employee wages, salaries and benefits, taxes, customer programs and certain vendors and other providers essential to the Debtors’ businesses. On May 24, 2019, the Bankruptcy Court approved the relief sought in these motions on interim bases. On June 27, 2019, the Bankruptcy Court entered orders approving the relief sought in these motions on final bases.

On the Petition Date, the Debtors filed a joint Chapter 11 plan of reorganization and an accompanying disclosure statement, which set forth, among other things, the terms of the Debtors’ proposed treatment of claims against and interests in the Debtors. On June 3, 2019, the Debtors filed a motion (“Disclosure Statement Motion”) with the Bankruptcy Court requesting, among other relief, approval of the disclosure statement and procedures to solicit votes to accept or reject the Chapter 11 plan. As a result of negotiations with various parties, the Debtors agreed to make certain changes to the Chapter 11 plan (“First Amended Chapter 11 Plan”) and the disclosure statement (“Disclosure Statement”), which the Debtors filed with the Bankruptcy Court on July 9, 2019. On July 11, 2019, the Bankruptcy Court entered an order (“Disclosure Statement Order”) approving the Disclosure Statement and establishing procedures to solicit votes to accept or reject the First Amended Chapter 11 Plan.

On May 30, 2019, the Debtors filed a motion (“KEIP/KERP Motion”) with the Bankruptcy Court requesting authority to implement a key executive incentive plan (“KEIP”) and a key employee retention plan (“KERP”). As a result of negotiations with various parties, the Debtors agreed to make certain changes to the KEIP and the KERP, which changes are set forth in a statement the Debtors filed with the Bankruptcy Court on July 9, 2019. On July 11, 2019, the Court entered an order (“KEIP/KERP Order”) approving the KEIP/KERP Motion, as revised. Pursuant to the KEIP/KERP Order, the Debtors are permitted to, among other things, reimburse Novelion for payments that Novelion makes on account of its key employee incentive plan pursuant to certain shared services agreements between Aegerion, Novelion and its subsidiary, Novelion Services USA, Inc.

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On September 10, 2019, the Bankruptcy Court entered an order (“Confirmation Order”) confirming Aegerion’s First Amended Joint Chapter 11 Plan (“Plan”), as modified to reflect certain resolutions agreed to among various parties. As a result of confirmation of the Plan, the Bankruptcy Court authorized Aegerion to consummate the transactions contemplated by the Plan, including the Amryt Transaction. Following the entry of the Confirmation Order, Amryt’s shareholders approved the Amryt Transaction at a meeting held on September 19, 2019.

Restructuring Support Agreement

In connection with its bankruptcy filing, on May 20, 2019, the Debtors entered into a Restructuring Support Agreement (“Restructuring Support Agreement”) with (a) Novelion as holder of 100% of the outstanding equity interests of Aegerion and holder of 100% by principal amount of claims under the Novelion Loan, (b) the holders of 100% by principal amount under the Bridge Loans, (c) holders of in excess of 67% by principal amount of the Convertible Notes, ((a), (b) and (c) collectively the “Consenting Lenders”), and (d) Amryt (collectively with the Consenting Lenders and the Debtors, the “RSA Parties”). In addition, on May 20, 2019, Aegerion and Amryt entered into the Plan Funding Agreement setting forth the terms and conditions of the Amryt Transaction.

The Restructuring Support Agreement and the Plan Funding Agreement set forth, subject to certain conditions, the commitments and obligations of the Debtors, the Consenting Lenders and Amryt to support the Debtors’ plan of reorganization and the acquisition of Aegerion by Amryt (“Restructuring Transactions”), which involve the following key elements that are relevant to Aegerion:

• Amryt acquiring 100% of the outstanding new equity interests in recapitalized Aegerion;

• Ordinary equity of Amryt representing 61.4% of the outstanding ordinary shares of Amryt, after giving effect to the Restructuring Transactions but before giving effect to shares underlying the New Convertible Notes, the Deal Equity Raise (each as described below), ordinary shares that may be issuable in satisfaction of the contingent value right if the relevant milestones are achieved, and equity that is reserved for issuance under any management equity compensation plan adopted by Amryt, will be distributed to certain existing creditors of Aegerion in complete or partial satisfaction of their claims, including in partial satisfaction of the claims of the holders of the existing Convertible Notes and in complete satisfaction of the outstanding Novelion Loan;

• The equity interests of Aegerion held by Novelion being terminated;

• Aegerion issuing $125 million of new 5% convertible notes (“New Convertible Notes”). The New Convertible Notes will be issued to certain existing creditors of Aegerion in satisfaction of their claims (and not for cash), including in satisfaction of a portion of the existing Convertible Notes, the approximately $22 million of Roll Up Loans under the Bridge Loans, and any amounts drawn down under Aegerion’s DIP Financing (defined below) that are not otherwise satisfied in cash at the closing of the Restructuring Transactions;

• Aegerion’s existing Bridge Loans in the original principal amount of $50 million, held by certain funds managed by and Athyrium Capital Management, LP (“Athyrium”) and Highbridge Capital Management, LLC (“Highbridge”), as well as Amryt’s existing approximately €20 million (in principal) of secured debt, will be converted into new first-lien secured debt of Aegerion and Amryt, which will have a cash interest rate of 6.5% per annum and an additional 6.5% PIK (Paid in Kind) and mature five years from the closing date of the Restructuring Transactions;

• In connection with the closing of the Restructuring Transactions, Amryt plans to raise $60 million through the issuance of new equity of Amryt (“Deal Equity Raise”). The proceeds from the Deal Equity Raise will be used as provided in the plan of reorganization to pay certain expenses and for general corporate purposes. The new equity will be priced at a 20 percent discount to Amryt’s implied valuation pro forma to the Restructuring Transaction with $18 million of the new equity offered to certain investors of Amryt and $42 million to certain creditors of Aegerion on a pro rata basis, including Novelion;

• Aegerion intends to, and the plan of reorganization provides that Aegerion will, continue to fully honor all obligations to the U.S. Department of Justice (“DOJ”), the U.S. Securities and Exchange Commission (“SEC”) and other U.S. and state government agencies and courts, which obligations will not be impaired by the Restructuring Transactions;

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• Aegerion intends to continue to pay all trade and other ordinary operating expenses that arise during the course of the Chapter 11 Cases and, upon consummation of the Restructuring Transactions, repay 100% of the trade claims;

Debtor-In-Possession Financing

On the Petition Date, the Debtors filed a motion (“DIP Financing Motion”) requesting authority to use cash collateral and enter into a $20.0 million super-priority debtor-in-possession multi-draw term loan facility (“DIP Facility”) with Athyrium and Highbridge on the terms and conditions set forth in a proposed Debtor-in-Possession Credit Agreement entered into between Aegerion Pharmaceuticals, Inc., as borrower, Aegerion Pharmaceuticals Holdings, Inc., as guarantor, Cantor Fitzgerald Securities, as administrative agent, and the lenders party thereto. On May 24, 2019, the Bankruptcy Court authorized the use of cash collateral on an interim basis. On June 27, 2019, the Bankruptcy Court entered an order granting the relief sought in the DIP Financing Motion on a final basis (“Final DIP Order”). As of June 30, 2019, Aegerion did not draw any portion of the DIP Facility.

Amended Shared Services Agreements

The Debtors entered into shared services agreements with Novelion and its subsidiary, Novelion Services USA, Inc., dated as of December 1, 2016, but effective as of November 29, 2016 (“Shared Services Agreements”), pursuant to which the Debtors provide to Novelion and Novelion provides to the Debtors, certain services, including, but not limited to administrative support, human resources, information technology support, accounting, finance and legal services.

In connection with the execution of the Restructuring Support Agreement and to facilitate the Restructuring Transactions, the Debtors and Novelion executed an amendment to the Shared Services Agreements (together, “Amended Shared Services Agreements”), which modified the Shared Services Agreements to provide, among other things, for Aegerion to make certain cash payments to Novelion on account of certain services Novelion provided or will provide to Aegerion. Pursuant to the Amended Shared Services Agreement, Aegerion made a payment to Novelion of approximately $3.1 million prior to the Petition Date and committed to make additional cash payments of up to approximately $2.0 million.

On the Petition Date, the Debtors filed a motion requesting authority to assume the Amended Shared Services Agreements. Following negotiations between the Debtors, Novelion, and the Official Committee of Unsecured Creditors appointed in the Chapter 11 Cases (“Creditors’ Committee”) and the Office for the United States Trustee, the parties agreed to defer final approval of the motion and the proposed assumption of the Amended Shared Services Agreements but that, subject to the Bankruptcy Court’s approval, to approve the motion on an interim basis and permit the Debtors to continue to operate under the terms of the Amended Shared Services Agreements on a post-petition basis in the ordinary course of business, subject to certain limitations. On June 27, 2019, the Bankruptcy Court entered an interim order granting the relief as agreed by the parties (“Interim Shared Services Agreements Order”).

On September 24, 2019, Novelion, Amryt and Aegerion entered into a master services agreement (“MSA”) whereby Amryt will reimburse Novelion for certain compensation and benefits that Novelion will be paying to two of Novelion’s three remaining employees until the earlier of each employee’s last date of employment with Novelion and December 31, 2019, in exchange for such employees providing transitional executive and integration services to Amryt during such period. Under the MSA, Amryt will also provide Novelion with certain accounting and reporting support, if requested, for an agreed monthly fee, and certain administrative support at no cost.

Financial Reporting in Reorganization

Effective on the Petition Date, Aegerion began to apply accounting standards applicable to reorganizations, which are applicable to companies under Chapter 11 bankruptcy protection. Such accounting standards require the financial statements for periods subsequent to the Petition Date to distinguish transactions and events that are directly associated with the reorganization from the ongoing operations of the business. Expenses, realized gains and losses, and provisions for losses that are directly associated with reorganization proceedings must be reported separately as reorganization items, net in the Consolidated Statement of Operation. In addition, the balance sheet must distinguish pre-petition liabilities subject to compromise (“LSTC”) of the Debtors from pre-petition liabilities that are not subject to compromise, post-petition liabilities and liabilities of non-Debtor entities in the

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accompanying Consolidated Balance Sheet. LSTC are pre-petition obligations that are not fully secured and have at least a possibility of not being repaid at the full claim amount. If there is uncertainty about whether a secured claim will be paid or impaired pursuant to the Chapter 11 Cases, the Company has classified the entire amount of the claim as a LSTC.

Furthermore, the realization of assets and the satisfaction of liabilities are subject to uncertainty. While operating as debtors-in-possession, certain claims against the Debtors in existence before the filing of the petitions for relief under the federal bankruptcy laws are stayed while the Debtors continue business operations as debtors in possession. These claims are reflected in the Consolidated Balance Sheet at June 30, 2019 as liabilities subject to compromise.

Liabilities Subject to Compromise

As a result of the commencement of the Chapter 11 Cases, the payment of pre-petition liabilities is generally subject to compromise pursuant to a plan of reorganization. Generally, actions to enforce or otherwise effect payment of pre-petition liabilities are stayed. Although payment of pre-petition claims generally is not permitted, the Bankruptcy Court granted the Debtors authority to pay certain pre-petition claims in designated categories and subject to certain terms and conditions. This relief generally was designed to preserve the value of the Debtors’ business and assets. Among other things, the Bankruptcy Court authorized the Debtors to pay certain pre-petition claims relating to employee wages and benefits, taxes and certain vendors.

Pre-petition liabilities that are subject to compromise are required to be reported at the amounts expected to be allowed by the Bankruptcy Court, even if they may be settled for different amounts. The amounts classified as LSTC may be subject to future adjustments depending on Bankruptcy Court actions, further developments with respect to disputed claims, the determination of secured status of certain claims, the determination as to the value of any collateral securing claims, proof of claims, or other events.

Liabilities subject to compromise as of June 30, 2019 include the following components:

	June 30, 2019
	(in thousands)
Short-term debt	$75,213
Short-term debt due to Novelion	36,340
Convertible notes, net	296,712
Accounts Payable	3,379
Payable due to Novelion	9,007
Total liabilities subject to compromise	$420,651

Reorganization Items, Net

Reorganization items, net represent amounts incurred after the Petition Date as a direct result of the Chapter 11 Cases and are comprised of the following for the six months ended June 30, 2019. There was no cash paid for reorganization items, net during six months ended June 30, 2019.

	Six Months Ended June 30, 2019
	(in thousands)
Professional fees	$2,145
Total	$2,145

3. Revenue Recognition

The Company’s net revenues are primarily derived from product sales; and its remaining revenues are derived from the royalties on product sales made by its sublicensees in the EU, Japan and other territories. During the second quarter of 2019, the Company also earned licensing revenues as a result of the license agreement entered with Recordati. The following summarizes the revenue recognition for the respective revenue streams.

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Product Sales Revenues

The Company recognizes revenue from sales of metreleptin and lomitapide at the point in time when control transfers, typically upon transfer of product to the carrier or delivery of product to customers. Revenue is recognized net of estimated discounts, rebates and any taxes collected from customers which are subsequently remitted to governmental authorities. Payment terms vary by contract, but payment is typically due within 30 to 120 days of delivery to the customer. Generally, the period between when the Company transfers or delivers the products and when payments are received is in one year or less; as such, the Company deems it unnecessary to assess whether a significant financing component exists and does not adjust the transaction price for the time value of money.

Variable Consideration

Product sales revenues are recognized at the net sales price (“transaction price”) which includes estimated reserves for variable consideration, upon the transfer of control of the Company’s products. Variable consideration primarily includes government rebates, prompt payment discounts and distribution service fees. Estimates of variable consideration are made at contract inception and based on historical experience, market trends, industry data and statutory requirements are considered when determining such estimates. Variable consideration is included in the transaction price to the extent it is probable that a significant reversal of revenue will not occur. The Company reassesses variable consideration at the end of each reporting period as additional information becomes available with the variance recorded to product sales revenue.

Government Rebates: The Company is subject to government mandated rebates for Medicare, Medicaid, Tricare and other government programs in the U.S. and other countries. These rebates are estimated based on actual payer information. The Company records an accrued liability for unpaid rebates related to products for which control has been transferred to distributors.

The following table summarizes combined activity for the allowances for the government rebates incurred in connection with the product sales of MYALEPT and JUXTAPID for the period indicated:

	Amount
	(in thousands)
Balance as of December 31, 2018	$19,637
Provision	15,679
Payments	(18,239)
Balance as of June 30, 2019	$17,077

Prompt Payment Discounts: The Company provides discounts to certain distributors if they pay for product within a defined period of time after title transfers, which terms are explicitly stated in the contract. These discounts are recorded as a reduction of revenue upon receipt of full payment from such distributors.

Distributor Service Fees: Certain distributors provide distribution services to the Company for a fee, and the costs associated with these services are generally recorded as a reduction of revenue.

Other Incentives: The Company offers other incentives that vary by contract; these incentives take into account specific relevant factors and are analyzed for revenue recognition purposes on a case by case basis.

Other Revenues

The Company has entered into agreements where the Company licenses certain rights to its products to sublicensees and earns royalties from product sales made by the sublicensees and milestone payments upon the achievement of certain levels of sales. Under ASC Topic 606, the Company recognizes royalty revenue and sales-related milestone payments, when applicable, at the later of (1) the time that the subsequent sale or usage occurs, or (2) the performance obligation to which some or all of the sales-based or usage-based royalty has been allocated has been satisfied (or partially satisfied).

On February 5, 2019, the Company entered into a license agreement with Recordati for the commercialization of JUXTAPID in Japan. Refer to Note 4, License Agreement, for further discussion and the revenue recognition related to this transaction.

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4. License Agreement

On February 5, 2019 (“Transaction Effective Date”), the Company entered into a license agreement (“License Agreement”) with Recordati for the commercialization of JUXTAPID in Japan. Under the terms of the License Agreement, and subject to the conditions set forth therein, the Company granted to Recordati an exclusive license in Japan, for the current marketed indication for HoFH. During the term of the License Agreement, Recordati also has an exclusive right of first negotiation to any new indications for JUXTAPID in Japan that may be developed by the Company and the right to grant sub-licenses and to manufacture and commercialize JUXTAPID, under specific circumstances.

Pursuant to the terms of the License Agreement, and subject to the conditions set forth therein, Recordati is required to make the following payments to the Company: (i) $25.0 million as a one-time upfront payment on the Transaction Effective Date (which was paid in the first quarter of 2019), (ii) $5.0 million as a one-time payment within 45 days following the date on which the Japan marketing authorization for JUXTAPID was transferred to Recordati (“Completion Date”), which was paid in the second quarter of 2019, (iii) quarterly royalty payments, during the term of the License Agreement, equal to 22.5% of all net sales of JUXTAPID in Japan, and (iv) 20% of all other sublicense revenues received by Recordati or any of its affiliates.

In addition, pursuant to the terms of the License Agreement, the Company may receive from Recordati commercial milestone payments (up to a total of $80.0 million) for net sales in Japan, conditioned and based upon the achievement of certain net sales levels in Japan, the first $12.5 million installment of which becomes payable at the end of the first quarter in which cumulative net sales in Japan reach $70.0 million, and which are payable in incremental installments thereafter at the end of each quarter in which cumulative net sales in Japan increase by $70.0 million (in incremental payments of $12.5 million until cumulative net sales reach $280.0 million and then in incremental installments of $5.0 million for each incremental $70.0 million of revenues until cumulative net sales reach $700.0 million).

Further, pursuant to the Company’s current license agreement with The Trustees of the University of Pennsylvania (“UPenn”), UPenn received 15% of the $25 million upfront payment and 15% of the marketing authorization transfer milestone in the second half of 2019. Additionally, UPenn is entitled to receive 15% of any subsequent sales milestone payments received from Recordati and 25% of royalty payments received by the Company from Recordati under the License Agreement.

The Company and Recordati have also entered into a customary supply agreement under which the Company will supply JUXTAPID to Recordati (or its affiliate) at cost plus an agreed upon markup for an initial term of two years with automatic renewal for successive two year terms, and a customary transitional services agreement under which the Company performed certain commercialization and administrative services on Recordati’s behalf until the Completion Date (during which time, in lieu of paying royalties and cost-plus supply and transitional services during this period, the Company retained 40% of the net sales of JUXTAPID in Japan and remitted the remaining 60% of net sales to Recordati) and certain other customary transitional services (if so requested by Recordati) at mutually agreed hourly rates for a term not to exceed six months from the Completion Date.

The Company and Recordati have made customary representations and warranties and have agreed to certain other customary covenants, including confidentiality, limitation of liability and indemnity provisions. The initial term of the License Agreement continues until the latest of: (i) expiration of the last valid claim of the licensed patents covering JUXTAPID in Japan, (ii) expiration of data or regulatory exclusivity in relation to JUXTAPID in Japan or (c) ten years from the Completion Date. Thereafter the term of the License Agreement will automatically renew for a single five-year term, and then thereafter for successive five-year terms unless either party provides written notice at least 18 months prior to the end of the then current renewal term. Either party may terminate the License Agreement for cause if the other party materially breaches or defaults in the performance of its obligations, and, if curable, such material breach remains uncured for 90 days (15 days for non-payment).

At the date of agreement inception, three performance obligations, consisting of (1) the license (inclusive of the Japan marketing authorization for JUXTAPID and other related intellectual property), (2) the JUXTAPID supply on hand at the Completion Date, and (3) the supply of JUXTAPID to Recordati, were identified. Revenue for each of the performance obligations will be recognized at a point in time, as further discussed below:

• License: The consideration for the license performance obligation consists of the $30.0 million in one-time, fixed payments as well as a reduction related to the 60% of net sales benefit to Recordati during the transition period, from the Transaction Effective Date through the Completion Date (“the

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Transition Period”). Revenue allocated to the license was recognized at the delivery of the Japan marketing authorization for JUXTAPID in May 2019, and was reduced by the 60% of net sales benefit to Recordati as Recordati did not transfer a distinct good or service to the Company during the Transition Period. During the second quarter of 2019, total of $30.0 million was recognized as licensing revenue upon the Japan marketing authorization for JUXTAPID delivered to Recordati, and such revenue was reduced by $1.5 million, which represented the 60% of net sales benefit owed to Recordati for the six months ended June 30, 2019.

• JUXTAPID supply on hand at the Completion Date: The consideration for JUXTAPID supply on hand at the Completion Date, pursuant to the transitional services agreement, was variable consideration dependent on the inventory on hand at the Completion Date multiplied by the cost plus markup. Revenue allocated to the JUXTAPID supply on hand at the Completion Date will be recognized when such supply is made available to Recordati at the manufacturing facility engaged by the Company. During the six months ended June 30, 2019, the Company earned $1.4 million of revenue from Recordati for the sales of JUXTAPID supply on hand at the Completion Date.

• Supply of JUXTAPID to Recordati: The consideration for the supply of JUXTAPID to Recordati will be variable consideration dependent on the inventory ordered pursuant to the supply agreement multiplied by the cost plus markup. Revenue allocated to the JUXTAPID supply will be recognized when such supply is made available to Recordati at the manufacturing facility engaged by the Company. During the six months ended June 30, 2019, there was no such supply sales made to Recordati.

Additionally, in accordance with the License Agreement, the Company has earned royalty revenue equal to 22.5% of all net sales of JUXTAPID in Japan. The Company recognizes royalty revenue at the later of (1) the time that the subsequent sale or usage occurs, or (2) the performance obligation to which some or all of the sales-based or usage-based royalty has been allocated has been satisfied (or partially satisfied). During the six months ended June 30, 2019, the Company earned $0.7 million of royalty revenue from Recordati.

5. Inventories

The components of inventories are as follows:

	June 30, 2019	December 31, 2018
	(in thousands)
Work-in-process	$26,727	$26,676
Finished goods	25,065	22,271
Total	51,792	48,947
Less: Inventories - current	(13,486)	(12,745)
Inventories - non-current	$38,306	$36,202

Non-current inventories primarily consist of the active pharmaceutical ingredients which do not expire. Additionally, a portion of finished goods is classified as non-current as of June 30, 2019 and December 31, 2018 based on forecasted consumption exceeding one year. There was no charge for excess or obsolete inventory during the six months ended June 30, 2019 and an immaterial charge for excess or obsolete inventory in the Consolidated Statement of Operations during the six months ended June 30, 2018.

6. Intangible Assets

The intangible assets are amortized over their estimated useful lives, which are the remaining patent lives of approximately 7 - 9 years, and reviewed for impairment when events and changes in circumstances indicate that the carrying amount may not be recoverable. During the six months ended June 30, 2019 and 2018, there were no impairment charges recorded.Additionally, the Company reviewed the useful lives of the intangibles as of June 30, 2019 and believes the useful lives are still reasonable.

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Intangible asset balances as of June 30, 2019 and December 31, 2018 are as follows:

	June 30, 2019
	Gross Carrying Value	Accumulated Amortization	Net Carrying Value
	(in thousands)
Developed technology - metreleptin	$210,158	$(54,664)	$155,494
Developed technology - lomitapide	42,300	(10,165)	32,135
Total intangible assets	$252,458	$(64,829)	$187,629

	December 31, 2018
	Gross Carrying Value	Accumulated Amortization	Net Carrying Value
	(in thousands)
Developed technology - metreleptin	$210,158	$(44,084)	$166,074
Developed technology - lomitapide	42,300	(8,198)	34,102
Total intangible assets	$252,458	$(52,282)	$200,176

Amortization expense was $12.5 million for each of the six months ended June 30, 2019 and 2018.

As of June 30, 2019, the estimated amortization expense related to intangibles for future periods is as follows:

	Amount
Years Ending December 31,	(in thousands)
2019 (remaining 6 months)	$12,549
2020	25,095
2021	25,095
2022	25,095
2023	25,095
Thereafter	74,700
Total intangible assets subject to amortization	$187,629

7. Accrued Liabilities

Accrued liabilities consist of the following:

	June 30, 2019	December 31, 2018
	(in thousands)
Accrued employee compensation and related costs	$2,110	$2,108
Accrued professional fees	3,838	592
Accrued allowances: government rebates	17,077	19,637
Accrued royalties	9,950	5,112
Other accrued liabilities	16,774	14,907
Total	$49,749	$42,356

8. Leases

The Company leases office space in the U.S. and foreign countries for its operational offices. The Company’s U.S. operational office, which was located in Cambridge, Massachusetts, expired April 30, 2019. In April 2019, the Company entered two 12-month term leases for its U.S. operational offices, one located in Cambridge, Massachusetts, and one located in Boston, Massachusetts. The aggregate lease payment amounts for these two leases over the lease term are approximately $0.4 million. The international lease agreements expire at various dates through the year 2025. In addition, the Company leases certain office facilities, office equipment as well as vehicles on behalf of certain employees in the European regions.

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As of June 30, 2019, the right-of-use assets associated with the Company’s operating leases were $1.1 million, which were recorded in other non-current assets as reflected on the Company’s Consolidated Balance Sheet. The corresponding lease liabilities for the operating leases were $1.1 million as of June 30, 2019, of which $0.4 million represents as short-term lease liability and $0.7 million represents as long-term lease liability, and they were recorded in accrued liabilities and other non-current liabilities, respectively, as reflected on the Company’s Consolidated Balance Sheet as of June 30, 2019. The right-of-use assets represent the Company’s right to use an underlying asset during the lease term and the related lease liabilities represent the Company’s obligation to make lease payments arising from the lease. Both the right-of-use assets and the corresponding liabilities are recognized at the commencement date of the lease based upon the present value of lease payments over the lease term. As the Company’s leases do not provide an implicit rate, when determining the lease liabilities, the Company estimated the incremental borrowing rate based on the interest rate from the Bridge Loans entered in November 2018. The discount rate is calculated as the midpoint between the incremental borrowing rate and the implied yields available from U.S. Treasury securities equal to the original lease term.

The Company’s lease agreements generally do not contain purchase options. Certain leases have renewal options that can be exercised at the discretion of the Company, and the Company only includes renewal option in the lease term when it is reasonably certain to exercise such option.

As of June 30, 2019, the weighted average remaining lease term on the Company’s existing leases was 3.3 years, and the weighted average discount rate used to calculate the lease liabilities was 6.7%. The non-cash lease expense for the Company’s leases totaled $0.9 million for the six months ended June 30, 2019.

The following table summarizes the components of the lease expenses for the six months ended June 30, 2019. The variable lease expenses generally include common area maintenance, rent adjustment based on index and real estate taxes. All the lease expenses are recorded as operating expenses in the Consolidated Statement of Operations as of June 30, 2019. Cash paid for amounts included in the measurement of the operating lease liabilities for the six months ended June 30, 2019 was $0.9 million.

	Six Months Ended June 30, 2019
	(in thousands)
Operating lease expense	$931
Variable lease expense	20
Short-term lease expense	92
Total lease expense	$1,043

As of June 30, 2019, the estimated minimum lease payments for the next five years and thereafter is as follows:

	Amount
Year Ending December 31,	(in thousands)
2019 (remaining 6 months)	$245
2020	451
2021	211
2022	138
2023	98
Thereafter	41
Total lease payments	1,184
Less: Present value adjustment using incremental borrowing rate	122
Present value of operating lease liabilities	$1,062

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As of December 31, 2018, prior to the adoption of ASU 2016-02, the estimated minimum lease payments for the next five years and thereafter is as follows:

	Amount
Years Ending December 31,	(in thousands)
2019	$1,246
2020	432
2021	190
2022	134
2023	97
Thereafter	41
Total	$2,140

9. Loan Facilities

As a result of Aegerion’s bankruptcy filing in May 2019, the outstanding balance of the loan facilities, including the New Money Loans, Roll Up Loans and the Novelion Loan, were classified as liabilities subject to compromise as of June 30, 2019. Refer to Note 2, Bankruptcy, for further discussion and Note 15, Subsequent Events, for discussion related to the treatment of the loan facilities upon Aegerion’s emergence from bankruptcy in September 2019.

Short-term debt, exclusive of convertible notes, consists of the following as of June 30, 2019 and December 31, 2018:

	June 30, 2019
	New Money Loans	Roll Up Loans	Novelion Loan	Total
	(in thousands)
Short-term principal and commitment fee	$51,000	$22,500	37,264	$110,764
Exit fee payable	1,500	—	—	1,500
Accrued unpaid interest	2,967	242	1,182	4,391
Repayment of short-term principal, including exit fee	(2,996)	—	(2,106)	(5,102)
Total short-term debt	$52,471	$22,742	$36,340	$111,553

	December 31, 2018
	New Money Loans	Roll Up Loans	Novelion Loan	Total
	(in thousands)
Short-term principal and commitment fee	$51,000	$22,500	$37,777	$111,277
Exit fee payable	1,500	—	—	1,500
Accrued unpaid interest	826	66	2,987	3,879
Unamortized debt issuance costs	(1,054)	—	—	(1,054)
Debt discount	(1,161)	—	—	(1,161)
Repayment of principal	—	—	(3,500)	(3,500)
Total short-term debt	$51,111	$22,566	$37,264	$110,941

Bridge Loans

On November 8, 2018, Aegerion entered into a bridge credit agreement (“Bridge Credit Agreement”) with certain funds managed by Highbridge, and Athyrium, as lenders (“Bridge Lenders”), and Cantor Fitzgerald Securities, as agent (“Bridge Agent”), under which Aegerion borrowed from the Bridge Lenders secured first lien term loans in cash in an original aggregate principal amount of $50.0 million (“New Money Loans”) and $22.5 million of secured term loans that were funded, on behalf of Aegerion, to repurchase and retire an equal amount of Convertible Notes, at par, held by certain funds managed by the Bridge Lenders (“Roll Up Loans”). The Roll Up Loans and the New Money Loans comprise the Bridge Loans referenced above.

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The Bridge Loans mature on the earliest to occur of (i) certain restructuring or bankruptcy events, (ii) June 30, 2019, and (iii) the acceleration after occurrence of an event of default. Refer to Note 15, Subsequent Events, for further discussion related to Aegerion’s bankruptcy filing in May 2019 and the related impact to the Bridge Loans. In January 2019, the maturity date of the Bridge Loans was extended to June 30, 2019, upon the exercise of Aegerion’s option and the satisfaction by Aegerion of the conditions stated in the Bridge Credit Agreement. In connection with the extension of the maturity date, Aegerion repaid $3.0 million of New Money Loans principal, including exit fee, and paid an extension fee in the amount of $1.5 million to the Bridge Lenders.

The New Money Loans accrue interest at the rate of 11.00% per annum and the Roll Up Loans accrue interest at the rate of 2.00% per annum. Following an event of default and so long as an event of default is continuing, the interest rate on each of the New Money Loans and the Roll Up Loans would increase by 2.00% per annum. Interest on the New Money Loans and the Roll Up Loans accrue and compound quarterly in arrears and will not be payable in cash until the maturity date or any earlier time that the Bridge Loans become due and payable under the Bridge Credit Agreement. Aegerion incurred a commitment fee equal to 2.00% of the New Money Loans, which will be paid in kind and is included in the outstanding principal amount of the New Money Loans. The New Money Loans may be prepaid, in whole or in part, by Aegerion at any time subject to payment of an exit fee (including at maturity) equal to 3.00% of the commitments with respect to New Money Loans.

Aegerion’s obligations under the Bridge Credit Agreement are guaranteed by each domestic subsidiary of Aegerion other than Aegerion Securities Corporation, a Massachusetts corporation (“Guarantors”), and secured by a lien on substantially all of the assets of Aegerion and the Guarantors, including a pledge of 65% of Aegerion’s and the Guarantors’ first-tier foreign subsidiaries’ equity interests and substantially all of the intellectual property and related rights in respect of MYALEPT and JUXTAPID, subject to certain contractual limitations and exclusions set forth in the Bridge Credit Agreement and related documentation. The liens on the assets of Aegerion and the Guarantors granted to secure Aegerion’s obligations to the Bridge Lenders with respect to New Money Loans are senior to the liens granted to secure Aegerion’s obligations to Novelion with respect to the Novelion Loan. The liens on the assets of Aegerion and the Guarantors granted to secure Aegerion’s obligations to the Bridge Lenders with respect to Roll Up Loans are junior to the liens granted to secure Aegerion’s obligations to Novelion with respect to the Novelion Loan.

The Bridge Credit Agreement includes affirmative and negative covenants binding on Aegerion and its subsidiaries, including prohibitions on the incurrence of additional indebtedness, granting of liens, certain asset dispositions, investments and restricted payments, in each case, subject to certain exceptions set forth in the Bridge Credit Agreement. The Bridge Credit Agreement also includes customary events of default for a transaction of this type, and includes (i) a cross-default to the occurrence of any event of default under material indebtedness of Aegerion, including the Convertible Notes or the Novelion Loan, and (ii) Novelion or any of its subsidiaries being subject to bankruptcy or other insolvency proceedings. Upon the occurrence of an event of default, the Bridge Lenders may declare all of the outstanding Bridge Loans and other obligations under the Bridge Credit Agreement to be immediately due and payable and exercise all rights and remedies available to the Bridge Lenders under the Bridge Credit Agreement and related documentation. Refer to Note 15, Subsequent Events, for further discussion related to Aegerion’s bankruptcy filing in May 2019 and the related impact to the Bridge Loans.

Pursuant to the terms of the Bridge Credit Agreement and after taking into account amounts payable to UPenn, as discussed in Note 4, License Agreement, Aegerion retained $15.0 million of the remaining upfront payment, of which $12.0 million is to be used in accordance with a proceeds reinvestment budget (which primarily relates to the development activities related to MYALEPT as a potential treatment for PL in the U.S.), and the balance is to be used in accordance with a general budget of Aegerion, in each case, subject to certain restrictions. Forty-two percent of the remaining net cash proceeds (less amounts owed to UPenn and amounts paid as transaction costs) was paid to Novelion to repay a portion of the outstanding Novelion Loan comprising the portion of the loan that the Bridge Lenders agreed not be contested and 58% was paid to the Bridge Lenders to repay a portion of the outstanding Bridge Loans.

In connection with the Amended and Restated Novelion Loan Agreement (defined below) and the Bridge Credit Agreement, Aegerion was required to enter into separate deposit account control agreements with each of the lenders in order to perfect each lender’s security interest in the cash collateral in Aegerion’s operating account. In the event of a default under either loan agreement, subject to the terms of the subordination agreement with the Bridge Lenders and Bridge Agent (“Bridge Intercreditor Agreement”), the respective lender would have the right to take control of the operating account and restrict Aegerion’s access to the operating account and the funds therein.

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In addition to the repurchase and cancellation of certain Convertible Notes with the proceeds of the Roll Up Loans, Aegerion used proceeds of the New Money Loans to repay, at par, (a) the amounts outstanding under the Shareholder Term Loan Agreement described below, in an aggregate principal amount of approximately $21.2 million, and (b) principal prepayments of the Novelion Loan in an amount of $3.5 million, in 2018.

Shareholder Term Loan Agreement

On March 15, 2018, Aegerion entered into a loan and security agreement with affiliates of Broadfin Capital, LLC and Sarissa Capital Management LP (“Shareholder Term Loan Agreement”), pursuant to which the lenders made a single-draw term loan to Aegerion in an aggregate amount of $20.0 million (“Shareholder Term Loans”), and secured by substantially all of Aegerion’s assets. The lenders or their affiliates were Novelion’s shareholders given their investments in Novelion’s common shares, and two members of Novelion’s Board of Directors at that time were affiliates of the lenders.

The Company accrued unpaid interest and recorded amortization of debt issuance costs, which was recognized as interest expense, in the Consolidated Statement of Operations during the six months ended June 30, 2018.

The Company determined that the acceleration of the maturity date upon the occurrence of a Convertible Notes restructuring was an embedded derivative, which required bifurcation and was separately ascribed with a fair value. The fair value of the embedded derivative liability on the Shareholder Term Loans issuance date was calculated by determining the fair value of the Shareholder Term Loans with and without the acceleration of the maturity date upon an occurrence of a Convertible Notes restructuring, using the same methodology and inputs in determining the fair value of the Shareholder Term Loans. The difference between the two fair values was determined to be the fair value of the embedded derivative liability. Accordingly, the Company initially recorded a derivative liability of $0.9 million as a reduction to debt payable, and the derivative liability was revalued on each reporting date, prior to the repayment of the Shareholder Term Loans in November 2018.

In connection with the entry into the Bridge Loans in November 2018, as discussed above, the Shareholder Term Loans were paid in full in 2018. At the time of repayment, the outstanding principal of the Shareholder Term Loans totaled approximately $21.2 million, including paid in kind interest that had been added to the principal of the Shareholder Term Loans. There were no other penalties associated with the repayments.

Novelion Loan

On June 14, 2016, Novelion and Aegerion entered into a term loan agreement, in which Novelion agreed to make term loans, from time to time, in an aggregate principal amount not to exceed (i) $3.0 million in any calendar month and (ii) $15.0 million in the aggregate, excluding any paid in kind interest that has been capitalized. Interest was accrued at 8% and would be paid in kind and added to the outstanding principal amount of the term loan.

In connection with the entry into the Shareholder Term Loan Agreement, on March 15, 2018 Aegerion and Novelion entered into an amended and restated senior secured term loan agreement, which has a maturity date of July 1, 2019 (“Amended and Restated Novelion Loan Agreement”), which amends and restates the secured loan facility between Aegerion and Novelion that was first entered into in connection with the Novelion Loan prior to the acquisition of Aegerion. As of June 30, 2019, the principal amount outstanding under the Novelion Loan was approximately $36.3 million, which includes $1.2 million of accrued unpaid interest for the six months ended June 30, 2019. During the first quarter of 2019, Aegerion used the net cash proceeds from the upfront payment received from Recordati, as discussed in Note 4, License Agreement, to repay $2.1 million of the outstanding Novelion loan.

In connection with, and as a condition to, Aegerion’s entering into the Bridge Credit Agreement, Novelion, as lender under the existing Amended and Restated Novelion Loan Agreement consented to Aegerion’s incurrence of the Bridge Loans and the repayment of the Shareholder Term Loans, and amended the Amended and Restated Novelion Loan Agreement (“Consent and Amendment”). Novelion also agreed to subordinate the Novelion Loan to the New Money Loans pursuant to a subordination agreement with the Bridge Lenders and Bridge Agent (“Bridge Intercreditor Agreement”). Under the terms of the Bridge Intercreditor Agreement, the New Money Loans and the liens securing the New Money Loans are senior to the liens securing the Novelion Loan. The Novelion Loan is not subordinated to the Roll Up Loans; however, under the terms of the Bridge Intercreditor Agreement, the liens securing the Roll Up Loans rank junior to the liens securing the Novelion Loan. Refer to Note 15, Subsequent Events, for further discussion related to Aegerion’s bankruptcy filing in May 2019 and the related impact to the Novelion Loan.

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10. Convertible Notes, net

The Convertible Notes are senior unsecured obligations of Aegerion and bear interest at a rate of 2.0% per year, payable semi-annually in arrears on February 15 and August 15, and had an effective interest rate of 16.42%, established as of the consummation of Novelion’s acquisition of Aegerion until the effective interest rate became 17.56% as a result of the retirement of a portion of the Convertible Notes in November 2018, as discussed in Note 9, Loan Facilities. The Convertible Notes matured on August 15, 2019, unless earlier repurchased, converted or renegotiated. Refer to Note 15, Subsequent Events, for further discussion related to Aegerion’s bankruptcy filing in May 2019 and the related impact to the Convertible Notes.

The outstanding Convertible Notes balances as of June 30, 2019 and December 31, 2018 was $296.7 million and $274.8 million, respectively. As permitted in Aegerion’s First Amended Joint Chapter 11 Plan (the “Plan”), which was filed with the Bankruptcy Court on July 2, 2019, the Convertible Noteholders were entitled to vote on the Plan by August 15, 2019. As discussed in Note 15, Subsequent Events, the Bankruptcy Court entered an order confirming the Plan in September 2019. Based on that, the Company did not have sufficient information available to reasonably estimate the amount of the allowed claim for the Convertible Notes as of June 30, 2019. Accordingly, the Company did not adjust the net carrying amount of the Convertible Notes to the amount of the allowed claim as of June 30, 2019.

The following table sets forth total interest expense recognized related to the Convertible Notes during the six months ended June 30, 2019 and 2018:

	Six Months Ended June 30,
	2019	2018
	(in thousands)
Contractual interest expense	$3,025	$3,250
Amortization of debt discount	21,897	18,605
Total	$24,922	$21,855

Subsequent to June 30, 2019, the future minimum payments under the Convertible Notes are as follows, and they were originally due on August 15, 2019 as governed by the terms. Refer to Note 15, Subsequent Events, for further discussion related to Aegerion’s bankruptcy filing in May 2019 and the related impact to the Convertible Notes.

	Amount
	(in thousands)
Principal	$302,498
Interest	3,025
Total	$305,523

11. Related Party Transactions

The Company has the following related party transactions with Novelion:

Novelion Loan

As described in Note 9, Loan Facilities, in June 2016, the Company entered into a term loan agreement with Novelion, which was amended and restated from time to time. As of June 30, 2019 and December 31, 2018, the principal amount outstanding under the Novelion Loan was approximately $36.3 million and $37.3 million, respectively which included accrued unpaid interest of $1.2 million and $3.0 million, respectively.

Shared Service Agreement

Aegerion entered into a shared service agreement with Novelion. As part of the agreement, Novelion provides executive, business development and administrative service to Aegerion. Aegerion provides back office type services such as finance and human resources to Novelion. Each party is compensated for their services rendered with a reimbursement of costs plus a margin on a quarterly basis. As part of the shared service agreement,

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Aegerion recognized $0.8 million and $1.9 million of related party income and incurred $1.2 million and $2.5 million of related party expense for the six months ended June 30, 2019 and 2018, respectively. As of June 30, 2019 and December 31, 2018, the net payables due to Novelion was $9.0 million and $11.0 million, respectively.

12. Enterprise-wide information

Net Revenues

The following table summarizes total net revenues from external customers by product and by geographic region, based on the location of the customer, for the six months ended June 30, 2019 and 2018.

	Six Months Ended June 30, 2019
	U.S.	Japan	Germany	Brazil	Other Foreign Countries	Total
	(in thousands)
Metreleptin	$24,891	$62	$5,191	$—	$8,771	$38,915
Lomitapide	18,710	32,642	79	—	5,511	56,942
Total	$43,601	$32,704	$5,270	$—	$14,282	$95,857

	Six Months Ended June 30, 2018
	U.S.	Japan	Germany	Brazil	Other Foreign Countries	Total
	(in thousands)
Metreleptin	$22,248	$48	$787	$1,170	$5,498	$29,751
Lomitapide	19,100	4,803	—	—	5,734	29,637
Total	$41,348	$4,851	$787	$1,170	$11,232	$59,388

Net revenues generated from customers outside of the U.S., Japan, Germany and Brazil, as listed in the column “Other Foreign Countries,” were primarily derived from Colombia and France during the six months ended June 30, 2019 and from Colombia during the six months ended June 30, 2018.

Significant Customers

For the six months ended June 30, 2019, two customers accounted for 76% of the Company’s net revenues and one of them accounted for 47% of its June 30, 2019 accounts receivable balance. For the six months ended June 30, 2018, one customer accounted for 70% of Aegerion’s net revenues and 51% of its June 30, 2018 accounts receivable balance.

Long-lived Assets

The Company’s long-lived assets are primarily comprised of intangible assets and property and equipment. As of June 30, 2019 and December 31, 2018, 64% and 65% of the intangible assets were attributable to Aegerion’s U.S. business, with the remaining intangible assets attributable to its European holding company, respectively.

As of June 30, 2019 and December 31, 2018, 57% and 75%, respectively, of Aegerion’s property and equipment resided in its U.S. subsidiaries, with the remaining assets residing in the foreign subsidiaries.

13. Commitments and Contingencies

The Company accrues a liability for legal contingencies when it believes that it is both probable that a liability has been incurred and that it can reasonably estimate the amount of the loss. The Company reviews these accruals and adjusts them to reflect ongoing negotiations, settlements, rulings, advice of legal counsel and other relevant information. To the extent new information is obtained and the Company’s views on the probable outcomes of claims, suits, assessments, investigations or legal proceedings change, changes in the Company’s loss contingency accrual would be recorded in the period in which such determination is made.

F-121

DOJ/SEC Investigations

In late 2013, Aegerion received a subpoena from the DOJ, represented by the U.S. Attorney’s Office in Boston, requesting documents regarding its marketing and sale of JUXTAPID in the U.S., as well as related public disclosures (“DOJ investigation”). In late 2014, Aegerion received a subpoena from the SEC requesting certain information related to Aegerion’s sales activities and disclosures related to JUXTAPID. The SEC also requested documents and information on a number of other topics, including documents related to the investigations by government authorities in Brazil into whether Aegerion’s activities in Brazil violated Brazilian anti-corruption laws, and whether Aegerion’s activities in Brazil violated the Foreign Corrupt Practices Act (“FCPA”). As a result of the SEC’s investigation, Aegerion consented to the entry of a final judgment, on September 25, 2017, in connection with a complaint filed by the SEC without admitting or denying the allegations set forth in the complaint (“SEC Judgment”). The complaint alleged negligent violations of Sections 17(a)(2) and (3) of the Securities Act of 1933, as amended, related to certain statements made by Aegerion in 2013 regarding the conversion rate for JUXTAPID prescriptions.

The SEC Judgment, which was approved by a U.S. District Court judge on September 25, 2017, provides that Aegerion must pay a civil penalty in the amount of $4.1 million, to be paid in installments over three years, plus interest on any unpaid balance at a rate of 1.75% per annum. As of June 30, 2019, $0.9 million remains due as a current liability, and $0.2 million remains due as a non-current liability. Aegerion’s payment of this civil penalty is subject to acceleration in the event of certain change of control transactions or certain transfers of Aegerion’s rights in MYALEPT or JUXTAPID. Aegerion’s payment schedule is also subject to acceleration in the event that Aegerion fails to satisfy its payment obligations under the SEC Judgment.

In connection with the DOJ investigation, Aegerion entered into a Plea Agreement, a Deferred Prosecution Agreement (“DPA”), a Civil Settlement, certain State Settlement Agreements and a Consent Decree of Permanent Injunction (“FDA Consent Decree”). Under the Court-approved DOJ Plea Agreement, Aegerion pled guilty to two misdemeanor misbranding violations of the Federal Food, Drug, and Cosmetic Act (“FDCA”) and on January 30, 2018, a U.S. District Court Judge sentenced Aegerion.

The Court did not impose a criminal fine and instead ordered Aegerion to pay restitution, in the amount of $7.2 million payable over three years, plus interest on any unpaid balance at a rate of 1.75% per annum, into a fund managed by an independent claims administrator. As of June 30, 2019, $1.9 million remains due as a current liability, and $1.4 million remains due as a non-current liability. As contemplated by the Plea Agreement, Aegerion was further sentenced to a three-year term of probation. Among the terms of probation, Aegerion must (i) comply with federal, state and local laws, (ii) notify its probation officer of any prosecution, major civil litigation or administrative proceeding, (iii) seek permission of its probation officer prior to selling, assigning or transferring assets, (iv) notify its probation officer of any material change in its economic circumstances, (v) forbear from disparaging the factual basis of Aegerion’s plea or denying that Aegerion itself is guilty, and (vi) comply with the DPA and Corporate Integrity Agreement (“CIA”) (and submit certain reports prepared thereunder to its probation officer). Under the terms of the DPA, Aegerion admitted it engaged in conduct that constituted a conspiracy to violate the Health Insurance Portability and Accountability Act (“HIPAA”). The DPA provides that Aegerion must continue to cooperate fully with the DOJ concerning its investigation into other individuals or entities. The DPA provides that Aegerion must maintain a robust compliance and ethics program that includes significant certification, training, monitoring and other requirements. Aegerion, as well as Novelion’s Board of Directors (or a designated committee thereof), must also conduct regular reviews of its compliance and ethics program, provide certifications to the DOJ that the program is believed to be effective and notify the DOJ of any probable violations of HIPAA. In the event Aegerion breaches the DPA, there is a risk the government would seek to impose remedies provided for in the DPA, including instituting criminal prosecution against Aegerion and/or seeking to impose stipulated penalties against Aegerion. The DPA is subject to supervision by a U.S. District Court judge.

Aegerion also entered into the DOJ Civil Settlement Agreement to resolve allegations by the DOJ that false claims for JUXTAPID were submitted to governmental healthcare programs. The DOJ Civil Settlement Agreement requires Aegerion to pay a civil settlement in the amount of $28.8 million, which includes up to $2.7 million designated for certain U.S. states relating to Medicaid expenditures for JUXTAPID, to be paid in installments over three years. As of June 30, 2019, $11.3 million remains due as a current liability, and $10.3 million remains due as a non-current liability. Aegerion’s payment of this civil settlement amount is subject

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to acceleration in the event of certain change of control transactions or certain transfers of Aegerion’s rights in MYALEPT or JUXTAPID. In the event that Aegerion fails to satisfy its obligations under the DOJ Civil Settlement Agreement, Aegerion could be subject to additional penalties or litigation.

Aegerion also agreed to enter into the State Settlement Agreements to resolve claims under state law analogues to the federal False Claims Act. The terms of the State Settlement Agreements are substantially similar to those set forth in the DOJ Civil Settlement Agreement. As noted above, participating states will receive up to $2.7 million in the aggregate from the $28.8 million amount to be paid pursuant to the DOJ Civil Settlement Agreement.

Aegerion also agreed to the FDA Consent Decree with the DOJ and the FDA to resolve a separate civil complaint alleging that Aegerion violated the FDCA by failing to comply with the JUXTAPID REMS program and the requirement to provide adequate directions for all of the uses for which it distributed JUXTAPID. The FDA Consent Decree requires Aegerion, among other things, to comply with the JUXTAPID REMS program; retain a qualified independent auditor to conduct annual audits of its compliance with the JUXTAPID REMS program; and remediate any noncompliance identified by the auditor within specified timeframes. In the event Aegerion fails to comply with the JUXTAPID REMS program or any other provisions of the FDA Consent Decree, Aegerion could be subject to additional administrative remedies, civil or criminal penalties and/or stipulated damages. Aegerion is required to notify the FDA in advance of certain changes in control, or changes in its business that may affect its operations, assets, rights or liabilities in the United States. On March 20, 2019, the Court entered the FDA Consent Decree.

Separately, Aegerion entered into a CIA with the Department of Human Services Office of the Inspector General (“OIG”). The CIA requires Aegerion, among other things, to maintain a compliance program with significant requirements relating to, among other things, training, monitoring, annual risk assessment and mitigation processes, independent review of Aegerion’s compliance and other activities, a disclosure program and an executive financial recoupment program. Under the CIA, Aegerion, as well as the Board of Directors of the Company (or a designated committee thereof), must also conduct regular reviews of Aegerion’s compliance program and provide an annual resolution or certification to OIG that the program is believed to be effective. Additionally, Aegerion has certain certification and reporting obligations under the CIA. In the event Aegerion breaches the CIA, there is a risk the government would seek to impose remedies provided for in the CIA, including seeking to impose stipulated penalties against Aegerion and/or seeking to exclude Aegerion from participation in federal healthcare programs.

Investigations in Brazil

Federal prosecutors in Brazil are conducting an investigation to determine whether there have been violations of Brazilian laws related to the sales of JUXTAPID in Brazil. In July 2016, the Ethics Council of Interfarma fined Aegerion’s subsidiary in Brazil (“Aegerion Brazil”) approximately $0.5 million for violations to its Code of Conduct, to which Aegerion Brazil is bound due to its affiliation with Interfarma. Also, the Board of Directors of Interfarma imposed an additional penalty of suspension of Aegerion Brazil’s membership, without suspension of Aegerion Brazil’s membership contribution, for a period of 180 days for Aegerion Brazil to demonstrate the implementation of effective measures to cease alleged irregular conduct, or exclusion of the Company’s membership in Interfarma if such measures are not implemented. Aegerion Brazil paid the fine of approximately $0.5 million during the third quarter of 2016. In March 2017, after the suspension period ended, Interfarma’s Board of Directors decided to reintegrate Aegerion Brazil, enabling it to participate regularly in Interfarma activities, subject to meeting certain obligations. In April 2019, the Board of Directors of Interfarma agreed that Aegerion Brazil has successfully met all of the requirements imposed by the association, and the investigation was closed.

Also, in July 2016, Aegerion Brazil received an inquiry from a Public Prosecutor Office of the Brazilian State of Paraná asking it to respond to questions related to media coverage regarding JUXTAPID and its relationship with a patient association to which Aegerion made donations for patient support. This preliminary inquiry was later reclassified as a civil inquiry, which is a preliminary procedure by the Public Prosecutor’s Office that aims to verify if there are enough elements for it to file a formal lawsuit or to dismiss the inquiry. In March 2018, the Paraná State Public Prosecutor’s Office sent the civil inquiry to the Federal Public Prosecutor’s Office, after deciding that the potential case should be subject to federal jurisdiction. The Federal Public Prosecutor dismissed the case in January 2019.

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In June 2017, the Federal Public Prosecutor of the City of São José dos Campos, State of São Paulo, in connection with its criminal investigation into former employees of Aegerion Brazil, requested that a Brazilian federal court provide federal investigators with access to the bank records of certain individuals and entities, including Aegerion Brazil, certain former Aegerion Brazil employees, a Brazilian patient association, and certain Brazilian physicians. The Federal Trial Court Judge issued a decision on July 12, 2018 authorizing the access to the banking records on the terms that the Federal Public Prosecutor of the City of São José dos Campos had requested. On July 16, 2018, Aegerion Brazil filed an appeal of the decision that authorized the breach of the banking secrecy, which was denied by the Federal Court Judge. The Public Prosecutor in São José dos Campos continues to gather information in connection with this investigation. At this time, the Company does not know whether the inquiry of the Public Prosecutor in São José dos Campos will result in the commencement of any formal proceeding against Aegerion, but if Aegerion’s activities in Brazil are found to violate any laws or governmental regulations, Aegerion may be subject to significant civil lawsuits to be filed by the Public Prosecution office, and administrative penalties imposed by Brazilian regulatory authorities and additional damages and fines. Under certain circumstances, Aegerion could be barred from further sales to federal and/or state governments in Brazil, including sales of JUXTAPID and/or MYALEPT, due to penalties imposed by Brazilian regulatory authorities or through civil actions initiated by federal or state public prosecutors. Additionally, the SEC conducted inquiries with Aegerion concerning the investigations by Brazilian authorities, and in July 2019, the SEC concluded the investigation and no enforcement action was made against Aegerion. As of June 30, 2019, the Company cannot determine if a loss is probable as a result of the investigations and inquiry in Brazil and whether the outcome will have a material adverse effect on the Company’s business and, as a result, no amounts have been recorded for a loss contingency.

Qui Tam Litigation

In March 2014, an amended qui tam complaint was filed under seal in the District of Massachusetts against Aegerion, two former executive officers and a former employee. On September 22, 2017, the U.S. filed a notice of intervention as to Aegerion. On September 27, 2017, the qui tam relators filed a second amended complaint naming additional parties, including a former board member, former executives, and former employees of Aegerion, as well as other third parties. The second amended complaint noted that the relators would file a joint stipulation of dismissal with respect to Aegerion upon the completion of certain conditions set forth in the Civil Settlement Agreement. On October 27, 2017, the court granted Aegerion and relators’ joint motion to stay proceedings until sentencing in the criminal matter is complete. On February 20, 2018, Aegerion was dismissed from the qui tam lawsuit. On June 5, 2018, two of the remaining defendants were dismissed from the lawsuit and on June 19, 2018, the remaining individual defendants filed a motion to dismiss the qui tam lawsuit. On March 31, 2019, the Court granted the Motion to Dismiss with respect to one defendant, and denied the motion with respect to the other remaining defendants. On April 17, 2019, the remaining defendants filed a motion to dismiss for lack of jurisdiction. Although Aegerion is not a party to the lawsuit, it could be liable for certain defense costs and damages for defendants remaining in the lawsuit. Although the Company does not believe the outcome of the lawsuit will have a material adverse effect on the Company, the Company cannot determine if a loss is probable as a result of the lawsuit and, as a result, no amounts have been recorded for a loss contingency.

14. Condensed Combined Debtor-in-Possession Financial Information

The financial statements below represent the unaudited condensed combined financial statements of the Debtors. As of and for the six months ended June 30, 2019, the results of the Non-Filing Entities are not included in these unaudited condensed combined financial statements. Intercompany transactions among the Debtors have been eliminated in the financial statements contained herein. Intercompany transactions among the Debtors and the Non-Filing Entities have not been eliminated in the Debtors’ financial statements.

F-124

Debtors’ Unaudited Condensed Combined Balance Sheet

	June 30, 2019
	(In thousands)
Assets
Current assets:
Cash and cash equivalents	$28,462
Accounts receivable, net	16,138
Inventories - current	3,779
Prepaid expenses and other current assets	4,896
Total current assets	53,275
Inventories - non-current	10,733
Property and equipment, net	429
Intangible assets, net	120,754
Other non-current assets	1,731
Investment in subsidiaries	100,830
Intercompany receivable	56,763
Total assets	$344,515
Liabilities and shareholders’ deficit
Current liabilities:
Accounts payable	$500
Accrued liabilities	39,075
Provision for legal settlements - current	14,070
Total current liabilities	53,645
Long-term liabilities:
Provision for legal settlements - non-current	11,962
Other non-current liabilities	32
Intercompany payables	8,708
Total liabilities not subject to compromise	74,347
Liabilities subject to compromise	420,651
Total liabilities	494,998
Commitments and contingencies (Note 13)
Shareholders’ deficit:
Total shareholders’ deficit	(150,483)
Total liabilities and shareholders’ deficit	$344,515

F-125

Debtors’ Unaudited Condensed Combined Statement of Operations

	Six Months Ended June 30, 2019
	(In thousands)
Net revenues	$81,551
Cost of product sales	34,887
Operating expenses:
Selling, general and administrative	37,298
Research and development	10,256
Intercompany expense, net	546
Related party expense, net	397
Total operating expenses	48,497
Loss from operations	(1,833)
Reorganization items, net	(2,145)
Interest expense, net (contractual interest of $690)	(29,672)
Interest expense due to Novelion (contractual interest of $352)	(1,182)
Other income, net	289
Loss before provision for income taxes	(34,543)
Provision for income taxes	(186)
Net loss	$(34,729)
Comprehensive loss	$(34,729)

F-126

Debtors’ Condensed Combined Statement of Cash Flows

	Six Months Ended June 30, 2019
	(In thousands)
Cash provided by (used in) operating activities
Net loss	$(34,729)
Adjustments to reconcile net loss to net cash used in operating activities:
Depreciation	461
Non-cash lease expense	686
Amortization of intangible assets	8,614
Stock-based compensation	676
Non-cash interest expense	24,214
Non-cash interest expense due to Novelion	1,182
Unrealized foreign exchange (gain) loss	(414)
Amortization of debt issuance costs and debt discount	2,215
Other non-cash operating activities	(8)
Loss on disposal of property and equipment	140
Changes in assets and liabilities:
Accounts receivable	(42)
Inventories	1,246
Prepaid expenses and other assets	1,898
Intercompany activities	4,461
Accounts payable	1,781
Accrued liabilities and other liabilities	(200)
Payable due to Novelion	(2,659)
Net cash provided by operating activities	9,522
Net cash used in investing activities	—
Cash used in financing activities
Repayment of Novelion Loan	(2,106)
Repayment of Bridge Loans	(2,996)
Net cash used in financing activities	(5,102)
Exchange rate effect on cash	—
Net decrease in cash and cash equivalents	4,420
Cash and cash equivalents, beginning of period	24,042
Cash and cash equivalents, end of period	$28,462

F-127

15. Subsequent Events

The Company has evaluated subsequent events through February 18, 2020, which is the date that financial statements were available to be issued. Refer to the below for the material subsequent events occurred since June 30, 2019.

Chapter 11 Bankruptcy Filing and Amryt Acquisition

On May 20, 2019, Aegerion and its U.S. subsidiary, Aegerion Pharmaceuticals Holdings, Inc. filed the Bankruptcy Petitions under Chapter 11 of the Bankruptcy Code in the Bankruptcy Court. On September 10, 2019, the Bankruptcy Court entered the Confirmation Order confirming Aegerion’s First Amended Joint Chapter 11 Plan (“Plan”), as modified to reflect certain resolutions agreed to among various parties. The effective date of the Plan (“Effective Date”) is subject to the satisfaction of all conditions precedent to the Plan, as further described in the Plan.

In accordance with the Plan, the Debtors will continue to exist after the Effective Date as Reorganized Debtors. On and after the Effective Date, all property of the estates, wherever located, including all claims, rights and causes of action, as defined in the Plan, and any property, wherever located, acquired by the Debtors under or in connection with this Plan, shall revest in the Reorganized Debtors, as applicable, free and clear of all claims, liens, charges, other encumbrances and interests.

As a result of the Plan, a significant amount of the Company’s indebtedness, as summarized in the following, was discharged upon Aegerion emergence from bankruptcy in 2019.

• New Money Loans and Roll Up Loans − the full amount, including the accrued unpaid interest, was discharged. Each holder of the New Money Loans received secured credit facility issued by Aegerion at the Closing on a dollar for dollar basis on account of its New Money Loan claim; each holder of the Roll Up Loans received new convertible notes issued by Aegerion at the Closing on a dollar for dollar basis on account of its Roll Up Loan claim.

• Novelion Loan – the full amount, including the accrued unpaid interest, was discharged. Novelion received American depositary receipts representing approximately 14.0 million Ordinary Shares, as discussed below.

• Convertible Notes – the outstanding unpaid principal and interest were discharged. Each Convertible Noteholders received its pro rata share of the net remaining new convertible notes and certain Amryt common stock.

• Outstanding settlements due to DOJ and SEC – The Company will continue to pay the outstanding settlements to DOJ and SEC in accordance with payment terms as set forth in the original settlement agreements.

• Ongoing trade transactions – The Company will continue to follow the respective payment terms with each vendor. Any outstanding amount that was associated to the pre-Petition period but was put on hold for payment during the bankruptcy period was fully paid in cash in September 2019, plus post-petition interest at the applicable interest rate.

• Payables due to Novelion – the outstanding payables due to Novelion were discharged.

Additionally, as a result of confirmation of the Plan, the Bankruptcy Court has authorized Aegerion to consummate the transactions contemplated by the Plan, including the Amryt Transaction. Following the entry of the Confirmation Order, Amryt’s shareholders approved the Amryt Transaction at a meeting held on September 19, 2019.

On September 24, 2019, Aegerion completed the Amryt Transaction (“Closing”). The consideration for the Amryt Transaction has been satisfied through the issuance of ordinary Amryt shares (“Ordinary Shares”), or American depositary receipts representing Ordinary Shares, to stakeholders of Aegerion. Through the Amryt Transaction, Novelion has been divested of its operating subsidiary, Aegerion. In full satisfaction of its claims as creditor under Novelion Loan, Novelion received American depositary receipts representing approximately 14.0 million Ordinary Shares (“Novelion Consideration”). Novelion only received the Novelion Consideration and did not receive any other equity or property in connection with the Amryt Transaction.

*** ***

F-128

Through and including      , 2020 (the 25^th day after the date of this prospectus), all dealers effecting transactions in these securities, whether or not participating in this offering, may be required to deliver a prospectus.

162,876,633 Ordinary Shares

 

AMRYT PHARMA PLC

Represented by Approximately 32,575,327 American Depositary Shares

 

     , 2020

PART II — INFORMATION NOT REQUIRED IN PROSPECTUS

Item 6.
Indemnification of Directors and Officers.

To the extent permitted by law, pursuant to our Articles of Association, current and former members of the Company’s board of directors and its current and former officers shall be entitled to, and current and former directors and officers of certain affiliated companies may, at the discretion of the Company’s board of directors, be indemnified by the Company against all liabilities, costs, charges and expenses incurred by him or her in the execution and discharge of his or her duties to the Company and certain affiliated companies, including any liability incurred in defending any proceedings, civil or criminal, relating to the execution and discharge of his or her duties to the Company and certain affiliated companies.

However, pursuant to the Companies Act, no such indemnity shall extend to any liability attaching to a director in connection with any negligence, default, breach of duty or breach of trust in relation to the Company. This prohibition does not apply to “qualifying third party indemnities,” being indemnities provided against liability incurred by a director to a person other than the Company or an associated company (as defined in the Companies Act), except that no such qualifying third party indemnities can provide indemnification against:

(i)
any fine imposed in any criminal proceedings;

(ii)
any sum payable to a regulatory authority by way of a penalty in respect of non-compliance with any requirement of a regulatory nature howsoever arising;

(iii)
any liability incurred in defending any criminal proceedings in which he or she is convicted and such conviction has become final;

(iv)
any liability incurred in defending any civil proceedings brought by the Company or any associated company in which a final judgment has been given against him or her; and

(v)
any liability incurred in connection with any application for relief under certain provisions of the Companies Act in which the court refuses to grant him or her relief and such refusal has become final.

In addition, the Company is not prevented from purchasing and maintaining for a director of the Company, or of an associated company, insurance against any such liability.

Insofar as indemnification of liabilities arising under the Securities Act may be permitted to our board, executive officers, or persons controlling us pursuant to the foregoing provisions, we have been informed that, in the opinion of the SEC, such indemnification is against public policy as expressed in the Securities Act and is therefore unenforceable.

Item 7.
Recent Sales of Unregistered Securities.

Issuances of Capital Stock

• On October 9, 2017, the registrant issued 66,477,651 ordinary shares (11,079,608 equivalent ordinary shares post the six for one share consolidation in July 2019) to certain new and existing investors for aggregate consideration of £13.3 million; and

• On August 27, 2019, the registrant's predecessor agreed to issue 7,346,189 ordinary shares to certain new and existing investors for aggregate consideration of $8 million.

II-1

Options

Since May 1, 2017, the registrant (or its predecessor) has granted stock options to purchase an aggregate of 12,324,310 ordinary shares with exercise prices ranging from £0.7584 to £1.5528 per share, to certain employees, contractors and directors in connection with services provided to the registrant by such parties, as follows:

Grant Date	Number of options	Exercise price per share
May 25, 2017	55,572	£1.5528
July 12, 2017	98,563	£1.3500
September 12, 2017	31,909	£1.4250
September 19, 2017	88,210	£1.5000
November 28, 2017	719,415	£1.2072
May 21, 2019	1,115,241	£0.7584
November 5, 2019	10,215,400	£1.2150

Certain of these options have since lapsed. The number of options and exercise price per share in the table above have been adjusted to reflect the six-for-one share consolidation in July 2019.

Acquisition of Aegerion

On September 24, 2019, in connection with its acquisition (the “Acquisition”) of Aegerion Pharmaceuticals, Inc. (“Aegerion”), the registrant issued the following securities:

• 77,027,423 ordinary shares (including 48,739,975 ordinary shares represented by 9,747,995 American Depositary Shares) and 8,065,000 zero cost warrants to former creditors of Aegerion as consideration for the Acquisition;

• 27,541,944 ordinary shares (including 1,693,275 ordinary shares represented by 338,655 American Depositary Shares) and 5,911,722 zero cost warrants in connection with a $60 million equity offering to new and existing investors and former creditors of Aegerion;

• $125 million aggregate principal amount of Convertible Notes due 2025 (convertible at initial rate of 386.75 ordinary shares for each $1,000 principal amount, which is subject to customary anti-dilution adjustments as well as adjustments following certain fundamental changes); and

• Contingent Value Rights to holders of the registrant's ordinary shares and to employee option holders entitling them to proceeds of up to $85 million upon the occurrence of specified milestones related to the regulatory approval and commercialization of AP101.

On November 14, 2019, the registrant issued 4,864,656 zero cost warrants in exchange for an equivalent number of ordinary shares to holders who, on December 19, 2019, elected to exercise 1,645,105 warrants in exchange for 1,645,105 ordinary shares.

All of the foregoing issuances were made pursuant to exemptions from the registration requirements of the Securities Act, namely, outside of the United States pursuant to Regulation S, to U.S. entities pursuant to Section 4(a)(2) of the Securities Act or pursuant to Section 1145 of the Bankruptcy Reform Act of 1978.

Item 8.
Exhibits and Financial Statements.

(a)
Exhibits. The exhibits to this registration statement are listed in the Exhibit Index to this registration statement and incorporated herein by reference.

(b)
Financial Statement Schedules. None. Schedules have been omitted because the information required to be set forth therein is not applicable or is shown in the consolidated financial statements or notes thereto.

Item 9.
Undertakings.

The undersigned registrant hereby undertakes:

(1)
To file, during any period in which offers or sales are being made, a post-effective amendment to this registration statement:

(i)
To include any prospectus required by Section 10(a)(3) of the Securities Act of 1933;

II-2

(ii)
To reflect in the prospectus any facts or events arising after the effective date of the registration statement (or the most recent post-effective amendment thereof) which, individually or in the aggregate, represent a fundamental change in the information set forth in the registration statement. Notwithstanding the foregoing, any increase or decrease in volume of securities offered (if the total dollar value of securities offered would not exceed that which was registered) and any deviation from the low or high end of the estimated maximum offering range may be reflected in the form of prospectus filed with the Commission pursuant to Rule 424(b) if, in the aggregate, the changes in volume and price represent no more than 20 percent change in the maximum aggregate offering price set forth in the "Calculation of Registration Fee" table in the effective registration statement; and

(iii)
To include any material information with respect to the plan of distribution not previously disclosed in the registration statement or any material change to such information in the registration statement.

(2)
That, for the purpose of determining any liability under the Securities Act of 1933, each such post-effective amendment shall be deemed to be a new registration statement relating to the securities offered therein, and the offering of such securities at that time shall be deemed to be the initial bona fide offering thereof.

(3)
To remove from registration by means of a post-effective amendment any of the securities being registered which remain unsold at the termination of the offering.

(4)
To file a post-effective amendment to the registration statement to include any financial statements required by Item 8.A. of Form 20-F at the start of any delayed offering or throughout a continuous offering. Financial statements and information otherwise required by Section 10(a)(3) of the Act need not be furnished, provided, that the registrant includes in the prospectus, by means of a post-effective amendment, financial statements required pursuant to this paragraph and other information necessary to ensure that all other information in the prospectus is at least as current as the date of those financial statements.

(5)
That, for purposes of determining any liability under the Securities Act, the information omitted from the form of prospectus filed as part of the registration statement in reliance upon Rule 430A and contained in a form of prospectus filed by the registrant pursuant to Rule 424(b)(1) or (4) or 497(h) under the Securities Act shall be deemed to be part of the registration statement as of the time it was declared effective.

(6)
That, for the purpose of determining any liability under the Securities Act, each post-effective amendment that contains a form of prospectus shall be deemed to be a new registration statement relating to the securities offered therein, and the offering of such securities at that time shall be deemed to be the initial bona fide offering thereof.

Insofar as indemnification for liabilities arising under the Securities Act may be permitted to directors, officers and controlling persons of the registrant pursuant to the foregoing provisions, or otherwise, the registrant has been advised that in the opinion of the U.S. Securities and Exchange Commission such indemnification is against public policy as expressed in the Securities Act and is, therefore, unenforceable. In the event that a claim for indemnification against such liabilities (other than the payment by the registrant of expenses incurred or paid by a director, officer or controlling person of the registrant in the successful defense of any action, suit or proceeding) is asserted by such director, officer or controlling person in connection with the securities being registered, the registrant will, unless in the opinion of its counsel the matter has been settled by controlling precedent, submit to a court of appropriate jurisdiction the question whether such indemnification by it is against public policy as expressed in the Securities Act and will be governed by the final adjudication of such issue.

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EXHIBIT INDEX

Exhibit Number	Exhibit Description
2.1	Plan Funding Agreement, dated May 20, 2019, between Amryt Pharma plc and Aegerion Pharmaceuticals, Inc.
2.2	Share Purchase and Transfer Agreement, dated October 16, 2015, among Amryt Pharmaceuticals DAC, Software AG – Stiftung, Dr. Armin Schiffler and Birken AG
3.1	Articles of Association of Amryt Pharma plc
4.1	Deposit Agreement
4.1.1	Form of amendment to Deposit Agreement
4.2	Form of American Depositary Receipt (included in Exhibit 4.1)
4.3	Form of Zero Cost Warrant
5.1*	Opinion of Gibson, Dunn & Crutcher UK LLP
10.1	Debtor’s Modified First Amended Joint Chapter 11 Plan, dated August 29, 2019, of Aegerion Pharmaceuticals, Inc., et al.
10.2	Restructuring Support Agreement, dated May 20, 2019, among Aegerion Pharmaceuticals, Inc. and each of its subsidiaries party thereto, Amryt Pharma plc, as plan investor, and Athyrium Opportunities II Acquisition LP, Athyrium Opportunities III Acquisition LP, Highbridge MSF International Ltd., 1992 Tactical Credit Master Fund, L.P., Highbridge SCF Special Situations SPV, L.P., Highbridge SCF Loan SPV, L.P., Whitebox Relative Value Partners, LP, Whitebox GT Fund, LP, Whitebox Multi-Strategy Partners, LP, Pandora Select Partners, LP, Nineteen77 Global Multi-Strategy Alpha Master Limited and Nineteen77 Global Convertible Bond Master Limited, as consenting lenders
10.3	Backstop Agreement, dated July 10, 2019, among Amryt Pharma plc and Highbridge MSF International Ltd., Highbridge SCF Special Situations SPV, L.P., 1992 Tactical Credit Master Fund, L.P., Athyrium Opportunities II Acquisition, 2 LP, Athyrium Opportunities III Acquisition 2 LP, Whitebox Relative Value Partners, LP, Whitebox GT Fund, LP, Whitebox Multi-Strategy Partners, LP, Pandora Select Partners, LP, Nineteen77 Global Multi-Strategy Alpha Master Limited and Nineteen77 Global Convertible Bond Master Limited, as backstop parties
10.4†	Registration Rights Agreement, dated September 25, 2019, among Amryt Pharma Holdings plc, Highbridge MSF International Ltd., Highbridge Tactical Credit Master Fund, L.P., Highbridge SCF Special Situations SPV, L.P., Athyrium Opportunities II Acquisition 2 LP and Athyrium Opportunities III Acquisition 2 LP
10.5†	Senior Secured Credit Agreement, dated September 24, 2019, among Aegerion Pharmaceuticals, Inc., as borrower, Amryt Pharma Holdings plc, the lenders party thereto and Cantor Fitzgerald Securities as administrative agent and collateral agent for the lenders
10.6†	Indenture, dated September 24, 2019, among Aegerion Pharmaceuticals, Inc., as issuer, Amryt Pharma Holdings plc, Amryt Pharma plc and the additional guarantors party thereto and GLAS Trust Company LLC, as the trustee, relating to the issuer’s 5.00% Convertible Senior Notes due 2025
10.7†	License Agreement, effective as of March 14, 2018, between Amryt Genetics Limited and University College Dublin, National University of Ireland
10.8.1	Patent License Agreement, effective as of May 19, 2006, between Aegerion Pharmaceuticals, Inc. and The Trustees of the University of Pennsylvania (incorporated by reference to Exhibit 10.6 to Aegerion Pharmaceutical Inc.’s Registration Statement on Form S-1, as amended, filed with the SEC on August 10, 2010)
10.8.2	First Amendment to Patent License Agreement, effective as of September 27, 2006, between Aegerion Pharmaceuticals, Inc. and The Trustees of the University of Pennsylvania (included in Exhibit 10.8.1)
10.9.1	Asset Purchase Agreement, dated November 5, 2014, by and among Aegerion Pharmaceuticals, Inc., Amylin Pharmaceuticals, LLC and AstraZeneca Pharmaceuticals LP (incorporated by reference to Exhibit 10.29 to Aegerion Pharmaceuticals, Inc.’s Amendment No. 1 to the Annual Report on Form 10-K, filed with the SEC on July 7, 2015)

II-4

Exhibit Number	Exhibit Description
10.9.2	First Amendment to Asset Purchase Agreement, dated January 9, 2015, by and among Aegerion Pharmaceuticals, Inc., Amylin Pharmaceuticals, LLC and AstraZeneca Pharmaceuticals LP (incorporated by reference to Exhibit 10.30 to Aegerion Pharmaceuticals, Inc.’s Annual Report on Form 10-K, filed with the SEC on March 2, 2015)
10.10	License Agreement, dated February 7, 2006, by and between Amgen Inc. and Amylin Pharmaceuticals, Inc. (incorporated by reference to Exhibit 10.32 to Aegerion Pharmaceuticals, Inc.’s Annual Report on Form 10-K, filed with the SEC on March 2, 2015).
10.11.1†	Contract Manufacturing Agreement, dated September 30, 2010, by and between Amylin Pharmaceuticals, Inc. and Sandoz GmbH
10.11.2†	First Amendment, dated September 1, 2011, to Contract Manufacturing Agreement, dated September 30, 2010, by and between Amylin Pharmaceuticals, Inc. and Sandoz GmbH
10.11.3†	Amendment No. 2, dated December 18, 2012, to Contract Manufacturing Agreement, dated September 30, 2010, by and between Amylin Pharmaceuticals, Inc. and Sandoz GmbH
10.11.4	Amendment No. 3, dated July 8, 2013, to Contract Manufacturing Agreement, dated September 30, 2010, by and between Amylin Pharmaceuticals, Inc. and Sandoz GmbH
10.11.5	Amendment No. 4, dated June 23, 2014, to Contract Manufacturing Agreement, dated September 30, 2010, by and between Amylin Pharmaceuticals, Inc. and Sandoz GmbH
10.11.6†	Amendment No. 5, dated October 13, 2014, to Contract Manufacturing Agreement, dated September 30, 2010, by and between Amylin Pharmaceuticals, Inc. and Sandoz GmbH
10.11.7†	6th Amendment, dated June 1, 2017, to Contract Manufacturing Agreement, dated September 30, 2010, by and between Aegerion Pharmaceuticals, Inc. and Sandoz GmbH
10.11.8†	7th Amendment, dated August 1, 2017, to Contract Manufacturing Agreement, dated September 30, 2010, by and between Aegerion Pharmaceuticals, Inc. and Sandoz GmbH
10.11.9†	8th Amendment, dated April 30, 2019, to Contract Manufacturing Agreement, dated September 30, 2010, by and between Aegerion Pharmaceuticals, Inc. and Sandoz GmbH
10.11.10†	9th Amendment, dated February 11, 2020, to Contract Manufacturing Agreement, dated September 30, 2010, by and between Aegerion Pharmaceuticals, Inc. and Sandoz GmbH
10.12.1†	Master Services Agreement, dated as of December 6, 2013 between Bristol-Meyers Squibb Company and Accredo Health Group, Inc.
10.12.2	1st Amendment, dated January 9, 2014, to Master Services Agreement, dated as of December 6, 2013 between Bristol-Meyers Squibb Company and Accredo Health Group, Inc.
10.12.3†	Second Amendment, dated June 1, 2014, to Master Services Agreement, dated as of December 6, 2013 between Astrazeneca Pharmaceuticals LP and Accredo Health Group, Inc.
10.12.4	Third Amendment, dated June 20, 2016, to Master Services Agreement, dated as of December 6, 2013 between Aegerion Pharmaceuticals, Inc. and Accredo Health Group, Inc.
10.12.5†	Fourth Amendment, dated October 19, 2017, to Master Services Agreement, dated as of December 6, 2013 between Aegerion Pharmaceuticals, Inc. and Accredo Health Group, Inc.
10.13#	Amryt Pharma plc Employee Share Option Plan 2019
16.1	Letter from BDO LLP
21.1	List of Subsidiaries
23.1	Consent of Grant Thornton with respect to financial statements of Amryt Pharma plc
23.2	Consent of Deloitte & Touche, LLP with respect to financial statements of Aegerion Pharmaceuticals, Inc.
23.3	Consent of Gibson, Dunn & Crutcher UK LLP (included in Exhibit 5.1)
24.1	Powers of Attorney (included on signature page to the registration statement)

*
To be filed by amendment.

†
Portions of this exhibit (indicated by asterisks) have been redacted in compliance with Regulation S-K Item 601(b)(10)(iv).

#
Indicates senior management contract or compensatory plan.

II-5

SIGNATURES

Pursuant to the requirements of the Securities Act of 1933, the registrant certifies that it has reasonable grounds to believe that it meets all of the requirements for filing on Form F-1 and has duly caused this registration statement to be signed on its behalf by the undersigned, thereunto duly authorized, in London, the United Kingdom on June 23, 2020.

AMRYT PHARMA PLC
By:	/s/ Dr. Joseph A. Wiley
	Name:	Dr. Joseph A. Wiley
	Title:	Chief Executive Officer

II-6

We, the undersigned directors, officers and/or authorized representative in the United States of Amryt, hereby severally constitute and appoint Joseph Wiley and Rory Nealon, and each of them singly, our true and lawful attorneys, with full power to any of them, and to each of them singly, to sign for us and in our names in the capacities indicated below the registration statement on Form F-1 filed herewith, and any and all pre-effective and post-effective amendments to said registration statement, and any registration statement filed pursuant to Rule 462(b) under the Securities Act of 1933, as amended, in connection with the registration under the Securities Act of 1933, as amended, of equity securities of Amryt Pharma plc, and to file or cause to be filed the same, with all exhibits thereto and other documents in connection therewith, with the Securities and Exchange Commission, granting unto said attorneys, and each of them, full power and authority to do and perform each and every act and thing requisite and necessary to be done in connection therewith, as fully to all intents and purposes as each of them might or could do in person, and hereby ratifying and confirming all that said attorneys, and each of them, or their substitute or substitutes, shall do or cause to be done by virtue of this Power of Attorney.

Pursuant to the requirements of the Securities Act of 1933, as amended, this registration statement has been signed by the following persons on June 23, 2020 in the capacities indicated:

Name	Title
/s/ Dr. Joseph A. Wiley	Chief Executive Officer and Director (Principal Executive Officer)
Dr. Joseph A. Wiley
/s/ Rory P. Nealon	Chief Financial Officer and Chief Operating Officer (Principal Financial and Accounting Officer)
Rory P. Nealon
/s/ Raymond T. Stratford	Chairman of the Board
Raymond T. Stratford
/s/ Donald K. Stern	Director
Donald K. Stern
/s/ Dr. Alain H. Munoz	Director
Dr. Alain H. Munoz
/s/ Dr. Patrick V.J.J Vink	Director
Dr. Patrick V.J.J. Vink
/s/ Stephen T. Wills	Director
Stephen T. Wills
/s/ George P. Hampton, Jr.	Director
George P. Hampton, Jr.

II-7

SIGNATURE OF AUTHORIZED US REPRESENTATIVE OF REGISTRANT

Pursuant to the requirements of the Securities Act of 1933, as amended, the undersigned, the duly authorized representative in the United States of Amryt Pharma plc has signed this registration statement on June 23, 2020.

By:	/s/ Donald Puglisi
	Name:	Donald Puglisi
	Title:	Authorized Representative in the United States

II-8

EX-2.1

Exhibit 2.1 

Execution Copy

AEGERION PHARMACEUTICALS, INC.

AND

AMRYT PHARMA PLC

PLAN FUNDING AGREEMENT

May 20, 2019

TABLE OF CONTENTS

		Page
ARTICLE I. DEFINITIONS		5
Section 1.1.	Definitions	5
Section 1.2.	Other Definitions	17
ARTICLE II. ACQUISITION; ISSUANCE OF CLOSING SHARES		18
Section 2.1.	Acquisition	18
Section 2.2.	Transaction Consideration	19
ARTICLE III. THE CLOSING		19
Section 3.1.	The Closing	19
Section 3.2.	Closing Deliverables	19
ARTICLE IV. REPRESENTATIONS AND WARRANTIES OF THE COMPANY		20
Section 4.1.	Organization	20
Section 4.2.	Qualification; Due Authorization; Power and Authority	21
Section 4.3.	Consents and Approvals	21
Section 4.4.	Capitalization	22
Section 4.5.	Financial Statements	22
Section 4.6.	No Undisclosed Liabilities	23
Section 4.7.	Recent Events	23
Section 4.8.	Contracts and Commitments	24
Section 4.9.	Real Property	26
Section 4.10.	Intellectual Property	26
Section 4.11.	Privacy and Data Security	28
Section 4.12.	Legal Compliance; Permits	28
Section 4.13.	Environmental Compliance and Conditions	29
Section 4.14.	Litigation	30
Section 4.15.	Tax Matters	30
Section 4.16.	Insurance	31
Section 4.17.	Illegal or Improper Payments	31
Section 4.18.	Related Party Transactions	32
Section 4.19.	Brokers’ Fees	32
Section 4.20.	Employees.	32
Section 4.21.	Healthcare Compliance Matters	33
Section 4.22.	No Other Representations or Warranties	35
ARTICLE V. REPRESENTATIONS AND WARRANTIES OF THE PLAN INVESTOR		35
Section 5.1.	Organization	35
Section 5.2.	Qualification; Due Authorization; Power and Authority	36
Section 5.3.	Consents and Approvals	36
Section 5.4.	Capitalization	36
Section 5.5.	Financial Statements	38
Section 5.6.	No Undisclosed Liabilities	38
Section 5.7.	Recent Events	38
Section 5.8.	Contracts and Commitments	39
Section 5.9.	Real Property	42

Section 5.10.	Intellectual Property	42
Section 5.11.	Privacy and Data Security	44
Section 5.12.	Legal Compliance; Permits	44
Section 5.13.	Environmental Compliance and Conditions	45
Section 5.14.	Litigation	45
Section 5.15.	Tax Matters	46
Section 5.16.	Insurance	47
Section 5.17.	Illegal or Improper Payments	47
Section 5.18.	Related Party Transactions	48
Section 5.19.	Brokers’ Fees	48
Section 5.20.	Employees.	48
Section 5.21.	Healthcare Compliance Matters	49
Section 5.22.	No Other Representations or Warranties	50
ARTICLE VI. COVENANTS		51
Section 6.1.	Conduct of Business of the Company Pending the Closing	51
Section 6.2.	Conduct of Business of the Plan Investor Pending the Closing	53
Section 6.3.	Cooperation; Access to Information; Interim Financial Information	55
Section 6.4.	Further Actions; Reasonable Efforts	55
Section 6.5.	Listing on AIM	57
Section 6.6.	U.S. Registration	58
Section 6.7.	Regulatory Filings	59
Section 6.8.	Financing	60
Section 6.9.	Company Solicitations; Company Alternative Transactions	61
Section 6.10.	Plan Investor Solicitations; Plan Investor Alternative Transactions	64
Section 6.11.	Plan Investor Stockholder Approval	64
Section 6.12.	Intercompany Obligations	65
Section 6.13.	Scheme	65
Section 6.14.	Other Governance Matters	65
Section 6.15.	Communication Materials	66
Section 6.16.	American Depository Shares	66
ARTICLE VII. CONDITIONS TO CLOSING		66
Section 7.1.	Conditions to the Obligations of Each Party	66
Section 7.2.	Conditions to the Obligations of the Plan Investor	68
Section 7.3.	Conditions to the Obligations of the Company	68
Section 7.4.	Frustration of Closing Conditions	69
ARTICLE VIII. TERMINATION		69
Section 8.1.	Termination	69
Section 8.2.	Effect of Termination	71
Section 8.3.	Fees and Expenses	71
ARTICLE IX. BANKRUPTCY COURT MATTERS		74
Section 9.1.	PFA Order	74
ARTICLE X. MISCELLANEOUS		74
Section 10.1.	Governing Law	74
Section 10.2.	Jurisdiction; Forum; Service of Process; Waiver of Jury Trial	74
Section 10.3.	Successors and Assigns	74
Section 10.4.	Entire Agreement; Amendment	74

Section 10.5.	Disclosure Schedule References; Data Room Disclosures	75
Section 10.6.	Notices	75
Section 10.7.	Delays or Omissions	76
Section 10.8.	Counterparts	76
Section 10.9.	Severability	76
Section 10.10.	Headings	77
Section 10.11.	No Third-Party Beneficiaries	77
Section 10.12.	No Survival	77
Section 10.13.	Fees and Expenses	77
Section 10.14.	No Public Announcement	77
Section 10.15.	Specific Performance	77
Section 10.16.	Construction	77

Exhibits

Exhibit A: Form of Voting Agreement

Exhibit B: Form of Shared Services Agreement

Exhibit C: Form of CVR Instrument

Exhibit D: Form of Loan Notes Deed Poll

Exhibit E: Form of Registration Rights Agreement

Disclosure Schedules

Company Disclosure Schedule
Plan Investor Disclosure Schedule

PLAN FUNDING AGREEMENT

THIS PLAN FUNDING AGREEMENT (as it may be amended, restated, supplemented or otherwise modified from time to time, this “Agreement”) is made as of May 20, 2019, by and between Aegerion Pharmaceuticals, Inc., a Delaware corporation (the “Company”), and Amryt Pharma plc, a company incorporated in England and Wales with the registered number 05316808 and registered address at Dept 920a 196 High Road, Wood Green, London, England, N22 8HH (the “Plan Investor” and, together with the Company, the “Parties” and each a “Party”). Capitalized terms used herein and not otherwise defined herein shall have the meanings ascribed to them in Article I hereof.

RECITALS

WHEREAS, the Company is a wholly-owned indirect subsidiary of Novelion Therapeutics Inc., a Canadian corporation (“Novelion”);

WHEREAS, Novelion holds the Novelion Intercompany Loan Claim;

WHEREAS, the Company and the Plan Investor desire to undertake the transactions contemplated by this Agreement and the other Transaction Documents;

WHEREAS, in order to facilitate the transactions contemplated by the Transaction Documents, the Company intends to take certain actions, including filing voluntary petitions for relief along with Aegerion Pharmaceuticals Holdings, Inc. (the “Bankruptcy Cases”) under chapter 11 of Title 11 of the United States Code, 11 U.S.C. §§ 101 et seq. (the “Bankruptcy Code”), in the United States Bankruptcy Court for the Southern District of New York (the “Bankruptcy Court”);

WHEREAS, in support of the Bankruptcy Cases, the Plan Investor, the Company and certain lenders to the Company (including Novelion) have entered into the Restructuring Support Agreement, dated as of the date hereof (as it may be amended, modified or supplemented from time to time in accordance with its terms, the “Restructuring Support Agreement”);

WHEREAS, to implement the restructuring described in the Restructuring Support Agreement (the “Restructuring”) and in connection with the transactions contemplated by the Transaction Documents, (i) the Plan Investor desires to purchase the Reorganized Company Interests (the “Acquisition”) in consideration for the Closing Shares (as hereinafter defined), upon the terms and subject to the conditions set forth herein, and (ii) simultaneously with the Acquisition and the issuance of the Closing Shares, the Company desires to distribute the Closing Shares to Novelion and certain other lenders or creditors of the Company in satisfaction of certain obligations of the Company to such Persons upon the terms and subject to the conditions set forth in this Agreement, the Plan and the other Transaction Documents;

WHEREAS, contemporaneously with the execution of this Agreement, certain stockholders of the Plan Investor have entered into a Voting Support Agreement (the “Voting Agreement”), a form of which is attached hereto as Exhibit A;

WHEREAS, contemporaneously with the execution of this Agreement, the Company and Novelion have entered into an amendment to that certain Master Service Agreement, dated as of December 1, 2016, by and between the Company and Novelion (as so amended, the “Shared Services Agreement”), a form of which is attached hereto as Exhibit B;

4

WHEREAS, upon the recommendation of the Restructuring Committee, the Company Board has unanimously approved the Transaction Documents and transactions contemplated thereby;

WHEREAS, the Plan Investor Board has unanimously approved the Transaction Documents and transactions contemplated thereby and will recommend that the holders of the voting securities (the “Plan Investor Shares”) of the Plan Investor (the “Plan Investor Stockholders”) vote in favor of the Acquisition and other transactions contemplated by the Transaction Documents; and

WHEREAS, in order to facilitate the transactions contemplated by this Agreement and the Transaction Documents, the Plan Investor proposes to enter into a scheme of arrangement under Part 26 of the Companies Act with the Plan Investor Stockholders whereby the Plan Investor Stockholders (and the holders of options over Plan Investor Shares (the “Plan Investor Options”) (and the holders of the Plan Investor Options, the “Plan Investor Optionholders”) will exchange the Plan Investor Shares held by such Plan Investor Stockholders (and the Plan Investor Options held by such Plan Investor Optionholders) for new ordinary shares (or new options over such ordinary shares) issued by a special purpose vehicle, together with one (1) CVR Security for each Plan Investor Share (or Plan Investor Option), incorporated to be the new ultimate holding company of the Plan Investor Group (“New Atlas TopCo”), whereupon the rights and obligations of the Plan Investor under this Agreement shall be assumed by New Atlas TopCo in accordance with the terms and conditions of this Agreement and the Scheme Document (such scheme of arrangement, the “Scheme”).

NOW, THEREFORE, in consideration of the mutual covenants, agreements, representations and warranties contained herein, and for other good and valuable consideration, the receipt and sufficiency of which are hereby acknowledged, the Parties hereby agree as follows:

ARTICLE I.

DEFINITIONS

Section 1.1.          Definitions. For the purposes of this Agreement, the following terms shall have the meanings set forth below. Capitalized terms used but not defined herein shall have the meanings attributed to them in the Restructuring Support Agreement or the Plan that is attached to the Restructuring Support Agreement.

“Acceptable Confidentiality Agreement” means a confidentiality agreement containing terms and conditions no less favorable to the Company, in any material respect, than the terms set forth in the Confidentiality Agreement; provided, however, that such confidentiality agreement shall not (x) limit or adversely affect the rights of the Company pursuant to Section 6.9 or (y) otherwise prohibit compliance by the Company with any provision of this Agreement or any of the Transaction Documents.

“Affiliate” means, with respect to any Person, any other Person that directly, or indirectly through one or more intermediaries, controls, or is controlled by, or is under common control with, such Person. As used in this definition, the term “control,” including the correlative terms “controlling,” “controlled by” and “under common control with,” means the possession, directly or indirectly, of the power to direct or cause the direction of the management or policies of an entity, whether through ownership of voting securities, by Contract, by Charter Documents or otherwise. Following the Closing, the Reorganized Company Group Members shall be deemed to be Affiliates of the Plan Investor. For purposes of this Agreement, except where expressly set forth to the contrary, Novelion shall not be deemed an Affiliate of any Company Group Member.

5

“AIM” means the Alternative Investment Market operated by the London Stock Exchange plc.

“Anti-corruption Laws” means Laws relating to anti-bribery or anti-corruption (governmental or commercial), including Laws that prohibit the corrupt payment, offer, promise or authorization of the payment or transfer of anything of value (including gifts or entertainment), directly or indirectly, to any foreign Government Official or other Person to obtain a business advantage, including the FCPA, the U.K. Bribery Act of 2010 and all national and international Laws enacted to implement the OECD Convention on Combating Bribery of Foreign Officials in International Business Transactions.

“Athyrium” means Athyrium Opportunities II Acquisition LP, a Delaware limited partnership and Athyrium Opportunities III Acquisition LP, a Delaware limited partnership;

“Backstop Agreement” means the agreement to be entered into between the Plan Investor and certain lenders of the Company on or about the date of this Agreement in connection with the Plan Investor Equity Raise;

“Business Day” means any day other than a Saturday, Sunday, a “legal holiday,” as defined in Federal Rule of Bankruptcy Procedure 9006(a), or a day on which banks are not open for general business in Dublin, Ireland, New York, New York or London, United Kingdom, as applicable in the context.

“Charter Documents” means (i) the articles of incorporation, the certificate of incorporation or the articles of association and the bylaws of a corporation; (ii) the partnership agreement and any statement of partnership of a general partnership; (iii) the limited partnership agreement and the certificate of limited partnership of a limited partnership; (iv) the limited liability company agreement, the operating agreement and the certificate of organization of a limited liability company; or (v) any document adopted or filed with any Governmental Entity in connection with the creation, formation or organization of the applicable Person; together with any amended, amended and restated or otherwise modified or supplemented version of any of the foregoing.

“Claim” shall have the meaning set forth in Section 101(5) of the Bankruptcy Code.

“Closing Shares” means a number of newly issued ordinary shares of the Plan Investor (or, at the election of the Plan Investor, American Depositary Shares representing such ordinary shares or, if applicable, equivalent ordinary shares in New Atlas Topco), equal to (a) 1.59 multiplied by (b) the aggregate amount of all issued and outstanding ordinary shares of the Plan Investor (or New Atlas Topco as appropriate) immediately prior to the Closing (but prior to giving effect to the Plan Investor Equity Raise and the Company Rights Offering Transactions and any ordinary shares issuable upon conversion of the New Convertible Notes) plus an amount of shares equal to the additional shares represented by all warrants and/or options outstanding immediately prior to the Closing (excluding any warrants and/or options issued under the existing Amryt Pharma plc Employee Share Option Plan, as amended on 25 May 2017). The Closing Shares shall be calculated based on information available as of the date five (5) Business Days prior to the Closing; provided from and after the date of such calculation the Plan Investor shall take all actions reasonably necessary not to alter the amounts used therefor other than in de minimis amounts. By way of illustration, this would result in the Closing Shares being equal to 61.4% of the issued and outstanding ordinary shares of the Plan Investor determined as provided above, after giving effect to such issuance of Closing Shares calculated as provided above.

“Code” means the U.S. Internal Revenue Code of 1986, as amended.

6

“Companies Act” means the Companies Act 2006 (UK).

“Company Alternative Proposal” means any solicited bona fide proposal or offer during the Go-Shop Period or any unsolicited bona fide proposal or offer other than during the Go-Shop Period, from any Person (other than the Plan Investor or any of its Affiliates) with respect to a Company Alternative Transaction.

“Company Board” means the board of directors of the Company.

“Company Business” means the business of the Company Group substantially as conducted as of the date hereof.

“Company Employee Benefit Plan” means any: (a) employee benefit plan (within the meaning of Section 3(3) of ERISA, whether or not subject to ERISA) that is maintained or sponsored by any Company Group Member or to which any such Person contributes (or is required to contribute) or for which any Company Group Member otherwise has or may have any Liability, either directly or as a result of being an ERISA Affiliate or otherwise, whether or not funded and whether or not terminated, (b) personnel policies of any Company Group Member (including any employee handbooks), and (c) fringe or other benefit or compensation plans, policies, programs and arrangements, whether or not subject to ERISA, whether or not funded and whether or not terminated, including stock bonus or other equity compensation, deferred compensation, incentive compensation, pension, severance, retention, change of control, bonus, vacation, travel, incentive, and health or other medical, disability, life and welfare plans or insurance (whether insured or self-insured), policies, programs or arrangements, share purchase, share option, stock appreciation, phantom stock, savings, profit sharing or termination pay, supplementary unemployment benefit, retirement and supplementary retirement plans, programs, agreements and arrangements, that are maintained or sponsored by any Company Group Member or to which any such Person contributes (or is required to contribute), or, in each case, for which any Company Group Member has any Liability, either directly or as a result of being an ERISA Affiliate or otherwise.

“Company Fundamental Representations” means the representations set forth in Section 4.1 (Organization), Section 4.2 (Qualification; Due Authorization; Power and Authority), and Section 4.19 (Brokers’ Fees).

“Company Group” means the Company and its direct or indirect Subsidiaries, as of the date hereof and prior to the Effective Date.

“Company Group Member” means each of the Company and its direct or indirect Subsidiaries, in each case, as of the date hereof and prior to the Effective Date.

“Company’s Knowledge” or other words of similar import means the actual knowledge, after a reasonable best efforts inquiry, of those individuals identified in Section 1.1(b) of the Company Disclosure Schedule.

“Company Latest Balance Sheet” means the unaudited consolidated balance sheet of Novelion and its Subsidiaries as of March 31, 2019 as set forth on Section 1.1(a) of the Company Disclosure Schedule.

“Company Material Adverse Effect” means any consideration, effect, occurrence, condition, change, development, event, state of facts or circumstance that: (a) has had, or would reasonably be expected to have, individually or in the aggregate, a material adverse effect on the business, results of operations, financial condition, assets or Liabilities or property of the Company Group, taken as

7

a whole, or (b) has impacted or would reasonably be expected to impact, the ability of the Company to consummate the transactions set forth in, or perform its obligations under, any Transaction Document, in each case in any material respect; provided, that none of the following, either alone or in combination, shall be deemed to constitute, or be taken into account in determining whether there has been, a Company Material Adverse Effect: any consideration, effect, occurrence, condition, change, development, event, state of facts or circumstance (i) arising from general economic or political conditions or financial, banking, credit or securities market conditions, including any disruption thereof and any interest or exchange rate fluctuations; (ii) directly arising from the announcement or performance of, or compliance with, the pendency of, or the public or industry knowledge of, this Agreement or the transactions contemplated by this Agreement (other than compliance with Article IV and it being understood that this clause (b)(ii) shall not apply with respect to any representation or warranty contained in this Agreement to the extent the purpose of such representation or warranty is to address the consequences resulting from the execution and delivery of this Agreement or the consummation of the transactions contemplated by this Agreement or the compliance with the terms of this Agreement) or the filing of the Bankruptcy Cases; (iii) arising from any changes in Laws or GAAP that the Company Group is required to adopt; (iv)    arising from natural disasters, acts of terrorism or war (whether or not declared) or epidemics or pandemics; (v) arising from the failure to meet any projections or forecasts (but not the underlying causes thereof which shall be considered in determining whether a Company Material Adverse Effect has occurred unless otherwise excluded pursuant to this definition); or (vi) arising out of any action taken or omitted to be taken at the written request or with the written consent of the other Party; provided that in each case of the clauses (i), (iii), (iv) and (v) above, such consideration, effect, occurrence, condition, change, development, event, state of facts or circumstance does not have a disproportionate impact on the Company Group, taken as a whole, compared to other companies operating in the industry in which the Company Group operates and in such event, only any such disproportionate impact shall be considered in determining whether a Company Material Adverse Effect has occurred. For the avoidance of doubt, any Proceeding brought before the Bankruptcy Court (whether under the Bankruptcy Cases or otherwise) shall not constitute a Company Material Adverse Effect as long as the Company has the ability to consummate the transactions contemplated by this Agreement and the other Transaction Documents in accordance with their respective terms and the Confirmation Order, in each case, in all material respects; provided, such consideration, effect, occurrence, condition, change, development, event, state of facts or circumstance of such Proceeding may be taken into account when determining whether a Company Material Adverse Effect has occurred.

“Company Rights Offering Transactions” means the Rights Offering as defined in the Plan.

“Company Superior Proposal” means a written Company Alternative Proposal in which any Person or group of Persons would acquire fifty percent (50%) or more of the outstanding equity securities of the Reorganized Company or fifty percent (50%) or more of the assets of the Company and its Subsidiaries, taken as a whole, that the Company Board determines in good faith (after consultation with the Company’s outside counsel and financial advisors) (w) is not subject to any financing or due diligence contingency of any kind, (x) was not made as a result of, or otherwise in connection with, any breach of this Agreement (y) is reasonably likely to be consummated in accordance with its terms and (z) if consummated, would result in a transaction that is more favorable to the Company from a financial point of view, after taking into account all relevant factors (including the timing, financing and other legal and regulatory aspects of such Company Alternative Proposal (including the identity of the Person or group making such proposal)), than the transactions contemplated by this Agreement and the other Transaction Documents (after giving effect to all adjustments to the terms hereof and thereof that may be offered by the Plan Investor pursuant to Section 6.9(f)).

“Company Termination Fee” shall be an amount equal to $11,850,000.

8

“Confidentiality Agreement” means that certain Confidentiality Agreement, dated April 11, 2019, by and among the Company, Novelion, Plan Investor and certain creditors of the Company.

“Confirmation Order” shall have the meaning set forth in the Plan.

“Contracts” means all contracts, agreements, leases, understandings and arrangements to which any Person is a party or by which any such Person or any of its assets is bound or under which any such Person has rights, including any Real Property Leases, in each case including any amendments, amendments and restatements and other modifications and supplements thereto.

“CVR Instrument” means the deed poll to be entered into by New Atlas TopCo constituting the CVR Securities to be issued to the Plan Investor Stockholders and the Plan Investor Optionholders in relation to the Scheme in accordance with the terms and conditions of the Scheme Document, in the form attached hereto as Exhibit C, containing the Loan Notes Deed Poll in the form attached hereto as Exhibit D.

“Data Protection Laws” means all applicable laws pertaining to data protection, data privacy, data security, data breach notification, and cross-border data transfer in the United States of America and elsewhere in the world, including the EU General Data Protection Regulation (EU) 2016/679 as implemented on May 25, 2018.

“Data Protection Requirements” means all applicable (i) Data Protection Laws; (ii) Privacy Policies; (iii) terms of any Contracts relating to the Party’s collection, use, storage, disclosure, or cross-border transfer of Personal Data; and (iv) industry standards and/or codes-of-conduct to which the Party is bound which govern the collection, use, storage, disclosure, or cross-border transfer of Personal Data.

“Debtors” shall have the meaning set forth in the Plan.

“DIP Financing Agreement” shall have the meaning set forth in the Plan.

“Distribution Agreement” means a distribution agreement entered into by Plan Investor or any of its Subsidiaries and a distributor, in any applicable territory, for the sales and marketing (if and when applicable), importation into a territory and distribution of a Plan Investor product or Plan Investor licensed product within a territory covered by the applicable license agreement in the ordinary course of business.

“DTIF Agreement” means an agreement that the Plan Investor may enter into with respect to the Plan Investor’s collaborative Disruptive Technology Innovation Fund agreement relating to Plan Investors AP103 product.

“Effective Date” shall have the meaning set forth in the Plan.

“Electronic Data Room” means the electronic data rooms established by the Plan Investor or Company as the case may be, in connection with the transactions contemplated by this Agreement.

“Encumbrance” means any mortgage, deed of trust, lien, pledge, security interest, hypothecation, transfer restriction, easement, purchase right, right of first refusal, conditional sale agreement or any other encumbrance.

9

“Environmental Claim” means any Proceeding by any Person or entity alleging actual or potential Liability (including actual or potential liability for investigatory costs, cleanup costs, response costs, natural resources damages, property damages, personal injuries, attorneys’ fees or penalties) arising out of, based on, resulting from or relating to any Environmental Laws, Environmental Permits or the presence, or Release into the environment, of, or exposure to, any Hazardous Materials at any location, but shall not include any claims relating to products liability.

“ERISA” means the Employee Retirement Income Security Act of 1974, as amended, and all rules and regulations promulgated thereunder.

“ERISA Affiliate” means any Person and that, together with such Person and any of the Subsidiaries of such Person, is or was at any time treated as a “single employer” under Section 414 of the Code or Section 4001(b)(1) of ERISA. Notwithstanding the foregoing, Novelion shall not be considered an ERISA Affiliate of the Company Group.

“Euronext” means the Euronext Growth Market of Euronext Dublin.

“Exchange Act” means the Securities Exchange Act of 1934.

“Fairly Disclosed” means disclosed in such manner to enable the Plan Investor or the Company (as the case may be) to identify the nature and scope of the matter so disclosed.

“FCPA” means the United States Foreign Corrupt Practices Act of 1977.

“Final Order” shall have the meaning set forth in the Plan.

“Financing” means any financing procured, or proposed to be procured, by the Plan Investor or any of its Affiliates in connection with the transactions contemplated by this Agreement.

“GAAP” means generally accepted accounting principles and practices, as in effect on the date hereof, in the United States or Canada, as applicable.

“Government Official” means (i) any official, officer, employee, representative or any person acting in an official capacity for or on behalf of any Governmental Entity; (ii) any political party or party official or candidate for political office; (iii) any public international organization or any department or agency thereof; or (iv) any Person or other entity owned in whole or in part, or controlled by any Person described in the foregoing clauses (i), (ii) or (iii) of this definition.

“Governmental Entity” means any supranational, national, foreign, federal, state, provincial or local judicial, legislative, executive, administrative, regulatory or arbitral body or authority or other instrumentality of the United States of America, any foreign jurisdiction, or any state, provincial, county, municipality or local governmental unit thereof, including any Tax authority.

“Health Care Laws” means: (i) the Federal Food, Drug, and Cosmetic Act (21 U.S.C. § 301 et seq.); (ii) the Controlled Substances Act (21 U.S.C. § 801 et seq.); (iii) the Public Health Service Act (42 U.S.C. § 201 et seq.); (iv) all federal, state, local and all applicable foreign health care related fraud and abuse, false claims, and anti-kickback laws, including, without limitation, the U.S. Anti-Kickback Statute (42 U.S.C. § 1320a-7b(b)), the U.S. Physician Payment Sunshine Act (42 U.S.C. § 1320a-7h) and similar gift and disclosure Laws, the U.S. Civil False Claims Act (31 U.S.C. § 3729 et seq.), the criminal False Claims Law (42 U.S.C. § 1320a-7b(a)), all criminal laws relating to health care fraud and abuse, including but not limited to 18 U.S.C. §§ 286 and 287, and the health care fraud criminal

10

provisions under the U.S. Health Insurance Portability and Accountability Act of 1996 (42 U.S.C. § 1320d et seq.), the exclusion laws (42 U.S.C. § 1320a-7), the civil monetary penalties law (42 U.S.C. § 1320a-7a), and Laws relating to price reporting requirements and the requirements relating to the processing of any applicable rebate, chargeback or adjustment, under applicable rules and regulations relating to the Medicaid Drug Rebate Program (42 U.S.C. § 1396r-8), any state supplemental rebate program, and Medicare average sales price reporting (42 U.S.C. § 1395w-3a); (v) state Laws relating to the manufacture, sale and distribution of pharmaceutical and medical products; (vi) Medicare (Title XVIII of the Social Security Act); and (vii) Medicaid (Title XIX of the Social Security Act).

“Highbridge” means Highbridge MSF International Ltd., an exempted company incorporated under the laws of the Cayman Islands, and 1992 Tactical Credit Master Fund, L.P., an exempted limited partnership organized under the laws of the Cayman Islands and Highbridge SCF Special Situations SPV, L.P., an exempted limited partnership formed under the laws of the Cayman Islands.

“HSR Act” means the Hart-Scott-Rodino Antitrust Improvements Act of 1976.

“IFRS” means International Financial Reporting Standards as adopted by the European Union.

“Independent” means a person who has been proposed as a Director of the Plan Investor who satisfies the criteria of an ‘independent director’ for the purposes of the NASDAQ rules, the AIM Rules, the Euronext Rules and standards and the Quoted Companies Alliance corporate governance code.

“Intellectual Property” means all intellectual property and rights related thereto, whether or not registrable, patentable or otherwise formally protectable, and whether or not registered, patented, otherwise formally protected or the subject of a pending application for registration, patent or any other formal protection, including: (a) patents, patent applications, statutory invention registrations, including reissues, divisionals, continuations, continuations-in-part, and reexaminations; (b) trademarks, trademark applications, trademark registrations, trade names, fictitious business names (d/b/as), service marks, service mark applications, service mark registrations, URLs, domain names, trade dress, logos, and other indicia of source or origin, and all goodwill associated with the foregoing; (c) copyrights and original works of authorship, whether or not registered, copyright registrations, and copyright applications; (d) computer software programs and software systems and related documentation, whether in source code or object code form; (e) data and database rights; and (f) trade secret and confidential information, including all confidential source code, know-how, processes, formulae, customer lists, inventions, and marketing information.

“IT Assets” means computers, software, servers, workstations, routers, hubs, switches, circuits, networks, data communications lines and all other information technology equipment.

“Law” or “Laws” means any applicable law, statute, ordinance, rule, regulation, order, judgment or decree of any Governmental Entity.

“Liability” or “Liabilities” means any and all assessments, charges, costs, damages, debts, obligations, expenses, fines, liabilities, losses and penalties, accrued or fixed, absolute or contingent, matured or unmatured or determined or determinable, liquidated or unliquidated, asserted or unasserted, disputed or undisputed, due or to become due, including those arising under any Law or in connection with any counterclaim or class action with respect to any claim by any Governmental Entity and those arising under any Contract and costs and expenses of any proceeding, assessment, judgment,

11

settlement or compromise relating thereto, and all interest, fines and penalties and reasonable legal fees and expenses incurred in connection therewith.

“NASDAQ” means the NASDAQ Global Select Market.

“New Convertible Notes” shall have the meaning set forth in the Plan.

“New Term Loan Agreement” shall have the meaning set forth in the Plan.

“Novelion Intercompany Loan Claim” shall have the meaning set forth in the Plan.

“Other Covered Party” means any political party or party official, or any candidate for political office.

“Outside Date” means the date that is one hundred and fifty (150) days after the Petition Date, as extended in accordance with Section 8.1(b)(ii).

“Permit” means any authorization, approval, consent, certificate, declaration, clearance, filing, notification, qualification, registration, license, permit or franchise or any waiver or exemption of any of the foregoing, of or from, or to be filed with or delivered to, any Person or pursuant to any Law.

“Permitted Encumbrances” means: (a) any Encumbrance permitted under the Plan or the Restructuring Support Agreement; (b) mechanics’, materialmen’s, and similar liens arising by operation of law and incurred in the ordinary course of business; (c) liens for Taxes not yet due and payable or for Taxes that the taxpayer is contesting through appropriate proceedings and for which adequate accruals or reserves have been established in accordance with GAAP (in the case of the Company or IFRS (in the case of the Plan Investor)); (d) purchase money liens with respect to equipment and liens securing rental payments under capital lease arrangements with respect to equipment, in each case, incurred in the ordinary course of business; (e) liens incurred in connection with any indebtedness for borrowed money, as identified in the Plan (including the DIP Financing Agreement), that shall be released at or prior to the Closing; (f) liens identified on Section 1.1(c) of the Company Disclosure Schedule; provided, however, for purposes of this definition as it is applied on the Closing Date, only those Encumbrances identified on Section 1.1(c) of the Company Disclosure Schedule that the Company designates pursuant to the Plan that will survive the Closing Date shall be deemed “Permitted Encumbrances”, and (g) restrictions on transfer imposed by federal and state securities laws.

“Person” shall have the meaning set forth in the Plan.

“Personal Data” has the same meaning as the terms “personal data,” “personal information,” or the equivalent under the applicable Data Protection Requirement.

“Petition Date” shall have the meaning set forth in the Plan.

“PFA Order” means an order of the Bankruptcy Court approving (i) the allowance and payment of the Company Termination Fee and the Company Expense Reimbursement Amount payable to the Plan Investor as permitted pursuant to Section 8.3 of this Agreement, as actual, necessary costs and expenses of preserving the Debtors’ estates entitled to priority as administrative expense claims against the Debtors under sections 503(b) and 507(a)(2) of the Bankruptcy Code and which amounts shall be senior in the Bankruptcy Cases to (x) all other administrative expense claims, (y) all liens securing any prepetition collateral, other than Prepetition Prior Liens (as defined in the Final DIP Order (as defined in the Restructuring Support Agreement)), and (z) any and all adequate protection liens and claims, in each

12

case other than any DIP Lien pursuant to section 364(d)(1) of the Bankruptcy Code or DIP Superpriority Claim (each as defined in the Final DIP Order) granted in connection with the DIP Financing Agreement and the indebtedness thereunder, (ii) the market-check procedures as provided in Section 6.9 of this Agreement, and (iii) the termination and remedy provisions of this Agreement and the Restructuring Support Agreement, including that the automatic stay provided in section 362 of the Bankruptcy Code shall be deemed automatically lifted and/or vacated with respect to any Plan Investor action related thereto, including, without limitation, exercise all of its rights and remedies pursuant to the terms of this Agreement, without further action or order of the Bankruptcy Court; provided, however, that any Company Termination Fee or Company Expense Reimbursement Amount shall be paid to the Plan Investor as required pursuant to Section 8.3 of this Agreement.

“Plan” means the proposed chapter 11 plan for the Company and Aegerion Pharmaceuticals Holdings, Inc., in the form attached to the Restructuring Support Agreement and as amended, as permitted in the Restructuring Support Agreement.

“Plan Investor Additional Equity Issuance” means the issuance, after the date of this Agreement and prior to the Closing, by the Plan Investor to certain Plan Investor Stockholders or other Persons, of a number of Plan Investor Shares representing, in the aggregate, no more than 10% of the total number of the issued and outstanding Plan Investor Shares as of the date of the Plan Investor Additional Equity Issuance, determined on a fully diluted basis, in exchange for cash, in circumstances where the approval of the Plan Investor Stockholders is not required in connection with such issuance; provided in such issuance, the Plan Investor shall use reasonable best efforts to obtain assurances from the Plan Investor Stockholders or other Persons, as applicable, obtaining Plan Investor Shares that such Plan Investor Stockholder or other Person will execute and become party to the Voting Agreement.

“Plan Investor Alternative Transaction” means any (a) firm offer made by any Person (other than the Company or any of its Affiliates or any other Person who is party to the Restructuring Support Agreement) to acquire the entire issued share capital of the Plan Investor in accordance with the requirements of the Takeover Code or (b) scheme of arrangement proposed by the Plan Investor to the Plan Investor Stockholders (other than the Scheme) pursuant to which any Person (other than the Company or any of its Affiliates or any other Person who is a party to the Restructuring Support Agreement) would acquire the entire issued share capital of the Plan Investor.

“Plan Investor Board” means the board of directors of the Plan Investor.

“Plan Investor Business” means the business of the Plan Investor Group substantially as conducted as of the date hereof.

“Plan Investor Employee Benefit Plan” means any: (a) employee benefit plan (within the meaning of Section 3(3) of ERISA, whether or not subject to ERISA) that is maintained or sponsored by any Plan Investor Group Member or to which any such Person contributes (or is required to contribute) or for which any Plan Investor Group Member otherwise has or may have any Liability, either directly or as a result of being an ERISA Affiliate or otherwise, whether or not funded and whether or not terminated, (b) personnel policies of any Plan Investor Group Member (including any employee handbooks), and (c) fringe or other benefit or compensation plans, policies, programs and arrangements, whether or not subject to ERISA, whether or not funded and whether or not terminated, including stock bonus or other equity compensation, deferred compensation, incentive compensation, pension, severance, retention, change of control, bonus, vacation, travel, incentive, and health or other medical, disability, life and welfare plans or insurance (whether insured or self-insured), policies, programs or arrangements, share purchase, share option, stock appreciation, phantom stock, savings, profit sharing or termination pay, supplementary unemployment benefit, retirement and supplementary retirement plans, programs,

13

agreements and arrangements, that are maintained or sponsored by any Plan Investor Group Member or to which any such Person contributes (or is required to contribute), or, in each case, for which any Plan Investor Group Member has any Liability, either directly or as a result of being an ERISA Affiliate or otherwise, and includes the Employee Share Option Plan (as adopted 18 April 2016 by the Plan Investor and as amended 25 May 2017), pursuant to which the Plan Investor Optionholders have been issued the Plan Investor Options.

“Plan Investor Equity Raise” shall have the meaning set forth in the Plan.

“Plan Investor Fundamental Representations” means the representations set forth in Section 5.1 (Organization), Section 5.2 (Qualification; Due Authorization; Power and Authority), and Section 5.19 (Brokers’ Fees).

“Plan Investor Group” means Plan Investor and its direct or indirect Subsidiaries.

“Plan Investor Group Member” means each of the Plan Investor and its direct or indirect Subsidiaries.

“Plan Investor’s Knowledge” or other words of similar import means the actual knowledge, after a reasonable best efforts inquiry, of those individuals identified in Section 1.1(a) of the Plan Investor Disclosure Schedule.

“Plan Investor Latest Balance Sheet” means the unaudited consolidated balance sheet of the Plan Investor and its Subsidiaries as of March 31, 2019 as set forth on Section 1.1(b) of the Plan Investor Disclosure Schedule.

“Plan Investor Material Adverse Effect” means any consideration, effect, occurrence, condition, change, development, event, state of facts or circumstance that: (a) has had, or would reasonably be expected to have, individually or in the aggregate, a material adverse effect on the business, results of operations, financial condition, assets or Liabilities or property of the Plan Investor Group, taken as a whole, or (b) has impacted or would reasonably be expected to impact, the ability of the Plan Investor to consummate the transactions set forth in, or perform its obligations under, any Transaction Document, in each case in any material respect; provided, that none of the following, either alone or in combination, shall be deemed to constitute, or be taken into account in determining whether there has been, a Plan Investor Material Adverse Effect: any consideration, effect, occurrence, condition, change, development, event, state of facts or circumstance (i) arising from general economic or political conditions or financial, banking, credit or securities market conditions, including any disruption thereof and any interest or exchange rate fluctuations; (ii) directly arising from the announcement or performance of, or compliance with, the pendency of, or the public or industry knowledge of, this Agreement or the transactions contemplated by this Agreement (other than compliance with Article V and it being understood that this clause (b)(ii) shall not apply with respect to any representation or warranty contained in this Agreement to the extent the purpose of such representation or warranty is to address the consequences resulting from the execution and delivery of this Agreement or the consummation of the transactions contemplated by this Agreement or the compliance with the terms of this Agreement) or the Bankruptcy Cases; (iii) arising from any changes in Laws or IFRS that the Plan Investor Group is required to adopt; (iv) arising from any actions required to be taken or not taken under this Agreement; (v)   arising from natural disasters, acts of terrorism or war (whether or not declared) or epidemics or pandemics; (vi) arising from the failure to meet any projections or forecasts (but not the underlying causes thereof which shall be considered in determining whether a Plan Investor Material Adverse Effect has occurred unless otherwise excluded pursuant to the terms of this definition); or (vii) arising out of any action taken or omitted to be taken at the written request or with the written consent of the other Party;

14

provided that in each case of the clauses (i), (iii), (iv), (v), and (vi) above, such consideration, effect, occurrence, condition, change, development, event, state of facts or circumstance does not have a disproportionate impact on the Plan Investor Group, taken as a whole compared to other companies operating in the industry in which the Plan Investor Group operate and in such event, only any such disproportionate impact shall be considered in determining whether a Plan Investor Material Adverse Effect has occurred.

“Plan Investor Stockholder Approval” means the requisite approval by resolution of the Plan Investor Stockholders to (i) approve, effect and implement the Acquisition and the other transactions contemplated by the Transaction Documents; (ii) approve the Plan Investor Equity Raise; (iii) confer authorities for the issue and allotment of the Closing Shares to be issued in connection with the Acquisition and the Plan Investor Shares to be issued in connection with the Plan Investor Equity Raise; (iv)  dis-apply all relevant preemption rights in respect of the allotment of the Closing Shares to be issued in connection with the Acquisition and the allotment of the Plan Investor Shares to be issued in connection with the Plan Investor Equity Raise; and (iv) approve any amendment of the Charter Documents of the Plan Investor required in connection with the foregoing.

“Plan Investor Termination Fee” shall be $2,050,000.

“Privacy Policies” means all published, posted and internal policies, procedures, agreements and notices relating to the Party’s collection, use, storage, disclosure, or cross-border transfer of Personal Data.

“Proceeding” means any suit, action, proceeding, arbitration, mediation, audit, hearing, inquiry or, to the knowledge of the Person in question, investigation (in each case, whether civil, criminal, administrative, investigative, formal or informal) commenced, brought, conducted or heard by or before, or otherwise involving, any Governmental Entity.

“Real Property Leases” means Company Real Property Leases and Plan Investor Real Property Leases.

“Registration Rights Agreement” means the agreement to be entered into between the Plan Investor or New Atlas Topco and certain lenders of the Company substantially in the form of which is attached hereto as Exhibit E.

“Release” means disposing, discharging, injecting, spilling, leaking, pumping, pouring, leaching, dumping, emitting, escaping or emptying into or upon the indoor or outdoor environmental, including any soil, sediment, subsurface strata, surface water, groundwater, ambient air, the atmosphere or any other media.

“Reorganized Company” means the Company following the Effective Date.

“Reorganized Company Group” means the Company and its direct or indirect Subsidiaries, following the Effective Date.

“Reorganized Company Group Member” means each of the Company and its Subsidiaries on and after the Effective Date and after giving effect to the Restructuring.

“Reorganized Company Interests” means all of the issued and outstanding equity interests of the Reorganized Company immediately following the Closing.

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“Restructuring Committee” means the restructuring committee of the Company Board.

“Rights Offering Documentation” means the documentation pursuant to which the Plan Investor Rights Offering Transactions and the Company Rights Offering Transactions will be made.

“Scheme Document” means the document to be sent to Plan Investor Stockholders and, for information purposes only, to persons with information rights and to Plan Investor Optionholders, containing, amongst other things, the Scheme and the notices convening the meetings of the Plan Investor Stockholders to consider and, if thought fit, approve the Scheme in accordance with the requirements of the Companies Act.

“SEC” means the U.S. Securities and Exchange Commission.

“Securities Act” means the Securities Act of 1933.

“Senior Officers” means, (a) with respect to the Company, the ten most highly-compensated officers of Novelion, the Company and the Company’s controlled Affiliates (collectively) and (b) with respect to the Plan Investor, the ten most highly-compensated officers of all Plan Investor Group Members (collectively).

“Subsidiaries” or “Subsidiary”, with respect to a Person, means each corporation, limited liability company, partnership, business association or other Person in which such Person owns, directly or indirectly, a majority of the voting power.

“Takeover Code” means the UK City Code on Takeovers and Mergers.

“Tax” or “Taxes” means all taxes, assessments, charges, dues, duties, rates, fees, imposts, levies and similar charges of any kind lawfully levied, assessed or imposed by any Governmental Entity under any applicable Tax Legislation, including United States federal, United Kingdom, German, Irish, provincial, state, territorial, county, municipal and local, foreign or other income, capital, capital gains, goods and services, sales, use, consumption, excise, value added, business, real property, personal property, transfer, franchise, withholding, payroll, or employer health taxes, customs, import, anti-dumping or countervailing duties, employment insurance premiums, and provincial workers’ compensation payments, levy, assessment, tariff, impost, imposition, toll and duty, whether computed on a separate, combined, unitary or consolidated basis or in any other manner, including any interest, penalties and fines associated therewith.

“Tax Legislation” means, collectively, all federal, provincial, state, territorial, county, municipal and local, foreign or other statutes, ordinances or regulations imposing a Tax, including all treaties, conventions, rules, regulations, orders and decrees of any jurisdiction.

“Tax Returns” means all returns, reports, declarations, elections, forms, slips, notices, filings, information returns, and statements in respect of Taxes that are required to be filed with any applicable Governmental Entity, including all amendments, schedules, attachments or supplements thereto and whether in tangible or electronic form.

“Transaction Documents” means this Agreement, the Restructuring Support Agreement, the Plan, the Voting Agreement, the CVR Instrument, the Scheme Document, the Backstop Agreement, the Registration Rights Agreement, the Shared Services Agreement, and each other Contract, exhibit, schedule, certificate and other document being delivered pursuant to, or in furtherance of the transactions contemplated by, this Agreement, the Restructuring Support Agreement or the Plan.

16

“Treasury Regulations” means the U.S. Treasury Regulations promulgated under the Code, including any successor regulations.

“Willful Breach” means a material breach of this Agreement that is the consequence of an act or omission by a Party with the actual knowledge that the taking of such act or failure to take such action would be a material breach of this Agreement.

Section 1.2.           Other Definitions. The following terms shall have the meanings defined in the Section indicated:

Acquisition	Recitals
Admission Document	Section 6.5(a)
Agreement	Preamble
AIM Rules	Section 5.4(c)
Antitrust Laws	Section 6.7(a)
Bankruptcy Cases	Recitals
Bankruptcy Code	Recitals
Bankruptcy Court	Recitals
Closing	Section 3.1
Closing Date	Section 3.1
Company	Preamble
Company Alternative Transaction	Section 6.9(c)
Company Alternative Transaction Agreement	Section 6.9(a)
Company Audited Financial Statements	Section 4.5(a)
Company Board Approval	Section 4.2
Company Closing Certificate	Section 7.2(a)
Company Disclosure Schedule	Article IV
Company Expense Reimbursement Amount	Section 8.3(f)
Company Financial Statements	Section 4.5(a)
Company Governmental Requirements	Section 4.3
Company Group Material Contracts	Section 4.8(a)
Company Group Material IP	Section 4.10(d)
Company Latest Balance Sheet Date	Section 4.5(a)
Company Notice of Intended Recommendation Change	Section 6.9(f)
Company Real Property Leases	Section 4.9(a)
Company Unaudited Financial Statements	Section 4.5(a)
CVR Distributions	Section 6.13
CVR Securities	Section 6.13
DEA	Section 4.21(b)
EIB Payoff Letter	Section 3.2(b)(iv)
Employment Agreements	Section 6.14(c)
Environmental Laws	Section 4.13(a)
Environmental Permits	Section 4.13(a)
Equity Transactions	Section 6.8(b)
Euronext Rules	Section 5.4(c)
FDA	Section 4.21(b)
Form F-1	Section 6.6(a)
Go-Shop Period	Section 6.9(b)
Governmental Requirements	Section 5.3
Hazardous Materials	Section 4.13(a)
Health Care Permits	Section 4.21(b)

17

NASDAQ Listing Application	Section 6.6(b)
New Atlas TopCo	Recitals
New Term Loan Financing	Section 7.1(j)
Nomad	Section 6.5(b)
Novelion	Recitals
Owned Company IP	Section 4.10(a)
Owned Plan Investor IP	Section 5.10(a)
Panel	Section 6.2(a)
Parties	Preamble
Party	Preamble
Plan Investor	Preamble
Plan Investor Audited Financial Statements	Section 5.5(a)
Plan Investor Board Recommendation	Section 5.2
Plan Investor Closing Certificate	Section 7.3(a)
Plan Investor Disclosure Schedule	Article V
Plan Investor Financial Statements	Section 5.5(a)
Plan Investor Governmental Requirements	Section 5.3
Plan Investor Group Material IP	Section 5.10(d)
Plan Investor IP	Section 5.10(b)
Plan Investor Latest Balance Sheet Date	Section 5.5(a)
Plan Investor Material Contracts	Section 5.8(a)
Plan Investor Optionholders	Recitals
Plan Investor Options	Recitals
Plan Investor Real Property Leases	Section 5.9(a)
Plan Investor Shares	Recitals
Plan Investor Stockholder Approval	Section 6.11
Plan Investor Stockholders	Recitals
Plan Investor Unaudited Financial Statements	Section 5.5(a)
Representatives	Section 6.9(b)
Restraining Order	Section 7.1(d)
Restructuring	Recitals
Restructuring Support Agreement	Recitals
Scheme	Recitals
Selected Court	Section 10.2(a)
Shared Services Agreement	Recitals
Voting Agreement	Recitals

ARTICLE II.

ACQUISITION; ISSUANCE OF CLOSING SHARES

Section 2.1.            Acquisition. Upon the terms and subject to the conditions set forth herein and in the Plan (as approved by the Bankruptcy Court pursuant to the Confirmation Order), at the Closing, the Company shall sell, issue, transfer and convey to the Plan Investor, and the Plan Investor shall purchase and acquire from the Company, the Reorganized Company Interests, free and clear of any Claims or Encumbrances (other than restrictions on transfer imposed by federal and state securities laws), together with all rights attaching to the Reorganized Company Interests. As a result of the Acquisition, the Company shall become a wholly-owned subsidiary of the Plan Investor and any equity interests of the Company issued and outstanding immediately prior to the Closing shall automatically be forfeited and cancelled in accordance with the Plan without any action by the Parties.

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Section 2.2.         Transaction Consideration.

(a)           Closing Shares. Upon the terms and subject to the conditions set forth herein and in the Plan, at the Closing, in consideration for the Acquisition, the Plan Investor shall issue the Closing Shares to the Company free and clear of all Encumbrances and deemed fully paid. Notwithstanding the foregoing, the Plan Investor shall issue New Warrants (as defined in the Plan) in lieu of the Closing Shares as and to the extent contemplated by the Plan.

(b)           Adjustment to Closing Shares. The Plan Investor may effect any stock split, reverse stock split, stock dividend (including any dividend or distribution of securities convertible into Plan Investor Shares) or other like change with respect to Plan Investor Shares occurring on or after the date hereof and prior to Closing, which shall be taken into account in determining the Closing Shares.

(c)            Distribution of the Closing Shares. Immediately following the issuance of the Closing Shares, the Company shall distribute such Closing Shares to Novelion and other creditors of the Company pursuant to the terms of the Plan and the other Transaction Documents (as approved by the Bankruptcy Court pursuant to the Confirmation Order).

ARTICLE III.

THE CLOSING

Section 3.1.          The Closing. The closing of the purchase and sale of the Reorganized Company Interests hereunder (the “Closing”) shall take place remotely via the electronic exchange of documents and signatures following the satisfaction or waiver of each of the conditions set forth in Article VII (other than those conditions that by their nature can only be satisfied at the Closing, but subject to the satisfaction or waiver of those conditions at such time) and on the same day as the Effective Date (as defined in the Plan), or on such other date or at such other time and place as the Parties mutually agreed upon in writing (the “Closing Date”). For the avoidance of doubt, the Closing Date shall be the same date as the Effective Date and any Party has the right to request an in-person Closing location, which shall occur at 10:00 a.m., New York time on the Closing Date at the offices of Gibson, Dunn & Crutcher LLP, 200 Park Avenue, New York, NY 10166. All proceedings to be taken and all documents to be executed and delivered by the Parties at the Closing will be deemed to have been taken and executed simultaneously and no proceedings will be deemed to have been taken nor documents executed or delivered until all have been taken, executed and delivered.

Section 3.2.        Closing Deliverables.

(a) At the Closing, the Company shall deliver to the Plan Investor, the following:

(i) evidence, in form and substance reasonably satisfactory to the Plan Investor, of transfer of the Reorganized Company Interests to the Plan Investor, free and clear of all Encumbrances (other than restrictions on transfer imposed by federal and state securities laws);

(ii) the Company Closing Certificate;

(iii) an affidavit issued to the Plan Investor by an officer of the Company as required by Treasury Regulations Section 1.1445-2(c)(3) certifying that the Company has not been a United States real property holding corporation (as the term is defined in the Code and the Treasury

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Regulations) at any time during the five (5)-year period ending on the Closing Date;

(iv) the New Term Loan Agreement shall have been entered into or shall be contemporaneously entered into providing for (i) the conversion of that certain Bridge Credit Agreement dated as of November 8, 2018 among Aegerion Pharmaceuticals, Inc., as borrower, the lenders party thereto and Cantor Fitzgerald Securities, as administrative agent, and (ii) the repayment in full of all obligations under that certain finance contract dated as of December 1, 2016 between Amryt Pharmaceuticals DAC and European Investment Bank;

(v) the New Convertible Notes shall have been issued or shall be contemporaneously issued; and

(vi) the Transaction Documents to which it is a party or to which it is contemplated to become a party at the Closing, duly executed by the Company.

(b) At the Closing, the Plan Investor shall deliver to the Company, the following:

(i) evidence, in form and substance reasonably satisfactory to the Company, of the issuance of the Closing Shares to the Company, free and clear of all Encumbrances (other than restrictions on transfer imposed by federal and state securities laws and the Registration Rights Agreement);

(ii) the Plan Investor Closing Certificate;

(iii) the Transaction Documents to which it is a party or to which it is contemplated to become a party at the Closing, duly executed by the Plan Investor; and

(iv) a payoff letter in form and substance reasonably satisfactory to the Company with respect to all indebtedness of the Plan Investor to European Investment Bank under that certain finance contract dated as of December 1, 2016 between Amryt Pharmaceuticals DAC and European Investment Bank (the “EIB Payoff Letter”).

ARTICLE IV.

REPRESENTATIONS AND WARRANTIES OF THE COMPANY

Except (i) as set forth in the disclosure schedule prepared by the Company (the “Company Disclosure Schedule”) and delivered to the Plan Investor simultaneously with the execution and delivery hereof and (ii) contemplated by Section 10.5 of this Agreement, the Company represents and warrants to the Plan Investor as follows:

Section 4.1.         Organization. Each Company Group Member is, and as of the Closing each Reorganized Company Group Member will be, duly incorporated, formed or organized, validly existing and (in the jurisdictions recognizing the concept) in good standing under the Laws of the jurisdiction in which such Person is incorporated, formed or domiciled. Each Company Group Member is, and as of the

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Closing each Reorganized Company Group Member will be, licensed or qualified to do business in each jurisdiction where the conduct or nature of its business or the ownership, leasing or holding of its properties makes such qualification necessary, except where the failure to be so licensed or qualified has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect. Each Company Group Member has the requisite power and authority to own, lease and operate its properties and to conduct its business as it is now being conducted, in each case, except where such failure has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect.

Section 4.2.           Qualification; Due Authorization; Power and Authority. Subject to approval of the Bankruptcy Court for actions to be taken after the Petition Date, the Company has all power and authority to execute and deliver this Agreement and the other Transaction Documents and to consummate the transactions contemplated hereby and thereby. The making, execution and delivery of this Agreement and the other Transaction Documents, and the performance of the obligations and covenants contained herein and therein have been duly and validly authorized by all necessary corporate actions of the Company. The Company Board, upon recommendation of the Restructuring Committee, at a meeting duly called and held, duly adopted resolutions (i) approving this Agreement and the other Transaction Documents, and (ii) determining that the terms of this Agreement and the other Transaction Documents are fair and in the best interests of the Company (the “Company Board Approval”). The Company Board Approval has not been rescinded, modified or withdrawn as of the date of this Agreement. This Agreement has been duly and validly executed and delivered by the Company and, assuming the due authorization, execution and delivery hereof by the Plan Investor, this Agreement will constitute the valid and binding obligations of the Company, and as of the Effective Date, the Reorganized Company, enforceable against the Company and the Reorganized Company, as applicable, in accordance with its terms (except as such enforcement may be limited by insolvency, reorganization, moratorium, receivership, conservatorship and by general equity principles).

Section 4.3.          Consents and Approvals. Neither the execution and delivery of this Agreement by the Company nor the consummation of the transactions contemplated hereby will (a) require any consent, approval, authorization, registration or filing under any Law to which the Company Group is subject or by which any of the assets of the Company Group is bound (the “Company Governmental Requirements”); (b) require the consent or approval of any other party to, or conflict with, result in any breach of, or constitute a default (or an event that, with notice or lapse of time or both, would become a default) under, any Contract to which any Company Group Member is a party; (c) give rise to a right of termination, cancellation or acceleration of any obligation or to loss of a material benefit under, or result in the creation of any Encumbrances (other than Permitted Encumbrances) upon any of the properties or assets of any Company Group Member; or (d) conflict with or result in a violation or breach of, or default under, any provision of the Charter Documents of any Company Group Member; in each case, other than (i)  on or after the Petition Date, the authorization or approval of the Bankruptcy Court, (ii) authorizations, consents, orders or approvals of, or registrations or declarations with, any Governmental Entity or other Person set forth on Section 4.3 of the Company Disclosure Schedule, (iii) authorizations, consents or approvals required under any applicable Antitrust Laws, and (iv) where the failure to obtain such consents, approvals, authorizations or registrations or to make such filings has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect. Any such authorization, consent, approval, order, registration or declaration that has been obtained, effected or given is in full force and effect as of the date hereof. Except as a result of the commencement of the Bankruptcy Cases, no Company Group Member is in default under, and no event has occurred that with the lapse of time or action by a third party could result in a default under, the terms of any judgment, order, writ, decree, Permit or license of any Governmental Entity where such default would reasonably be expected to have a Company Material Adverse Effect.

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Section 4.4.        Capitalization. With respect to each Company Group Member, Section 4.4 of the Company Disclosure Schedule sets forth a true, correct and complete list of the (i) name, (ii) type of entity, (iii) jurisdiction, (iv) the number and type of all authorized capital stock or other equity interests thereof, (v) the number and type of all issued and outstanding capital stock or other equity interests thereof, and (vi) the ownership of such capital stock or other equity interests as of the date of this Agreement. Except as set forth on Section 4.4 of the Company Disclosure Schedule, there are no other corporations, limited liability companies, partnerships, joint ventures, associations or other entities or Persons in which any Company Group Member owns as of the date of this Agreement, of record or beneficially, any direct or indirect equity or other interest or any right (contingent or otherwise) to acquire the same. Except as set forth on Section 4.4 of the Company Disclosure Schedule, all outstanding shares of capital stock or other equity interests of the Company Group Members have been duly authorized and validly issued as of the date of this Agreement. There are no outstanding warrants, options, rights, “phantom” stock rights, agreements, convertible or exchangeable securities or other commitments (other than this Agreement) (a) pursuant to which any Company Group Member is obligated to issue, sell, purchase, return or redeem any shares of capital stock or other equity securities of such Person or (b) that give any Person the right to receive any benefits or rights similar to any rights enjoyed by or accruing to the holders of shares of capital stock or other equity securities of any Company Group Member (including any rights to receive any payment in respect thereof) as of the date of this Agreement. There are no outstanding bonds, debentures, notes or other indebtedness having the right to vote on any matters on which equityholders of any Company Group Member may vote as of the date of this Agreement.

Section 4.5.         Financial Statements.

(a)         The Company has previously provided the Plan Investor with the following financial statements (collectively, the “Company Financial Statements”): (i) the audited consolidated balance sheets of Novelion which includes the Company Group as of December 31, 2018 and the related statements of income, cash flows and changes in owners’ equity for the fiscal year then ended, together with the notes to such Company Financial Statements and the opinion of the Novelion’s independent auditor thereon (the Financial Statements set forth in this clause (i), the “Company Audited Financial Statements”), and (ii) the unaudited consolidated balance sheet of Novelion which includes the Company Group as of March 31, 2019 (the “Company Latest Balance Sheet Date”) and the related statements of income and cash flows for the three (3)-month period then ended (the “Company Unaudited Financial Statements”). The Company Financial Statements have been prepared in all material respects in accordance with GAAP applied on a consistent basis throughout the periods indicated therein (except as set forth in footnote disclosures thereto) and except for (x) footnote disclosures thereto, and (y) with respect to the Company Unaudited Financial Statements, normal and recurring year-end adjustments (none of which, individually or in the aggregate, are material to the Company Group Members taken as a whole), the Company Financial Statements fairly present, in all material respects, the financial position, and results of operations, stockholders’ equity and cash flows of Novelion, on a consolidated basis, as of the dates and for the periods indicated therein. The Company Financial Statements were derived from the books and records of Novelion and the Company Group Members and present fairly in all material respects the financial condition of Novelion as of the respective dates they were prepared and the results of operations of the Novelion for the periods indicated therein.

(b)          Each Company Group Member maintains a system of internal accounting controls sufficient to provide reasonable assurances that: (i) transactions are executed in accordance with management’s general or specific authorization, (ii) subject to the disclosure set forth on Section 4.5(b) of the Company Disclosure Schedule, transactions are recorded as necessary to permit preparation of financial statements in conformity with GAAP and to maintain asset accountability, (iii) access to assets is permitted only in accordance with management’s general or specific authorization, and (iv) the

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recorded accountability for assets is compared with the existing assets at reasonable intervals and appropriate action is taken with respect to any differences.

Section 4.6.           No Undisclosed Liabilities. No Company Group Member has any Liabilities of a nature that would be required to be disclosed on a balance sheet prepared in accordance with GAAP (as in effect on the date hereof) except for (i) any Liability identified in the Company Latest Balance Sheet; (ii)  current Liabilities that have arisen after the Company Latest Balance Sheet Date in the ordinary course of business; (iii) Liabilities arising in the ordinary course of business under any Contract (but, in each case, not Liabilities for breaches thereof); (iv) Liabilities incurred in connection with the Bankruptcy Cases, the DIP Financing Agreement or the transactions contemplated thereby, which Liabilities described in this clause (iv) will be treated pursuant to the Plan, and (v) Liabilities incurred in connection with this Agreement or other Transaction Documents. No Company Group Member has any “off-balance sheet arrangements” (as such term is defined in Item 303(a)(4) of Regulation S-K promulgated under the Exchange Act).

Section 4.7.         Recent Events.

(a)          Since the Company Latest Balance Sheet Date until the date hereof, (i) each Company Group Member has conducted its business in all material respects in the ordinary course of business (except in connection with the transactions contemplated by this Agreement and the other Transaction Documents) and (ii) there has not been a Company Material Adverse Effect.

(b)           Without limiting the generality of the foregoing Section 4.7(a), except as expressly contemplated by any Transaction Document or as set forth on Section 4.7(b) of the Company Disclosure Schedule, no Company Group Member has since the Company Latest Balance Sheet Date and through the date hereof:

(i) subjected a material portion of its properties or assets to any Encumbrances, except for Permitted Encumbrances;

(ii) sold, assigned or transferred a material portion of its assets, except in the ordinary course of business and except for sales of obsolete assets or assets with de minimis book value;

(iii) amended its Charter Documents;

(iv) made any material change in any method of accounting or accounting practice of the Company, except as required by GAAP or as disclosed in the notes to the Company Audited Financial Statements;

(v) incurred, assumed or guaranteed any indebtedness for borrowed money, except unsecured current obligations and Liabilities incurred in the ordinary course of business;

(vi) cancelled any material debts or claims or waived any material rights against a Person that is not a Company Group Member;

(vii) taken any action to make, change or rescind any material Tax election, amend any material Tax Return or taken any position on any Tax Return, taken any action, omitted to take any action or entered into any other transaction that would have the effect of increasing the Tax liability of any Company Group Member in

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 respect of any Tax period starting after the Closing Date, in each case other than in the ordinary course of business; or

(viii) entered into any Contract to do any of the foregoing.

Section 4.8.         Contracts and Commitments.

(a)           Section 4.8 of the Company Disclosure Schedule lists the following Contracts (including all amendments, modifications and supplements thereto) to which a Company Group Member is a party as of the date hereof (collectively, the “Company Group Material Contracts”):

(i) (A) any material Contract relating to the borrowing of money or to the issuance of any note, bond, debenture or other evidence of indebtedness, or to mortgaging, pledging or otherwise placing a material Encumbrance on any securities or assets of any Company Group Member; (B) any Contract in the nature of a letter of credit, bankers’ acceptance and similar facilities involving any Company Group Member as an account party or beneficiary; (C) any Contract in the nature of a capital or direct financing lease that is required by GAAP to be treated as a long-term liability involving payments above $250,000 annually; and (D) any Contract containing material earn-out obligations or other contingent payment obligations for the deferred purchase price of property or services, in each case other than any such Contracts whose liabilities will be fully discharged under the Bankruptcy Code;

(ii) any Contract involving any guaranty of any obligation for borrowed money or other material guaranty, performance or completion bond or indemnity or surety arrangement or otherwise relating to the assumption or guarantee of any obligation by or of any Company Group Member, other than any such Contracts whose liabilities will be fully discharged under the Bankruptcy Code;

(iii) any license, sublicense, development, collaboration or royalty agreement or other Contract relating to the use by any Company Group Member of any material third-party Intellectual Property (other than commercially available software or software subject to click-through or shrink-wrap agreements);

(iv) any license, sublicense, development, collaboration or royalty agreement or other Contract relating to the use of any Intellectual Property of any Company Group Member by any third party (other than licenses granted to customers, resellers and distributors in the ordinary course of business) pursuant to which any Company Group Member receives annual payments above $250,000;

(v) any Contract including a covenant not to compete with any Person, Contracts granting any exclusivity or preferential right of first refusal or right of first offer to any Person or otherwise creating an exclusive relationship with a Person, in each case, to the extent such Contract materially restricts or limits the activities of any Company Group Member or the ability of any Company Group Member to engage or

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compete in any line of business or any geographic area or from developing or commercializing any pharmaceutical products;

(vi) any Contract for the acquisition or disposition of any business, any merger, consolidation, plan or scheme of arrangement or reorganization, or acquisition or disposition of a material amount of stock or assets of any Person or any material real property (whether by merger, sale of stock, sale of assets or otherwise) to the extent any Company Group Member has any remaining material obligations thereunder;

(vii) other than as contemplated by the applicable Transaction Documents, any Contract that by its terms limits the payment of dividends or other distributions by the Company;

(viii) any Contract involving consideration in excess of $250,000 individually, and $500,000 in aggregate for Contracts involving substantially the same customer, supplier or subject matter, and which, in each case, cannot be cancelled by the applicable Company Group Member without penalty or without more than thirty (30) days’ notice;

(ix) employment agreements and Contracts with independent contractors or consultants which are not cancellable without material penalty or without more than thirty (30) days’ notice;

(x) any Contracts between any directors or officers of any Company Group Member or any of their Affiliates, on the one hand, and such Company Group Member or any other Company Group Member, on the other hand;

(xi) material Contract that provides for any joint venture, partnership or similar arrangement or any Contract involving a sharing of revenues, profits, losses, costs or Liabilities between any Company Group Member, on the one hand, and any other Person, on the other hand;

(xii) any “single source” supply Contract pursuant to which goods or materials that are material to the Company Business are supplied to any Company Group Member from an exclusive source; or

(xiii) any Contract with any Governmental Entity.

(b)          The Plan Investor either has been supplied with, or has been given access to, a true, correct and complete copy of all written Company Group Material Contracts or a summary of all oral Company Group Material Contracts. Except as (i) set forth in the Plan, or (ii) has not had and would not reasonably be expected to have a Company Material Adverse Effect, each Company Group Material Contract (assuming due power and authority of, and due execution and delivery by, the other party or parties thereto) is in full force and effect and is valid, binding and enforceable against the applicable Company Group Member and, to the Company’s Knowledge, the other parties thereto, in accordance with their respective terms (except as may be limited by bankruptcy, insolvency, moratorium or other similar laws affecting creditors rights).

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(c)          Except as has not had and would not reasonably be expected to have a Company Material Adverse Effect or except as set forth in the Plan or on Section 4.8 of the Company Disclosure Schedule, (A) within the one-year period preceding the date of this Agreement, no Company Group Member has violated or breached, or committed any default in any respect under, any Company Group Material Contract that remains uncured as of the date hereof, and (B) to the Company’s Knowledge, as of the date of this Agreement, no other Person has violated or breached, or committed any default in any respect under, any Company Group Material Contract that remains uncured as of the date hereof; and (C) as of the date of this Agreement, no event has occurred and is continuing through any Company Group Member’s actions or inactions, as applicable, that will result in a violation or breach in any respect of any of the provisions of any Company Group Material Contract.

Section 4.9.         Real Property.

(a)          No Company Group Member owns any real property or any interest in real property other than the leaseholds created under the real property leases or subleases for the properties identified on Section 4.9(a) of the Company Disclosure Schedule (including all amendments, modifications, terminations and extensions thereof, the “Company Real Property Leases”). Section 4.9(a) of the Company Disclosure Schedule contains a true, correct and complete list of all Company Real Property Leases with respect to all real property leased, licensed, subleased or otherwise used or occupied by any Company Group Member.

(b)          The Company Real Property Leases are in full force and effect and are valid and binding against the applicable Company Group Member and, to the Company’s Knowledge, the other parties thereto in accordance with their respective terms (except as may be limited by bankruptcy, insolvency, moratorium or other similar laws affecting creditors’ rights). No Company Group Member has leased, subleased or granted to any Person a right to possess, lease or occupy any portion of the real property subject to any Company Real Property Lease.

(c)           The Company has delivered or made available to the Plan Investor complete and accurate copies of each of the Company Real Property Leases, and none of such Company Real Property Leases has been materially amended, modified, terminated or extended as of the date hereof in any respect, except to the extent that such amendments, modifications, terminations or extensions are disclosed by copies delivered or made available to the Plan Investor.

(d)          No Company Group Member is in default under any of the Company Real Property Leases that remains uncured as of the date hereof, in each case, except as has not had and would not reasonably be expected to have, individually or in the aggregate with other uncured defaults, a Company Material Adverse Effect

Section 4.10.       Intellectual Property.

(a)           Section 4.10(a) of the Company Disclosure Schedule sets forth a complete and accurate list of all (i) (A) issued patents and pending patent applications, (B) trademark and service mark registrations and applications, (C) copyright registrations and applications, and (D) internet domain name registrations, in each case that are owned by the Company Group Members (collectively, the “Owned Company IP”), and (ii) material (A) issued patents and pending patent applications, (B) trademark and service mark registrations and applications, (C) copyright registrations and applications, and (D) internet domain name registrations, in each case that are licensed to the Company Group Members. Except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect, the Company Group Members own all right, title and interest in the Owned Company IP, free and clear of all Encumbrances (other than Permitted Encumbrances). Each item of

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material Owned Company IP has been duly registered in, filed in or issued by, as applicable, an official governmental register and/or issuer (or officially recognized register or issuer) and each such registration, filing, issuance and/or application, (x) has not been abandoned or cancelled, (y) has been maintained effective by all requisite filings, renewals and payments, and (z) to the Knowledge of the Company remains in full force and effect.

(b)           The Company Group Members own and possess all right, title and interest in and to (or have the right pursuant to a valid and enforceable license or otherwise possess legally enforceable rights to use) all Intellectual Property that is necessary for or used or held for use in the conduct of the Company Business (the “Company IP”). Neither the execution and delivery of this Agreement by the Company, nor the performance of this Agreement by the Company, will result in the loss, forfeiture, termination, or impairment of, or give rise to a right of any Person to limit, terminate, or consent to the continued use of, any rights of any Company Group Member in any Company IP.

(c)           No Company Group Member is infringing, misappropriating, diluting, or otherwise violating the Intellectual Property rights of any Person. No Company Group Member has received any written charge, complaint, claim, demand, or notice since January 1, 2017 (or earlier, if presently not resolved) alleging any such infringement, misappropriation, dilution, or violation (including any claim that any Company Group Member must license or refrain from using any Intellectual Property rights of any Person) which alleged infringement, misappropriation, dilution, or violation, if true, would reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect. To the Knowledge of the Company, no Person is infringing, misappropriating, diluting or otherwise violating any Owned Company IP. No Company Group Member has made or asserted any charge, complaint, claim, demand or notice since January 1, 2017 (or earlier, if presently not resolved) alleging any such infringement, misappropriation, dilution, or violation which alleged infringement, misappropriation, dilution, or violation, if true, would reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect.

(d)            Each applicable Company Group Member has taken reasonable best effort steps to maintain, police and protect the Intellectual Property that is material to the Company Business (“Company Group Material IP”). All Company Group Material IP that derives actual or potential economic value from not being generally known to the public or to other persons who can obtain economic value from its disclosure or use has been maintained in confidence in accordance with protection procedures that are in accordance with procedures customarily used in the industry to protect rights of like importance and, to the Knowledge of the Company, adequate for protection against unauthorized disclosure or use. To the Knowledge of the Company, there has been no unauthorized disclosure of any Company Group Material IP. Except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect, to the Knowledge of the Company, all former and current officers, directors, employees, personnel, consultants, advisors, agents, and independent contractors of any Company Group Member, and each of its predecessors, who have contributed to or participated in the conception and development of Intellectual Property for such entities have entered into valid and binding proprietary rights agreements with the applicable Company Group Member or one of its predecessors, vesting ownership of such Intellectual Property in the applicable Company Group Member. No such Person has asserted, and to the Knowledge of the Company, no such Person has, any right, title, interest or other claim in, or the right to receive any royalties or other consideration with respect to, any Company Group Material IP.

(e)           The IT Assets of the Company Group Members operate in all material respects in accordance with their documentation and functional specifications and as required to operate the Company Business and have not, since January 1, 2017, materially malfunctioned or failed. Each Company Group Member has implemented reasonable best effort measures to protect the confidentiality

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and security of such IT Assets and information stored or contained therein against any unauthorized use, access, interruption or corruption, and to the Knowledge of the Company, there has been no such unauthorized use, access, interruption or corruption that has not been remedied in all material respects. Each Company Group Member has implemented reasonable best effort procedures regarding data backup, data storage, system redundancy and disaster avoidance procedures with respect to their IT Assets.

Section 4.11.       Privacy and Data Security.

(a)           Each of the Company Group Members comply with, and since January 1, 2017 have complied with, in all material respects, all Data Protection Requirements.

(b)         There are no restrictions on any Company Group Member’s collection, use, disclosure and retention of Personal Data, except as provided by the Data Protection Requirements. There are no ongoing material Proceedings, and to the Company’s Knowledge, there are no pending or to the Knowledge of the Company threatened Proceedings, with respect to any Company Group Member’s violation of any Data Protection Requirement. No decision, judgment or order, whether statutory or otherwise, is pending or has been made, and no notice, complaint, claim, enforcement action, or litigation of any kind has been served on or initiated against any of the Company Group Members pursuant to any Data Protection Requirement.

(c)           Each of the Company Group Members have taken reasonable best effort steps, compliant with applicable Data Protection Requirements, to protect (i) the operation, confidentiality, integrity, and security of the Company Group’s software, systems, and websites that are involved in the collection and/or processing of Personal Data, and (ii) Personal Data in the Company Group’s possession and/or control from unauthorized use, access, disclosure, and modification.

(d)           None of the Company Group Members have experienced any failures, crashes, security breaches, unauthorized access, use, or disclosure, or other adverse events or incidents related to Personal Data that would require notification of individuals, law enforcement, or any Governmental Entity, any remedial action under any applicable Data Protection Requirement, or that have caused any substantial disruption of or interruption in the use of the Company Group’s software, equipment or systems

Section 4.12.         Legal Compliance; Permits. Except as set forth on Section 4.12 of the Company Disclosure Schedule:

(a)           Since January 1, 2017 and as of the date hereof, each Company Group Member has been in compliance with all Laws applicable to such Company Group Member other than any such noncompliance that has not had or would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect. All Permits required to conduct the Company Business are in the possession of the applicable Company Group Member, are in full force and effect and are being complied with, in each case, except when such failure would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect. Except as would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect, the current conduct of the Company Business is not, and has not been since January 1, 2017, in default or violation under any Permit (except for such violation that has been remedies and imposes no continuing Liability) and, to the Knowledge of the Company, no event has occurred which, with notice or the lapse of time or both, would constitute a default or violation of any term, condition or provision of any applicable Permit. There are no actions pending, or to the Knowledge of the Company, threatened in writing, that seek revocation, cancellation or modification of any applicable Permit, except where such revocation,

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cancellation or modification has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect.

(b)          The Company is and shall remain in full compliance with each of the Agreements with Governmental Entities provided on Section 4.12 of the Company Disclosure Schedule. There is no material proceeding or disciplinary action (including fines) by any Governmental Entity currently pending or, to the Company’s Knowledge, threatened in writing against any Company Group Member, any of their respective assets, rights or properties or any of their respective officer or directors, in each case, except for those that have not had, and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect.

Section 4.13.       Environmental Compliance and Conditions.

(a)          The Company Group Members have obtained and possess all material Permits (“Environmental Permits”) required under Laws and regulations concerning occupational health and safety, pollution or protection of the environment that were enacted and in effect on or prior to the date hereof, including all such Laws and regulations relating to the emission, discharge, release or threatened release of any chemicals, petroleum, pollutants, contaminants or hazardous or toxic materials, substances or wastes (“Hazardous Materials”) into ambient air, surface water, groundwater or lands or otherwise relating to the manufacture, processing, distribution, use, treatment, storage, disposal, transport or handling of any Hazardous Materials (“Environmental Laws”), in each case, except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect.

(b)           The Company Group Members are, and since January 1, 2017 have been, in compliance in all material respects with all terms and conditions of such Environmental Permits and are, and since January 1, 2017 have been, in compliance in all material respects with all other Environmental Laws or any written notice or demand letter issued, entered, promulgated or approved thereunder.

(c)            There are no Environmental Claims pending, nor to the Knowledge of the Company, threatened against any Company Group Member, and to the Knowledge of the Company, no Company Group Member has received any notification of any allegation of actual or potential responsibility for any Release or threatened Release of any Hazardous Materials with respect to any location currently or formerly owned, leased, operated or used by such Company Group Member. There have been no Releases of Hazardous Materials at any properties that are operated, leased or used by any Company Group Member, or to the Knowledge of the Company, at properties that were formerly owned, operated, leased or used by any Company Group Member, that are reasonably likely to cause any Company Group Member to incur any material Liability pursuant to applicable Environmental Law. No Company Group Member (i) has entered into or agreed to any consent decree or consent order or is otherwise subject to any judgment, decree, or judicial or administrative order relating to compliance with Environmental Laws or Environmental Permits, the investigation, sampling, monitoring, treatment, remediation, response, removal or cleanup of Hazardous Materials, and no Proceeding is pending, or to the Knowledge of the Company is threatened, with respect thereto, and (ii) is an indemnitor by contract or otherwise in connection with any claim, demand, suit or action threatened or asserted by any third-party for any Liability under any Environmental Law or otherwise relating to any Hazardous Materials.

(d)         The representations and warranties in this Section 4.13 constitute the sole and exclusive representations and warranties of the Company with respect to any environmental, health or safety matters, including any arising under Environmental Law, and no other representation or warranty contained in any other Section of this Agreement shall apply to any such matters and no other representation or warranty, express or implied, is being made with respect thereto.

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Section 4.14.         Litigation. Except as set forth on Section 4.14 of the Company Disclosure Schedule, since January 1, 2017 and as of the date hereof, except for the anticipated Bankruptcy Cases, there has not been (a) any material pending action, suit, proceeding, claim, administrative or court action or other litigation, or to the Company’s Knowledge, any material investigation by any Governmental Entity, pending, or, (b) to the Company’s Knowledge, any material threatened action, suit, proceeding, claim, administrative or court action or other litigation threatened in writing, in each case (X) against any Company Group Member or (Y) that involves any Company Group Member, that is reasonably expected to have a Company Material Adverse Effect. Except as set forth on Section 4.14 of the Company Disclosure Schedule, no Company Group Member or any of such Person’s assets or its Liabilities are subject to any judicial or administrative or other order issued by, or agreement entered into with, a Governmental Entity except as would not be material to the Company Group Members, taken as a whole.

Section 4.15.       Tax Matters.

(a)           Since January 1, 2017, the Company Group has filed (or have had filed) all federal and other material Tax Returns that it was required to file (or to have filed), taking into account any extensions of time to file. All such Tax Returns were correct and complete in all material respects. All material Taxes of the Company Group (whether or not shown as owing by such Person on such Person’s Tax Returns) have been fully paid or properly accrued and reserved for in accordance with GAAP. No material claim has ever been made by an authority in a jurisdiction where the Company Group does not file Tax Returns that the Company Group is or may be subject to taxation by that jurisdiction. There are no material liens for Taxes (other than Taxes not yet due and payable) upon any of the assets of any Company Group Member.

(b)          No Company Group Member is, as of the date hereof, the subject of a Tax audit or examination with respect to material Taxes. There are no outstanding agreements or waivers extending the statutory period of limitations applicable to any federal or other material Tax Return of the Company Group. No Company Group Member has granted a power of attorney that is in effect with respect to any Tax matters.

(c)           The Company Group does not have any current material Liability for Taxes of any Person other than itself, including (i) under Treasury Regulations Section 1.1502-6 or (ii) as a transferee or successor, by Contract or otherwise.

(d)          No Company Group Member has been a United States real property holding corporation within the meaning of Section 897(c)(2) of the Code during the applicable period specified in Section 897(c)(1)(A)(ii) of the Code.

(e)           To the Company’s Knowledge, the Company has not been a party to a “listed transaction,” as such term is defined in Section 6707A(c)(1) of the Code and Treasury Regulations Section 1.6011-4(b)(2).

(f)           All material Taxes that any Company Group Member was obligated to withhold from amounts owing to any person, including any employee, independent contractor, stockholder, creditor or third party, in each case, prior to the date hereof, have been fully and timely paid, withheld and remitted or properly accrued.

(g)           No Company Group Member has distributed stock of another Person, or has had its stock distributed by another Person, in a transaction that was purported or intended to be governed in whole or in part by Section 355 or Section 361 of the Code.

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(h)          There are no Tax rulings, requests for rulings, closing agreements, or any other Contracts with any Tax authorities that relate to any Company Group Member that could have a material effect on the liability of any Reorganized Company Group Member for Taxes for any Tax period ending after the Closing Date.

(i)            The representations and warranties in this Section 4.15 constitutes the sole and exclusive representations and warranties of the Company with respect to Taxes related to the Company Group, and no other representation or warranty contained in any other section of this Agreement shall apply to any such Tax matters and no other representation or warranty, express or implied, is being made with respect thereto.

Section 4.16.         Insurance. Section 4.16 of the Company Disclosure Schedule lists each insurance policy maintained by each Company Group Member as of the date hereof, and the deductibles and coverage limits for each such policy. The Company has made available to the Plan Investor a copy of such policies. All such insurance policies are in full force and effect, and applicable Company Group Member is not in default with respect to any material obligations under any such insurance policy. All premiums in respect of each insurance policy maintained by any of Company Group Member have been paid when due; to the Company’s Knowledge as of the date of this Agreement no material default on the part of the counterparty to such policy exists. The applicable Company Group Member has not received written notice of cancellation of any insurance policies listed on Section 4.16 of the Company Disclosure Schedule. There is no claim pending under any such insurance policies as to which, to the Company’s Knowledge, coverage has been questioned, denied or disputed by the underwriters of such policies

Section 4.17.       Illegal or Improper Payments.

(a)          Each Company Group Member (i) is in compliance, and since January 1, 2017 has been in compliance, in all material respects with the FCPA and any other applicable Anti-corruption Laws; (ii) since January 1, 2017 has not been investigated by any Governmental Entity with respect to, and to the Knowledge of the Company, has not been given notice in writing by a Governmental Entity or any other Person of, any actual or alleged violation by any Company Group Member of the FCPA or any other Anti-corruption Laws; and (iii) during the past five (5) years has had an operational and effective FCPA and anticorruption compliance program that includes, at a minimum, policies, procedures and training intended to enhance awareness of and compliance by each Company Group Member with the FCPA and any other applicable Anti-corruption Laws.

(b)           To the Knowledge of the Company, no Company Group Member has, directly or indirectly through its Representatives or any Person authorized to act on its behalf (including any distributor, agent, sales intermediary or other third party), offered, promised, paid, authorized or given money or anything of value to any Person for the purpose of: (i) influencing any act or decision of any Government Official or Other Covered Party; (ii) inducing any Government Official or Other Covered Party to do or omit to do an act in violation of a lawful duty; (iii) securing any improper advantage; or (iv) inducing any Government Official or Other Covered Party to influence the act or decision of a government or government instrumentality, in order to obtain or retain business, or direct business to, any Person or entity, in any way.

(c)         To the Knowledge of the Company, since January 1, 2017, each Company Group Member has maintained complete and accurate books and records, including records of payments to any agents, consultants, representatives, third parties and Government Officials, in accordance with GAAP, in all material respects. There have been no false or fictitious entries made in the books and records of any Company Group Member relating to any unlawful offer, payment, promise to pay, or authorization of the payment of any money, or unlawful offer, gift, promise to give, or authorization of the giving of anything

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of value, including any bribe, kickback or other illegal or improper payment, and no Company Group Member has established or maintained a secret or unrecorded fund.

(d)           To the Knowledge of the Company, since January 1, 2017, no Company Group Member has had a customer or supplier or other business relationship with, is a party to any Contract with, or has engaged in any transaction with, any Person (i) that is organized or domiciled in or that is a citizen of Crimea, Cuba, Iran, North Korea or Syria (including any Governmental Entity within such country) or (ii) that is the subject of any international economic or trade sanction administered or enforced by the Office of Foreign Assets Control of the United States Department of the Treasury, the United Nations Security Council, the European Union, Her Majesty’s Treasury, the United Kingdom Export Control Organization or other relevant sanctions authority.

Section 4.18.         Related Party Transactions. No officer, member of the board of directors or managers (or equivalent governing body) of any Company Group Member or, to the Company’s Knowledge, any individual in such officer’s, director’s or manager’s immediate family or Affiliate of any such Person is a party to any material Contract or transaction with any Company Group Member or has any material interest in any material property that is currently used by any Company Group Member, other than under an Company Employee Benefit Plan or pursuant to an employment agreement or as contemplated by this Agreement or the Transaction Documents.

Section 4.19.        Brokers’ Fees. Except as set forth on Section 4.19 of the Company Disclosure Schedule, neither the Company nor any of its officers or directors on behalf of the Company has employed any financial advisor, broker or finder or incurred any liability for any financial advisory fee, broker’s fees, commissions or finder’s fees in connection with any of the transactions contemplated by the Transaction Documents.

Section 4.20.         Employees. Section 4.20(a) of the Company Disclosure Schedule sets out, with respect to each Company Group Member, the name, age, position, title, length of employment, status such as full time, part time, exempt or non-exempt, employee or independent contractor, total annual remuneration (including a breakdown of salary and bonus) or other incentive compensation and other terms and conditions of employment (other than Company Employee Benefit Plans) of all employees of such Company Group Member including, solely for the purposes of such schedule, Novelion.

(b)         No Company Group Member is bound by or a party to any collective bargaining agreement, agreement with any works council, or labor contract. There are no actual, or to the Knowledge of the Company, threatened or pending organizing activities of any trade union, council of trade unions, employee bargaining agency or affiliated bargaining agent or any actual, threatened or pending unfair labor practice complaints, strikes, work stoppages, picketing, lock-outs, hand-billings, boycotts, slowdowns, arbitrations, grievances, complaints, charges or similar labor-related disputes or proceedings pertaining to any of the Company Group Members, and there have not been any such activities or disputes or proceedings January 1, 2017, in each case, except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect.

(c)           Each Company Group Member is, and since January 1, 2016 has been, in compliance with all Laws respecting employment and employment practices, including, without limitation, all Laws respecting terms and conditions of employment, health and safety, wage payment, wages and hours, child labor, collective bargaining, immigration and work authorizations, employment discrimination, retaliation, civil rights, veterans’ rights, disability rights or benefits, equal opportunity, plant closures and layoffs, affirmative action, workers’ compensation, labor relations, social welfare obligations, proper classification of employees as exempt and non-exempt for purposes of wage and hour laws and as employees and independent contractors, unemployment insurance and the collection and

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payment of withholding and/or social security Taxes and any similar Tax, except for noncompliance that has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect.

(d)           Section 4.20(d) of the Company Disclosure Schedule contains a list of every Company Employee Benefit Plan. The Company has delivered to the Plan Investor true, complete and up-to-date copies of all Company Employee Benefit Plans and all amendments thereto together with, if applicable, all summary descriptions thereof, past or present participants therein, the statement of investment policies for each such Company Employee Benefit Plans, all funding agreements and service provider Contracts or other Contracts (including insurance Contracts, investment management agreements, subscription and participation agreements and recordkeeping agreements) relating thereto, the two most recent actuarial reports, the financial statements and evidence of any registration or qualification in respect thereof, in each case, to the extent any Company Group Members may have Liability under such Company Employee Benefit Plans.

(e)         All of the Company Employee Benefit Plans are duly registered or qualified where required by applicable Law (including registration or qualification with the relevant Tax authorities where such registration or qualification is required to qualify for Tax exemption or other beneficial Tax treatment) and have always been administered in compliance with their terms and all applicable Laws. Each Company Employee Benefit Plan intended to be tax-qualified within the meaning of Section 401(a) of the Code is subject to a favorable determination or opinion letter from the Internal Revenue Service and, to the Knowledge of the Company, nothing has occurred that could reasonably be expected to adversely impact such tax-qualified status. The Company Group Members have no direct or contingent obligation with respect to any plan subject to Title IV of ERISA or any obligation to provide post-employment welfare benefits except to the extent required by Section 4980B of the Code or similar law.

(f)          Neither the execution, delivery or performance of this Agreement nor the consummation of any of the transactions contemplated herein will result in any bonus, golden parachute, severance or other payment, obligation or liability to any current or former employee or director of any of Company Group Member (whether or not under any Company Employee Benefit Plan), materially increase the benefits payable or provided under any Company Employee Benefit Plan, result in any acceleration of the time of payment or vesting of any such benefit, increase or accelerate employer contributions thereunder or result in any payment that could be nondeductible pursuant to Section 280G of the Code.

(g)           There are no claims pending or, to the Knowledge of the Company, threatened against any of the Company Group Members with respect to any Company Employee Benefit Plans and their funding agent, the insurers or the fund of such Company Employee Benefit Plans, other than claims for benefits in the ordinary course.

(h)           All of the Company Employee Benefit Plans are fully funded in accordance with their terms and all applicable Laws and generally accepted actuarial principles and practices.

Section 4.21.       Healthcare Compliance Matters.

(a)          Except as set forth on Section 4.21(a) of the Company Disclosure Schedule (i) each Company Group Member is in compliance and since January 1, 2017 has been in compliance with all Health Care Laws applicable to such Company Group Member or any assets owned or used by it and (ii)   no Company Group Member has received any written communication or has been subject to any Proceeding (other than routine FDA inspections) since January 1, 2017 from a Governmental Entity that alleges that such Company Group Member is not in compliance with any Health Care Law, except in the

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case of the immediately foregoing clauses (i) and (ii) where any non-compliance has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect. Except as set forth on Section 4.21(a) of the Company Disclosure Schedule, (i) no Company Group Member is party to and has any ongoing obligations pursuant to or under any corporate integrity agreements, deferred prosecution agreements, monitoring agreements, consent decrees, settlement orders, plans of correction or similar agreements with or imposed by any Governmental Entity, and (ii) no Company Group Member or any of its employees, officers or directors has been excluded, suspended or debarred from participation in any U.S. state or federal health care program or, to the Knowledge of the Company, has been convicted of any crime or is subject to any Proceeding by any Governmental Entity or other similar action, or has engaged in any conduct, that could reasonably be expected to result in debarment, suspension, or exclusion.

(b)          Each Company Group Member has, maintains and is operating in material compliance with all Permits of the United States Food and Drug Administration (“FDA”), Drug Enforcement Administration (“DEA”), and comparable Governmental Entities which are required for the conduct of the Company Business (collectively, the “Health Care Permits”), and all such Health Care Permits are valid, subsisting, and in full force and effect, except where the failure to have, maintain or operate in compliance with the Health Care Permits has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect. Each Company Group Member has fulfilled and performed all of its obligations with respect to the Health Care Permits, and no event has occurred which allows, or with notice or lapse of time or both, would allow revocation or termination thereof or results in any other material impairment of the rights of the holder of any Health Care Permit, except where the failure to so fulfill or perform, or the occurrence of such event, has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect. There is no Proceeding pending or, to the Knowledge of the Company, threatened in writing that could result in the suspension, termination, revocation, cancellation, limitation or impairment of any such Health Care Permit other than those that have not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect.

(c)          Except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect, all applications, notifications, submissions, information, claims, reports and statistics, and other data and conclusions derived therefrom, utilized as the basis for or submitted in connection with any and all requests for a Health Care Permit relating to any Company Group Member, its business and product candidates, when submitted to the FDA, DEA or other Governmental Entity were true, complete and correct as of the date of submission and any necessary or required updates, changes, corrections or modification to such applications, submissions, information and data have been submitted to the FDA, DEA or other Governmental Entity.

(d)           Except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect, January 1, 2017, no Company Group Member had any product, product candidate or manufacturing site subject to a Governmental Entity (including FDA or DEA) shutdown or import or export prohibition, and has not received any FDA Form 483 or other Governmental Entity notice of inspectional observations, “warning letters,” “untitled letters” or written requests or requirements to make changes to a product candidate, or similar correspondence or written notice from the FDA, DEA or other Governmental Entity alleging or asserting noncompliance with any applicable Health Care Law, Health Care Permit or such requests or requirements of a Governmental Entity.

(e)         Except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Company Material Adverse Effect, (i) the clinical, pre-clinical and other studies and tests conducted by or on behalf of or sponsored by any Company Group Member or in which any

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Company Group Member, or any of its product candidates have participated were, and if still pending are, being conducted in accordance with standard medical and scientific research procedures and all applicable Laws, including, but not limited to, the Federal Food, Drug and Cosmetic Act and its applicable implementing regulations and (ii) no investigational new drug application filed by or on behalf of any Company Group Member with the FDA has been terminated or suspended by the FDA, and neither the FDA nor any applicable foreign Governmental Entity has commenced, or, to the Knowledge of the Company, threatened to commence, any action to place a clinical hold order on, or otherwise terminate, delay or suspend, any proposed or ongoing clinical investigation conducted or proposed to be conducted by or on behalf of any Company Group Member.

(f)           No Company Group Member is the subject of any pending or, to the Knowledge of the Company, threatened investigation in respect of such Company Group Member or its product candidates, by the FDA pursuant to its “Fraud, Untrue Statements of Material Facts, Bribery, and Illegal Gratuities” Final Policy set forth in 56 Fed. Reg. 46191 (September 10, 1991) and any amendments thereto. The Company has provided the Plan Investor with accurate and complete copies of all Health Care Permits and correspondence with any Governmental Entity related to all product candidates of any Company Group Member.

Section 4.22.        No Other Representations or Warranties. NOTWITHSTANDING ANY PROVISION OF THIS AGREEMENT TO THE CONTRARY, EXCEPT FOR THE REPRESENTATIONS AND WARRANTIES EXPRESSLY MADE BY THE COMPANY IN THIS Article IV, NO COMPANY GROUP MEMBER, ANY AFFILIATE THEREOF, OR ANY OTHER PERSON MAKES ANY REPRESENTATION OR WARRANTY WITH RESPECT TO ANY COMPANY GROUP MEMBER, AFFILIATE THEREOF OR ANY OTHER PERSON OR THEIR RESPECTIVE BUSINESSES, OPERATIONS, ASSETS, LIABILITIES, CONDITION (FINANCIAL OR OTHERWISE) OR PROSPECTS, NOTWITHSTANDING THE DELIVERY OR DISCLOSURE TO THE PLAN INVESTOR OR ANY OF ITS RESPECTIVE AFFILIATES OR REPRESENTATIVES OF ANY DOCUMENTATION, FORECASTS, PROJECTIONS OR OTHER INFORMATION WITH RESPECT TO ANY ONE OR MORE OF THE FOREGOING. EXCEPT FOR THE REPRESENTATIONS AND WARRANTIES EXPRESSLY MADE BY THE COMPANY IN THIS Article IV, ALL OTHER REPRESENTATIONS AND WARRANTIES, WHETHER EXPRESS OR IMPLIED, ARE EXPRESSLY DISCLAIMED BY THE COMPANY.

ARTICLE V.

REPRESENTATIONS AND WARRANTIES OF THE PLAN INVESTOR

Except (i) as set forth in the disclosure schedule prepared by the Plan Investor (the “Plan Investor Disclosure Schedule”) and delivered to the Company simultaneously with the execution and delivery hereof and (ii) contemplated by Section 10.5 of this Agreement, the Plan Investor represents and warrants to the Company as follows:

Section 5.1.          Organization. Each Plan Investor Group Member is duly incorporated, formed or organized, validly existing and (in the jurisdictions recognizing the concept) in good standing under the Laws of the jurisdiction in which such Person is incorporated, formed or domiciled. Each Plan Investor Group Member is licensed or qualified to do business in each jurisdiction where the conduct or nature of its business or the ownership, leasing or holding of its properties makes such qualification necessary, except where the failure to be so licensed or qualified has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect. Each Plan Investor Group Member has the requisite power and authority to own, lease and operate its properties and to conduct its business as it is now being conducted, in each case, except where such failure has not had and

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would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect.

Section 5.2.           Qualification; Due Authorization; Power and Authority. Subject to obtaining the Plan Investor Stockholder Approval, the Plan Investor has all power and authority to execute and deliver this Agreement and the other Transaction Documents and to consummate the transactions contemplated hereby and thereby. The making, execution and delivery of this Agreement and the other Transaction Documents, and the performance of the obligations and covenants contained herein and therein have been duly and validly authorized by all necessary corporate actions of the Plan Investor. The Plan Investor Board, at a meeting duly called and held, duly adopted resolutions (i) approving this Agreement and the other Transaction Documents, (ii) determining that the terms of this Agreement and the other Transaction Documents are fair and in the best interests of the Plan Investor and its stockholders, and (iii) recommending that Plan Investor stockholders approve the Acquisition, the issuance of the Closing Shares and the other transactions contemplated by this Agreement (as set forth in the Plan Investor Stockholder Approval) (the “Plan Investor Board Recommendation”). The Plan Investor Board Recommendation has not been rescinded, modified or withdrawn as of the date of this Agreement. The consummation of the Acquisition, the issuance of the Closing Shares and the other transactions contemplated by this Agreement by the Plan Investor requires the affirmative vote of at least 50% (or 75% in the case of certain resolutions) of the votes cast by Plan Investor stockholders at a meeting duly called for purposes of obtaining such vote, assuming a quorum is present. This Agreement has been duly and validly executed and delivered by the Plan Investor and, assuming the due authorization, execution and delivery hereof by the Company, this Agreement will constitute the valid and binding obligations of the Plan Investor in accordance with its terms (except as such enforcement may be limited by insolvency, reorganization, moratorium, receivership, conservatorship and by general equity principles).

Section 5.3.            Consents and Approvals. Neither the execution and delivery of this Agreement by the Company nor the consummation of the transactions contemplated hereby will (a) require any consent, approval, authorization, registration or filing under any Law to which the Plan Investor Group is subject or by which any of the assets of the Plan Investor Group is bound (the “Plan Investor Governmental Requirements” and, together with the Company Governmental Requirements, the “Governmental Requirements”); (b) require the consent or approval of any other party to, or conflict with, result in any breach of, or constitute a default (or an event that, with notice or lapse of time or both, would become a default) under, any Contract to which any Plan Investor Group Member is a party; (c) give rise to a right of termination, cancellation or acceleration of any obligation or to loss of a material benefit under, or result in the creation of any Encumbrances (other than Permitted Encumbrances) upon any of the properties or assets of any Plan Investor Group Member; or (d) conflict with or result in a violation or breach of, or default under, any provision of the Charter Documents of any Plan Investor Group Member; in each case, other than (i) on or after the Petition Date, the authorization or approval of the Bankruptcy Court, (ii) authorizations, consents, orders or approvals of, or registrations or declarations with, any Governmental Entity or other Person set forth on Section 5.3 of the Plan Investor Disclosure Schedule, (iii)   authorizations, consents or approvals required under any applicable Antitrust Laws, and (iv) where the failure to obtain such consents, approvals, authorizations or registrations or to make such filings has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect. Any such authorization, consent, approval, order, registration or declaration that has been obtained, effected or given is in full force and effect as of the date hereof. No Plan Investor Group Member is in default under, and no event has occurred that with the lapse of time or action by a third party could result in a default under, the terms of any judgment, order, writ, decree, Permit or license of any Governmental Entity where such default would reasonably be expected to have a Plan Investor Material Adverse Effect.

Section 5.4.         Capitalization.

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(a)           With respect to each Plan Investor Group Member, Section 5.4(a) of the Plan Investor Disclosure Schedule sets forth a true, correct and complete list of the (i) name, (ii) type of entity, (iii) jurisdiction, (iv) the number and type of all authorized capital stock or other equity interests thereof, (v) the number and type of all issued and outstanding capital stock or other equity interests thereof, and (vi)  the ownership of such capital stock or other equity interests as of the date of this Agreement. Except as set forth on Section 5.4(a) of the Plan Investor Disclosure Schedule, there are no other corporations, limited liability companies, partnerships, joint ventures, associations or other entities or Persons in which any Plan Investor Group Member owns, as of the date of this Agreement, of record or beneficially, any direct or indirect equity or other interest or any right (contingent or otherwise) to acquire the same. Except as set forth on Section 5.4(a) of the Plan Investor Disclosure Schedule, all outstanding shares of capital stock or other equity interests of the Plan Investor Group Members have been duly authorized and validly issued as of the date of this Agreement. There are no outstanding warrants, options, rights, “phantom” stock rights, agreements, convertible or exchangeable securities or other commitments (other than this Agreement) (a) pursuant to which any Plan Investor Group Member is or may become obligated to issue, sell, purchase, return or redeem any shares of capital stock or other equity securities of such Person or (b) that give any Person the right to receive any benefits or rights similar to any rights enjoyed by or accruing to the holders of shares of capital stock or other equity securities of any Plan Investor Group Member (including any rights to receive any payment in respect thereof) as of the date of this Agreement. Each grant of a stock option or other equity award in respect of shares of capital stock or other equity interests of the Plan Investor Group Members was made in accordance with the terms of the applicable Plan Investor Employee Benefit Plan and all other applicable Law. There are no outstanding bonds, debentures, notes or other indebtedness having the right to vote on any matters on which the equityholders of any Plan Investor Group Member may vote as of the date of this Agreement.

(b)          The Closing Shares, when issued to the Company in accordance with the terms of this Agreement, will be duly and validly issued, fully paid and nonassessable and free and clear of all Encumbrances (other than restrictions on transfer imposed by federal and state securities laws and Permitted Encumbrances), will not have been issued in violation of preemptive or similar rights to subscribe for or purchase securities of the Plan Investor, and will be issued in compliance with all applicable Laws and its constitutional documents and the holder thereof will have good, valid and marketable title thereto upon the issuance of such Closing Shares. Except as contemplated by this Agreement, the issue and sale of the Closing Shares will not result in the right of any holder of Plan Investor securities to adjust the exercise, conversion or exchange price under such securities.

(c)          To the Knowledge of the Plan Investor, the Plan Investor is and since January 1, 2017 has been, in compliance in all material respects with all applicable listing and corporate governance rules and requirements applicable to companies traded on AIM and Euronext. The Plan Investor has no reason to believe that it will not, upon the issuance of the Closing Shares, continue to be, in compliance with the listing and maintenance requirements for continued listing or trading on AIM and Euronext in all material respects. Assuming the representations and warranties of the Company are true and correct in all material respects, to the Plan Investor’s Knowledge the consummation of the transactions contemplated by this Agreement will not contravene the rules and regulations applicable to companies traded on AIM or Euronext. There are no proceedings pending or threatened against the Plan Investor relating to the continued listing or trading of the Plan Investor ordinary shares on AIM or Euronext, and the Plan Investor has not received any notice of, nor to the Knowledge of the Plan Investor is there any basis for, the delisting of the Plan Investor ordinary shares from AIM or Euronext. For the avoidance of doubt, the Plan Investor shall not be in breach of this Section 5.4(c) should its ordinary shares be suspended from trading on AIM or Euronext as a result of the transactions contemplated by this Agreement being made public prior to an admission document being published pursuant to the AIM Rules for Companies (the “AIM Rules”) and the Euronext Rule Book (the “Euronext Rules”).

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Section 5.5.         Financial Statements.

(a)          The Plan Investor has previously provided the Company with the following financial statements (collectively, the “Plan Investor Financial Statements”): (i) the audited consolidated balance sheets of the Plan Investor Group as of December 31, 2018 and the related statements of income, cash flows and changes in owners’ equity for the fiscal year then ended, together with the notes to such Plan Investor Financial Statements and the opinion of the Plan Investor’s independent auditor thereon (the Financial Statements set forth in this clause (i), the “Plan Investor Audited Financial Statements”), and (ii) the unaudited consolidated balance sheet of the Company Group as of March 31, 2019 (the “Plan Investor Latest Balance Sheet Date”) and the related statements of income and cash flows for the three (3)-month period then ended (the “Plan Investor Unaudited Financial Statements”). The Plan Investor Financial Statements have been prepared in all material respects in accordance with IFRS applied on a consistent basis throughout the periods indicated therein (except as set forth in footnote disclosures thereto) and except for (x) footnote disclosures thereto, and (y) with respect to Plan Investor Unaudited Financial Statements, normal and recurring year-end adjustments thereto (none of which, individually or in the aggregate, are material to the Company Group Members taken as a whole), the Plan Investor Financial Statements fairly present, in all material respects, the financial position, and results of operations, stockholders’ equity and cash flows of the Plan Investor Group, on a consolidated basis, as of the dates and for the periods indicated therein. The Plan Investor Financial Statements were derived from the books and records of the Plan Investor Group Members and present fairly in all material respects the financial condition of the Plan Investor Group as of the respective dates they were prepared and the results of operations of the Plan Investor Group for the periods indicated therein. Each Plan Investor Group Member maintains a standard system of accounting established and administered in accordance with IFRS.

(b)         Each Plan Investor Group Member maintains a system of internal accounting controls sufficient to provide reasonable assurances that: (i) transactions are executed in accordance with management’s general or specific authorization, (ii) subject to the disclosure set forth on Section 5.5(b) of the Plan Investor Disclosure Schedule, transactions are recorded as necessary to permit preparation of financial statements in conformity with IFRS and to maintain asset accountability, (iii) access to assets is permitted only in accordance with management’s general or specific authorization, and (iv) the recorded accountability for assets is compared with the existing assets at reasonable intervals and appropriate action is taken with respect to any differences.

Section 5.6.          No Undisclosed Liabilities. No Plan Investor Group Member has any Liabilities of a nature that would be required to be disclosed on a balance sheet prepared in accordance with IFRS (as in effect on the date hereof) except for (i) any Liability identified in the Plan Investor Latest Balance Sheet; (ii) current Liabilities that have arisen after the Plan Investor Latest Balance Sheet Date in the ordinary course of business; (iii) Liabilities arising in the ordinary course of business under any Contract (but, in each case, not Liabilities for breaches thereof); or (iv) Liabilities incurred in connection with this Agreement or other Transaction Documents. No Plan Investor Group Member has any “off-balance sheet arrangements” (as such term is defined in Item 303(a)(4) of Regulation S-K promulgated under the Exchange Act).

Section 5.7.         Recent Events.

(a)           Since the Plan Investor Latest Balance Sheet Date until the date hereof, (i) each Plan Investor Group Member has conducted its business in all material respects in the ordinary course of business (except in connection with the transactions contemplated by this Agreement and the other Transaction Documents) and (ii) there has not been a Plan Investor Material Adverse Effect.

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(b)           Without limiting the generality of the foregoing Section 5.7(a), except as expressly contemplated by any Transaction Document or as set forth on Section 5.7(b) of the Plan Investor Disclosure Schedule, no Plan Investor Group Member has since the Plan Investor Latest Balance Sheet Date and through the date hereof:

(i) subjected a material portion of its properties or assets to any Encumbrances, except for Permitted Encumbrances;

(ii) sold, assigned or transferred a material portion of its assets, except in the ordinary course of business and except for sales of obsolete assets or assets with de minimis book value;

(iii) amended its Charter Documents;

(iv) made any material change in any method of accounting or accounting practice of the Company, except as required by the IFRS or as disclosed in the notes to the Plan Investor Audited Financial Statements;

(v) incurred, assumed or guaranteed any indebtedness for borrowed money, except unsecured current obligations and Liabilities incurred in the ordinary course of business;

(vi) cancelled any material debts or claims or waived any material rights against a Person that is not a Plan Investor Group Member;

(vii) taken any action to make, change or rescind any material Tax election, amend any material Tax Return or taken any position on any Tax Return, taken any action, omitted to take any action or entered into any other transaction that would have the effect of increasing the Tax liability of the Company in respect of any Tax period starting after the Closing Date, in each case other than in the ordinary course of business; or

(viii) entered into any Contract to do any of the foregoing.

Section 5.8.         Contracts and Commitments.

(a)          Section 5.8 of the Plan Investor Disclosure Schedule lists the following Contracts (including all amendments, modifications and supplements thereto) to which a Plan Investor Group Member is a party as of the date hereof (each a “Plan Investor Material Contract” and collectively, the “Plan Investor Material Contracts”), in each case, other than Contracts expressly contemplated by this Agreement or the other Transaction Documents:

(i) (A) any material Contract providing for the borrowing of money or to the issuance of any note, bond, debenture or other evidence of funded indebtedness, or to mortgaging, pledging or otherwise placing a material Encumbrance on any securities or assets of any Plan Investor Group Member; (B) any Contract in the nature of a letter of credit, bankers’ acceptance and similar facilities involving any Plan Investor Group Member as an account party or beneficiary; (C) any Contract in the nature of a capital or direct financing lease that is required by IFRS to be treated as a long-term liability involving annual payments above

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$250,000 individually; and (D) any Contract containing material earn-out obligations or other contingent payment or contingent obligations for the deferred purchase price of property or services;

(ii) any material Contract involving any guaranty by a third party of any obligation for borrowed money or other material guaranty, performance or completion bond or indemnity or surety arrangement;

(iii) any license, sublicense, development, collaboration or royalty agreement or other Contract relating to the use by any Plan Investor Group Member of any material third-party Intellectual Property (other than commercially available software or software subject to click-through or shrink-wrap agreements);

(iv) any license, sublicense, development, collaboration or royalty agreement or other Contract relating to the use of any Intellectual Property of any Plan Investor Group Member by any third party (other than licenses granted to customers, resellers and distributors in the ordinary course of business) pursuant to which any Plan Investor Group Member receives annual payments above $250,000 individually;

(v) any Contract binding any Plan Investor Group Member in respect of a covenant not to compete with any Person, Contracts (other than Distribution Agreements and Contracts entered into in the ordinary course of business) in which any Plan Investor Group Member grants any exclusivity or preferential right of first refusal or right of first offer to any Person or otherwise creates an exclusive relationship binding on any Plan Investor Group Member with a Person, in each case, to the extent such Contract materially restricts or limits the activities of any Plan Investor Group Member or the ability of any Plan Investor Group Member to engage or compete in any line of business or any geographic area or from developing or commercializing any pharmaceutical products;

(vi) any Contract for the acquisition or disposition of any business, any merger, consolidation, plan or scheme of arrangement or reorganization, or acquisition or disposition of a material amount of stock or material portion of assets of any Person outside the ordinary course of business, or any material real property (whether by merger, sale of stock, sale of assets or otherwise) to the extent any Plan Investor Group Member has any remaining payment or indemnity obligations thereunder in excess of $250,000 individually, in each case other than sales of inventory in the ordinary course of business;

(vii) any Contract that by its terms limits the payment of dividends or other distributions by the Plan Investor;

(viii) any Contract, other than a Distribution Agreement or any employment agreements, involving consideration in excess of $250,000 individually, and $500,000 in aggregate for Contracts with substantially the same customer, supplier or subject matter, and which, in each case, cannot be

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cancelled by the applicable Plan Investor Group Member (a) without penalty or (b) with less than ninety (90) days’ notice;

(ix) Contracts with independent contractors or consultants which are not cancellable without material penalty or without more than ninety (90) days’ notice;

(x) any material Contract between any directors of any Plan Investor Group Member, any Senior Officers or (in both cases) any of their Affiliates, on the one hand, and such Plan Investor Group Member or any other Plan Investor Group Member, on the other hand;

(xi) any material Contract, involving consideration in excess of $250,000 individually, and $500,000 in aggregate, that provides for any joint venture, partnership or similar arrangement or any Contract, involving consideration in excess of $250,000 individually, and $500,000 in aggregate, involving a sharing of revenues, profits, losses, costs or Liabilities between any Plan Investor Group Member, on the one hand, and any other Person, on the other hand excluding, in each case, (A) Distribution Agreements, (B) Contracts among Plan Investor Group Members which are directly or indirectly wholly owned by the Plan Investor and (C) any Contract that would be covered by this clause (x) solely by virtue of an obligation to pay customary royalties on account of product sales;

(xii) any “single source” supply Contract pursuant to which goods or materials that are material to the Plan Investor Business are supplied to any Plan Investor Group Member from an exclusive source which source cannot be replaced without a material increase in cost within ninety (90) days of termination of such Contract; or

(xiii) any material Contract with any Governmental Entity outside of the ordinary course of business.

(b)           The Company either has been supplied with, or has been given access to, a true, correct and complete copy of all written Plan Investor Material Contracts or a summary of all oral Plan Investor Material Contracts. Except as has not had and would not reasonably be expected to have a Plan Investor Material Adverse Effect and except as set forth in the Plan, each Plan Investor Material Contract (assuming due power and authority of, and due execution and delivery by, the other party or parties thereto) is in full force and effect and is valid, binding and enforceable against the applicable Plan Investor Group Member and, to the Plan Investor’s Knowledge, the other parties thereto, in accordance with their respective terms (except as may be limited by bankruptcy, insolvency, moratorium or other similar laws affecting creditors rights).

(c)          Except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect or except as set forth on Section 5.8 of the Plan Investor Disclosure Schedule, (i) within the one-year period preceding the date of this Agreement, no Plan Investor Group Member has violated or breached, or committed any default in any respect under, any Plan Investor Material Contract that remains uncured as of the date hereof, and (ii) to the Plan Investor’s Knowledge, as of the date of this Agreement, no other Person has violated or breached, or committed any default in any respect under, any Plan Investor Material Contract that remains uncured as

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of the date hereof; and (iii) as of the date of this Agreement, no event has occurred and is continuing through any Plan Investor Group Member’s actions or inactions, as applicable, that will result in a violation or breach in any respect of any of the provisions of any Plan Investor Material Contract.

Section 5.9.         Real Property.

(a)           No Plan Investor Group Member owns any real property or any interest in real property other than the leaseholds created under the real property leases or subleases for the properties identified on Section 5.9 of the Plan Investor Disclosure Schedule (including all amendments, modifications, terminations and extensions thereof, the “Plan Investor Real Property Leases”). Section  5.9 of the Plan Investor Disclosure Schedule contains a true, correct and complete list of all Plan Investor Real Property Leases with respect to all real property leased, licensed, subleased or otherwise used or occupied by any Plan Investor Group Member.

(b)         The Plan Investor Real Property Leases are in full force and effect in all material respects and are valid and binding against the applicable Plan Investor Group Member and, to the Plan Investor’s Knowledge, the other parties thereto in accordance with their respective terms (except as may be limited by bankruptcy, insolvency, moratorium or other similar laws affecting creditors’ rights). No Plan Investor Group Member has leased, subleased or granted to any Person a right to possess, lease or occupy any portion of the real property subject to any Plan Investor Real Property Lease.

(c)          The Plan Investor has delivered or made available to the Company complete and accurate copies of each of the Plan Investor Real Property Leases, and none of such Plan Investor Real Property Leases has been materially amended, modified, terminated or extended as of the date hereof in any respect, except to the extent that such amendments, modifications, terminations or extensions are disclosed by copies delivered or made available to the Company.

(d)          No Plan Investor Group Member is in default under any of the Plan Investor Real Property Leases that remains uncured as of the date hereof, in each case, except as has not had and would not reasonably be expected to have, individually or in the aggregate with other uncured defaults, a Plan Investor Material Adverse Effect.

Section 5.10.       Intellectual Property.

(a)          Section 5.10(a) of the Plan Investor Disclosure Schedule sets forth a complete and accurate list of all (i)(A) issued patents and pending patent applications, (B) trademark and service mark registrations and applications, (C) copyright registrations and applications, and (D) internet domain name registrations, in each case that are owned by the Plan Investor Group Members (collectively, the “Owned Plan Investor IP”) and (ii) material (A) issued patents and pending patent applications, (B) trademark and service mark registrations and applications, (C) copyright registrations and applications, and (D) internet domain name registrations, in each case that are licensed to the Plan Investor Group Members. Except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect, the Plan Investor Group Members own, all right, title and interest in the Owned Plan Investor IP, free and clear of all Encumbrances (other than Permitted Encumbrances). Each item of material Owned Plan Investor IP has been duly registered in, filed in or issued by, as applicable, an official governmental register and/or issuer (or officially recognized register or issuer) and each such registration, filing, issuance and/or application, (x) has not been abandoned or cancelled, (y) has been maintained effective by all requisite filings, renewals and payments, and (z) to the Knowledge of the Plan Investor, remains in full force and effect.

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(b)          The Plan Investor Group Members own and possess all right, title and interest in and to (or have the right pursuant to a valid and enforceable license or otherwise possess legally enforceable rights to use) all Intellectual Property that is necessary for or used or held for use in the conduct of the Plan Investor Business (the “Plan Investor IP”). Neither the execution and delivery of this Agreement by the Plan Investor, nor the performance of this Agreement by the Plan Investor, will result in the loss, forfeiture, termination, or impairment of, or give rise to a right of any Person to limit, terminate, or consent to the continued use of, any rights of any Plan Investor Group Member in any Plan Investor IP.

(c)           No Plan Investor Group Member is infringing, misappropriating, diluting, or otherwise violating the Intellectual Property rights of any Person. No Plan Investor Group Member has received any written charge, complaint, claim, demand, or notice since January 1, 2017 (or earlier, if presently not resolved) alleging any such infringement, misappropriation, dilution, or violation (including any claim that any Plan Investor Group Member must license or refrain from using any Intellectual Property rights of any Person) which alleged infringement, misappropriation, dilution, or violation, if true, would reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect. To the Knowledge of the Plan Investor, no Person is infringing, misappropriating, diluting or otherwise violating any Owned Plan Investor IP. No Plan Investor Group Member has made or asserted any charge, complaint, claim, demand or notice since January 1, 2017 (or earlier, if presently not resolved) alleging any such infringement, misappropriation, dilution, or violation which alleged infringement, misappropriation, dilution, or violation, if true, would reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect.

(d)          Each applicable Plan Investor Group Member has taken reasonable best effort steps to maintain, police and protect the Intellectual Property that is material to the Plan Investor Business (“Plan Investor Group Material IP”). All Plan Investor Group Material IP that derives actual or potential economic value from not being generally known to the public or to other persons who can obtain economic value from its disclosure or use has been maintained in confidence in accordance with protection procedures that are in accordance with procedures customarily used in the industry to protect rights of like importance and, to the Knowledge of the Plan Investor, adequate for protection against unauthorized disclosure or use. To the Knowledge of the Plan Investor, there has been no unauthorized disclosure of any Plan Investor Group Material IP. Except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect, to the Knowledge of the Plan Investor, all former and current officers, directors, employees, personnel, consultants, advisors, agents, and independent contractors of any Plan Investor Group Member, and each of its predecessors, who have contributed to or participated in the conception and development of Intellectual Property for such entities have entered into valid and binding proprietary rights agreements with the applicable Plan Investor Group Member or one of its predecessors, vesting ownership of such Intellectual Property in the applicable Plan Investor Group Member. No such Person has asserted, and to the Knowledge of the Plan Investor, no such Person has, any right, title, interest or other claim in, or the right to receive any royalties or other consideration with respect to, any Plan Investor Group Material IP.

(e)          The IT Assets of the Plan Investor Group Members operate in all material respects in accordance with their documentation and functional specifications and as required to operate the Plan Investor Business and have not, since January 1, 2017, materially malfunctioned or failed. Each Plan Investor Group Member has implemented reasonable best effort measures protect the confidentiality and security of such IT Assets and information stored or contained therein against any unauthorized use, access, interruption or corruption, and to the Knowledge of the Plan Investor, there has been no such unauthorized use, access, interruption or corruption that has not been remedied in all material respects. Each Plan Investor Group Member implemented reasonable best effort procedures regarding data backup, data storage, system redundancy and disaster avoidance procedures with respect to their IT Assets.

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Section 5.11.      Privacy and Data Security.

(a)           Each of the Plan Investor Group Members comply with, and since January 1, 2017 have complied with, in all material respects, all Data Protection Requirements.

(b)          There are no restrictions on any of the Plan Investor Group Members’ collection, use, disclosure and retention of Personal Data, except as provided by the Data Protection Requirements. There are no ongoing material Proceedings, and to the Plan Investor’s Knowledge, there are no pending or, to the Knowledge of the Plan Investor, threatened Proceedings, with respect to any Plan Investor Group Member’s violation of any Data Protection Requirement. No decision, judgment or order, whether statutory or otherwise, is pending or has been made, and no notice, complaint, claim, enforcement action, or litigation of any kind has been served on or initiated against any of the Plan Investor Group Members pursuant to any Data Protection Requirement.

(c)           Each of the Plan Investor Group Members have taken reasonable best effort steps, compliant with applicable Data Protection Requirements, to protect (i) the operation, confidentiality, integrity, and security of the Plan Investor’s software, systems, and websites that are involved in the collection and/or processing of Personal Data, and (ii) Personal Data in the Plan Investor’s possession and/or control from unauthorized use, access, disclosure, and modification.

(d)          None of the Plan Investor Group Members have experienced any failures, crashes, security breaches, unauthorized access, use, or disclosure, or other adverse events or incidents related to Personal Data that would require notification of individuals, law enforcement, or any Governmental Entity, any remedial action under any applicable Data Protection Requirement, or that have caused any substantial disruption of or interruption in the use of the Plan Investor’s software, equipment or systems.

Section 5.12.        Legal Compliance; Permits. Except as set forth on Section 5.12 of the Plan Investor Disclosure Schedule:

(a)        since January 1, 2017 and as of the date hereof, each Plan Investor Group Member has been in compliance with all Laws applicable to such Plan Investor Group Member other than any such noncompliance that has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect. All Permits required to conduct the Plan Investor Business are in the possession of the applicable Plan Investor Group Member, are in full force and effect and are being complied with, in each case, except when such failure would not reasonably be excepted to have, individually or in the aggregate, a Plan Investor Material Adverse Effect. Except as would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect, the current conduct of the Plan Investor Business is not, and has not been since January 1, 2017, in default or violation under any Permit (except for such violation that has been remedies and imposes no continuing Liability) and, to the Knowledge of the Plan Investor, no event has occurred which, with notice or the lapse of time or both, would constitute a default or violation of any term, condition or provision of any applicable Permit. There are no actions pending, or to the Knowledge of the Plan Investor, threatened in writing, that seek revocation, cancellation or modification of any applicable Permit, except where such revocation, cancellation or modification has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect.

(b)           there is no material proceeding or disciplinary action (including fines) by any Governmental Entity currently pending or, to the Plan Investor’s Knowledge, threatened in writing against any Plan Investor Group Member, any of their respective assets, rights or properties or any of

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their respective officer or directors, in each case, except for those that have not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect.

Section 5.13.      Environmental Compliance and Conditions.

(a)           The Plan Investor Group Members have obtained and possess all material Environmental Permits required under the Environmental Laws, in each case, except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect.

(b)          The Plan Investor Group Members are, and since January 1, 2017 have been, in compliance in all material respects with all terms and conditions of such Environmental Permits and are, and since January 1, 2017 have been, in compliance in all material respects with all other Environmental Laws or any written notice or demand letter issued, entered, promulgated or approved thereunder.

(c)           There are no Environmental Claims pending, nor to the Knowledge of the Plan Investor, threatened against any Plan Investor Group Member, and to the Knowledge of the Plan Investor, no Plan Investor Group Member has received any notification of any allegation of actual or potential responsibility for any Release or threatened Release of any Hazardous Materials with respect to any location currently or formerly owned, leased, operated or used by such Plan Investor Group Member. There have been no Releases of Hazardous Materials at any properties that are operated, leased or used by any Plan Investor Group Member, or to the Knowledge of the Plan Investor, at properties that were formerly owned, operated, leased or used by any Plan Investor Group Member, that are reasonably likely to cause any Plan Investor Group Member to incur any material Liability pursuant to applicable Environmental Law. No Plan Investor Group Member (i) has entered into or agreed to any consent decree or consent order or is otherwise subject to any judgment, decree, or judicial or administrative order relating to compliance with Environmental Laws or Environmental Permits, the investigation, sampling, monitoring, treatment, remediation, response, removal or cleanup of Hazardous Materials, and no Proceeding is pending, or to the Knowledge of the Plan Investor is threatened, with respect thereto, and (ii) is an indemnitor by contract or otherwise in connection with any claim, demand, suit or action threatened or asserted by any third-party for any Liability under any Environmental Law or otherwise relating to any Hazardous Materials.

(d)          The representations and warranties in this Section 5.13 constitute the sole and exclusive representations and warranties of the Plan Investor with respect to any environmental, health or safety matters, including any arising under Environmental Law, and no other representation or warranty contained in any other Section of this Agreement shall apply to any such matters and no other representation or warranty, express or implied, is being made with respect thereto.

Section 5.14.       Litigation. Except as set forth on Section 5.14 of the Plan Investor Disclosure Schedule, since January 1, 2017 and as of the date hereof, there has not been (a) any material pending action, suit, proceeding, claim, administrative or court action or other litigation, or to the Plan Investor’s Knowledge, any material investigation by any Governmental Entity, pending, or, (b) to the Plan Investor’s Knowledge, any material threatened action, suit, proceeding, claim, administrative or court action or other litigation threatened in writing, in each case (X) against any Plan Investor Group Member or (Y) that involves any Plan Investor Group Member, that is reasonably expected to have a Plan Investor Material Adverse Effect. Except as set forth on Section 5.14 of the Plan Investor Disclosure Schedule, no Plan Investor Group Member or any of such Person’s assets or its Liabilities are subject to any judicial or administrative or other order issued by, or agreement entered into with, a Governmental Entity except as would not be material to the Plan Investor Group Members, taken as a whole.

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Section 5.15.      Tax Matters.

(a)           Since January 1, 2017, the Plan Investor Group has filed (or have had filed) all federal and other material Tax Returns that it was required to file (or to have filed), taking into account any extensions of time to file. All such Tax Returns were correct and complete in all material respects. All material Taxes of the Plan Investor Group (whether or not shown as owing by such Person on such Person’s Tax Returns) have been fully paid or properly accrued and reserved for in accordance with GAAP. No material claim has ever been made by an authority in a jurisdiction where the Plan Investor Group does not file Tax Returns that the Plan Investor Group is or may be subject to taxation by that jurisdiction. There are no material liens for Taxes (other than Taxes not yet due and payable) upon any of the assets of any Plan Investor Group Member.

(b)           No Plan Investor Group Member is, as of the date hereof, the subject of a Tax audit or examination with respect to material Taxes. There are no outstanding agreements or waivers extending the statutory period of limitations applicable to any federal or other material Tax Return of the Plan Investor Group. No Plan Investor Group Member has granted a power of attorney that is in effect with respect to any Tax matters.

(c)         The Plan Investor Group does not have any current material Liability for Taxes of any Person other than itself, including (i) under Treasury Regulations Section 1.1502-6 or (ii) as a transferee or successor, by Contract or otherwise.

(d)          No Plan Investor Group Member has been a United States real property holding corporation within the meaning of Section 897(c)(2) of the Code during the applicable period specified in Section 897(c)(1)(A)(ii) of the Code.

(e)          To the Plan Investor’s Knowledge, the Company has not been a party to a “listed transaction,” as such term is defined in Section 6707A(c)(1) of the Code and Treasury Regulations Section 1.6011-4(b)(2).

(f)           All material Taxes that any Plan Investor Group Member was obligated to withhold from amounts owing to any person, including any employee, independent contractor, stockholder, creditor or third party, in each case, prior to the date hereof, have been fully and timely paid, withheld and remitted or properly accrued.

(g)           No Plan Investor Group Member has distributed stock of another Person, or has had its stock distributed by another Person, in a transaction that was purported or intended to be governed in whole or in part by Section 355 or Section 361 of the Code.

(h)           There are no Tax rulings, requests for rulings, closing agreements, or any other Contracts with any Tax authorities that relate to any Plan Investor Group Member that could have a material effect on the liability of the Company for Taxes for any Tax period ending after the Closing Date.

(i)            The representations and warranties in this Section 5.15 constitutes the sole and exclusive representations and warranties of the Plan Investor with respect to Taxes related to the Plan Investor Group, and no other representation or warranty contained in any other section of this Agreement shall apply to any such Tax matters and no other representation or warranty, express or implied, is being made with respect thereto.

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Section 5.16.         Insurance. Section 5.16 of the Plan Investor Disclosure Schedule lists each insurance policy maintained by each Plan Investor Group Member as of the date hereof, and the deductibles and coverage limits for each such policy. The Plan Investor has made available to the Company a copy of such policies. All such insurance policies are in full force and effect, and applicable Plan Investor Group Member is not in default with respect to any material obligations under any such insurance policy. All premiums in respect of each insurance policy maintained by any of Plan Investor Group Member have been paid when due; to the Plan Investor’s Knowledge as of the date of this Agreement no material default on the part of the counterparty to such policy exists. The applicable Plan Investor Group Member has not received written notice of cancellation of any insurance policies listed on Section 5.16 of the Plan Investor Disclosure Schedule. There is no claim pending under any such insurance policies as to which, to the Plan Investor’s Knowledge, coverage has been questioned, denied or disputed by the underwriters of such policies.

Section 5.17.          Illegal or Improper Payments.

(a)           Each Plan Investor Group Member (i) is in compliance, and since January 1, 2017 has been in compliance, in all material respects with the FCPA and any other applicable Anti-corruption Laws; (ii) since January 1, 2017 has not been investigated by any Governmental Entity with respect to, and to the Knowledge of the Plan Investor, has not been given notice in writing by a Governmental Entity or any other Person of, any actual or alleged violation by any Plan Investor Group Member of the FCPA or any other Anti-corruption Laws; and (iii) during the past five (5) years has had an operational and effective FCPA and anticorruption compliance program that includes, at a minimum, policies, procedures and training intended to enhance awareness of and compliance by each Plan Investor Group Member with the FCPA and any other applicable Anti-corruption Laws.

(b)           To the Knowledge of the Plan Investor, no Plan Investor Group Member has, directly or indirectly through its Representatives or any Person authorized to act on its behalf (including any distributor, agent, sales intermediary or other third party), offered, promised, paid, authorized or given money or anything of value to any Person for the purpose of: (i) influencing any act or decision of any Government Official or Other Covered Party; (ii) inducing any Government Official or Other Covered Party to do or omit to do an act in violation of a lawful duty; (iii) securing any improper advantage; or (iv) inducing any Government Official or Other Covered Party to influence the act or decision of a government or government instrumentality, in order to obtain or retain business, or direct business to, any Person or entity, in any way.

(c)           To the Knowledge of the Plan Investor, since January 1, 2017, each Plan Investor Group Member has maintained complete and accurate books and records, including records of payments to any agents, consultants, representatives, third parties and Government Officials, in accordance with IFRS, in all material respects. There have been no false or fictitious entries made in the books and records of any Plan Investor Group Member relating to any unlawful offer, payment, promise to pay, or authorization of the payment of any money, or unlawful offer, gift, promise to give, or authorization of the giving of anything of value, including any bribe, kickback or other illegal or improper payment, and no Plan Investor Group Member has established or maintained a secret or unrecorded fund.

(d)           To the Knowledge of the Plan Investor, since January 1, 2017, no Plan Investor Group Member has had a customer or supplier or other business relationship with, is a party to any Contract with, or has engaged in any transaction with, any Person (i) that is organized or domiciled in or that is a citizen of Crimea, Cuba, Iran, North Korea or Syria (including any Governmental Entity within such country) or (ii) that is the subject of any international economic or trade sanction administered or enforced by the Office of Foreign Assets Control of the United States Department of the Treasury, the

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United Nations Security Council, the European Union, Her Majesty’s Treasury, the United Kingdom Export Control Organization or other relevant sanctions authority.

Section 5.18.         Related Party Transactions. No officer, member of the board of directors or managers (or equivalent governing body) of any Plan Investor Group Member or, to the Plan Investor’s Knowledge, any individual in such officer’s, director’s or manager’s immediate family or an Affiliate of any such Person, is a party to any material Contract or transaction with any Plan Investor Group Member or has any material interest in any material property that is currently used by any Plan Investor Group Member, other than under a Plan Investor Employee Benefit Plan or pursuant to an employment agreement or as contemplated by this Agreement or the Transaction Documents.

Section 5.19.         Brokers’ Fees. Except as set forth on Section 5.19 of the Plan Investor Disclosure Schedule, neither the Plan Investor nor any of its officers or directors on behalf of the Plan Investor has employed any financial advisor, broker or finder or incurred any liability for any financial advisory fee, broker’s fees, commissions or finder’s fees in connection with any of the transactions contemplated by the Transaction Documents.

Section 5.20.         Employees. No Plan Investor Group Member is bound by or a party to any collective bargaining agreement, agreement with any works council, or labor contract. There are no actual, or to the Knowledge of the Plan Investor, threatened or pending organizing activities of any trade union, council of trade unions, employee bargaining agency or affiliated bargaining agent or any actual, threatened or pending unfair labor practice complaints, strikes, work stoppages, picketing, lock-outs, hand-billings, boycotts, slowdowns, arbitrations, grievances, complaints, charges or similar labor-related disputes or proceedings pertaining to any of the Plan Investor Group Members, and there have not been any such activities or disputes or proceedings since January 1, 2017, in each case, except as has not had or would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect.

(b)           Each Plan Investor Group Member is, and since January 1, 2016 has been, in compliance with all Laws respecting employment and employment practices, including, without limitation, all Laws respecting terms and conditions of employment, health and safety, wage payment, wages and hours, child labor, collective bargaining, immigration and work authorizations, employment discrimination, retaliation, civil rights, veterans’ rights, disability rights or benefits, equal opportunity, plant closures and layoffs, affirmative action, workers’ compensation, labor relations, social welfare obligations, proper classification of employees as exempt and non-exempt for purposes of wage and hour laws and as employees and independent contractors, unemployment insurance and the collection and payment of withholding and/or social security Taxes and any similar Tax, except for noncompliance that has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect.

(c)           Section 5.20(c) of the Plan Investor Disclosure Schedule contains a list of every Plan Investor Employee Benefit Plan. The Plan Investor has delivered to the Company true, complete and up-to-date copies of all Plan Investor Employee Benefit Plans and all amendments thereto together with, if applicable, all summary descriptions thereof, past or present participants therein, the statement of investment policies for each such Plan Investor Employee Benefit Plans, all funding agreements and service provider Contracts or other Contracts (including insurance Contracts, investment management agreements, subscription and participation agreements and recordkeeping agreements) relating thereto, the two most recent actuarial reports, the financial statements and evidence of any registration or qualification in respect thereof, in each case, to the extent any Plan Investor Group Members, may have any Liability under such Plan Investor Employee Benefit Plans.

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(d)           All of the Plan Investor Employee Benefit Plans are duly registered or qualified where required by applicable Law (including registration or qualification with the relevant Tax authorities where such registration or qualification is required to qualify for Tax exemption or other beneficial Tax treatment) and have always been administered in compliance with their terms and all applicable Laws. Each Plan Investor Employee Benefit Plan intended to be tax-qualified within the meaning of Section 401(a) of the Code is subject to a favorable determination or opinion letter from the Internal Revenue Service and, to the Knowledge of the Plan Investor, nothing has occurred that could reasonably be expected to adversely impact such tax-qualified status. The Plan Investor Group Members have no direct or contingent obligation with respect to any plan subject to Title IV of ERISA or any obligation to provide post-employment welfare benefits except to the extent required by Section 4980B of the Code or similar law.

(e)           Neither the execution, delivery or performance of this Agreement nor the consummation of any of the transactions contemplated herein will result in any bonus, golden parachute, severance or other payment, obligation or liability to any current or former employee or director of any of Plan Investor Group Member (whether or not under any Plan Investor Employee Benefit Plan), increase the benefits payable or provided under any Plan Investor Employee Benefit Plan, result in any acceleration of the time of payment or vesting of any such benefit, increase or accelerate employer contributions thereunder, or result in any payment that could be nondeductible pursuant to Section 280G of the Code.

(f)            All of the Plan Investor Employee Benefit Plans are fully funded in accordance with their terms and all applicable Laws and generally accepted actuarial principles and practices.

Section 5.21.          Healthcare Compliance Matters.

(a)           Except as set forth on Section 5.21 of the Plan Investor Disclosure Schedule (i) each Plan Investor Group Member is in compliance and since January 1, 2017 has been in compliance with all Health Care Laws applicable to such Plan Investor Group Member or any assets owned or used by it and (ii) no Plan Investor Group Member has received any written communication or has been subject to any Proceeding (other than routine FDA inspections) since January 1, 2017 from a Governmental Entity that alleges that such Plan Investor Group Member is not in compliance with any Health Care Law, except in the case of the immediately foregoing clauses (i) and (ii) where any non-compliance has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect. Except as set forth on Section 5.21 of the Plan Investor Disclosure Schedule, (i) no Plan Investor Group Member is party to and has any ongoing obligations pursuant to or under any corporate integrity agreements, deferred prosecution agreements, monitoring agreements, consent decrees, settlement orders, plans of correction or similar agreements with or imposed by any Governmental Entity, and (ii) no Plan Investor Group Member or any of its employees, officers or directors has been excluded, suspended or debarred from participation in any U.S. state or federal health care program or, to the Knowledge of the Plan Investor, has been convicted of any crime or is subject to any Proceeding by any Governmental Entity or other similar action, or has engaged in any conduct, that could reasonably be expected to result in debarment, suspension, or exclusion.

(b)           Each Plan Investor Group Member has, maintains and is operating in material compliance with all Health Care Permits, and all such Health Care Permits are valid, subsisting, and in full force and effect, except where the failure to have, maintain or operate in compliance with the Health Care Permits has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect. Each Plan Investor Group Member has fulfilled and performed all of its obligations with respect to the Health Care Permits, and no event has occurred which allows, or with notice or lapse of time or both, would allow revocation or termination thereof or results in any other

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material impairment of the rights of the holder of any Health Care Permit, except where the failure to so fulfill or perform, or the occurrence of such event, has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect. There is no Proceeding pending or, to the Knowledge of the Plan Investor, threatened in writing that could result in the suspension, termination, revocation, cancellation, limitation or impairment of any such Health Care Permit other than those that have not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect.

(c)           Except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect, all applications, notifications, submissions, information, claims, reports and statistics, and other data and conclusions derived therefrom, utilized as the basis for or submitted in connection with any and all requests for a Health Care Permit relating to any Plan Investor Group Member, its business and product candidates, when submitted to the FDA, DEA or other Governmental Entity were true, complete and correct as of the date of submission and any necessary or required updates, changes, corrections or modification to such applications, submissions, information and data have been submitted to the FDA, DEA or other Governmental Entity.

(d)           Except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect, since January 1, 2017, no Plan Investor Group Member had any product, product candidate or manufacturing site subject to a Governmental Entity (including FDA or DEA) shutdown or import or export prohibition, and has not received any FDA Form 483 or other Governmental Entity notice of inspectional observations, “warning letters,” “untitled letters” or written requests or requirements to make changes to a product candidate, or similar correspondence or written notice from the FDA, DEA or other Governmental Entity alleging or asserting noncompliance with any applicable Health Care Law, Health Care Permit or such requests or requirements of a Governmental Entity.

(e)           Except as has not had and would not reasonably be expected to have, individually or in the aggregate, a Plan Investor Material Adverse Effect, (i) the clinical, pre-clinical and other studies and tests conducted by or on behalf of or sponsored by any Plan Investor Group Member or in which any Plan Investor Group Member, or any of its product candidates have participated were, and if still pending are, being conducted in accordance with standard medical and scientific research procedures and all applicable Laws, including, but not limited to, the Federal Food, Drug and Cosmetic Act and its applicable implementing regulations and (ii) no investigational new drug application filed by or on behalf of any Plan Investor Group Member with the FDA has been terminated or suspended by the FDA, and neither the FDA nor any applicable foreign Governmental Entity has commenced, or, to the Knowledge of the Plan Investor, threatened to commence, any action to place a clinical hold order on, or otherwise terminate, delay or suspend, any proposed or ongoing clinical investigation conducted or proposed to be conducted by or on behalf of any Plan Investor Group Member.

(f)           No Plan Investor Group Member is the subject of any pending or, to the Knowledge of the Plan Investor, threatened investigation in respect of such Plan Investor Group Member or its product candidates, by the FDA pursuant to its “Fraud, Untrue Statements of Material Facts, Bribery, and Illegal Gratuities” Final Policy set forth in 56 Fed. Reg. 46191 (September 10, 1991) and any amendments thereto. The Plan Investor has provided the Company with accurate and complete copies of all Health Care Permits and correspondence with any Governmental Entity related to all product candidates of any Plan Investor Group Member.

Section 5.22.        No Other Representations or Warranties. NOTWITHSTANDING ANY PROVISION OF THIS AGREEMENT TO THE CONTRARY, EXCEPT FOR THE REPRESENTATIONS AND WARRANTIES EXPRESSLY MADE BY THE PLAN INVESTOR IN

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THIS Article V, NO PLAN INVESTOR GROUP MEMBER, ANY AFFILIATE THEREOF, OR ANY OTHER PERSON MAKES ANY REPRESENTATION OR WARRANTY WITH RESPECT TO ANY PLAN INVESTOR GROUP MEMBER, ANY AFFILIATE THEREOF OR ANY OTHER PERSON OR ITS RESPECTIVE BUSINESSES, OPERATIONS, ASSETS, LIABILITIES, CONDITION (FINANCIAL OR OTHERWISE) OR PROSPECTS, NOTWITHSTANDING THE DELIVERY OR DISCLOSURE TO THE COMPANY OR ANY OF ITS RESPECTIVE AFFILIATES OR REPRESENTATIVES OF ANY DOCUMENTATION, FORECASTS, PROJECTIONS OR OTHER INFORMATION WITH RESPECT TO ANY ONE OR MORE OF THE FOREGOING. EXCEPT FOR THE REPRESENTATIONS AND WARRANTIES EXPRESSLY MADE BY THE PLAN INVESTOR IN THIS Article V, ALL OTHER REPRESENTATIONS AND WARRANTIES, WHETHER EXPRESS OR IMPLIED, ARE EXPRESSLY DISCLAIMED BY THE PLAN INVESTOR.

ARTICLE VI.

COVENANTS

Section 6.1.            Conduct of Business of the Company Pending the Closing.

(a)           Except as (i) otherwise contemplated by any Transaction Document including the Plan, (ii) set forth on Section 6.1 of the Company Disclosure Schedule, (iii) consented to in writing by the Plan Investor or (iv) required by Law or the Bankruptcy Court, during the period from the date of this Agreement until the Closing, the Company shall, and shall cause each of the Company Group Members to, use its reasonable best efforts to conduct its operations and business in the ordinary course of business and in accordance with applicable Law, and to keep available the services of their respective current officers, employees and consultants and to preserve the goodwill and current relationships with Persons with which they have business relations.

(b)           Without limiting the generality of Section 6.1, except as (i) otherwise contemplated by any Transaction Document including the Plan, (ii) set forth on Section 6.1 of the Company Disclosure Schedule, (iii) consented to in writing by the Plan Investor, (iv) required by Law or the Bankruptcy Court or (v) necessary to implement the Scheme in accordance with the Scheme Document, the Company shall not, and shall not permit any of the Company Group Members to:

(i) amend any of their respective Charter Documents except as consistent with the Transaction Documents, including the Plan;

(ii) merge or consolidate with or into any other Person;

(iii) excluding debtor in possession financing, sell, assign, lease, sublease, license, sublicense, pledge or otherwise transfer, dispose of or grant any option, warrant or rights in, to or under or subject or allow to be subjected to any Encumbrance (other than a Permitted Encumbrance), any portion of the Company Group Members’ debt or equity securities, properties or assets (including tangible and intangible assets) other than, in the case of such properties or assets, in the ordinary course of business;

(iv) issue any equity shares or instrument convertible into, or exchangeable or exercisable for, any equity securities or other ownership interest of the Company or any other Company Group Member;

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(v) make, declare, set aside, establish a record date for or effect a distribution (whether payable in cash, shares, property or a combination thereof) to holders of its capital stock;

(vi) reclassify, combine, split, subdivide or amend the terms of, or redeem, purchase or otherwise acquire, directly or indirectly, any of its equity securities;

(vii) form any new Subsidiary of the Company, or merge or consolidate any Company Group Member with any Person or adopt a plan of complete or partial liquidation or resolutions providing for a complete or partial liquidation, dissolution, restructuring, recapitalization or other reorganization of any Company Group Member;

(viii) enter into any Contract with respect to the voting and registration of its equity securities;

(ix) acquire any Person, business or assets of any Person (other than in the ordinary course of business);

(x) make or authorize any loans, advances or capital contributions to, or investments in, any other Person;

(xi) forgive any loans to the directors, officers or employees of any Company Group Member;

(xii) change any material Tax election or material accounting or Tax accounting method, file any amendment to a material Tax Return, enter into any closing agreement, waive or extend any statute of limitations with respect to material Taxes, settle or compromise any Tax claim or assessment or consent to any Tax claim or assessment, surrender any right to claim a refund of material Taxes;

(xiii) (A) materially reduce the amount of any material insurance coverage provided by existing insurance policies, or (B) fail to maintain in full force and effect insurance coverage materially consistent with past practices;

(xiv) change in any material respect its practices related to the collection of accounts receivable or the payment of accounts payables outside the ordinary course of business or otherwise in a manner not permitted by the terms thereof;

(xv) make any changes in its methods of accounting or accounting practices (including with respect to reserves), or write down, write up or write off the book value of any assets (except for depreciations or amortization in ordinary course), in each case, other than as required by GAAP;

(xvi) sell, assign, transfer, license, permit to lapse, abandon, or otherwise dispose of any Owned Company IP, other than in the ordinary course of business;

(xvii) amend or terminate any Company Group Material Contract, involving consideration in excess of $250,000 individually, and $500,000 in aggregate, or enter into any Contract, involving consideration in excess of $250,000 individually, and $500,000 in aggregate that, if entered into prior to the date

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hereof, would be a Company Group Material Contract, other than in the ordinary course of business; or

(xviii) take, or agree (in writing or otherwise) to take, any of the actions prohibited by this Section 6.1(b).

Section 6.2.            Conduct of Business of the Plan Investor Pending the Closing.

(a)           Except as (i) otherwise contemplated by any Transaction Document, (ii) set forth on Section 6.2 of the Plan Investor Disclosure Schedule, (iii) consented to in writing by the Company or (iv) required by the UK Panel on Takeovers and Mergers (the “Panel”), during the period from the date of this Agreement until the Closing, the Plan Investor shall, and shall cause each of the Plan Investor Group Members to, use its reasonable best efforts to conduct its operations and business in the ordinary course of business and in accordance with applicable Law, and to keep available the services of their respective current officers, employees and consultants and to preserve the goodwill and current relationships with Persons with which they have business relations.

(b)           Without limiting the generality of Section 6.2(a), except as (i) otherwise contemplated by any Transaction Document, (ii) set forth on Section 6.2 of the Plan Investor Disclosure Schedule, (iii) consented to in writing by the Company, (iv) required by Law or (v) required by the Panel, the Plan Investor shall not, and shall not permit any of the Plan Investor Group Members to:

(i) amend any of their respective Charter Documents;

(ii) merge or consolidate with or into any other Person;

(iii) sell, issue or distribute or allow to be subjected to any Encumbrance (other than Permitted Encumbrances), any equity securities or instrument convertible into, or exchangeable or exercisable for, any equity securities or other ownership interest of the Plan Investor or any other Plan Investor Group Member; other than in connection with (A) the Plan Investor Rights Offering Transactions, (B) the Plan Investor Additional Equity Issuance, (C) the CVR Securities and the CVR Distributions, or (D) the issuance of equity securities in connection with the conversion or exercise of any security convertible into or exercisable for equity securities which are outstanding as of the date of this Agreement and disclosed in accordance with Section 5.4;

(iv) sell, assign, lease, sublease, license, sublicense, pledge or otherwise transfer, dispose of or grant any option or rights in, to or under or subject or allow to be subjected to any Encumbrance (other than Permitted Encumbrances), any portion of the Plan Investor Group Members’ properties or assets (including tangible and intangible assets) other than the disposition of inventory in the ordinary course of business;

(v) other than in connection with the CVR Securities and the CVR Distributions, make, declare, set aside, establish a record date for or effect a distribution (whether payable in cash, shares, property or a combination thereof) to holders of its capital stock;

(vi) form any new Subsidiary (other than a direct or indirect wholly owned Subsidiary) of the Plan Investor, or merge or consolidate any Plan Investor

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Group Member with any Person or adopt a plan of complete or partial liquidation or resolutions providing for a complete or partial liquidation, dissolution, restructuring, recapitalization or other reorganization of any Plan Investor Group Member;

(vii) enter into any Contract with respect to the voting and registration of its equity securities other than any Contract entered into in connection with the Plan Investor Additional Equity Issuance, subject to the terms and obligations under the Registration Rights Agreement;

(viii) acquire any Person, business or assets of any Person other than the acquisition of inventory in the ordinary course of business;

(ix) make or authorize any loans, advances or capital contributions to, or investments in, any other Person (other than a direct or indirect wholly owned Subsidiary of the Plan Investor);

(x) forgive any loans to the directors, officers or employees of any Company Group Member;

(xi) adopt or change any material Tax election or material accounting or Tax accounting method, file any amendment to a material Tax Return, enter into any closing agreement, waive or extend any statute of limitations with respect to material Taxes, settle or compromise any Tax claim or assessment or consent to any Tax claim or assessment, surrender any right to claim a refund of material Taxes;

(xii) (A) materially reduce the amount of any material insurance coverage provided by existing insurance policies, or (B) fail to maintain in full force and effect insurance coverage materially consistent with past practices;

(xiii) make any changes in its methods of accounting or accounting practices (including with respect to reserves), or write down, write up or write off the book value of any assets (except for depreciations or amortization in ordinary course), in each case, other than as required by IFRS;

(xiv) sell, assign, transfer, license, permit to lapse, abandon, or otherwise dispose of any Owned Plan Investor IP, other than in the ordinary course of business;

(xv) amend in any material respect or terminate any Plan Investor Material Contract (other than a Distribution Agreement or services agreement), involving consideration in excess of $250,000 individually, and $500,000 in aggregate, or enter into any Contract (other than a Distribution Agreement, DTIF Agreement or services agreement), involving consideration in excess of $250,000 individually, and $500,000 in aggregate, that, if entered into prior to the date hereof, would be a Plan Investor Material Contract, other than in the ordinary course of business;

(xvi) change in any material respect its practices related to the collection of accounts receivable or the payment of accounts payables outside the ordinary course of business or otherwise in a manner not permitted by the terms thereof ; or

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(xvii) take, or agree (in writing or otherwise) to take, any of the actions prohibited by this Section 6.2(b).

Section 6.3.            Cooperation; Access to Information; Interim Financial Information.

(a)           Subject to Section 6.3(c), from the date hereof through the earlier of termination hereof and the Closing Date, each Party shall, and shall cause each of its directors, officers and employees and Representatives to, (i) give the other Party and its Representatives reasonable access, during normal business hours upon reasonable notice, to the books, Contracts, records and other documents, properties, facilities and personnel of the first Party and its Subsidiaries; provided, however, that none of the foregoing shall unreasonably interfere with the conduct of the business of the first Party or any of its applicable Subsidiaries, (ii) as promptly as reasonably practicable, furnish to the other Party all such information concerning its business, properties, facilities, operations and personnel as such other Party may reasonably request; provided, however, that none of the foregoing provisions of this paragraph shall require any Party to provide such access or furnish any such information that, in such Party’s reasonable judgment based on the advice of outside counsel does, or would reasonably be expected to, violate any Law or Data Protection Laws. All documents and information obtained by the Parties or any of their respective Affiliates and Representatives, in each case, that is obtained by virtue of the rights granted by, or otherwise in connection with or pursuant to, this Agreement (including, for the avoidance of doubt, this Section 6.3) shall be subject to the terms and conditions of the Confidentiality Agreement and, for the avoidance of doubt, no information about, or any documents related to, any Plan Investor Group Member shall be disclosed by any Company Group Member in the Bankruptcy Cases or otherwise without the prior written consent of the Plan Investor, except for any information or documents (A) contained or described in the Plan disclosure statement or (B) required by Law or the Bankruptcy Court.

(b)           Subject to Section 6.3(c), each Party shall give prompt notice to the other Party if (i) such Party receives any notices or other communication in writing from any Person alleging that the consent or approval of such Person is or may be required in connection with the transactions contemplated by this Agreement and the other Transaction Documents, (ii) such Party receives any communications from any Governmental Entity (including the SEC), AIM, Euronext or NASDAQ in connection with the transactions contemplated by this Agreement and the other Transaction Documents, or (iii) such Party becomes aware of any occurrence of an event that is reasonably likely to prevent or delay beyond the Outside Date the consummation of the transactions contemplated by this Agreement and the other Transaction Documents or that would reasonably be expected to result in any of the conditions set forth in Article VII not being satisfied at the Closing; provided, however, that the provision of such notice shall not cure any breach of any representation, warranty or covenant contained in this Agreement or otherwise limit or effect the remedies available to each Party hereunder.

(c)           Notwithstanding Section 6.3(a) or Section 6.3(b) to the contrary, the Plan Investor shall not be required to disclose to the Company any such information that in the Plan Investor’s reasonable judgment: (i) is material non-public information, the disclosure of which by the Plan Investor to the Company would constitute a breach by the Plan Investor of its obligations under the Market Abuse Regulation, Data Protection Laws or any other Law; (ii) any information which is subject to legal professional privilege or similar rights under any Laws or (iii) any material competitively sensitive information (including any such information, the disclosure of which could, if required to be disclosed by the Plan Investor pursuant to Rule 21.3 of the Takeover Code to a third party offeror or potential offeror who is a competitor of the Plan Investor (as a result of the disclosure by the Plan Investor to the Company hereunder), be materially detrimental to the Plan Investor).

Section 6.4.           Further Actions; Reasonable Efforts.

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(a)           Upon the terms and subject to the conditions hereof and of the Restructuring Support Agreement, the Plan Investor and the Company each agree to use their respective reasonable best efforts to take, or cause to be taken, all actions, and to do, or cause to be done, and to assist and cooperate with the other Party and its Representatives in doing, all things necessary, proper or advisable to consummate and make effective, in the most expeditious manner practicable, the transactions contemplated by any of the Transaction Documents in accordance with the terms of the Transaction Documents, including the obtaining of all Governmental Requirements, the Plan Investor Stockholder Approval and the execution and delivery of any additional instruments consistent with the terms of the Transaction Documents and necessary to consummate the transactions contemplated by, and to fully carry out the purposes of, the Transaction Documents. The Plan Investor and the Company each agree to use their respective reasonable best efforts to deliver the required notices to, and obtain the required consents or waivers from any third parties in connection with the transactions contemplated by the Transaction Documents, and each Party shall keep the other Party reasonably informed of the status of the delivery and receipt of such third-party consents.

(b)           Notwithstanding anything to the contrary herein, prior to the consummation of the Closing, neither the Plan Investor nor the Company, nor any of their respective Subsidiaries or Affiliates or any Person acting on the behalf of the foregoing, shall unilaterally extend any waiting period or comparable period under any Law in connection with any regulatory filing or enter into any agreement with any Governmental Entity not to consummate the transactions contemplated hereby, without the prior written consent of the other Party.

(c)           Until the earlier of the Closing Date or termination of this Agreement in accordance with the terms hereof, the Plan Investor hereby agrees to, and to cause its Subsidiaries to: (i) use reasonable best efforts to take any and all necessary and appropriate actions in furtherance of the restructuring transactions contemplated under the Restructuring Support Agreement, the Plan and this Agreement and consummation of the transactions contemplated by the Transaction Documents within the time frames contemplated by the Restructuring Support Agreement; (ii) use its reasonable best efforts to support (and not object to) the “first day” motions; (iii) refrain from taking any action not required by Law which is materially inconsistent with, or that would materially delay or materially impede approval, confirmation or consummation of the Plan or that is otherwise materially inconsistent with the express terms of the Restructuring Support Agreement, this Agreement or any other Transaction Document; (iv) not, directly or indirectly, propose, support, solicit, encourage, or participate in the formulation of any chapter 11 plan in the Bankruptcy Cases other than the Plan or other restructuring or reorganization of the Company; (v) use reasonable best efforts to obtain any and all required regulatory approvals and third-party approvals of the transactions contemplated by the Transaction Documents; and (vi) not take any actions materially inconsistent with the Restructuring Support Agreement, the Plan, this Agreement or any other related documents executed by the Company or the Company’s efforts to expeditiously consummate the Restructuring and other transactions contemplated by the Transaction Documents. For the avoidance of doubt, nothing in this Section 6.4 shall prohibit the Plan Investor from exercising its rights under this Agreement or any other Transaction Document, including Section 6.9 hereof, in accordance with the terms set forth therein.

(d)           Until the earlier of the Closing Date or termination of this Agreement in accordance with the terms hereof, the Company hereby agrees to, and to cause its Affiliates to: (i) use reasonable best efforts to take any and all necessary and appropriate actions in furtherance of the restructuring transactions contemplated under the Restructuring Support Agreement, the Plan and this Agreement and consummation of the transactions contemplated by the Transaction Documents within the time frames contemplated by the Restructuring Support Agreement; (ii) commence the Bankruptcy Cases within the time frame provided in this Agreement and the Transaction Documents; (iii) file and prosecute the “first day” motions and all other motions necessary to effectuate the Restructuring pursuant to the

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Plan in accordance with the terms of this Agreement and the Transaction Documents; (iv) refrain from taking any action not required by Law which is materially inconsistent with, or that would materially delay or materially impede approval, confirmation or consummation of the Plan or that is otherwise materially inconsistent with the express terms of the Restructuring Support Agreement, this Agreement or any other Transaction Document; (v) not, directly or indirectly, propose, support, solicit, encourage, or participate in the formulation of any chapter 11 plan in the Bankruptcy Cases other than the Plan or other restructuring or reorganization of the Company; (vi) use reasonable best efforts to obtain any and all required regulatory approvals and third-party approvals of the transactions contemplated by the Transaction Documents; and (vii) not take any actions materially inconsistent with the Restructuring Support Agreement, the Plan, this Agreement or any other related documents executed by the Plan Investor or the Plan Investor’s efforts to expeditiously consummate the Restructuring and other transactions contemplated by the Transaction Documents. Without limiting the generality of the foregoing, the Company agrees to support and use its reasonable best efforts, and agrees to cause the other Company Group Members to use their respective reasonable best efforts, to prosecute the Bankruptcy Cases to accomplish the foregoing. For the avoidance of doubt, nothing in this Section 6.4 shall prohibit the Company from exercising its rights under this Agreement or any other Transaction Document including, Section 6.8 hereof in accordance with the terms set forth therein.

(e)           Consultation Right. The Company may consult with the Plan Investor in respect of, and the Plan Investor may provide consultation in respect of, any material and non-ordinary course of business matters pertaining to the launch of any products of the Company or any Subsidiary thereof in Europe or Latin America, provided that neither the Company nor the Plan Investor shall have any monetary obligation or liability of any kind to any person or entity in respect of any such consultation; provided further, that failure to comply with this Section 6.4 shall not be deemed a failure to satisfy the covenants in this Article VI.

Section 6.5.            Listing on AIM.

(a)           The Parties shall use their reasonable best efforts to cause all of the issued and to be issued ordinary shares of the Company to be listed and admitted to trading on AIM and Euronext at Closing. If an admission document (an “Admission Document”) is required under the AIM Rules for Companies and/or the Euronext Rules for Companies, as soon as reasonably practicable following the date of this Agreement, the Plan Investor shall prepare a draft copy of the Admission Document (together with any applications to AIM or Euronext) and the Plan Investor shall cause the Admission Document to comply as to form and substance in all material respects with the requirements of applicable Laws, and the Admission Document shall contain a notice for the general meeting at which the Plan Investor Stockholder Approval is sought in accordance with the Plan Investor’s Charter Documents. The Plan Investor shall provide such copy of the Admission Document to the Company in advance of submission and will provide the Company a reasonable opportunity to review and comment thereon. The Company shall furnish all information concerning itself, its affiliates and its stockholders to the Plan Investor and provide such other assistance as may be reasonably required in respect of the preparation and approval of the Admission Document. Each of the Company and the Plan Investor shall use its reasonable best efforts to enable the Admission Document to be published promptly after the date of this Agreement, including by supplying all such information, executing all such documents and paying all such fees incurred by each such Party as may be reasonably necessary or required. If at any time prior to the Closing Date any information relating to the Plan Investor, the Company, or any of their respective Affiliates, officers or directors should be discovered by Plan Investor or the Company that should be set forth in an amendment of, or a supplement to, the Admission Document so that it would not include any misstatement of a material fact or omit to state any material fact necessary to make the statements therein, in light of the circumstances under which they were made, not misleading, the Party that discovers such information shall promptly notify the other Party and the Parties shall cooperate in the prompt publication of any

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necessary amendment of, or supplement to, the Admission Document, and to the extent required by applicable Law, in disseminating the information contained in such amendment or supplement to the stockholders of the Plan Investor. Notwithstanding any other provision herein to the contrary, no amendment or supplement (including by incorporation by reference) to the Admission Document shall be made without prior written approval of both Parties, which approval shall not be unreasonably withheld, conditioned or delayed.

(b)           The Plan Investor shall use its reasonable best efforts to publish the Admission Document, in accordance with applicable Laws and as promptly as practicable following the date hereof. The Plan Investor shall advise the Company, promptly when the Admission Document has been approved by its nominated adviser (the “Nomad”). The Plan Investors agrees to provide the Company with copies of any written comments, and shall inform the Company of any oral comments, from the Nomad (or any other person) relating to drafts of the Admission Document or notification that the Admission Document is formally approved by the Nomad. The Plan Investor shall give due consideration to the additions, deletions or changes suggested by the Company in response to communication from the Nomad and the Plan Investor shall use its reasonable best efforts to respond as promptly as practicable to any comments from the Nomad relating to drafts of the Admission Document.

(c)           The Company shall use reasonable best efforts to create, as promptly as practicable following the date of this Agreement, a consolidated balance sheet of the Company Group and the related statements of income, cash flows and changes in owners’ equity for each of the fiscal years of the Company as required by the AIM Rules (and any derogation therefrom granted by AIM) prior to the date of this Agreement. The Company shall use reasonable best efforts to have such financial statements audited by an internationally recognized accounting firm and, upon receipt of the audit opinion of such accounting firm, the Company shall promptly deliver such financial statements and such audit opinion to the Plan Investor. The Company shall permit the Plan Investor to use such financial statements, and will use reasonable best efforts to cause the accounting firm to use such audit opinion in any filings with the Nomad or any Governmental Entity, or in any disclosure document (including the Admission Document), necessary to consummate the transactions contemplated by this Agreement and the other Transactions Documents. The Company shall keep the Plan Investor reasonably informed of the status of the preparation of such financial statements and the receipt of such audit opinion.

Section 6.6.            U.S. Registration.

(a)           As promptly as practicable after the execution of this Agreement, the Parties shall jointly prepare and cause to be confidentially submitted to the SEC, a Form F-1 or other form appropriate for registration under the Securities Act (the “Form F-1”) in connection with the registration for resale of certain of the ordinary shares of the Plan Investor or American Depositary Shares representing such ordinary shares to be issued hereunder. Each Party shall use its reasonable best efforts to cause the Form F-1 to be declared effective as promptly as practicable after Closing (including by responding to comments of the SEC, if any). Each Party shall furnish all information as may be reasonably requested by the other Party in connection with any such action and the preparation, filing and distribution of the Form F-1. Prior to the Closing, no filing of, or amendment or supplement to, the Form F-1 will be made by either Party without providing the other Party with a reasonable opportunity to review and comment thereon. Each Party agrees to provide the other Party with copies of any written comments, and shall inform the other Party of any oral comments, that such Party or its counsel may receive prior to the Closing from the SEC or its staff with respect to Form F-1 promptly after receipt of such comments, and any written or oral responses thereto. Each Party shall be given a reasonable opportunity to review any such written responses and each Party shall give due consideration to the additions, deletions or changes suggested thereto by the other Party. If at any time prior to the time that the Form F-1 is declared effective by the SEC any information relating to a Party or its Affiliates,

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directors or officers should be discovered by such Party which should be set forth in an amendment or supplement to the Form F-1, so that either such document would not include any misstatement of a material fact or omit to state any material fact necessary to make the statements therein, in light of the circumstances under which they were made, not misleading, the Party that discovers such information shall promptly notify the other Party and an appropriate amendment or supplement describing such information shall be promptly filed with the SEC. The Plan Investor shall promptly notify the Company of (i) the time when the Form F-1 has been declared effective, and (ii) the issuance of any stop order or suspension of the qualification of the Closing Shares for offering or sale in any jurisdiction.

(b)           The Parties shall use their reasonable best efforts to cause the Closing Shares to be listed and approved for trading on NASDAQ (under a ticker symbol to be agreed upon in writing by the Parties) at Closing or as soon as reasonably practicable thereafter. Prior to Closing, the Parties shall submit an initial listing application with NASDAQ (the “NASDAQ Listing Application”) with respect to the Closing Shares. Each Party shall use its reasonable best efforts to cause the NASDAQ Listing Application to be approved (subject to official notice of issuance) as promptly as practicable following such submission (including by responding to any comments from NASDAQ). Each of Party shall furnish all information as may be reasonably requested by the other Party in connection with any such action and the preparation and submission of the NASDAQ Listing Application. No material submission of, or material amendment or supplement to, the NASDAQ Listing Application will be made by either Party without providing the other Party with a reasonable opportunity to review and comment thereon. In addition, each Party agrees to provide the other Party with copies of any written comments, and shall inform the other Party of any oral comments, that such Party or its counsel may receive prior to the Closing from NASDAQ or its staff with respect to the NASDAQ Listing Application promptly after receipt of such comments, and any written or oral responses thereto. Each Party shall be given a reasonable opportunity to review any such written responses and each Party shall give due consideration to the additions, deletions or changes suggested thereto by the other Party.

Section 6.7.            Regulatory Filings.

(a)           The Plan Investor and the Company shall cooperate to promptly (and in any event within ten (10) Business Days) after the date hereof make or cause, as applicable, to be made, all required filings and submissions under the HSR Act, if applicable, or any other applicable antitrust Laws (the HSR Act and any other applicable antitrust Law, in each case if applicable, “Antitrust Laws”). Each Party shall promptly comply with any additional requests for information, including requests for production of documents and production of witnesses for interviews or depositions by any Governmental Entity. The Parties shall cooperate in good faith in connection with all filings under applicable Antitrust Laws and use their respective reasonable best efforts to undertake promptly any and all action required to complete the transactions contemplated by any of the Transaction Documents. In furtherance and not in limitation of the covenants of the Parties contained in this Section 6.7, each of the Parties shall use its reasonable best efforts to resolve objections, if any, that may be asserted by any Governmental Entity in connection with any Antitrust Laws and to avoid the entry of, or effect the dissolution of, any order in any suit or proceeding that would otherwise have the effect of preventing the consummation of the transactions contemplated hereby (including by defending any lawsuits or other legal proceedings, whether judicial or administrative, challenging this Agreement or the transactions contemplated by any of the Transaction Documents).

(b)           If an action is threatened or instituted by any Governmental Entity or any other Person challenging the validity or legality or seeking to restrain the consummation of the transactions contemplated by any of the Transaction Documents, the Plan Investor and the Company shall each use their reasonable best efforts to avoid, resist, resolve or, if necessary, defend such action and shall afford the other Party a reasonable opportunity to participate therein at its own expense.

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(c)           Subject to the provisions of the Confidentiality Agreement, each Party shall cooperate with the other Party in preparing and filing any and all written communications that are to be submitted to any Governmental Entity in connection with the transactions contemplated by any of the Transaction Documents and in obtaining any governmental or third-party consents, waivers, authorizations or approvals that may be required to be obtained by either Party in connection with the transactions contemplated by any of the Transaction Documents, which assistance and cooperation shall include: (i) timely furnishing to the other Party all information concerning the first Party or any of its Affiliates that counsel to the requesting Party reasonably determines is required to be included in such documents or would be helpful in obtaining such required consent, waiver, authorization or approval; (ii) promptly providing the other Party with copies of all written communications to or from any Governmental Entity relating to Antitrust Laws; (iii) keeping the other Party reasonably informed of any communication received or given in connection with any Proceeding regarding the Restructuring and the transactions contemplated by any of the Transaction Documents; and (iv) permitting the other Party to review, and considering in good faith incorporating such other Party’s comments to, any written communication to any Governmental Entity or in connection with any proceeding related to Antitrust Laws, in each case regarding the Restructuring and transactions contemplated by the Transaction Documents.

(d)           Neither the Plan Investor nor the Company, nor any of their respective Representatives, shall initiate, or participate in any meeting or discussion with any Governmental Entity with respect to any filings, applications, investigation, or other inquiry regarding the Restructuring or filings under any Antitrust Laws without giving the other party reasonable prior notice of the meeting or discussion and, to the extent permitted by the relevant Governmental Entity, the opportunity to attend and participate in such meeting or discussion. The Plan Investor and the Company shall equally split all filing fees payable to Governmental Entities under any Antitrust Laws with respect to the transactions contemplated by the Transaction Documents.

(e)           Compliance with Agreements with Governmental Entities. The Company shall provide notice to the Plan Investor and shall use reasonable best efforts to take any actions requested by the Governmental Entities in connection with effecting the transactions contemplated by this Agreement in conformity with the Agreements with Governmental Entities provided on Section 4.12 of the Company Disclosure Schedule.

Section 6.8.            Financing.

(a)          The Company shall use its reasonable best efforts to take any actions, reasonably necessary to effectuate the issuance of the New Term Loan and New Convertible Notes and the Plan Investor shall reasonably cooperate in connection with issuance of the New Term Loan and New Convertible Notes Financing, including by delivery of information or execution of documents or instruments reasonably requested by the Company.

(b)           The Plan Investor shall use its reasonable best efforts to take any actions, reasonably necessary to consummate, on or prior to the Closing, the Company Rights Offering Transaction, the Plan Investor Equity Raise and the Plan Investor Additional Equity Issuance (the Plan Investor Equity Raise, the Company Rights Offering Transactions and the Plan Investor Additional Equity Issuance are collectively referred to herein as the “Equity Transactions”). The Company shall use its reasonable best efforts to take any actions, reasonably necessary to effectuate the Equity Transactions and the Parties shall reasonably cooperate in connection with consummation of the Equity Transactions, including by delivery of information or execution of documents or instruments reasonably requested by the other Party.

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Section 6.9.            Company Solicitations; Company Alternative Transactions.

(a)           No Change in Company Board Approval. Except as set forth in this Section 6.9, the Company Board shall not (i)(A) withhold or withdraw (or modify or qualify in any manner adverse to the Plan Investor), or (B) propose publicly to withhold or withdraw (or modify or qualify in any manner adverse to the Plan Investor) the Company Board Approval, or (ii) approve, recommend or declare advisable, or propose publicly to approve, recommend or declare advisable, any Company Alternative Proposal or Company Alternative Transaction or allow any Company Group Member to enter into any definitive agreement relating to any Company Alternative Transaction (a “Company Alternative Transaction Agreement”) constituting or relating to, or that is intended to or would reasonably be expected to result in or lead to, any Company Alternative Proposal or Company Alternative Transaction, or requiring, or that would reasonably be expected to cause, the Company to abandon, terminate, delay or fail to consummate, or that would otherwise impede, interfere with or be inconsistent with, the transactions contemplated by this Agreement and the other Transaction Documents, or requiring, or that would reasonably be expected to cause, the Company to fail to comply with the terms of this Agreement or other Transaction Documents.

(b)           Go-Shop Rights. Notwithstanding anything to the contrary set forth in this Agreement, during the period (the “Go-Shop Period”) (i) beginning on the Petition Date, the Company and its advisors, members, consultants, legal counsel and investment bankers that are providing services in connection with the transactions contemplated in the Transaction Documents (each a “Representative” and collectively, “Representatives”) shall have the right (subject to the entry into, and in accordance with, an Acceptable Confidentiality Agreement) to, furnish to any Person that has made an unsolicited proposal any non-public information relating to the Company and its Subsidiaries or afford to any Person access to the business, properties, assets, books, records or other non-public information, or to any personnel, of the Company and its Subsidiaries, and (ii) beginning on the date that the Bankruptcy Court enters the PFA Order and continuing until 12:00 p.m., Eastern time on the date that is fifty-five (55) days following the entry of such PFA Order, the Company and its Representatives shall have the right to: (A) solicit, initiate, propose or induce the making of, or knowingly encourage, any proposal that constitutes, or is reasonably expected to lead to, a Company Alternative Proposal; (B) subject to the entry into, and in accordance with, an Acceptable Confidentiality Agreement, furnish to any Person any non-public information relating to the Company and its Subsidiaries or afford to any Person access to the business, properties, assets, books, records or other non-public information, or to any personnel, of the Company and its Subsidiaries, in any such case, in connection with the actions permitted by this Section 6.9(b); provided, however, that with respect to clauses (i) and (ii), the Company will promptly (and in any event within 24 hours) provide to the Plan Investor, or provide the Plan Investor access to, any such non-public information concerning the Company and/or its Subsidiaries that is provided to any such Person or its Representatives that was not previously provided to the Plan Investor or its Representatives; and, provided, further that with respect to clauses (i) and (ii), the Company shall withhold such portions of documents or information, or provide pursuant to customary “clean-room” or other appropriate procedures, to the extent relating to any pricing or other matters that are highly sensitive or competitive in nature if the exchange of such information (or portions thereof) would reasonably be likely in the Company’s reasonable judgment to be harmful to the operation of the Company in any material respect and neither furnish nor otherwise provide access to any information to any Person pursuant to this Section 6.9(b) to the extent the Company reasonably determines that such furnishing or access would jeopardize any legal professional privilege or similar right; and (iii) subject to the restrictions contained in the immediately preceding clause (ii), participate and engage in discussions or negotiations with any Person with respect to a Company Alternative Proposal.

(c)           No Solicitation. Except as expressly permitted by this Section 6.9, the Company will, and will cause each other Company Group Member and its and their respective officers and directors

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to, and will cause its other Representatives to, (i) promptly cease and terminate all solicitations, discussions and negotiations with any Person that would be prohibited by this Section 6.9(c) (including any Person that received non-public information about the Company and its Subsidiaries or with whom the Company or its Representatives had discussions during the Go-Shop Period) and terminate all physical and electronic data-room access previously granted to any such Person or any of their Representatives in connection with the consideration of a Company Alternative Proposal (other than with respect to the Plan Investor and the other parties to the Restructuring Support Agreement and its and their respective Affiliates and Representatives); and (ii) not, directly or indirectly, (A) initiate, solicit, knowingly encourage or knowingly facilitate any inquiries, proposals or offers from any Person other than the Plan Investor and its Affiliates and its and their respective Representatives, relating to, or that could reasonably result in, any merger, acquisition, divestiture, sale of material assets or equity, business combination, recapitalization, joint venture, or other extraordinary transaction directly or indirectly involving the equity, voting power or all or a material portion of the assets of the Company Group, taken as a whole or any proposal that by its terms requires the Company to abandon, terminate or fail to consummate the transactions contemplated by this Agreement (any such transaction or proposal, a “Company Alternative Transaction”); (B) other than an Acceptable Confidentiality Agreement, enter into any agreement (including any acquisition agreement, restructuring support agreement, plan funding agreement, or similar definitive agreement, or any letter of intent, memorandum of understanding, agreement in principle or similar agreement) relating to any Company Alternative Transaction other than with the Plan Investor or one of its Affiliates; (C) participate in discussions or negotiations with any other Person with respect to, or that would reasonably be expected to result in, a Company Alternative Transaction with a party other than the Plan Investor or one of its Affiliates; (D) provide to any other Person, any material non-public information relating directly or indirectly to any Company Group Member, the purpose of which is to assist or facilitate a Company Alternative Proposal or a Company Alternative Transaction with any Person other than the Plan Investor or one of its Affiliates; or (E) publicly propose to do any of the actions prohibited by any of clauses (A) through (D), other than in connection with a transaction with the Plan Investor or its Affiliates. Without limiting the foregoing, it is agreed that any violation of the restrictions set forth in this Section 6.9(c) by any Company Group Member, and officer or director of the Company or any Company Group Member or any other Representative shall constitute a breach of this Section 6.9(c) by the Company. Notwithstanding anything to the contrary in Section 6.9, nothing herein shall prohibit the Company from releasing, waiving, modifying or not enforcing a standstill or confidentiality restriction with respect to any Person solely to the extent necessary to permit such Person to make a Company Alternative Proposal.

(d)           Permitted Actions. Notwithstanding anything to the contrary in Section 6.9(c), nothing in this Agreement shall prohibit or limit the Company or any of its Representatives from (i) indicating to any Person that the Company or such Representatives are not permitted to engage in any negotiations relating to any Company Alternative Transaction, (ii) making any (x) disclosure or (y) “stop-look-and-listen” communication or any other similar disclosure that, in either case, in the good faith determination of the Company Board (after consultation with its outside legal counsel) is required by applicable Laws, (iii) participating in any negotiations, or entering into any definitive agreements, with any other Person (or such Person’s Representatives) solely in connection with such Person or an Affiliate thereof providing debt financing to the Company Group (including under the DIP Financing Agreement) as contemplated by the Plan, or (iv) participating in any negotiations, or entering into any definitive agreements (including a joinder to the Restructuring Support Agreement), with any Person (or such Person’s Representatives) who is a debt holder or creditor of any Company Group Member, but in either instance of the immediately foregoing clauses (iii) or (iv), only in connection with the completion and consummation of the transactions contemplated by the Restructuring Support Agreement and the other Transaction Documents and making any related filings or petitions for relief under chapter 11 of the Bankruptcy Code or other similar Laws that are consistent with and in furtherance of the transactions contemplated by the Restructuring Support Agreement and the other Transaction Documents.

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(e)           Company Alternative Proposals. Notwithstanding anything to the contrary in this Section 6.9, and without limiting any rights of the Company under the Restructuring Support Agreement, prior to the confirmation of the Plan, if the Company receives a bona fide Company Alternative Proposal that did not result from a breach of this Section 6.9 from any Person, the Company and its Representatives may, in all cases subject to compliance with this Section 6.9(e): (i) communicate with such Person solely to clarify the terms and conditions thereof in order to determine whether such Company Alternative Proposal constitutes or would reasonably be expect to lead to a Company Superior Proposal, (ii) provide information (including non-public information and data) regarding, and afford access to the business, properties, assets, books, records and personnel of, the Company Group to such Person and its Representatives if the Company receives from such Person (or has received from such Person) an executed Acceptable Confidentiality Agreement; provided that the Company shall promptly make available to the Plan Investor any information concerning the Company and the Subsidiaries that is provided to any such Person and that was not previously made available to the Plan Investor or any of its Representatives; and, provided further that (A) the Company shall withhold such portions of documents or information, or provide pursuant to customary “clean-room” or other appropriate procedures, to the extent relating to any pricing or other matters that are highly sensitive or competitive in nature if the exchange of such information (or portions thereof) could reasonably be likely in the Company’s reasonable judgment to be harmful to the operation of the Company in any material respect or (B) the Company shall neither furnish nor otherwise provide access to any information to any Person pursuant to this Section 6.9(e) to the extent the Company reasonably determines that such furnishing or access would jeopardize any legal professional privilege or similar right; and (iii) engage in, enter into or otherwise participate in any discussions or negotiations with such Person (and the stakeholders in the Company that are party to the Restructuring Support Agreement) with respect to such Company Alternative Proposal, if prior to taking any action described in immediately foregoing clauses (ii)-(iii) above, the Company Board determines in good faith, after consultation with its outside counsel and financial advisors, that such Company Alternative Proposal constitutes a Company Superior Proposal or would reasonably be expected to result in a Company Superior Proposal and that the failure to take the actions set forth in immediately foregoing clauses (ii)-(iii) above would reasonably be expected to constitute a breach of the Company Board’s fiduciary duties. The Company shall promptly (and in any event within twenty-four (24) hours) notify the Plan Investor of (i) the receipt of any Company Alternative Proposal or any initial request for non-public information concerning the Company or any of its Subsidiaries, related to, or from any Person who could reasonably be expected to make any Company Alternative Proposal, or any other inquiry or initial request for discussions or negotiations related to any Company Alternative Proposal (including any material changes related to the foregoing), and in connection such notice, shall specify the material terms thereof (including, if applicable, by providing copies of any written requests, proposals, letters of intent or offers, including proposed agreements), and provide the identity of the Person making such Company Alternative Proposal, request or inquiry (including, if known, each of its beneficial owners and controlling persons), and (ii) the Company shall keep the Plan Investor informed, on a reasonably current basis (and, in any event within twenty-four (24) hours of the Company’s Knowledge of any such event), of any material developments, discussions, negotiations or modifications to the financial or other material terms and conditions of such Company Alternative Proposal, request or inquiry or any amendment thereto (including by providing copies of any written requests, proposals, letters of intent or offers, including).

(f)            Company Superior Proposals. Notwithstanding anything to the contrary set forth in this Agreement, prior to the confirmation of the Plan, if (i) the Company receives a Company Alternative Proposal that did not result from a breach of this Section 6.9 that the Company Board determines in good faith, after consultation with its outside counsel and financial advisors, constitutes a Company Superior Proposal, and (ii) the Company Board determines in good faith, after consultation with its outside counsel and financial advisors, that the failure to take the actions set forth in this Section 6.9(f) would reasonably be expected to constitute a breach of its fiduciary duties, then the Company

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Board may authorize, adopt, or approve such Company Superior Proposal and cause or permit the Company to terminate this Agreement pursuant to Section 8.1(b)(iii) in order to simultaneously enter into a Company Alternative Transaction Agreement with respect to such Company Superior Proposal if: (i) the Company shall have provided prior written notice (a “Company Notice of Intended Recommendation Change”) to the Plan Investor of the Company’s intention to take such actions described in this Section 6.9(f) at least five (5) Business Days in advance of taking such actions, which notice shall include a reasonably detailed description of the material terms and conditions of the Company Alternative Proposal received by the Company that constitutes a Company Superior Proposal, including a copy of the Company Alternative Transaction Agreement and any other proposed transaction agreements with, and the identity of, the party making such Company Alternative Proposal; (ii) after providing such notice and prior to terminating this Agreement, the Company shall have negotiated, and shall have caused its Representatives to negotiate, with the Plan Investor and its Representatives in good faith (to the extent the Plan Investor desires to so negotiate) during such five (5) Business Day period to make such adjustments to the terms and conditions of this Agreement and the other Transaction Documents as would result in such Company Alternative Proposal not constituting a Company Superior Proposal; (iii) the Company Board shall have considered in good faith any changes to this Agreement and the other Transaction Documents that may be offered in writing by the Plan Investor by 5:00 p.m. Eastern Time on the last Business Day of the period described in the foregoing clause (ii); and (iv) following the expiration of such five (5) Business Days’ notice period, the Company Board shall have determined in good faith, after consultation with its outside counsel and financial advisors, that the Company Alternative Proposal received by the Company would continue to constitute a Company Superior Proposal even if such changes offered in writing by the Plan Investor were given effect and that the failure to take the actions contemplated by this Section 6.9(f) would continue to be reasonably expected to constitute a breach of its fiduciary duties; provided, however, that any material amendment to the terms of any Company Superior Proposal (and, in any event, including any amendment to any price term thereof or the form of consideration payable in connection therewith), shall require delivery of a new Company Notice of Intended Recommendation Change and compliance with the five (5) Business Days’ period described in this Section 6.9(f). For the avoidance of doubt, in connection with any Company Alternative Proposal (including any Company Superior Proposal) pursuant to this Section 6.9(f), the Company shall continue to comply with the obligations set forth in the last sentence of Section 6.9(e).

Section 6.10.         Plan Investor Solicitations; Plan Investor Alternative Transactions. No provision of this Agreement shall require the Plan Investor to take any action in relation to any Plan Investor Alternative Transaction (or any potential Plan Investor Alternative Transaction) which is prohibited or restricted by the Takeover Code, nor shall any provision of this Agreement require the Plan Investor to refrain from taking any action in relation to any Plan Investor Alternative Transaction (or any potential Plan Investor Alternative Transaction) which is required by the Takeover Code.

Section 6.11.        Plan Investor Stockholder Approval. The Plan Investor shall, in accordance with its Charter Documents and applicable Law, as promptly as reasonably practicable following the date of this Agreement, take all actions that are reasonably necessary or required to receive the approval of the Plan Investor Stockholders, as required in accordance with Section 7.1(e) (the “Plan Investor Stockholder Approval”) in connection with the Acquisition, the issuance of the Closing Shares and other transactions contemplated herein and in the other Transaction Documents (including, without limitation, the approval of any waiver obtained from the Panel of any obligation that would otherwise arise on the recipients of the Closing Shares, either individually and collectively, to make a general offer to the remaining Plan Investor Stockholders pursuant to Rule 9 of the Takeover Code as a result of the allotment and issue of the Closing Shares to such recipients, to the extent that such waiver is required by the Panel). Without limiting the generality of the foregoing, the Plan Investor shall, in consultation with the Company, (i) establish a record date for, and duly call and give notice of, a meeting of the Plan Investor Stockholders entitled to vote on the transactions contemplated by this Agreement (at which meeting the Plan Investor

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shall seek the Plan Investor Stockholder Approval), (ii) cause appropriate proxy materials for such meeting to be mailed to the Plan Investor Stockholders and (iii) duly convene and hold such meeting. The Plan Investor shall use its reasonable best efforts to take, or cause to be taken, all actions, and do or cause to be done all things, necessary, proper or advisable on its part to cause the Plan Investor Stockholder Approval to be received at such meeting or any adjournment or postponement thereof, and shall comply with all legal requirements applicable to such meeting. The Plan Investor shall not, without the prior written consent of the Company, adjourn, postpone or otherwise delay such meeting; provided that the Plan Investor may, notwithstanding the foregoing, without the prior written consent of the Company, adjourn or postpone such meeting if, after consultation with the Company, the Plan Investor believes in good faith that such adjournment or postponement is reasonably necessary to allow reasonable additional time to solicit additional proxies necessary to obtain the Plan Investor Stockholder Approval. Without the prior written consent of the Company, the matters contemplated by the Plan Investor Stockholder Approval shall be the only matters (other than matters of procedure and matters required by applicable Law to be voted on by the Plan Investor Stockholders in connection therewith) that the Plan Investor shall propose to be voted on by the stockholders of the Plan Investor Stockholders at such meeting.

Section 6.12.        Intercompany Obligations. From and after the Closing, all intercompany agreements or obligations (including any intercompany account balances or cash pooling arrangements), between the Company, on the one hand, and any other Company Group Member, on the other hand, shall be treated in accordance with the Plan.

Section 6.13.        Scheme. Notwithstanding anything herein to the contrary, the Plan Investor shall (and shall be entitled to), in accordance with its Charter Documents and applicable Law, as promptly as reasonably practicable following the date of this Agreement, use reasonable best efforts to take all actions that are reasonably necessary or required to receive the approval of the Plan Investor Stockholders in connection with the Scheme, such that the Scheme shall become effective prior to Closing. The Parties agree that on and from the effectiveness of the Scheme, if determined by the Plan Investor by delivery of written notice to the Company, New Atlas TopCo shall assume all rights and obligations of the Plan Investor by hereunder (but such assumption shall not release the Plan Investor from its obligations hereunder unless and until the Closing shall occur), and shall be substituted for the Plan Investor as a party to this Agreement for all purposes hereunder, mutatis mutandis. The Parties shall, and the Plan Investor shall procure that New Atlas TopCo shall, enter into all such agreements and execute all such further contracts as are required to give effect to this Section 6.13. In connection with the Scheme, notwithstanding anything herein to the contrary (including Section 6.2), the Plan Investor may procure that New Atlas TopCo may issue securities to the Plan Investor Stockholders and Plan Investor Optionholders as of the record date established therefor (such securities, the “CVR Securities”) which CVR Securities entitle the holder thereof to receive certain distributions (such distributions, the “CVR Distributions”), with such CVR Distributions to be made, and such CVR Securities to have, the terms set forth on Exhibit C attached hereto.

Section 6.14.          Other Governance Matters. The Parties shall use reasonable best efforts to cause immediately following the Closing:

(a)           Headquarters. The global headquarters of the Plan Investor and its Subsidiaries to be located in Dublin, Ireland and the U.S. headquarters of the Plan Investor and its Subsidiaries to be located in Boston, Massachusetts.

(b)           Board Composition. The Plan Investor Board shall be comprised of seven (7) members appointed for terms of a period of two years following Closing and all such members shall be subject to the prior unanimous approval of the Plan Investor, Highbridge and Athyrium, which approval shall not be unreasonably withheld, and the Plan Investor Board shall be comprised of the following: (i)

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the chief executive officer of the Plan Investor as at the Closing, who shall be Joe Wiley, and, together with the chief executive officer of the Plan Investor, the Plan Investor shall be entitled to designate for nomination, appointment or reappointment two (2) members to the Plan Investor Board, both of whom shall be Independent, (ii) Highbridge shall be entitled to designate for nomination two (2) members to the Plan Investor Board and (iii) Athyrium shall be entitled to designate for nomination two (2) members to the Plan Investor Board, for a total of four (4) directors between each of Highbridge and Athyrium and their respective Affiliates, one (1) of whom shall not be an American citizen or U.S. resident. At least one (1) director nominated by each of Highbridge or its Affiliates and Athyrium or its Affiliates shall be Independent.

(c)           Employment Agreements. The Plan Investor to enter into employment agreements, as promptly as practicable following the Closing, in form and substance reasonably acceptable to the Plan Investor, with Joe Wiley and Rory Nealon, whereby each such Person shall be, respectively, the Chief Executive Officer and Chief Financial Officer of the Plan Investor as of and following consummation of the Closing (the “Employment Agreements”).

(d)           Employee Equity Incentive Plan. The Plan Investor to reserve ten percent of the Plan Investor’s Closing Shares (calculated on a fully-diluted basis) for issuance pursuant to an employee equity incentive plan to be adopted by the Plan Investor Board following the Closing.

Section 6.15.         Communication Materials. Prior to the Closing, the Parties shall use reasonable best efforts to (i) cooperate in good faith to jointly develop a communications plan with the employees of the Parties and their Subsidiaries regarding the transactions contemplated hereby and (ii) provide reasonable cooperation with each other in connection with communications with employees.

Section 6.16.         American Depository Shares. During the term of this Agreement, the Plan Investor Stockholder shall provide the Company with all notices and documentation related to the issuance of any American Depository Shares.

ARTICLE VII.

CONDITIONS TO CLOSING

Section 7.1.          Conditions to the Obligations of Each Party. The obligations of the Plan Investor and the Company to consummate the transactions contemplated by this Agreement are subject to the satisfaction or waiver (or the extent permitted by applicable Law), at or prior to the Closing, of the following conditions:

(a)           Plan Confirmation. The Confirmation Order, in form and substance reasonably acceptable to the Plan Investor and the Company, shall have become a Final Order and remain in full force and effect (and no stay of the Confirmation Order shall be in effect).

(b)           Plan Approval. The conditions precedent to the consummation of the Plan have each been satisfied or waived in accordance with the terms of the Plan and the transactions contemplated by the Plan (including the emergence of the relevant members of the Company Group from the Bankruptcy Cases) shall be consummated substantially concurrently with the Closing.

(c)           Regulatory Approval. Any applicable waiting period (and any extension thereof) under the applicable Antitrust Laws relating to the transactions contemplated by any of the Transaction Documents as set forth on Section 7.1(c) of the Company Disclosure Schedule and Section 7.1(c) of the Plan Investor Disclosure Schedule shall have been terminated or expired.

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(d)           No Injunction. No order, whether temporary, preliminary or permanent (each, a “Restraining Order”), shall have been enacted, entered, promulgated, adopted, issued or enforced by any Governmental Entity of competent jurisdiction that is in effect on the Closing Date and has the effect of making any of the transactions contemplated by any of the Transaction Documents illegal or otherwise prohibiting or preventing the consummation of the transactions contemplated by any of the Transaction Documents.

(e)           Plan Investor Stockholder Approval. The Plan Investor shall have duly passed the Plan Investor Stockholder Approval.

(f)            The Plan Investor Equity Raise and Company Rights Offering Transactions. The Plan Investor Equity Raise and the Company Rights Offering Transactions shall have been consummated in accordance with the Rights Offering Documentation .

(g)           The Restructuring Support Agreement. The Restructuring Support Agreement shall not have been terminated and shall remain in full force and effect and the PFA Order shall have become a Final Order and remain in full force and effect; provided that a termination of the Restructuring Support Agreement as to any party thereto where the termination occurs only as to such party and the Restructuring Support Agreement remains in full force and effect with respect to the other parties thereto, shall not mean the Restructuring Support Agreement has been terminated or is not in full force and effect for purposes of this paragraph.

(h)           AIM Listing. The Admission Document shall have been published and AIM shall have acknowledged to the Plan Investor or New Atlas Topco (and such acknowledgment shall not have been withdrawn) that the application for readmission of the ordinary shares of the Plan Investor or New Atlas Topco, as the case may be, to trading on the AIM has been approved and will become effective after satisfaction of any conditions to which such approval is expressed to be subject, and such conditions having been satisfied.

(i)            Whitewash. A waiver being granted by the Panel of the obligations which may otherwise arise pursuant to Rule 9 of the Takeover Code for certain lenders of the Company to make a general offer to the Plan Investor Stockholders for all the issued ordinary shares in the capital of the Plan Investor as a result of the distribution of the Closing Shares to such lenders following the issuance thereof to the Company as contemplated hereby, and such waiver being approved by the Plan Investor Stockholders by a resolution duly passed by the requisite majority of Plan Investor Stockholders entitled to vote on such resolution pursuant to the Takeover Code and any requirement or direction issued by the Panel in connection therewith.

(j)            New Term Loan Financing. The financing in connection with the New Term Loan Agreement (the “New Term Loan Financing) shall have been consummated prior to the Closing, or it shall be manifestly apparent that the New Term Loan Financing will be consummated simultaneously with the Closing (including by the lenders thereunder confirming that they are ready, willing and able to close the New Term Loan Financing pursuant to its terms and, if applicable, the proceeds thereof will be used to satisfy the amount(s) due under the EIB Payoff Letter as directed by the Plan Investor ).

(k)           New Convertible Notes. The New Convertible Notes shall have been issued or shall be issued contemporaneously with the Closing.

(l)            Scheme Resolutions. The approval of the Scheme, by the requisite majority in number and in value of the Plan Investor Stockholders who are on the register of members of the Plan Investor at the voting record time in respect of the Scheme as set forth pursuant to the terms of the

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Scheme Document, at the court meeting convened in respect of the Scheme and at any separate class meeting which may be required by the courts of England in connection with the Scheme (or any adjournment thereof).

(m)          Scheme Sanction. The sanction of the Scheme by the courts of England (with or without modification (but subject to such modification being acceptable to the Plan Investor and the Company)) and the delivery of the office copy of the court order to the Registrar of Companies in the United Kingdom.

Section 7.2.           Conditions to the Obligations of the Plan Investor. The obligation of the Plan Investor to consummate the transactions contemplated hereby shall be subject to the satisfaction at or prior to the Closing of each of the following conditions; provided, however, that the Plan Investor may, in its sole and absolute discretion, waive any or all of the following conditions:

(a)           Company Representations and Warranties; Company Covenants. (i) (A) The Company Fundamental Representations shall be true and correct in all material respects (without giving effect to any limitation as to “materiality”, “Company Material Adverse Effect” or other similar qualifications) both as of the date of this Agreement and as of the Closing Date as if made as of such date (except to the extent expressly made as of an earlier date, in which case, as of such earlier date) and (B) all other representations and warranties of the Company set forth in this Agreement shall be true and correct in all respects (without giving effect to any limitation as to “materiality”, “Company Material Adverse Effect” or other similar qualifications) both as of the date of this Agreement and as of the Closing Date as if made as of such date (except to the extent expressly made as of an earlier date, in which case, as of such earlier date), except where the failure of such representations and warranties to be true and correct would not, individually or in the aggregate, reasonably be expected to result in a Company Material Adverse Effect, (ii) the Company has performed, or caused to be performed, in all material respects the covenants in this Agreement that are required to be complied with by the Company Group between the date of this Agreement and the Closing Date, and (iii) the Plan Investor shall have received a certificate signed by an authorized officer of the Company, in a form reasonably acceptable to the Plan Investor, certifying that the conditions in the foregoing clauses (i) and (ii) have been satisfied at Closing.

(b)           Transaction Documents. Each of the parties to the Transaction Documents (other than the Plan Investor and its subsidiaries and Affiliates) shall have executed and delivered the applicable Transaction Documents.

Section 7.3. Conditions to the Obligations of the Company. The obligation of the Company to consummate the transactions contemplated hereby shall be subject to the satisfaction at or prior to the Closing of each of the following conditions; provided, however, that Company may, in its sole and absolute discretion, waive any or all of the following conditions:

(a)           Plan Investor Representations and Warranties; Plan Investor Covenants. (i) (A) The Plan Investor Fundamental Representations shall be true and correct in all material respects (without giving effect to any limitation as to “materiality”, “Plan Investor Material Adverse Effect” or other similar qualifications) both as of the date of this Agreement and as of the Closing Date as if made and as of such date (except to the extent expressly made as of an earlier date, in which case, as of such earlier date) and (B) all other representations and warranties of the Plan Investor set forth in this Agreement shall be true and correct in all respects (without giving effect to any limitation as to “materiality”, “Plan Investor Material Adverse Effect” or other similar qualifications) as of the date of this Agreement and the Closing Date (except to the extent expressly made as of an earlier date, in which case, as of such earlier date), except where the failure of such representations and warranties to be true and correct would not,

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individually or in the aggregate, reasonably be expected to result in a Plan Investor Material Adverse Effect and except where the failure of such representations and warranties to be true and correct arises solely out of the implementation of the Scheme, (ii) the Plan Investor has performed, or caused to be performed, in all material respects the covenants in this Agreement that are required to be complied with by the Plan Investor Group between the date of this Agreement and the Closing Date, and (iii) the Company shall have received a certificate signed by an authorized officer of the Plan Investor, in a form reasonably acceptable to the Company, certifying that this conditions in the foregoing clauses (i) and (ii) have been satisfied at Closing.

(b)           Transaction Documents. Each of the parties to the Transaction Documents (other than the Company and its subsidiaries and Affiliates) shall have executed and delivered the applicable Transaction Documents.

Section 7.4.           Frustration of Closing Conditions.

(a)         The Plan Investor may not rely on the failure of any condition set forth in Section 7.1 and Section 7.2 to be satisfied, if such failure was directly the result of the failure of any Plan Investor Group Member to perform and comply in all material respects with the covenants and agreements in this Agreement to be performed or complied with by Plan Investor Group Member prior to the Closing.

(b)         The Company may not rely on the failure of any condition set forth in Section 7.1 and Section 7.3 to be satisfied, if such failure was directly the result of the failure any Company Group Member to perform and comply in all material respects with the covenants and agreements in this Agreement to be performed or complied with by such Company Group Member prior to the Closing.

ARTICLE VIII.

TERMINATION

Section 8.1.         Termination. This Agreement may be terminated and the transactions contemplated hereby may be abandoned at any time prior to the Effective Date, notwithstanding the fact that any requisite authorization and approval of the transactions contemplated hereby (including the Plan Investor Stockholder Approval) shall have been received:

(a) by the mutual written consent of the Plan Investor and the Company;

(b) by either the Plan Investor or the Company:

(i) if there shall be any Law that makes consummation of the transactions contemplated hereby (including the Acquisition) illegal or otherwise prohibited, or if any Governmental Entity shall have issued a final and non-appealable Restraining Order which permanently prohibits, restrains or makes illegal the transactions contemplated this Agreement; provided, however, that the right to terminate this Agreement pursuant to this Section 8.1(b)(i) shall not be available to any Party whose material uncured breach of this Agreement or any of the Transaction Documents is the principal cause of the enactment or issuance of any such Law or Restraining Order;

(ii) if the Closing has not occurred by the Outside Date; provided, however, that (A) in the event that, as of the initial Outside Date, all conditions to Closing set forth in Article VII have been satisfied or waived (other than

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(x) such conditions that by their terms are satisfied at the Closing, which shall be reasonably capable of being satisfied as of such date and (y) the receipt of any required regulatory or other approval of a Governmental Entity (other than entry of applicable orders by the Bankruptcy Court) or shareholder approval of a Plan Investor as necessary for the occurrence of the Plan effective date), the Outside Date shall be deemed automatically extended a single time for sixty (60) days (for the avoidance of doubt, the Outside Date shall only be extended one time pursuant to this clause (A)); (B) in the event that, as of the initial Outside Date, all conditions to Closing set forth in Article VII have not been satisfied or waived (other than such conditions that by their terms are satisfied at the Closing, which shall be reasonably capable of being satisfied as of such date), the Plan Investor may elect to extend the Outside Date for a single time for thirty (30) days upon delivery of written notice thereof to the Company at least three (3) days prior to the initial Outside Date (for the avoidance of doubt, the Outside Date may only be extended one time pursuant to the foregoing provisions of this clause (B)) or (C) in the event that, as of the initial Outside Date, all conditions to Closing set forth in Article VII have not been satisfied or waived (other than such conditions that by their terms are satisfied at the Closing, which shall be reasonably capable of being satisfied as of such date), the Company may elect (subject to the prior written approval of each of Highbridge and Athyrium) to extend the Outside Date for a single time for sixty (60) days upon delivery of written notice thereof to the Plan Investor at least three (3) days prior to the initial Outside Date (for the avoidance of doubt, the Outside Date may only be extended one time pursuant to the foregoing provisions of this clause (C)); provided, however, for the avoidance of doubt, the Outside Date may not be extended to exceed sixty (60) days past the initial Outside Date; provided, further, that the right to terminate this Agreement pursuant to this Section 8.1(b)(ii) shall not be available to a Party whose material uncured breach of this Agreement or any of the Transaction Documents is the principal cause of the transactions contemplated by this Agreement to not be consummated by the Outside Date;

(iii) if the Company Board authorizes the Company to enter into a Company Alternative Transaction Agreement which constitutes a Company Superior Proposal pursuant to and in accordance with Section 6.9(f), provided, the Company may only terminate this Agreement pursuant to this Section 8.1(b)(iii) if the Company enters into such Company Alternative Transaction Agreement immediately following or concurrently with such termination;

(iv) if the Plan Investor Board approves, recommends, enters into or declares advisable, or proposes publicly to approve, recommend or declare advisable, a Plan Investor Alternative Transaction; or

(v) if the Plan Investor Stockholder Approval is not received upon a vote taken thereon at a meeting of the stockholders of the Plan Investor (including any adjournments or postponements thereof), provided that the Plan Investor may not terminate this Agreement pursuant to this

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Section 8.1(b)(v) if the Plan Investor is in breach of Section 6.11 of this Agreement.

(c) by the Plan Investor:

(i) if the Company is in breach of any representation or warranty or failure to perform any covenant or agreement such that the conditions to the obligations of the Plan Investor set forth in Section 7.2(a) could not be satisfied at or prior to the Closing, and such failure cannot be or has not been cured by the earlier of the Outside Date or thirty (30) days after the giving of written notice by the Plan Investor to the Company;

(ii) if (A) the Company breaches, in any material respect, any covenant or agreement set forth in Section 6.9 or (B) the Company delivers to the Plan Investor a Company Notice of Intended Recommendation Change or

(iii) upon termination of the Restructuring Support Agreement for any reason unless the termination of the Restructuring Support Agreement is principally due to a material uncured breach of this Agreement or the Restructuring Support Agreement by the Plan Investor.

(d) by the Company:

(i) if the Plan Investor is in breach of any representation or warranty or failure to perform any covenant or agreement such that the conditions to the obligations of the Company set forth in Section 7.3(a) could not be satisfied at or prior to the Closing, and such failure cannot be or has not been cured by the earlier of the Outside Date or thirty (30) days after the giving of written notice by the Company to the Plan Investor; or

(ii) upon termination of the Restructuring Support Agreement for any reason unless the termination of the Restructuring Support Agreement is principally due to a material uncured breach of this Agreement by the Company or the Restructuring Support Agreement by the Company or the Consenting Lenders (as defined in the Restructuring Support Agreement).

Section 8.2.           Effect of Termination.

(a)         If this Agreement is validly terminated in accordance with Section 8.1, this Agreement shall thereafter become void and have no effect, and neither Party shall have any liability to the other Party, its Subsidiaries, or its Affiliates or any of their respective Representatives in connection with this Agreement, except that (i) the obligations of the Parties contained in the Confidentiality Agreement, the provisions of Section 8.3, this Section 8.2 and Article IX shall survive such termination, and (ii) subjection to Section 8.3, such termination shall not relieve either Party from Liability for any fraud or Willful Breach of this Agreement prior to such termination.

Section 8.3.           Fees and Expenses.

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(a)           Certain Plan Investor Termination Fee and Expense Reimbursement Obligations. If this Agreement is terminated (i) in accordance with Section 8.1(b)(ii) and, at the time of such termination, any of the conditions set forth in Section 7.1(e), Section 7.1(l) or Section 7.1(m) have not been satisfied (solely with respect to Section 7.1(l) or Section 7.1(m), if the Parties have elected to pursue the Scheme), or (ii) in accordance with Section 8.1(b)(iv), Section 8.1(b)(v), Section 8.1(d)(i) or Section 8.1(d)(ii) (in the event that the termination of the Restructuring Support Agreement arose from the uncured material breach thereof by the Plan Investor), then the Plan Investor shall pay to the Company (or its designee or successor) an amount equal to the Plan Investor Termination Fee no later than two (2) Business Days after the date of such a termination of this Agreement (in the case of any such termination by the Company) or at or prior to, and as a condition precedent to, such a termination of this Agreement (in the case of any such termination by the Plan Investor); provided, however, that if at the time of any such termination the Plan Investor is in an offer period (as defined under the Takeover Code), then the Plan Investor shall have no obligation to pay the Plan Investor Termination Fee to the Company unless and until such offer period concludes (in accordance with the Takeover Code) without a Plan Investor Alternative Transaction becoming or being declared unconditional or becoming effective, in which case the Plan Investor Termination Fee shall be payable no later than two (2) Business Days after the conclusion of the offer period.

(b)           Certain Company Termination Fee and Expense Reimbursement Obligations. If this Agreement is terminated (i) in accordance with Section 8.1(b)(ii) (other than in those circumstances as contemplated by Section 8.3(a)), Section 8.1(c)(i), Section 8.1(c)(ii)(A) or Section 8.1(c)(iii) (in the event that the termination of the Restructuring Support Agreement arose from the uncured material breach thereof by the Company or any of the Consenting Lenders), then the Company shall pay to the Plan Investor (or its designee or successor) an amount equal to the Company Expense Reimbursement Amount no later than two (2) Business Days after the date of such a termination of this Agreement (in the case of any such termination by the Plan Investor) or at or prior to, and as a condition precedent to, such a termination of this Agreement (in the case of any such termination by the Company); provided, however, that if (i) prior to the date of such a termination of this Agreement, a bona fide Company Alternative Proposal shall have been publicly disclosed or announced and shall not have been publicly withdrawn prior to the date of such termination; and (ii) within the first (1st) year following such termination of this Agreement: (1) a Company Alternative Transaction is consummated; or (2) a Company Alternative Transaction Agreement is executed, the Company shall pay to the Plan Investor the Company Termination Fee, with such payment to be payable on the date on which the Company Alternative Transaction is consummated.

(c)           Termination Regarding Company Alternative Transactions. If this Agreement is terminated pursuant to Section 8.1(b)(iii), (i) the Company shall pay to the Plan Investor (or its designee or successor) an amount equal to the Company Expense Reimbursement Amount at or prior to, and as a condition precedent to, such a termination of this Agreement, and (ii) the Company shall pay to the Plan Investor (or its designee or successor) an amount equal to the Company Termination Fee upon the consummation of the Company Alternative Transaction contemplated by the Company Alternative Transaction Agreement that was entered into in connection with the termination of this Agreement (or, if earlier, upon consummation of any other Company Alternative Transaction that is consummated within one (1) year of the termination of this Agreement).

(d)           Payment. Any payment of the Company Termination Fee, the Plan Investor Termination Fee or the Company Expense Reimbursement Amount shall be made by wire transfer of immediately available funds to an account designated in writing by the Plan Investor or the Company, as applicable, if and when so provided.

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(e)           Effect of Payments. The Parties agree and understand that (x) in no event shall the Company be required to pay the Company Termination Fee on more than one occasion, and in no event shall the Plan Investor be required to pay the Plan Investor Termination Fee on more than one occasion, in each case, under any circumstances, and (y) except in the case of fraud or Willful Breach of any covenant or agreement set forth in this Agreement by the other Party, (1) in no event shall the Plan Investor be entitled to receive an amount greater than the Company Termination Fee and the Company Expense Reimbursement Amount, and (2) in no event shall the Company be entitled to receive an amount greater than the Plan Investor Termination Fee. Notwithstanding anything to the contrary in this Agreement, except in the case of fraud or Willful Breach of any covenant or agreement set forth in this Agreement by the other party, (i) if the Plan Investor receives the Company Termination Fee and any applicable Company Expense Reimbursement Amount from the Company, or if the Company receives the Plan Investor Termination Fee from the Plan Investor, such payment shall be the sole and exclusive remedy of the receiving Party against the paying Party and its Subsidiaries and their respective former, current or future partners, equityholders, managers, members, Affiliates and Representatives, (ii) if the Company receives any payments from the Plan Investor in respect of any breach of this Agreement and thereafter the Company receives the Plan Investor Termination Fee, the amount of such Plan Investor Termination Fee shall be reduced by the aggregate amount of such payments made by the Plan Investor in respect of any such breaches, and (iii) if the Plan Investor receives any payments from the Company in respect of any breach of this Agreement (other than the Company Expense Reimbursement Amount) and thereafter the Plan Investor receives the Company Termination Fee, the amount of such Company Termination Fee shall be reduced by the aggregate amount of such payments made by the Company in respect of any such breaches (other than the Company Expense Reimbursement Amount).

(f)            Company Expense Reimbursement Amount. In all circumstances when the Company is required to pay to the Plan Investor the Company Expense Reimbursement Amount hereunder, the Company shall pay to the Plan Investor an amount equal to the amount of all reasonable and documented fees and expenses incurred by the Plan Investor in connection with the negotiation, preparation and implementation of the Transaction Documents (such reimbursement obligation not to exceed $4,000,000 in the aggregate) (the “Company Expense Reimbursement Amount”).

(g)           Integral Part of Agreement. The Parties acknowledge that the agreements contained in this Section 8.3 are an integral part of the transactions contemplated hereby, that, without these agreements, the Parties would not enter into this Agreement and that any amounts payable pursuant to this Section 8.3 do not constitute a penalty. Accordingly, if any Party fails to promptly pay any amount due pursuant to this Section 8.3, such Party shall also pay any out-of-pocket costs and expenses (including reasonable legal fees and expenses) incurred by the Party entitled to such payment in connection with a legal action to enforce this Agreement that results in a judgment for such amount against the Party failing to promptly pay such amount. Any amount not paid when due pursuant to this Section 8.3 shall bear interest from the date such amount is due until the date paid at a rate equal to the prime rate as published in The Wall Street Journal, Eastern Edition in effect on the date of such payment.

(h)           Agreement Subject to PFA Order. The obligations of the Company to make any of the payments contemplated by this Section 8.3 shall become effective upon the date on which the Bankruptcy Court enters the PFA Order and shall have no effect on the Company until such PFA Order has been entered.

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ARTICLE IX.

BANKRUPTCY COURT MATTERS

Section 9.1.           PFA Order. On the Petition Date or as soon as reasonably practicable thereafter, the Company shall file with the Bankruptcy Court a motion seeking entry of the PFA Order, and the Company shall use reasonable best efforts to obtain the entry of the PFA Order on the time contemplated by the Restructuring Support Agreement. The Plan Investor agrees that it will promptly take such actions as are reasonably requested by the Company to assist in obtaining entry of the PFA Order.

ARTICLE X.

MISCELLANEOUS

Section 10.1.        Governing Law. This Agreement (and all Proceedings arising out of or related to this Agreement, whether based upon contract, tort or otherwise) shall be governed by, and construed in accordance with, the procedural and substantive Laws of the State of New York (including the Laws relating to the statutes of limitation) without giving effect to conflicts of Law principles thereof that would compel the application of the Laws of another jurisdiction, other than sections 5-1401 and 5-1402 of the New York General Obligations Law. Notwithstanding the foregoing, to the extent that the fiduciary duties of any director or officer of the Plan Investor and its Subsidiaries are governed by the Laws of England and Wales, then the Laws of England and Wales shall govern with respect to such duties.

Section 10.2.        Jurisdiction; Forum; Service of Process; Waiver of Jury Trial. With respect to any Proceeding arising out of or relating to this Agreement, each Party hereby irrevocably:

(a)        submits to the exclusive jurisdiction of the Bankruptcy Court, or in the event that the Bankruptcy Cases are no longer pending or the Bankruptcy Court does not have jurisdiction over the matters in question, the state and federal courts sitting in the Southern District of New York (any such court, the “Selected Court”), for any Proceeding arising out of or relating to this Agreement and the transactions contemplated hereby (and agrees not to commence any Proceeding relating hereto except in such court) and waives any objection to venue being laid in the Selected Court whether based on the grounds of forum non conveniens or otherwise; provided that each of the Company and the Plan Investor hereby irrevocably submits to the exclusive jurisdiction of the Bankruptcy Court for so long as the Bankruptcy Cases are pending;

(b)         consents to service of process in any Proceeding in accordance with Section 10.6 (other than email); and

(c)        WAIVES, TO THE FULLEST EXTENT PERMITTED BY LAW, ANY RIGHT IT MAY HAVE TO A TRIAL BY JURY IN ANY PROCEEDING DIRECTLY OR INDIRECTLY ARISING OUT OF, UNDER OR IN CONNECTION WITH THIS AGREEMENT.

Section 10.3.        Successors and Assigns. Except as otherwise provided herein, the provisions hereof shall inure to the benefit of, and be binding upon, the successors by operation of Law and permitted assigns of the Parties. No assignment of this Agreement may be made by a Party at any time, whether or not by operation of Law, without the other Party’s prior written consent.

Section 10.4.         Entire Agreement; Amendment. This Agreement (including the Exhibits and Schedules attached hereto), any confidentiality agreement between the Parties or their respective Affiliates (including the Confidentiality Agreement) and the other Transaction Documents constitute the

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full and entire understanding and agreement between the Parties with regard to the subject-matter hereof and supersede all prior agreements relating to the subject matter hereof (other than the Confidentiality Agreement); provided, however, that the Confidentiality Agreement shall terminate in accordance with the terms thereof. Except as expressly provided herein, neither this Agreement nor any term hereof may be amended, waived, discharged or terminated other than by a written instrument signed by each of the Parties. If any ruling is made by the Panel that any provision of this Agreement is not permitted by the Takeover Code, such provision shall be given no effect. The Parties shall negotiate in good faith to replace such provision with a valid and enforceable provision which is acceptable to the Panel and carries out, as closely as possible, the intentions of the Parties.

Section 10.5.         Disclosure Schedule References; Data Room Disclosures. The Parties agree that the disclosure set forth in any particular section or subsection of the Company Disclosure Schedule or the Plan Investor Disclosure Schedule provided in connection with this Agreement and/or the Restructuring Support Agreement shall be deemed to be an exception to (or, as applicable, a disclosure for purposes of) (a) the representations and warranties (or covenants, as applicable) of the disclosing Party that are set forth in the corresponding Section or subsection of this Agreement or the Restructuring Support Agreement; and (b) any other representations and warranties (or covenants, as applicable) of the disclosing party that are set forth in this Agreement or the Restructuring Support Agreement, but in the case of this clause (b) only if the relevance of that disclosure as an exception to (or a disclosure for purposes of) such other representations and warranties (or covenants, as applicable) is reasonably apparent in the face of such disclosure. In addition, solely for purposes of the representations and warranties set forth in this Agreement or the Restructuring Support Agreement, any information or documentation that is (i) disclosed in any Electronic Data Room at 11:59 p.m., New York time, on the date that is two (2) days prior to the date of this Agreement (excluding, for computational purposes the date of this Agreement), and (ii) Fairly Disclosed, shall be deemed to have been disclosed on the Company Disclosure Schedule or Plan Investor Disclosure Schedule, as applicable, for purposes of this Agreement or the Restructuring Support Agreement. Concurrently with the execution of this Agreement, for evidentiary purposes, the Parties have provided each other with a USB drive containing all of the information and documents contained in each Electronic Data Room as of 11:59 p.m., New York time, on the date that is two (2) days prior to the date of this Agreement (excluding, for computational purposes the date of this Agreement).

Section 10.6.         Notices. All notices, requests, consents and other communications hereunder to any Party shall be deemed to be sufficient if contained in a written instrument delivered in person or sent by email or nationally recognized overnight courier, addressed to such Party at the applicable address set forth below or such other address as may hereafter be designated in writing by such Party to the other Party from time to time:

(a) if to the Company:

c/o Aegerion Pharmaceuticals, Inc.

245 First Street

Riverview II, 18^th Floor

Cambridge, MA 02142

Attention: John R. Castellano

Email: JCastellano@alixpartners.com

with a copy to:

Willkie Farr & Gallagher LLP

787 Seventh Avenue

75

New York, NY 10019

Attention: Russell L. Leaf, Esq.; Jared Fertman, Esq.; Paul V. Shalhoub, Esq.; and Andrew S. Mordkoff, Esq.

Email: rleaf@willkie.com; jfertman@willkie.com;

pshalhoub@willkie.com; amordkoff@willkie.com

(b) if to the Plan Investor, to:

Amryt Pharma plc

90 Harcourt Street

Dublin 2, Ireland

Attention: Joe Wiley

Email: joe.wiley@amrytpharma.com

with a copy to:

Gibson, Dunn & Crutcher LLP

200 Park Avenue 

New York, NY 10166

Attention: George P. Stamas, Esq.; William B. Sorabella, Esq.; Robert Klyman, Esq.; and Matthew J. Williams, Esq.

Email: GStamas@gibsondunn.com; WSorabella@gibsondunn.com; RKlyman@gibsondunn.com; MJWilliams@gibsondunn.com

All such notices, requests, consents and other communications shall be deemed to have been given or made on the date so given or made, if and when delivered personally or by overnight courier to the applicable Party at the above addresses or sent by electronic transmission, with delivery confirmed (which may be electronic), or to the email addresses specified above (or at such other address for a Party as shall be specified by like notice).

Section 10.7.         Delays or Omissions. No delay or omission to exercise any right, power or remedy accruing to a Party upon any breach or default by the other Party under this Agreement shall impair any such right, power or remedy of the non-breaching or the non-defaulting Party nor shall it be construed to be a waiver of any such breach or default, or an acquiescence therein, or of or in any similar breach or default thereafter occurring; nor shall any waiver of any single breach or default be deemed a waiver of any other breach or default theretofore or thereafter occurring. Any waiver, consent or approval of any kind or character on the part of a Party of any breach or default under this Agreement by the other Party, or any waiver on the part of any such Party of any provisions or conditions of this Agreement, must be in writing and shall be effective only to the extent specifically set forth in such writing. All remedies, either under this Agreement or by Law or otherwise afforded to a Party, shall be cumulative and not alternative.

Section 10.8.         Counterparts. This Agreement may be executed in one or more counterparts, each of which shall be deemed an original and all of which shall constitute one and the same agreement. Delivery of an executed signature page of this Agreement by facsimile or portable document format shall be effective as delivery of a manually executed signature page of this Agreement.

Section 10.9.        Severability. In the event that any provision of this Agreement becomes or is declared by a final and non-appealable judgment of a court of competent jurisdiction to be illegal, unenforceable or void, this Agreement shall continue in full force and effect without said provision(s); provided that no such severability shall be effective if it materially changes the economic benefit of this Agreement to any Party.

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Section 10.10.       Headings. The table of contents and headings used in this Agreement are used for convenience only, do not constitute a part of this Agreement and are not to be considered in construing or interpreting this Agreement.

Section 10.11.        No Third-Party Beneficiaries. Nothing in this Agreement is intended to, or shall, confer any third-party beneficiary or other rights or remedies upon any Person other than the Parties.

Section 10.12.       No Survival. The representations and warranties of the Parties in this Agreement, other than as set forth in Section 4.22 and Section 5.22, shall not survive the Closing. The covenants or agreements of the Parties shall only survive the Closing if and as explicitly set forth in the applicable provision of this Agreement requiring a Party to perform such covenant(s) following the Closing.

Section 10.13.        Fees and Expenses. Except as otherwise expressly set forth in this Agreement, each Party shall bear all costs and fees and expenses that it incurs, or that may be incurred on its behalf, in connection with this Agreement and the transactions contemplated by any of the Transaction Documents.

Section 10.14.       No Public Announcement. The Parties agree that any press release or public announcement to be issued with respect to the transactions contemplated by the Transaction Documents shall be made in accordance with the provisions of Section 6.6 of the Restructuring Support Agreement.

Section 10.15.        Specific Performance. This Agreement shall be enforceable by a decree of specific performance issued by any court of competent jurisdiction, and appropriate injunctive relief may be applied for and granted in connection therewith without the posting of a bond or other security. Such remedies shall, however, be cumulative and not exclusive and shall be in addition to any other remedies that a Party may have under this Agreement or otherwise. The Parties agree to waive any defense in any action for specific performance to the effect that a remedy at law would be adequate.

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Section 10.16.        Construction. The Parties have participated jointly in the negotiation and drafting of this Agreement. If an ambiguity or question of intent or interpretation arises, then this Agreement will be construed as drafted jointly by the Parties and no presumption or burden of proof will arise favoring or disfavoring any Party by virtue of the authorship of any of the provisions of this Agreement. Unless otherwise indicated to the contrary herein by the context or use thereof: (a) any reference to any Law will be deemed also to refer to all rules and regulations promulgated thereunder and all amendments thereto; (b) all references to the preamble, recitals, Sections, Articles, Exhibits or Schedules are to the preamble, recitals, Sections, Articles, Exhibits or Schedules of or to this Agreement; (c) the words “herein,” “hereto,” “hereof” and words of similar import refer to this Agreement as a whole and not to any particular Section or paragraph hereof; (d) masculine gender shall also include the feminine and neutral genders and vice versa; (e) words importing the singular shall also include the plural and vice versa; (f) the words “include,” “including” and “includes” shall mean without limitation by reason of enumeration; (g) all references to “$” or dollar amounts are to lawful currency of the United States of America; (h) all reference to a “day” or a number of “days” (without explicit reference to “Business Days”) shall be interpreted as a reference to a calendar day or number of calendar days; and (i) the word “will” shall be construed to have the same meaning as the word “shall”, (j) the phrase “ordinary course” or “ordinary course of business” shall mean “ordinary course of business and consistent with past practices,” (k) subject to Section 10.5, the phrase “provided,” “delivered” or “made available” when used in reference to a document shall include all documents and materials that are made available by the applicable Party to the other Party via an Electronic Data Room as of 11:59 p.m., New York time, no later than the date that is two (2) days prior to the date of this Agreement (excluding, for computational purposes, the date of this Agreement), and (l) unless the context provides otherwise, the word “or” shall not be exclusive and shall mean “and/or”. If any action is to be taken or given on or by a particular calendar day, and such calendar day is not a Business Day, then such action shall automatically be deferred until the next Business Day.

[SIGNATURE PAGES TO FOLLOW]

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IN WITNESS WHEREOF, each of the undersigned has caused the foregoing Agreement to be executed as of the date first above written.

AEGERION PHARMACEUTICALS, INC.
By:	/s/ John R. Castellano
	Name:  John R. Castellano
	Title: Chief Restructuring Officer

AMRYT PHARMA PLC
By:
	Name:
	Title:

[Signature Page to Plan Funding Agreement]

IN WITNESS WHEREOF, each of the undersigned has caused the foregoing Agreement to be executed as of the date first above written.

AEGERION PHARMACEUTICALS, INC.
By:	/s/ John R. Castellano
	Name:  John R. Castellano
	Title: Chief Restructuring Officer

AMRYT PHARMA PLC
By:	/s/ Joe Wiley
	Name:  Joe Wiley
	Title: CEO

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Exhibit A

Form of Voting Agreement

80

Exhibit B

Form of Shared Services Agreement

81

Exhibit C

Form of CVR Instrument

82

Exhibit D

Form of Loan Notes Deed Poll

83

Exhibit E

Form of Registration Rights Agreement

84

EX-2.2

Exhibit 2.2

Execution Version

SHARE PURCHASE AND TRANSFER AGREEMENT

relating to shares (Aktien)
in
BIRKEN AG

between

Software AG-Stiftung, with its offices at Am Eichwäldchen 6, 64297 Darmstadt, Germany

(“Seller 1”),

and

Dr. Armin Scheffler, Bussardweg 15/1, 75223 Niefern-Öschelbronn, Germany
(“Seller 2”),

Seller 1 and Seller 2 are collectively referred to as the “Sellers”.

and

Amryt Pharmaceuticals Designated Activity Company, registered with company number 566448, with its
offices at 106B Pembroke Road, Dublin 4, Ireland

(“Buyer”),

and

(with regard to clauses 4.6.4 to 4.6.6, clause 7, and clauses 16 through 18)

Birken AG, registered with the commercial register of the Local Court of Mannheim under HRB 711487,

with its offices at Streiflingsweg 11, 75223 Niefern-Öschelbronn, Germany

(“Company”),

Sellers, Buyer and Company are collectively referred to as the “Parties” and each a “Party”.

 Table of Contents

Preamble		1
1.	Sale and Transfer	1
2.	Effective Dates	2
3.	Consideration	2
4.	Closing	3
5.	Independent Guarantee Undertakings of the Sellers	6
6.	Rules of Conduct	10
7.	Cooperation Obligations	12
8.	Legal Consequences in Case of Breach of Warranties; Violations of Rules of Conduct; Limitation of Liability	13
9.	Independent Guarantee Undertakings of the Buyer	14
10.	Pre-Closing Obligation of Buyer	16
11.	Post-Closing Obligations of Buyer	17
12.	Protective Covenants and Post-Closing Cooperation	17
13.	Confidentiality, Non-Compete	18
14.	Costs	19
15.	Liability of Sellers	19
16.	Final Provisions	19
17.	Severability	21
18.	Copies of this Agreement	21

List of Annexes:

Annex	Content
Annex 3.1	Consideration schedule
Annex 3.1.5	Reverse takeover and allotment of shares in Fastnet
Annex 4.2	Closing Conditions
Annex 4.6.3	Side letter by shareholders Buyer relating to Fastnet Shares
Annex 4.6.4	Agreement on the termination and dissolution of the silent partnership
Annex 4.6.5	Agreement on the termination of the royalty agreement between Seller 2 and the Company
Annex 4.6.6	Agreement providing for an option to extend the term of the two lease agreements regarding the production site in [***]
Annex 4.6.7	Resolution by the Sellers as the sole shareholders of the Company consenting to the sale and transfer of the Shares
Annex 4.6.9	Resolution by the advisory board of Seller 1 approving the transactions contemplated by this Agreement
Annex 4.6.10	Shareholders resolution with respect to appointment of members of the supervisory board
Annex 5.1.3	DVD (containing data room and Q&A responses)
Annex 5.2.6	Company’s articles of association dated April 2, 2015
Annex 5.2.7	Excerpt from the commercial register
Annex 5.3.2	Sale of Viscum TT
Annex 5.4.3	distribution agreement South Korea
Annex 5.5.1	Registered Intellectual Property
Annex 5.5.7	Publication relating to the use of Betulin
Annex 5.6.1	Employees
Annex 9.1.6	Buyer’s Constitution

Index of Definitions

Additional Cash Amount	shall have the meaning as defined in clause 3.1.2
Applications	means applications for Registered Intellectual property which is used by the Company
Agreement	shall have the meaning as defined in Preamble
Breach	shall have the meaning as defined in clause 8.1
Business Day	shall have the meaning as defined in clause 16.14
Business Intellectual Property	means the Intellectual Property which is owned or licensed by the Company
Buyer	shall have the meaning as defined in Definition of Parties
Buyer’s Constitution	shall have the meaning as defined in clause 9.1.6
Buyer Shareholders	shall have the meaning as defined in clause 6.3.1
Closing	shall have the meaning as defined in clause 4.1
Closing Conditions	shall have the meaning as defined in clause 4.2
Closing Date	shall have the meaning as defined in clause 2.2
Closing Minutes	shall have the meaning as defined in clause 4.9
Company	shall have the meaning as defined in Definition of Parties
Contribution	shall have the meaning as defined in clause 4.6.1
Counsel	Shall have the meaning as defined in clause 6.3.3
Disclosure	shall have the meaning as defined in clause 8.5
Disclosure Exhibits	shall have the meaning as defined in clause 5.1.3(b)
Draft Amryt Agreement	shall have the meaning as defined in clause 6.3.1
Due Diligence Information	shall have the meaning as defined in clause 5.1.3(a)
Equity Participation	shall have the meaning as defined in clause 3.1.5
Fastnet	shall have the meaning as defined in Preamble (F)
Intellectual Property	means all intellectual property rights, including (without limitation) rights in patents, supplementary protection certificates, petty patents, utility models, trademarks, database rights in designs, copyrights and topography rights (whether or not any of these rights

	are registered, and including applications and the right to apply for registration of any such rights) and all inventions, know-how, trade secrets, techniques and confidential information, and other proprietary knowledge and information, and all rights and forms of protection of a similar nature or having equivalent or similar effect to any of these which may subsist anywhere in the world, in each case for their full term, and together with any renewals or extensions
Licence	means any licence, permission or consent in respect of the use of any Intellectual Property (including, without limitation, any unwritten and/or informal licensing arrangement) and any arrangement of which any licence, permission or consent forms part
Long Stop Date	shall have the meaning as defined in clause 4.5
Milestone Payments	shall have the meaning as defined in clause 3.1.3
Parties	shall have the meaning as defined in Definition of Parties
Party	shall have the meaning as defined in Definition of Parties
Prepayment Amount	shall have the meaning as defined in clause 3.1.1
Purchase Price	shall have the meaning as defined in clause 3.1
Registered Intellectual Property	means patents, certificates of addition, supplementary certificates of addition, supplementary protection certificates, petty patents, utility models, registered or filed for
Roadshow	shall have the meaning as defined in clause 6.5
Royalty Payments	shall have the meaning as defined in clause 3.1.4
RTO	shall have the meaning as defined in Recital F
Sole Permitted Variations	shall have the meaning as defined in clause 6.3.5
Sompharma Transaction	shall have the meaning as defined in clause 3.1.5
Scheduled Closing Date	shall have the meaning as defined in clause 4.1
Seller 1	shall have the meaning as defined in Definition of Parties
Seller 1 Shares	shall have the meaning as defined in Preamble (B)
Seller 2	shall have the meaning as defined in Definition of Parties
Seller 2 Shares	shall have the meaning as defined in Preamble (B)
Sellers	shall have the meaning as defined in Definition of Parties
Sellers Knowledge	shall have the meaning as defined in clause 5.9

Shares	shall have the meaning as defined in Preamble (B)
Signing Date	shall have the meaning as defined in clause 2.1
Warranties	shall have the meaning as defined in clause 5.1.1
Warranty	shall have the meaning as defined in clause 5.1.1

Preamble

(A) This Agreement sets out the terms on which Buyer acquires all non-par value ordinary bearer shares (“Shares”) in Birken AG (“Company”) from the Sellers.

(B) The Company is registered with the commercial register of the local Court of Mannheim under HRB 711487, having its registered office at Streiflingsweg 11, 75223 Niefern-Öschelbronn, Germany. The Company is a German law stock corporation (Aktiengesellschaft) with a stated share capital (Grundkapital) of [***]. The Company’s stated share capital Is divided into [***] non-par value ordinary bearer shares (nennbetragslose Namensaktien) (the “Shares”) which are held as follows:

● [***] non-par value ordinary bearer shares are held by Seller 1 (the “Seller 1 Shares”), and

● [***] non-par value ordinary bearer shares are held by Seller 2 (the “Seller 2 Shares”).

(C) The Company has not issued any share certificates (Aktienurkunden). The Company does not have any other shareholders.

(D) Seller 1 and Buyer had agreed on a Heads of Terms in relation to this Agreement as of June 19, 2015.

(E) The Sellers intend to sell to the Buyer all their Shares in the Company, and the Buyer intends to acquire all such Shares, all subject to the terms and conditions of this Agreement.

(F) Buyer intends, after having entered into this agreement but before the Closing Date, to enter into an agreement with Fastnet Equity PLC, a company registered in England and Wales with registration number 5316808 with its offices at Ivybridge House, 1 Adam Street London WC2N 6LE (“Fastnet”) for the sale of the entire issued (and, in the case of the shares to be issued to the Seller 1 hereunder, to be issued) share capital of the Buyer in exchange for the issue by Fastnet of Fastnet’s shares. As Fastnet’s shares are traded on the AIM market of the London Stock Exchange, the sale of the Buyer to Fastnet will be a reverse takeover (“RTO”) for the purposes of the AIM Rules and will require the approval of Fastnet’s shareholders in a general meeting. Closing hereunder shall take place once each of the other conditions to the RTO shall have been satisfied. In connection with the RTO Fastnet will undertake a fundraising with the intention of raising approximately EUR [***] and with Net Proceeds (as this term is defined In Annex 3.1.5) of at least [***].

NOW THEREFORE, in consideration of the above and subject to the terms and conditions set forth herein, the Parties hereto enter into the following sale and purchase agreement (the “Agreement”):

1. Sale and Transfer

1.1 Sellers hereby individually sell their respective Shares, free and clear of all liens, charges, security interests, pre-emption rights, encumbrances and other limitations of any nature whatsoever, to the Buyer with economic effect as of the Closing Date. The Shares are sold to the Buyer including any and all rights and duties attaching to them, in particular the profit participation rights regarding the undistributed profits of the current and previous fiscal years. The Buyer hereby accepts the sale of the Shares.

1.2 The Buyer shall not be obliged to complete the purchase of any of the Shares unless the sale and purchase of all of the Shares occurs simultaneously.

1.3 Subject to the condition precedent (aufschiebende Bedingung) within the meaning of § 158 para. 1 BGB of the occurrence of Closing pursuant to clause 4.6

1.3.1 Seller 1 assigns to the Buyer the Seller 1 Shares,

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1.3.2 Seller 2 assigns to the Buyer the Seller 2 Shares, and

1.3.3 Seller 1 assigns to the Buyer its then already terminated participation in the silent partnership (Stille Gesellschaft) between Seller 1 and the Company dated 12 March 2015, the termination becoming effective December 31, 2016

in each case including all membership rights (Mitgliedschaftsrechte) attached to the respective Shares. The Buyer hereby accepts each such assignment.

2. Effective Dates

2.1 The signing date is the date on which this Agreement is executed (the “Signing Date”).

2.2 The closing date is the date on which Closing pursuant to clause 4.6 actually occurs (the “Closing Date”).

3. Consideration

3.1 The consideration for the purchase of the Shares (the “Purchase Price”) shall comprise the following elements, further provisions relating to which are set out in Annex 3.1 to this Agreement:

3.1.1 The sum of EUR 1,000,000 (“Prepayment Amount”) payable to the Sellers within [***] following the Signing Date;

3.1.2 The sum of EUR 150,000 (“Additional Cash Amount”) payableto the Sellers on the Scheduled Closing Date;

3.1.3 Milestone payments as further set out in clause 1.3 of Annex 3.1 (“Milestone Payments”);

3.1.4 Royalty payments as further set out in clause 2 of Annex 3.1 (“Royalty Payments”);

3.1.5 The allotment to the Sellers of the Consideration Shares and any Additional Consideration Shares (as defined in Annex 3.1.5) which shall, following completion of the Amryt Share Exchange Agreement (as defined in Annex 3.1.5) be exchanged for the requisite number of Fastnet Consideration Shares (as defined in Annex 4.2) as further outlined in Annex 3.1.5 (“Equity Participation”). The Consideration Shares, and any Additional Consideration Shares, and the Fastnet Consideration Shares shall be allotted as fully paid and free from all encumbrances and shall rank pari passu in all respects with the Ordinary Shares and B Ordinary Shares of the Buyer and Fastnet respectively in issue at the Closing Date and with any other shares issued in course of the Placing (as defined in Annex 4.2) or the issue of any shares pursuant to the Sompharma Transaction (as defined in Annex 3.1.5), including as to dividends declared, made or paid after the Closing Date.

3.2 The payment of the elements of the Purchase Price pursuant to clauses 3.1.1 through 3.1.4, and any allotment of Consideration Shares and any Additional Consideration Shares pursuant to clause 3,1.5, shall be made by the Buyer and be split between and allocated to the Sellers as follows:

Seller 1:	[***]%,
Seller 2:	[***]%.

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3.3 Payments to the Sellers shall be made to the following account in the name of Seller 1, and the receipt of payment by Seller 1 on behalf of Seller 2 shall be an absolute discharge in respect of the amount payable to Seller 2, unless any other account has been notified to the Buyer by the respective Seller:

Bank:	[***]
IBAN:	[***]
BIC:	[***]

4. Closing

4.1 Closing

The Parties shall consummate the transfer of the Shares as agreed in this Agreement and perform the closing actions set forth in clause 4.6 (collectively referred to as the “Closing”) on or before 31 March 2016 (the “ScheduledClosing Date”), provided that the Closing Conditions as specified in clause 4.2 have been satisfied (or, where permissible, waived).

4.2 Closing Conditions

The Parties are not obligated to carry out the Closing until all of the conditions for the Closing set forth in Section 2.1 of Annex 4.2 (the “Closing Conditions”) have been satisfied or waived pursuant to the provisions of Annex 4.2 and subject to waiver by the Buyer that the provisions of clauses 4.3 and 4.4 have been complied with.

4.3 Material Adverse Change

There has been no significant change in the Business Intellectual Property and no commercialisation rights have been granted to the Business Intellectual Property by the Company between the Signing Date and the Closing Date.

4.4 No Material Pending or Threatened Action

On the Closing Date there are no material pending or threatened actions or proceedings known to the Sellers or the Company by or before any court or other governmental body or agency which shall seek to restrain, prohibit or invalidate the sale and transfer of the Shares in the Company to the Buyer as contemplated by this Agreement.

4.5 Long Stop Date

If any or all of the Closing Conditions are not fulfilled or waived or extended by the Seller 1 (for and on behalf of the Sellers) on or before 31 March 2016 at the latest (or by such later date as the Parties may agree) (the “Long Stop Date”) this Agreement shall lapse and cease to have effect, save that clause 4.11 and clause 13 through 18 shall remain binding on the Parties, and no Party shall have any claim against any of the others with respect to the non-performance of any obligation under this Agreement falling due for performance prior to such lapse.

4.6 Closing

On the Scheduled Closing Date, the Parties shall undertake the following actions:

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4.6.1 Buyer shall contribute to the Company’s capital reserves (Kapitalrucklagen) within the meaning of § 272 para. 2 no. 4 German Commercial Code (Handelsgesetzbuch, HGB) the amount of EUR [***] (“Contribution”);

4.6.2 Buyer shall pay the Additional Cash Amount to the Sellers;

4.6.3 Cathal Friel and Michael Nolan shall by side letter, substantially in the form attached hereto as Annex 4.6.3, agree to vote such shares as they beneficially own in Fastnet in favour of the appointment of a named individual to be determined by Seller 1 (subject to the approval of the nomad (nominated advisor as defined under AIM Rules) of Fastnet) as a non-executive director of Fastnet, and the directors of Fastnet shall recommend to the shareholders of Fastnet that they vote, at the general meeting to approve the RTO, in favour of the appointment of said named individual as such a director of Fastnet. In the event that the shareholders do not vote in favour of this recommendation, the directors of Fastnet shall procure, within two weeks of the general meeting referred to above, and subject to the approval of the nomad of Fastnet, that said individual be appointed as director of Fastnet by way of directors’ resolution and shall include a resolution to appoint the nominee at the next following annual general meeting of the Company and such appointment shall be referred to in the Fastnet admission document;

4.6.4 Seller 1 has executed and wilt not alter an agreement on the termination and dissolution of the silent partnership (Stille Gesellschaft) between Seller 1 and the Company dated 12 March 2015, including a statement that the Company is not obliged to repay any stakes (Bareinlagen) paid by Seller 1, substantially in the form attached as Annex 4.6.4 and which shall be in full force and effect as of the Closing Date in substantially the same form as the document attached hereto;

4.6.5 Seller 2 has executed an agreement on the termination of the royalty agreement between Seller 2 and the Company dated 2 August 2013 an executed copy of which is attached hereto as Annex 4.6.5 such that the Company has no remaining obligations to Seller 2, and which shall come into full force and effect as of the Closing Date in the same form as the executed agreement attached hereto;

4.6.6 Sellers shall procure that the Company executes, to the extent not already executed, an agreement providing for an option to extend the term of the two lease agreements regarding the production site in Streiflingsweg 11, Niefern-Öschelbronn, Germany, until December 31, 2019, on the same terms, substantially in the form attached hereto as Annex 4.6.6;

4.6.7 Sellers as the sole shareholders of the Company have adopted a shareholders’ resolution unconditionally and irrevocably consenting to the sale and transfer of the Shares to the Buyer and irrevocably waiving any and all pre-emptive rights, rights of first refusal, and or any similar rights they may be entitled to in connection herewith, an executed copy of which is attached hereto as Annex 4.6.7 and which shall be in full force and effect as of the Closing Date in same form as the executed resolutions attached hereto;

4.6.8 The Sellers shall have executed the Amryt Share Exchange Agreement (as defined in Annex 4.2) the provisions of which shall not be inconsistent with the schedule to the MOU (as defined in Annex 4.2) and under which the Sellers shall have no obligations other than to transfer the Consideration Shares, and any Additional Consideration shares, as the case may be, to Fastnet free from encumbrances in exchange for

4

receiving the Fastnet Consideration Shares (as defined in Annex 4.2) and to provide customary warranties as to ownership, non-encumbrance of the Consideration Shares and authorisation of and due execution of the Amryt Share Exchange Agreement.

4.6.9 Seller 1 has presented an executed resolution by the executive board (Stiftungsvorstand) of the Seller 1 irrevocably approving the transactions contemplated by this Agreement, an executed copy of which is attached hereto as Annex 4.6.9 and which shall be in full force and effect as of the Closing Date in same form as the executed resolution attached hereto; and

4.6.10 Seller 2 as current chairman (Vorstand) of the Company and the existing members of the supervisory board (Aufsichtsrat) of the Company shall have resigned, effective as at the Closing Date. The current supervisory board of the Company shall have adopted a resolution appointing a nominee of the Buyer as Chairman of the Company. The Sellers as the sole shareholders of the Company shall have adopted a shareholders’ resolution unconditionally consenting to the appointment of three nominees of the Buyer as members of the advisory board, substantially in the form attached hereto as Annex 4.6.10 and which shall come into full force and effect as of the Closing Date In same form as attached hereto;

4.7 The actions listed above in clauses 4.6.1 to 4.6.3 are for the sole benefit of the Sellers and may be waived by Seller 1 (for and on behalf of all Sellers) (either in whole or in part) at any time by written notice to the Buyer.

4.8 The actions listed above in clauses 4.6.4 to 4.6.7 and 4.6.9 to 4.6.10 are for the sole benefit of the Buyer and may be waived by the Buyer (either in whole or in part) at any time by written notice to the Sellers. The action listed in clause 4.6.8 may only be waived by Seller 1 (for and on behalf of the Sellers) and the Buyer jointly.

4.9 The Buyer and Seller 1 (for and on behalf of all Sellers) shall record in the closing minutes that all Closing Conditions have been satisfied (or, where applicable, waived), all actions pursuant to clause 4.6 have been completed and that Closing has occurred.

4.10 If one of the Parties is unable to comply with any of the obligations specified in this clause 4, there shall be a grace period of ten (10) Business Days by written notice specifying the non-compliance; following the unsuccessful expiration of the grace period, the other Party may withdraw from this Agreement by written notice to the Party unable to comply with the applicable obligation (Rücktritt).

4.11 In the event that the Closing does not take place due to a failure by the Sellers to comply with the obligations specified in clauses 4.6.4 to 4.6.10, the Prepayment Amount shall be refundable. In the event that the Closing does not take place for any other reason, the Prepayment Amount shall not be refundable.

4.12 Power of attorney

As of Closing and until the point in time when the Buyer is towards the Company deemed to be the owner of the Shares, the Sellers hereby grant to the Buyer an irrevocable power of attorney to exercise all rights resulting from and attaching to the purchased Shares (in particular voting rights). The Buyer shall be released from the restrictions according to section 181 German Civil Code (BGB). The Buyer shall be entitled to use such power of attorney solely in its own best interest and without consultation of the Sellers. The Sellers hereby covenant towards the Buyer

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not to make use of the rights resulting from and attaching to the Shares during the validity of the power of attorney without written consent of the Buyer.

5. Independent Guarantee Undertakings of the Sellers

5.1 General Scope of Application

5.1.1 Each Seller warrants to the Buyer by way of an independent guarantee undertaking (selbständiges Garantieversprechen) pursuant to § 311 para. 1 BGB, subject to the requirements and limitations provided in the provisions of clause 8 which are an inseparable and integral part of these warranties, that the statements set forth below in this clause 5 (the “Warranties” and each a “Warranty”) are true and complete as of the Signing Date, as of the Closing Date, and/or any other date if so referred to in the respective Warranty.

5.1.2 Sellers and the Buyer agree that the Warranties shall be exhaustive and that the Sellers do not assume any guarantees, representations or warranties of any kind in addition to the Warranties. Sellers and the Buyers agree that the Warranties do not constitute guarantees of quality (Beschaffenheitsgarantie) pursuant to §§ 443, 444 BGB.

5.1.3 Sellers represent and warrant to the Buyer that:

(a) all information supplied in writing by the Sellers or their agents and advisors to the Buyer or its agents and advisors (including, for the avoidance of doubt, Raglan Capital) in the data room or the Q&A sessions, the contents of each of which are attached hereto in a DVD as Annex 5.1.3, (“Due Diligence Information”) is to Sellers’ Knowledge accurate and complete in all material aspects, and

(b) all information contained or referred to in the Disclosure Exhibits (as defined herein below) is fairly presented and nothing has been omitted from the Disclosure Exhibits which renders any of that information incomplete or misleading in any material respect. “Disclosure Exhibits” shall mean and include all Annexes to this Agreement containing disclosure information, particularly (without limitation) all Annexes to clause 5 of this Agreement.

5.1.4 Except as expressly provided to the contrary in any of the Warranties, all of the Warranties shall be treated as qualified by any actual knowledge on the part of the Buyer or any of its agents.

5.1.5 The Sellers acknowledge that the Warranties under this Agreement are material and the accuracy of the Warranties and compliance with the undertakings is essential to the Buyer’s decision to enter into and pay the Consideration set out in this Agreement.

5.2 The following Warranties with regard to the capacity of Seller 1 and Seller 2 are given by each Seller only with regard to itself and the Shares held by the respective Seller:

5.2.1 The Sellers have taken all necessary actions and have all requisite power and authority to enter into and perform this Agreement and the other documents referred to in it (to which it is a party) in accordance with their respective terms.

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5.2.2 This Agreement and the other documents referred to in it (to the extent that the respective Seller is a party to it) constitute (or shall constitute when executed) valid, legal and binding obligations on the Sellers in accordance with their respective terms.

5.2.3 No proceedings are current, pending or threatened to restrain or which would have the effect of restraining the entry into, the performance of, compliance with and enforcement of, any of the obligations of the Sellers hereunder and, so far as the Sellers are aware, there are no circumstances which might give rise to such proceedings.

5.2.4 On the Closing Date, the information about the Company provided in the Preamble sections (B) and (C) is true and complete. The Shares effectively exist and are, as of the Closing Date, free and clear of any encumbrance and other third party’s right of whatever nature. The capital contributions (Kapitaleinlagen) have been paid in in full and have not been paid back. Upon the passing of the resolutions pursuant to clauses 4.6.7 and 4.6.9 the Sellers may freely dispose of the Shares held by them in the Company and there do not exist any pre-emptive, option, usufructuary or other purchase rights and/or security interests or similar rights of third parties with regard to the Shares.

5.2.5 No person has any right to require, at any time, the transfer, creation, issue or allotment of any share, loan capital or other securities (or any rights or interest in them) of the Company, and neither the Sellers nor the Company have agreed to confer any such rights, and no person has claimed any such right.

5.2.6 The Company’s articles of association dated 2 April 2015 and set out in Annex 5.2.6 represent the current and applicable version of the Company’s articles of association. There are no collateral agreements to the relevant articles of associations except for the side letter on joint voting obligations between Seller 1 and Seller 2 dated 16 July 2010.

5.2.7 The excerpt from the commercial register attached hereto as Annex 5.2.7 truly and completely reflects the legal situation of the Company. Except for the execution of measures in connection herewith or on the basis hereof, no applications for registration have been filed and no shareholders’ resolutions passed, which may require the registration with the commercial register and which are not shown in the excerpt from the commercial register.

5.2.8 The Company does neither have any shareholdings in or ownership rights with regard to other companies or enterprises nor is under any obligation to acquire such shareholdings or rights. Apart from the silent partnership (Stille Gesellschaft) between Seller 1 and the Company dated 12 March 2015 (referred to inclause 4.6.3 above) the Company is neither a party to inter-company agreements within the meaning of §§ 291 et seq. of the German Stock Corporations Act (AktG) nor has it entered into agreements on the establishment of silent partnerships or other profit-sharing agreements.

5.3 Warranties with regard to the economic situation of the Company:

5.3.1 As at the Signing Date and as at the Scheduled Closing Date, the Company does not have any indebtedness or liabilities (including contingent liabilities) other than trade debts incurred in the ordinary course of business and is not insolvent (zahlungsunfählg).

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5.3.2 Since June 30, 2015,

(a) to the Sellers’ Knowledge, the Company has substantially been properly managed within the course of ordinary business and in accordance with past practice;

(b) there has been no disposal or acquisition of any material asset or supply of any service or business facility of any kind by or to the Company other than in the ordinary course of business, apart from the sale of “Viscum TT” to Seller 1 as disclosed in Annex 5.3.2; and

(c) the Company has not made any payment or incurred any liability, commitment to the Seller or any member of Seller 1’s group, except in the ordinary course of business on normal commercial terms, except as set forth in this Agreement;

5.3.3 The Company has full and unlimited title or effective usufructuary rights to assets owned by the Company, and – in the case of leased assets which are currently used in the course of business – there are effective lease agreements in force. Customary reservations of title (Eigentumsvorbehalt) of suppliers and liens of lessors or other third parties which may exist by virtue of law or contractual agreement with regard to the liabilities of the Company shall be reserved.

5.3.4 To the Sellers’ Knowledge there have been no environmental incidents, including explosions and fire incidents at the site of the Company in the five years prior to the Signing Date other than the incidents which occurred on 27 July 2010 and 2 September 2011 and, to the Sellers’ Knowledge, no personal injury claims of any person, claims by the landlord, and any other liabilities related to these incidents have been brought during that period.

5.4 Warranties with regard to the business of the Company:

5.4.1 To the Sellers’ Knowledge, (i) the Company is In the possession of any and all material public and private-law permits, permissions and licenses required for the management and the continuation of its relevant current businesses, (ii) the Company is in compliance with applicable law, permits and licenses, and (iii) third party contractors are in compliance with applicable law, permits and licenses in relation to the conduct of clinical trials carried out on behalf of the Company. For the avoidance of doubt, the granting of the pending Marketing Approval by the EMA (both as defined in Annex 3.1) shall not be considered as being required under this Warranty.

5.4.2 To the Sellers’s Knowledge, neither the Company, nor any of its respective officers, is involved in or subject to any pending or threatened litigation, claims or actions before any court, governmental or regulatory authority that the Company may be liable for.

5.4.3 The Company is not a party to any agency, license, distribution or other agreement or to any arrangement which restricts its freedom to carry on its business in any part of the world, apart from the agreements disclosed in Annex 5.4.3.

5.5 Warranties with regard to Intellectual property rights:

5.5.1 Summary details of all Business Intellectual Property which is Registered Intellectual Property (and any Applications) are set out in Annex 5.5.1.

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5.5.2 The Company is either the sole legal and beneficial owner of the Business Intellectual Property, or the Company has a valid Licence to use all Business Intellectual Property which it uses and no Licences are in existence in relation to Betulin (as defined in Annex 3.1) and the Company has not granted any Licences to Business Intellectual Property to any third party.

5.5.3 To the Sellers’ Knowledge, there is no material unauthorised use or infringement by any person of the Business Intellectual Property.

5.5.4 To the Sellers’ Knowledge, the operations of the business of the Company as conducted at the Signing Date and the Closing Date do not infringe or make unauthorised use of any Intellectual Property rights of a third party.

5.5.5 To the Sellers’ Knowledge, there are no pending court proceedings (either brought by or against the Company) or any court decisions in respect of the Business Intellectual Property. No third party has notified the Company or the Seller in writing of its intention to bring such proceedings in respect of the Business intellectual Property.

5.5.6 The Business Intellectual Property which is Registered Intellectual Property is subsisting and properly maintained.

5.5.7 Other than as disclosed in Annex 5.5.7, as at the Signing Date no publication or other disclosure has been made by or on behalf of the Company relating to the use of betulin for the treatment and/or amelioration of Epidermolysis Bullosa (EB), and no such publication shall be made between the Signing Date and the Completion Date.

5.6 Warranties with regard to employees:

5.6.1 Annex 5.6.1 lists all current employees and there have been no material changes to each of their applicable terms and conditions other than as disclosed in the Disclosure Exhibits and/or Due Diligence information.

5.6.2 To the Sellers’ knowledge, there are no pension schemes or other incentive schemes or (including, without limitation, any share option or share award plan, and any commission, profit sharing or bonus scheme) established by the Company in which any current or former director of the Company, or any employee (or any of their respective associates or nominees) participates or has participated.

5.7 Warranties with regard to insurance:

5.7.1 To the Sellers’ Knowledge (i) insurance claims made against the Company during the period of 48 months prior to the Signing Date or between the Signing Date and the Closing Date have been disclosed in the Disclosure Exhibits and/or Due Diligence Information and (ii) there are no circumstances likely to give rise to a claim under any of the foregoing policies.

5.8 The Buyer expressly acknowledges to acquire the Shares as they are as of the Closing Date in accordance with its own examinations and assessment of all circumstances and to execute the purchase transaction on the basis of its own decision, examination and assessment without applying to any express or implied warranties or guarantees of the Seller except for the Warranties expressly given by the Sellers herein. Notwithstanding the preceding sentence, the Buyer in particular agrees that the Sellers do not give any warranties or guarantee undertakings with regard to (i) forecasts, estimations or budgets referring to future earnings, profits, returns, cash flows, the future financial situation, the future volume of orders or the future business of the Company made available to the Buyer and (ii) other documents with regard to the business

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of the Company made available to the Buyer, his lawyers, auditors or other advisers within the course of the sales process and the due diligence, in particular the Due Diligence Information and information made available to the Company and its advisers and consultants (which for the avoidance of doubt shall include Raglan Capital) at formal management presentations (at which at least two representatives of the Buyer were present) save as otherwise expressly provided for herein.

5.9 The “Sellers’ Knowledge” within the meaning of this Agreement only refers to the actual knowledge of Seller 1 and/or of Seller 2.

6. Rules of Conduct

6.1 The Sellers shall, to the extent legally permissible, procure that during the period between the Signing Date and the Closing Date the business of the Company Is managed with due care and attention and exclusively within the course of ordinary business in accordance with past practice, with all required diligence and care and complies with all statutory and regulatory provisions applying to its activities. During the period between the Signing Date and the Closing Date, the Sellers will not resolve on or distribute any dividends or other distributions without the Buyer’s prior written approval.

6.2 In particular, the Sellers will procure that during the period between the Signing Date and the Closing Date the Company shall not, other than within the Company’s ordinary course of business:

6.2.1 dispose of any current or fixed assets,

6.2.2 sell inventories within the meaning of § 266 para, 2 B I German Commercial Code (Handelsgesetzbuch, HGB), and/or

6.2.3 delay payments on trade accounts payables.

6.3 The Sellers shall execute the Amryt Share Exchange Agreement according to the following procedure;

6.3.1 The Buyer agrees that promptly following the Signing Date it shall negotiate expeditiously and in good faith with Fastnet and [***] (“Buyer Shareholders”) to agree the terms of the Amryt Share Exchange Agreement. Once the Amryt Share Exchange Agreement is in an agreed form between the Buyer, the Buyer Shareholders and Fastnet, and incorporates or adequately reflects the terms referred to in the definition of “Amryt Share Exchange Agreement” in Annex 4.2 and in the schedule to the MOU (the “Draft Amryt Agreement”) (which the Buyer undertakes shall happen no later than 27 November 2015), the Buyer shall provide Seller 1 with an electronic copy of the Draft Amryt Agreement and each other document referred to in it.

6.3.2 The Sellers shall review the Draft Amryt Agreement and if they believe that it does not adequately reflect the terms referred to in the definition of “Amryt Share Exchange Agreement” in Annex 4.2 and in the schedule to the MOU (as defined in Annex 4.2), they and the Buyer shall negotiate In good faith to agree the terms of the Draft Amryt Agreement not later than three weeks from the date of receipt of the electronic copy referred to above.

6.3.3 Once the Draft Amryt Agreement is in a form agreed between the Sellers, the Buyer, the Buyer Shareholders and Fastnet, each Seller shall promptly execute the signature

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page of the Draft Amryt Agreement and shall provide the applicable signature pages to Seller 1’s counsel, Dentons Europe LLP, Berlin (“Counsel”) to be held for release upon exchange of the Amryt Share Exchange Agreement on the following terms:

(a) Counsel shall be irrevocably Instructed by the Sellers to hold such signature pages until (i) each of the parties to the Amryt Share Exchange Agreement (other than the parties referred to above in the first paragraph of this clause 6.3.3) shall have executed such agreement, and (ii) the Buyer shall have provided Counsel with a full undated pdf copy of such agreement (and each other agreement and document required to be executed in connection with the exchange of such agreement) evidencing execution by them; and

(b) Counsel shall be irrevocably instructed by the Sellers that, upon each of the matters set out in clause 6.3.3(a) having happened, and no changes having been made to the Draft Amryt Agreement (save for in relation to the Sole Permitted Variations referred to in clause 6.3.5), to release the Sellers’ signature pages, thereby effecting the execution of the Amryt Share Exchange Agreement.

6.3.4 For the avoidance of doubt, Seller 1’s right not to complete the sale and purchase of the Shares pursuant to clause 10.4 and, therefore, revoke above instructions, remains reserved.

6.3.5 For the further avoidance of doubt, as the final valuation of Amryt for the purposes of the RTO shall not be completed (and therefore not reflected in the Amryt Share Exchange Agreement) until the day the Amryt Share Exchange Agreement Is executed by all parties thereto, the Draft Amryt Agreement shall be revised prior to being executed for the purposes of including the number and/or value of Fastnet Shares to be issued to the Sellers and the other shareholders of the Buyer on completion of the RTO in accordance with this Agreement, and, in addition, may be revised to provide for additional warranties which are required to be provided by parties other than the Sellers under the terms of the Amryt Share Exchange Agreement (together the “Sole Permitted Variations’’). Accordingly, the Buyer undertakes to the Sellers to provide Seller 1 with regular updates (including on the day of exchange) as to the likely valuation of Amryt and, as soon as reasonably practicable (and in any event, prior to exchange), with a schedule showing the Fastnet Shares to be issued to each of the shareholders in the Buyer pursuant to the Amryt Share Exchange Agreement. The Parties agree that, the revision of the Draft Amryt Agreement for the purposes of the Sole Permitted Variations in accordance with this clause 6.3.3 shall have no Impact upon the other agreed terms of the Amryt Share Exchange Agreement or upon the release of the signature pages upon the RTO by Counsel.

6.3.6 For the avoidance of doubt, in the event that any changes to the Draft Amryt Agreement (as agreed and executed In accordance with clauses 6.3,2 and 6.3.3) are proposed beyond the Sole Permitted Variations, the Counsel shall not be obliged to release the signature pages, the Buyer shall present the revised Draft Amryt Agreement and the Sellers and the Buyer shall negotiate in good faith to agree the terms of the revised Draft Amryt Agreement within a period of one week from receipt of the revised document. Upon agreement of the terms of the Revised Draft Amryt Agreement the terms of clauses 6.3.3 to 6.3.5 shall apply equally to the revised Draft Amryt Agreement.

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6.4 Furthermore, the Sellers will procure, to the extent legally permissible, that the following agreements are executed on or before the ‘roadshow’ to be carried out by Fastnet to potential Investors which is scheduled to take place in [***] (the “Roadshow”): to the extent not already executed, an agreement providing for an option to extend the term of the two lease agreements regarding the production site in [***], until [***], as referred to in clause 4.6.6.

6.5 The Sellers shall immediately notify the Buyer of any matter or thing which arises or becomes known to them before Closing or in advance of the Roadshow (subject to the Sellers being given one week’s advance notice of the date of commencement of the Roadshow) which:

6.5.1 constitutes (or would after the lapse of time constitute) a misrepresentation or a Breach (as defined in clause 8.1) of any of the Warranties or the undertakings or other obligations on the part of the Sellers under this Agreement; or

6.5.2 would constitute (or be likely to constitute) a Breach (as defined in clause 8.1) of any of the Warranties when the Warranties are repeated at Closing.

6.6 Without prejudice to the generality of clause 6.5, the Sellers shall provide a confirmation within one week prior to the Roadshow of any material developments in relation to the Company and that they are not aware of any matters occurring after the Signing Date which would constitute (or would after the lapse of time constitute) a misrepresentation or a Breach (as defined in clause 8.1) of any of the Warranties or the undertakings or other obligations on the part of the Sellers under this Agreement, and will provide such details if there are any such matters.

7. Cooperation Obligations

7.1 For the period of time following the Signing Date until 31 January 2016, the Company shall procure that the employees of the Company shall provide the following directly to the nominated consultants of the Buyer (initially being [***]) (“Buyer’s Consultants”), in each case with the requirement of the Buyer’s Consultants seeking prior consent by the Company’s executive chairman (Vorstand):

7.1.1 procure that the Buyer’s Consultants are given access to the premises and to the books and records of the Seller’s that relate to the Company,

7.1.2 provide such information regarding the businesses and affairs of the Company as the foregoing consultants may reasonably require, and

7.1.3 keep Buyer’s Consultants informed as to all material developments in the operation of the Business (and in particular in relation to any matters concerning intellectual property),

in each case only if and to the extent required for preparing the reverse takeover (RTO) and the granting of the Consideration Shares and the Fastnet Consideration Shares as referred to in clause (F) of the Preamble in further outlined in Annex 4.2.

7.2 The Company’s obligation under clause 7.1 is subject to each of the Buyer’s Consultants to execute in favour of the Company customary non-disclosure and non-compete agreements, acceptable to the Company, protecting the Company’s confidential information and Intellectual Property. The maximum number of Buyer’s Consultants is limited to 2 individuals,

7.3 The Company agrees to provide, on cost of the Buyer, such reasonable cooperation, assistance, documents and information as is reasonably required by the Buyer and Fastnet to carry out the

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activities required to give effect to the RTO (as referred to in clause (F) of the Preamble in further outlined in Annex 4.2), including (i) the audit of the accounts of the Company by such auditors as may be nominated by the Buyer for the period from [***] to [***] or to [***] as may be determined by the Buyer, and (ii) to provide such assistance as is required to assist with the restatement of the annual accounts for the past three financial years (2012, 2013 and 2014) of the Company according to IFRS accounting principles. Any and all costs relating to and/or arising from such activities shall be borne by the Buyer.

7.4 Seller 2 shall procure that the article on “Betulin-based Triterpene Extract accelerates wound healing in Hereditary Epidermolysis Bullosa - Results of a prospective controlled phase II study” referred to in Annex 5.5.7 is not being published without the Buyer’s consent.

8. Legal Consequences in Case of a Breach of Warranties; Violations of Rules of Conduct; Limitation of liability

8.1 In the event that any (i) of the Warranties given under clause 5 above is breached or (ii) if any rule of conduct obligation pursuant to clause 6 has been violated (each a “Breach”) the Sellers have to put the Buyer into such position as the Buyer would have been in if the Warranty had been correct or the rule of conduct complied with (restitution in kind (Naturalrestitution)). Buyer shall notify the Sellers in writing without undue delay after the Buyer becomes aware of a Breach, however not later than (i) four weeks after discovery of the relevant circumstances if discovered before the Scheduled Closing Date and (ii) six months after discovery if discovered after the Scheduled Closing Date. If the Sellers fail to rectify the Breach by restitution in kind withina period of four weeks after having been properly notified by the Buyer of such Breach, the Buyer may demand a reduction of the purchase price or damages instead of performance (Schadensersatz statt der Leistung), subject to the other provisions of clause 8.

8.2 Claims for a Breach of the Warranties shall become time-barred two (2) years from the Closing Date.

8.3 De Minimis Amount, Threshold

The Buyer shall only be entitled to a claim against the Sellers for a Breach if and to the extent the individual claim for a Breach exceeds an amount of [***] and the aggregate amount of such claims exceeds an amount of [***]. If the total amount of [***] is exceeded, the Buyer may claim the entire amount.

8.4 Maximum Liability Amount and Remedies

Any claims of the Buyer against the Sellers for a Breach of the Warranties pursuant to clause 5 (which are required to be made within the two year period referenced in clause 8.2) shall be limited in total to the Prepayment Amount and the first to occur of Milestone Payment 1 or Milestone Payment 5 which have been paid to the Sellers or which may become payable to the Sellers upon the occurrence of the milestone events which trigger Milestone Payment 1 or Milestone Payment 5, pursuant to clause 3. For the avoidance of doubt, as long as neither Milestone 1 or Milestone 5 are triggered, the maximum liability amount is limited to the Prepayment Amount. Subject to the preceding sentence, Buyer shall as applicable be entitled to (i) seek repayment of the foregoing payments made to date to the Sellers and/or (ii) a right of set off against the foregoing payments to the extent these are payable in the future (including after the foregoing two year period), provided that such rights are approved by a final judicial decision or pursuant to agreement of the Sellers and the Buyer notwithstanding that such final judicial decision may not take place within the two year period referenced in clause 8.2.

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8.5 Exclusion of Liability in case of Disclosure

The Buyer shall not be entitled to assert any claim for a Breach of clause 5 and clause 6 if, at the Signing Date and/or the Closing Date, the Buyer has had actual knowledge of the respective facts or circumstances based on the Disclosure (as defined herein below). “Disclosure” shall include all facts, matters or circumstances fairly disclosed to the Buyer in the Disclosure Exhibits, including by reference to specific agreements or documents, and all Due Diligence Information as contained in Annex 5,1.3.

8.6 Exclusion and Limitation of Liability

8.6.1 No double consideration

Any claim of the Buyer under or in connection with this Agreement shall be excluded if and to the extent the claim is based on any amendment of a law, ordinance, statutes, administrative regulation, judgment, ruling, decision, permission, decree or another (administrative) act or other legal provision which occurred after the Closing Date.

8.6.2 No liability in case of actions taken by the Buyer

The Sellers shall not be liable for any facts or circumstances if and to the extent only that such facts or circumstances would not have been occurred without an arbitrary action or omission by the Buyer.

8.6.3 Exhaustive Provisions

Except as expressly provided for otherwise in this Agreement, the remedies provided for in this clause 8 shall be Buyers’ sole remedies. Any additional liability of the Sellers or their representatives for further rights or claims of the Buyer – including without limitation statutory, contractual and pre-contractual obligations (e. g. § 280 to 282, 311 BGB), the right of rescission (Anfechtung) or withdrawal (Rücktritt) – arising from or in connection with defects in quality or title or from a Breach are explicitly excluded. Such exclusion shall not apply in case of fraud (Arglist) or wilful misconduct (Vorsatz) by the Sellers.

8.7 Contributory Fault

Section 254 BGB (Mitverschulden) shall remain unaffected, The Buyer shall in particular be obliged to avert any damages and to mitigate the scope of damages suffered.

9. Independent Guarantee Undertakings of the Buyer

9.1 The Buyer hereby warrants to the Sellers by way of an independent guarantee undertaking pursuant to § 311 para. 1 BGB that the statements in this clause 9 are true and complete as of the Signing Date and the Closing Date:

9.1.1 The Buyer has been duly established under Irish law, effectively exists and has the required corporate power and authority in order to hold the assets and to carry on its business.

9.1.2 The Buyer has the required corporate power and authority and has been duly authorized by any and all required corporate actions to execute this Agreement and any legal transactions provided for herein.

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9.1.3 The execution and performance of this Agreement and any legal transactions provided for herein by the Buyer do not constitute a violation of the Buyer’s articles of association and no violation of applicable legal provisions, judgments, injunctions or other binding provisions. To the Buyer’s knowledge there neither exist pending legal proceedings, preliminary investigations or other proceedings against the Buyer before a court, an arbitral tribunal or an administrative authority that may prevent, modify or delay in any way the execution of the legal transactions provided for herein nor are they threatened.

9.1.4 The Buyer has no subsidiary companies and will not Incorporate any subsidiaries other than the incorporation of a subsidiary as an intellectual property holding company pursuant to the clause 10.1.1 Activities.

9.1.5 The only shares in the capital of the Buyer are Ordinary Shares and B Ordinary Shares and the Buyer has not issued or agreed to issue and will not issue or agree to issue, nor has it created nor will it create rights over or In respect of, any such shares or any shares of any other class or denomination at any time prior to completion of the Amryt Share Exchange Agreement.

9.1.6 The Buyer’s constitution (incorporating its memorandum and articles of association) dated 9 October 2015 (the “Buyer’s Constitution”); and set out in Annex 9.1.6 represents the current and applicable version of the Buyer’s Constitution. There are no collateral agreements to the Buyer’s Constitution and, for the avoidance of doubt, neither the Buyer nor its shareholders have elected to modify or adjust any of the “Optional Provisions” as referred to in Article 1 of the Buyer’s Constitution to the detriment of the Sellers. To the extent that the “Optional Provisions” (as defined in the Buyer’s Constitution) include any drag along or other right to require the Sellers to sell the Consideration Shares or any Additional Consideration Shares, the Buyer shall procure that such right is not exercised in respect of such shares.

9.2 Since its incorporation the Buyer has not (save as expressly contemplated by this Agreement):

9.2.1 Traded or carried on any business, other than pursuant to the clause 10.1.1 Activities

9.2.2 declared or paid any dividend or made any other distribution to its shareholders;

9.2.3 acquired or agreed to acquire any asset or property in excess of [***] and will not between the Closing Date do so save as relates to the clause 10.1.1. Activities; or

9.2.4 incurred any liability actual contingent or otherwise in excess of [***] and will not between the Closing Date do so save as relates to the clause 10.1.1. Activities.

9.3 No order has been made or petition presented or resolution passed for the winding-up of the Buyer and no distress, execution or other process has been levied on any of its assets, it has not stopped payment and is not insolvent or unable to pay its debts for the purpose of section 570 of the Companies Act 2014, no receiver or examiner has been appointed by any person of its business or assets or any part thereof, there is no unfulfilled or unsatisfied judgment or court order outstanding against it and there has been no delay by it in the payment of any obligation due for payment.

9.4 Save for such appropriate liabilities as may be incurred pursuant to the clause 10,1.1 Activities and as referred to in this clause 9.4, or pursuant to employment and consulting agreements, there are no loans made by the Buyer to any of its directors or shareholders and/or any person connected with any of them, and no debts or liabilities owing by the Buyer to any of its directors

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or shareholders and/or any person connected with them as aforesaid. A convertible loan note instrument up to the amount of [***] shall be granted by the Buyer on the basis that such loan notes shall be convertible to ordinary shares in the Buyer (the “Loan Notes”), and which for the avoidance of doubt, shall not affect the Sellers’ rights as set out in section 1.2 of Annex 3.1.5.

9.5 Save for such appropriate liabilities as may be incurred pursuant to the clause 10.1.1 Activities, and contracts of employment, consultancy agreements and the allotment of shares, there are no existing contracts or arrangements to which the Buyer is a party and in which any of its directors or shareholders and/or any person connected with any of them is interested.

9.6 Neither the Buyer, nor any person for whose acts or omissions it may be vicariously liable:

9.6.1 is engaged in or subject to any litigation, administrative, mediation or arbitration proceedings in relation to the Buyer; or

9.6.2 is the subject of any investigation, inquiry or enforcement proceedings by any governmental, administrative or regulatory body.

9.7 No such proceedings, investigation or inquiry as are mentioned in clause 9.6 have been threatened or are pending by or against the Buyer or against any such person, and there are no facts or circumstances likely to give rise to any such proceedings.

10. Pre-Closing Obligations of Buyer

10.1 During the period beginning on the Signing Date and ending at the Closing Date, or any earlier time at which this Agreement terminates, the Buyer shall not without the prior written consent of Seller 1, not to be unreasonably withheld, conditioned or delayed:

10.1.1 incur any liabilities in excess of [***] other than in relation to (i) the acquisition of the Company, (ii) the acquisition of [***] and [***] (together “[***]”), (iii) the RTO, (iv) the payment of employees, (v) the loan Notes, and (vi) general corporate activities, including the incorporation of a subsidiary as an intellectual property holding company (together the “clause 10.1.1 Activities”);

10.1.2 grant, issue or redeem any mortgage, charge, debenture or other security;

10.1.3 enter into any material agreement or materially change the terms of any material agreement to which it is a party at the date of this Agreement, or terminate or give notice to terminate any such agreement, save in each case as relates to the clause 10.1.1. Activities;

10.1.4 declare, make or pay any dividend or other distribution;

10.1.5 agree, conditionally or otherwise, to do any of the activities listed in clauses 10.1.1 to 10.1.4.

10.2 During the period beginning on the Signing Date and ending at the Closing Date, the Buyer shall not do or omit to do anything which would cause any of the independent guarantee undertakings of the Buyer in clause 9 to be untrue in any material respect if they were repeated immediately before Closing.

10.3 If before the Closing:

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10.3.1 Seller 1 becomes aware of any breach of any of the independent guarantee undertakings of the Buyer in clause 9 which is material in the context of this Agreement; or

10.3.2 the Buyer is in breach of any of its obligations under clause 10 and that breach is material in the context of this Agreement,

then Seller 1 shall provide notice to the Buyer within 5 Business Days of becoming aware of the relevant breach, requiring the Buyer to remedy the breach within a further period of 10 Business Days starting on the day after the day on which the Buyer receives Seller 1’s notice.

10.4 If the Buyer remedies the breach in question on or before the expiry of the 10 Business Day period referred to in clause 10.3 then, subject to the other provisions of this Agreement, Closing shall take place in accordance with clause 4 or, if Closing would otherwise have taken place during such 10 Business Day period it shall take place on the fifth Business Day after the expiry of such 10 Business Day period. If the Buyer is not able to remedy the breach in question on or before the expiry of the 10 Business Day period referred to in clause 10.3, then Seller 1 (for and on behalf of all Sellers) may, by notice to the Buyer during the 5 Business Day period immediately following the expiry of that 10 Business Day period, elect not to complete the sale and purchase of the Shares or elect to complete the sale and purchase of the shares notwithstanding the relevant breach or, with the consent of the Buyer, elect to postpone Closing again by a further 10 Business Days.

10.5 Seller 1’s right to elect to proceed or not to proceed to Closing or (with the consent of the Buyer) to grant the Buyer a further period in which to remedy the relevant breach, shall apply to each occasion on which such a period of remedy expires without the Buyer having remedied that breach in accordance with clause 10.4. Seller’s right to claim for damages remains reserved.

10.6 If Seller 1 elects not to complete the sale and purchase of the Shares in accordance with clause 10.4, this Agreement shall terminate and the parties shall have no further rights or obligations under this Agreement other than accrued rights and obligations at the time of that election in respect of prior breaches thereof, save that clauses 13 through 18 shall remain binding on the parties in accordance with their terms. In these circumstances, the Prepayment Amount shall not be refundable.

11. Post-Closing Obligations of Buyer

Following Closing the Buyer agrees:

11.1 to be bound by the protective covenants safeguarding the Milestone Payments and the Royalty Payments as further set out in Annex 3.1 to this Agreement.

11.2 to procure that the sum of the Contribution pursuant to clause 4.6.1 will be applied solely for the business of the Company. Upon request by Seller 1, the Buyer shall provide the Sellers with evidence (to the Sellers’ reasonable satisfaction) of its compliance with this obligation.

11.3 that if the acquisition of SOM Pharmaceuticals S.A and SOM Therapeutics, Inc. is not completed in advance of the Closing of this Agreement, the Buyer may not acquire, and procure that no Affiliate of the Buyer acquires, SOM Pharmaceuticals S.A or SOM Therapeutics, Inc. within six months of Closing without the prior written consent of Seller 1.

12. Protective Covenants and Post-Closing Cooperation

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12.1 The Sellers covenant to the Buyer and the Company that they shall not and shall procure (to the extent legally permissible) that no member of the Company and the Seller (including their respective wholly owned subsidiaries) shall:

12.1.1 for a period of two years from Closing be concerned in any business, apart from any participation in Fastnet, carrying on business in any part of the world which is competitive with any of the businesses carried on by the Company at Closing (not including the holding of a non-controlling stake of maximum 5% in a competing company); or

12.1.2 for a period of three years from Closing induce or attempt to induce any director or senior employee of the Company to leave the employment of the Company with a view to hiring such person; or

12.1.3 make use of or (except as required by law or any competent regulatory body) disclose or divulge to any third party any information of a secret or confidential nature relating to the business or affairs of the Company or its customers or suppliers, including information which may be disclosed by the Company or the Buyer after the Closing Date; or

12.1.4 for a period of three years from Closing induce or attempt to induce any supplier of the Company to cease to supply, or to restrict or vary the terms of supply to, the Company; and

12.1.5 after Closing use any trade name used by the Company at the Closing Date or any other name intended or likely to be confused with such a trade name.

12.2 For the purposes of this clause: The covenants in this clause may be enforced by the Company with the prior written consent of the Buyer against the Sellers.

13. Confidentiality, Non-Compete

13.1 The Parties to this Agreement undertake to maintain strict silence about the contents hereof as well as about the negotiations taking place between them. The contents hereof may, however, be disclosed:

13.1.1 to employees or advisers of the respective Party who are subject to customary or professional confidentiality obligations;

13.1.2 to existing or future investors or shareholders of the Seller to the extent they are subject to customary confidentiality obligations;

13.2 If any of the Parties is obliged for mandatory legal or official reasons (such as under stock exchange rules) to disclose this Agreement or individual provisions hereof, such Party shall be permitted to do so. This confidentiality obligation shall not apply to a notification of the change of control as such within the scope of a press release jointly agreed upon between the Parties after the execution of this Agreement.

13.3 In the event that the transaction contemplated by this Agreement is not completed for any reason,

13.3.1 the Buyer hereby undertakes not to use any confidential information disclosed to it by the Sellers, including any Due Diligence information, for the purposes of competing

18

with the business of the Company as carried on by the Company at the Signing Date; or

13.3.2 for a period of two years from Closing be concerned in any business carrying on business in any part of the world which is competitive with any of the businesses carried on by the Company at Closing (not including the holding of a non-controlling stake of maximum 5% in a competing company); or

13.3.3 for a period of three years from Closing induce or attempt to induce any director or senior employee of the Company to leave the employment of the Company with a view to hiring such person; or

13.3.4 make use of or (except as required by law or any competent regulatory body) disclose or divulge to any third party any information of a secret or confidential nature relating to the business or affairs of the Company or its customers or suppliers, including information which may be disclosed by the Company or the Buyer after the Closing Date; or

13.3.5 for a period of three years from Closing induce or attempt to induce any supplier of the Company to cease to supply, or to restrict or vary the terms of supply to, the Company.

13.4 For the purposes of clause 13.3: The covenants in this clause may be enforced by the Company with the prior written consent of Seller 1 (for and on behalf of the Sellers) against the Buyer.

14. Costs

Each Party shall bear their own costs and the costs of their own advisers. The Buyer shall be responsible for any stamp duties payable in relation to this Agreement and its execution.

15. Liability of Sellers

In case of a Breach of Warranties by either Seller pursuant to clause 5 or other claims for damages of the Buyer, each Seller shall be liable for all claims of Buyer under this Agreement according to the proportion of its respective shareholding in the Company. In case of a Breach of the Warranties pursuant to clause 5.2 each Seller shall only liable for the Shares held by it.

16. Final Provisions

16.1 Any and all declarations and notices in connection herewith must be made in writing, unless notarization or another form is required under mandatory law. In order to comply with the written form requirement a sending by telefax or letter shall be sufficient but not by way of another form of telecommunication. The electronic form, including email, does not replace the written form requirement.

16.2 Notices have to be addressed to:

Seller 1: [***], Software AG-Stiftung, [***]

Seller 2: [***]

19

Buyer: [***], Amryt Pharmaceuticals Designated Activity Company, 106B Pembroke Road, Dublin 4, Ireland

Company: [***], Birken AG, [***]

16.3 A Notice shall be deemed to have been received:

16.3.1 if delivered personally, at the time of delivery; or

16.3.2 in the case of a fax, at the time of transmission; or

16.3.3 if sent by courier or registered post 48 hours from the date of posting and if deemed receipt under the previous paragraphs of this § 15 is not within business hours (meaning 9.00 am to 5.30 pm Monday to Friday on a day that is not a public holiday in the place of receipt), when business next starts in the place of receipt.

16.4 To prove service it is sufficient to prove that the Notice was transmitted by fax to the fax number of the party or, if sent by courier or registered post, that the envelope containing the Notice was properly addressed and posted.

16.5 The Parties shall notify the remaining Parties of any change of their addresses stated in this clause 16. Until the receipt of such notice by the other Party, such address shall be effective.

16.6 Buyer appoints [***] at [***] to receive service (Empfangs- und Zustellungsvollmacht) for any communication, including service in any legal proceedings, arising out of or in connection with this Agreement.

16.7 This Agreement as well as any Annexes and documents referred to herein and which are an integral part hereof include any and all agreements between the Parties with regard to the subject-matter hereof and supersede all former agreements, if any, existing in this regard. There do not exist any collateral agreements of whatever nature.

16.8 If an agreement on the place of jurisdiction is permissible, the courts of Frankfurt shall exclusively be competent for any and all disputes under and in connection with this Agreement.

16.9 This Agreement shall exclusively be subject to and governed by the substantive laws of the Federal Republic of Germany excluding the conflict of law rules. Place of jurisdiction is Frankfurt.

16.10 Any amendment of or supplement to this Agreement as well as any declarations given hereunder must be made in writing by all Parties in order to be effective, unless notarization is required. The same applies regards to any waiver of the aforementioned written form requirement.

16.11 Unless otherwise agreed herein, neither this Agreement nor any right, remedy, liability or obligation resulting therefrom shall be assigned by any of the Parties without the prior approval of the other Party.

16.12 The headings in this Agreement are for convenience purposes only and shall not affect the interpretation of any of the provisions hereof.

20

16.13 Terms to which a German translation has been added in brackets shall be interpreted as having the meaning assigned to them by the German translation.

16.14 For the purpose of this Agreement, a “Business Day” is any day on which banks are open for business in Frankfurt/Main, Germany.

16.15 Words such as “hereof”, “herein” or “hereunder” refer (unless otherwise required by the context) to this Agreement as a whole and not to a specific provision of this Agreement. The term “including” shall mean “including, without limitation”.

16.16 Wherever this Agreement refers to a contract or other agreement, such reference shall apply to and include all ancillary agreements, arrangements, amendments, side letters, waivers and other legally binding statements, if any, related thereto.

16.17 The rights of each Party under this Agreement, unless provided otherwise in this Agreement with respect to certain rights:

16.17.1 may be exercised as often as necessary;

16.17.2 are cumulative and not exclusive of rights and remedies provided by law (except regarding damage claims to the extent exclusively regulated in this Agreement to the exclusion of such other rights and remedies); and

16.17.3 may be waived only in writing and specifically

16.17.4 delay in exercising or non-exercise of any such right is not a waiver of that right.

17. Severability

In the event any provision hereof is or shall become invalid or unenforceable, the validity of the other provisions shall remain unaffected. In lieu of the invalid or unenforceable provision, such valid and enforceable provision shall be deemed to be agreed upon which closely corresponds to the intended economic purpose of the invalid or unenforceable provision. The same shall apply to any supplementary interpretation (ergänzende Vertragsauslegung) of any of the terms of this Agreement.

18. Copies of this Agreement

18.1 Each Party shall receive a signed copy of this Agreement.

18.2 All Annexes are an integral part of this Agreement. This Agreement including the Annexes was approved by the Parties and signed by them as follows:

- Signature Page follows –

21

Share Purchase and Transfer Agreement relating to the sale of the shares in BIRKEN AG

- Signature Page –

For Seller 1:
Date: 16 October 2015	Date: 16 October 2015
/s/ Dr. h.c. Peter Schnell	/s/ Markus Ziener
Dr. h.c. Peter Schnell	Markus Ziener
Vorstandsvorsitzender	Geschäftsführender Vorstand
For Seller 2:
Date: 16 October 2015
/s/ Dr. Armin Scheffler
Dr. Armin Scheffler

For Buyer:
Date: 16 October 2015	Date: 16 October 2015
/s/Joe Wiley
Name/Position:	Name/Position:

For Company:

Date: 16 October 2015

/s/ Dr. Armin Scheffler

Dr. Armin Scheffler
Vorstand

EX-3.1

Exhibit 3.1

Company Number: 12107859

THE COMPANIES ACT 2006

PUBLIC COMPANY LIMITED BY SHARES

ARTICLES OF ASSOCIATION

OF

AMRYT PHARMA PLC

(formerly known as Amryt Pharma Holdings Limited and Amryt Pharma Holdings plc)

(adopted by special resolution passed on 23 September 2019 (effective 24 September 2019))

CONTENTS

REGULATIONS AND INTERPRETATION		1
1.	Interpretation	1
2.	Model Articles not to apply and application of contractual agreements with members	4
SHARE CAPITAL		4
3.	Share Capital	4
COMPANY BUSINESS		5
4.	Business	5
VARIATION OF CLASS RIGHTS		5
5.	Sanction to variation	5
6.	Deemed variation	6
SHARES		6
7.	Allotment of shares	6
8.	Purchase of own shares	7
9.	Commission and brokerage	7
10.	Trusts not to be recognised	7
SHARE CERTIFICATES		7
11.	Share certificates	7
12.	Replacement of share certificate	8
ISSUE OF SHARES		8
13.	Uncertificated shares	8
14.	Relevant Class	9
CALLS ON SHARES		9
15.	Calls	9
16.	Payment	10
17.	Interest on calls	10
18.	Sums treated as calls	10
19.	Power to differentiate	10
20.	Payment in advance of calls	10
FORFEITURE, SURRENDER, LIEN AND UNTRACED MEMBERS		10
21.	Notice if call not paid	10
22.	Forfeiture for non-compliance	11
23.	Disposal of forfeited shares	11
24.	Effect of forfeiture	11
25.	Lien	11
26.	Enforcement of lien by sale	12
27.	Application of proceeds of sale	12
28.	Untraced members	12
29.	Evidence of forfeiture	13
TRANSFER OF SHARES		14
30.	Transfer of title and interest	14
31.	Transfer of shares	14
32.	Right to refuse registration	14
33.	Notice of refusal	14
34.	Closing of register	14
35.	Fees on registration	15
36.	Retention	15

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37.	Transfer by renunciation	15
TRANSMISSION OF SHARES		15
38.	On death	15
39.	Election of person entitled by transmission	15
40.	Transfer notice	15
41.	Rights on transmission	16
INCREASE OF CAPITAL		16
42.	Increase of Capital	16
43.	New Shares	16
ALTERATION OF CAPITAL		16
44.	Alteration	16
GENERAL MEETINGS		17
45.	Annual general meetings	17
46.	General meetings	17
47.	Notice of general meetings	17
48.	Statement	18
49.	Omission of notice	18
PROCEEDINGS AT GENERAL MEETINGS		18
50.	Business of meetings	18
51.	Notice of resolution	18
52.	Quorum	18
53.	Quorum not present	19
54.	Chairman	19
55.	Power to adjourn	19
56.	Directors may attend and speak	19
57.	Amendment	19
VOTES OF MEMBERS		20
58.	Votes	20
59.	Joint holders	20
60.	Vote by proxy	20
61.	Restriction on voting rights	20
62.	Objection to error in voting	20
63.	Votes on a poll	20
POLLS		21
64.	Method of voting	21
65.	Proxy	21
66.	Error	21
67.	Procedure on a poll	21
68.	Poll to be taken forthwith	22
69.	Casting vote	22
70.	Demand for poll	22
71.	Withdrawal	22
PROXY		22
72.	Form of Proxy	22
73.	Appointment of proxy	22
74.	Deposit of proxy	23
75.	Validity	23
76.	Supply of proxy cards	24
77.	Corporate representative	24
DISCLOSURE OF INTERESTS		24

ii

78.	Section 793	24
79.	Default	24
80.	Restrictions	25
81.	Arms length transfer	25
82.	Relevant period	26
83.	Interest in shares	26
APPOINTMENT OF DIRECTORS		26
84.	Power of Company to appoint Directors	26
85.	Power of Board to appoint Directors	26
86.	Number of Directors	26
87.	Additional remuneration	27
ALTERNATE DIRECTORS		27
88.	Appointment	27
89.	Remuneration	27
INTERESTS OF DIRECTORS		28
90.	Other office of Director	28
91.	Disqualification	28
92.	Declaration of interest	28
93.	Material interest	28
94.	Voting	29
95.	Two Directors	29
96.	Directors interests	30
97.	Interest of connected person	30
98.	Suspension of provisions	30
99.	Directors’ conflict of interest	30
100.	Benefits	31
101.	Exercise of power	32
GENERAL POWERS OF DIRECTORS		32
102.	Management	32
103.	Delegation of Authority	32
104.	Power of Attorney	33
105.	Overseas registers	33
106.	Uncalled capital	33
DIRECTORS HOLDING EXECUTIVE OFFICE		33
107.	Office	33
108.	Remuneration	34
109.	Powers	34
RETIREMENT OF DIRECTORS		34
110.	Retirement	34
111.	Vacation of office	34
112.	Resolution as to a vacancy conclusive	35
ROTATION OF DIRECTORS		35
113.	Retirement by rotation	35
114.	Retirement in every year	35
115.	Vacated office	36
116.	Appointment	36
117.	Motion	36
PROCEEDINGS OF DIRECTORS		36
118.	Meetings	36
119.	Authorisation to vote	37

iii

120.	Quorum	37
121.	Minimum number of directors	37
122.	Chairman	37
123.	Resolutions	38
124.	Committees	38
125.	Validity	38
BORROWING POWERS		38
126.	Powers	38
OTHER DIRECTORS		39
127.	Appointment	39
MINUTES AND BOOKS		39
128.	Minutes	39
129.	Records	39
SECRETARY		40
130.	Appointment	40
131.	Office	40
THE SEAL		40
132.	Safe custody	40
133.	Application	40
134.	Seal for use abroad	40
135.	Issue	41
136.	Seal	41
AUTHENTICATION OF DOCUMENTS		41
137.	Authentication	41
DIVIDENDS		41
138.	Declaration of dividends	41
139.	Dividends payable	41
140.	Payment of dividends	42
141.	Interim dividends	42
142.	Profits and losses	42
143.	Calls or debts deducted from dividends	42
144.	Retention of dividends	42
145.	Unclaimed dividends	43
146.	Payment of dividends	43
147.	Receipts for dividends	43
148.	Scrip dividends	43
149.	General meeting to declare dividend	45
150.	Reserves	45
151.	Capitalisation	45
152.	Authority	46
153.	Record Dates	46
ACCOUNTS		46
154.	Accounting records	46
155.	Preparation of accounts	47
156.	Accounts to members	47
157.	Electronic means	47
AUDITORS		47
158.	Appointment	47
159.	Correctness	47
160.	Auditors to attend meetings	48

iv

161.	Change of auditors	48
SERVICE OF NOTICE ON MEMBERS		48
162.	Notices to be in writing	48
163.	Service of notice on members	48
164.	Notice in case of death, bankruptcy or mental disorder	49
165.	Evidence of service	49
166.	Notice binding on transferees	50
167.	Notice by advertisement	50
168.	Suspension of the postal services	50
169.	Service of notices on the Company	50
ELECTRONIC COMMUNICATION		51
170.	Electronic Communication	51
DESTRUCTION OF DOCUMENTS		51
171.	Destruction	51
172.	Correct entries	52
WINDING UP		52
173.	Authority to divide assets	52
INDEMNITY		53
174.	Right to indemnity	53
175.	Power to Insure	53

v

Company Number: 12107859

THE COMPANIES ACT 2006

PUBLIC COMPANY LIMITED BY SHARES

ARTICLES OF ASSOCIATION

OF

AMRYT PHARMA PLC

(the “Company”)

(adopted by special resolution passed on 23 September 2019 (effective 24 September 2019))

REGULATIONS AND INTERPRETATION

1. Interpretation

1.1 In these Articles, if not inconsistent with the subject or context:

(a) words importing the singular number include the plural, and vice versa;

(b) words importing one gender include any gender;

(c) a reference to a person includes a body corporate and an unincorporated body of persons;

(d) a reference to any statute or provision of a statute shall include any orders, regulations or other subordinate legislation made under it and shall, unless the context otherwise requires, include any statutory modification or re-enactment of it for the time being in force;

(e) the following words and expressions shall have the following meanings, unless the context otherwise requires:

“Act” means the Companies Act 2006 (as amended);

“Articles” means the articles of association of the Company as contained in this document or as amended from time to time;

“Bank” means the bank with which the Company has its main current account from time to time;

“City Code” means the City Code on Takeovers and Mergers;

“Directors” or “Board” means the directors of the Company from time to time or a quorum of such directors present at a board meeting and “Director” shall mean any one of them;

“dividend” includes bonus;

“communication” and “electronic communication” shall have the same meaning as in the Electronic Communications Act 2000;

“electronic form” and “electronic means” shall, where the context so admits, have the same meaning as in the Act;

“executed” includes any mode of execution;

“holder” means, in relation to shares, means a member whose name is entered in the register of members as the holder of the shares;

“Office” the registered office for the time being of the Company;

“Operator” the person from time to time who in respect of the Company’s securities carries out the functions of the operation of a relevant system for the purposes of the Regulations;

“Ordinary Shares” means ordinary shares with a nominal value of £0.06 each in the share capital of the Company;

“paid up” means paid up or credited as paid up;

“Panel” means the Panel on Takeovers and Mergers in the United Kingdom;

“Privileged Relation” means in relation to a shareholder who is an individual shareholder (or a deceased or former individual shareholder) means a spouse, civil partner, (as defined in the Civil Partnerships Act 2004) child or grandchild (including step or adopted or illegitimate child and their issue);

“Redeemable Shares” means redeemable shares with a nominal value of £49,999.94 each;

“Regulations” means the Uncertificated Securities Regulations 2001 (SI 2001 no. 3755) including any modification thereof or any regulations in substitution thereof and for the time being in force;

“Relevant Liability” means any cost, charge, loss, damage, expense or liability which any person may suffer or incur:

(a) as a result of anything he does, or does not do, in carrying out or trying to carry out his duties, or using or trying to use his powers in relation to the Company, or in relation to any of the other bodies corporate which are referred to in the definition of “Relevant Person” or, in the case of any current or past trustee of any pension fund, in relation to that pension fund; or

2

(b) in any other way in connection with his duties, powers or posts in relation to the Company or in relation to any of the other bodies corporate which are referred to in the definition of “Relevant Person” or, in the case of any current or past trustee of any pension fund, in relation to that pension fund including (without prejudice to the generality of the foregoing) any liability incurred in connection with defending any proceedings (whether civil or criminal) which relate to any of the matters referred to in this definition of “Relevant Liability”;

“Relevant Person” means any person who is or was at the time a Director, alternate director, officer or employee of:

(a) the Company, or any body corporate which is or was at any time a holding company of the Company;

(b) any body corporate in which the Company, or any body corporate which is or was at any time a holding company of the Company, has any kind of direct or indirect interest;

(c) any body corporate in which any of the predecessors of the Company, or of any body corporate which is or was at any time a holding company of the Company, has any kind of direct or indirect interest;

(d) any body corporate with which the Company is or was at any time allied, or associated; or

(e) any body corporate which is or was at any time a subsidiary undertaking of any body corporate referred to in this definition;

“relevant system” means a relevant system as defined by regulation 2(1) of the Regulations;

“Seal” means the common seal of the Company or if appropriate any official seal which the Company may have pursuant to Section 50 of the Act (the “Securities Seal”);

“Secretary” means the secretary of the Company and (subject to the provisions of the Act) any other person appointed by the Directors to perform any of the duties of the secretary of the Company, including a joint assistant or deputy secretary;

“Statutes” means the Act, and every other statute (and any regulations subordinate thereto) for the time being in force concerning companies and affecting the Company;

“United Kingdom” means the United Kingdom of Great Britain and Northern Ireland;

(f) “in writing” written, or produced by any other mode of reproducing or representing words in a permanent visible form, or partly one and partly another;

3

(g) save as aforesaid, and unless the context otherwise requires, words or expressions contained in these Articles shall bear the same meaning as in the Act and words and expressions used in the Regulations have the same meanings when used in these Articles;

(h) the headings are inserted for convenience only and shall not affect the construction of these Articles;

(i) words importing the singular number only shall include the plural, and vice versa;

(j) words importing the masculine gender only shall include the feminine gender;

(k) words importing individuals and words importing persons shall include bodies corporate and unincorporated associations.

(l) the words and phrases “other”, “including” and “in particular” do not limit the generality of any preceding words and any words which follow them shall not be construed as being limited in scope to the same class as the preceding words where a wider construction is possible;

(m) subject to the provisions of Article 51, where for any purpose an ordinary resolution of the Company is required a special resolution shall also be effective;

(n) references to Articles are references to these Articles and references to paragraphs and sub-paragraphs are, unless otherwise stated, references to paragraphs of the Article or references to sub-paragraphs of the paragraph in which the reference appears.

2. Model Articles not to apply and application of contractual agreements with members

2.1 No regulations set out in any statute or in any statutory instrument or other subordinate legislation concerning companies shall apply to the Company except insofar as they are repeated or contained in these Articles. This document constitutes the Articles of the Company.

2.2 Subject to the Statutes, the Articles shall be subject to any contractual agreement entered into by the Company and any of its members (in their capacity as shareholders of the Company only).

SHARE CAPITAL

3. Share Capital

3.1 The share capital of the Company is divided into Ordinary Shares and Redeemable Shares.

3.2 The Ordinary Shares shall confer the following rights and restrictions on their holders:

(a) the right to receive notice of, attend and vote at general meetings;

4

(b) the right to participate in the profits of the Company; and

(c) the right on a winding up or return of capital or otherwise to repayment of the amounts paid up or credited as paid up on them in respect of each Ordinary Share with the Ordinary Shares conferring a right to participate in any surplus assets of the Company in proportion to the number of shares held.

3.3 The Redeemable Shares shall confer the following rights and restrictions on their holders:

(a) no right to receive notice of, attend or vote at general meetings or (subject to the Act) at any meeting of the holders of any class of shares in the capital of the Company;

(b) no right to participate in the profits of the Company by way of receipt of any dividend or distribution of profits;

(c) the right to receive, on any payment or return of capital on a winding up or other return of assets, pro rata out of the assets of the Company available for distribution the nominal capital paid up or credited as paid up on the Redeemable Shares but only after the holders of Ordinary Shares have been paid the nominal capital paid up or credited as paid up on the Ordinary Shares (including any premium) held by them respectively together with the aggregate sum of £100,000,000,000 to the holders of such Ordinary Shares; and

(d) subject to the Act, the Redeemable Shares are redeemable at their nominal value at the option of the Company or the holder of the Redeemable Shares.

3.4 Without prejudice to any special rights previously conferred on the holders of any shares or class of shares already issued (which special rights shall not be modified or abrogated except with such consent or sanction as is provided in Articles 5 or 6), a share (whether forming part of the original capital or not) may be issued with such preferred, deferred or other special rights or such restrictions, whether in regard to dividend, return of capital, voting or otherwise, as the Company by ordinary resolution determines.

COMPANY BUSINESS

4. Business

Any branch or kind of business which the Company is either expressly or by implication authorised to undertake may be undertaken by the Directors at such times as they think fit, and may be permitted by them to be in abeyance, whether the branch or kind of business commenced or not, so long as the Directors deem it expedient not to commence or proceed with it.

VARIATION OF CLASS RIGHTS

5. Sanction to variation

5.1 Subject to the provisions of the Act if at any time the capital of the Company is divided into different classes of shares or groups, the rights attached to any class or group may be varied or abrogated, whether or not the Company is being wound up, either:

5

(a) in such manner (if any) as may be provided by such rights; or

(b) in the absence of any such provisions with the consent in writing of the holders of three-quarters in nominal value of the issued shares of that class or group, or with the sanction of a special resolution passed at a separate general meeting of the holders of the shares of the class or group, but not otherwise.

5.2 To every such separate general meeting of the holders of a class or group of shares all the provisions of these Articles relating to general meetings of the Company or to the proceedings at such general meetings shall, so far as applicable and with the necessary modifications, apply, except that:

(a) the necessary quorum at any such meeting other than an adjourned meeting shall be two persons holding or representing by proxy at least one-third in nominal value of the issued shares of the class or group in question and at an adjourned meeting one person holding shares of the class or group in question or his proxy;

(b) any holder of shares of the class or group in question present in person or by proxy may demand a poll; and

(c) the holders of shares of the class or group in question shall, on a poll, have one vote in respect of every share of the class or group held by them respectively.

6. Deemed variation

The special rights conferred upon the holders of any class or group of shares issued with preferred or other special rights shall not (unless otherwise expressly provided by these Articles or by the conditions of issue of such shares) be deemed to be varied by the creation or issue of further shares ranking in some or all respects pari passu with them.

SHARES

7. Allotment of shares

7.1 Subject to Article 7.2 and to any direction to the contrary given by the Company in general meeting, the shares and any right to subscribe for, or to convert any security into, shares in the Company for the time being (other than shares shown in the memorandum of association of the Company to have been taken by the subscribers or shares allotted in pursuance of an employee’s share scheme) may be allotted to such persons, at such times, in such proportions, upon such terms (other than at a discount) and with such rights or restrictions, including but without limit as to differentiation between members of calls, as the Directors, subject to the Articles and to the provisions of the Act shall think fit.

7.2 The Company may in accordance with and subject to sections 684 to 689 of the Act and all other relevant provisions (if any) in force for the time being:

(a) issue shares which are to be redeemed or are liable to be redeemed at the option of the Company or the holder thereof;

6

(b) make a payment in respect of the redemption or purchase of any of its own paid-up shares out of the distributable profits of the Company or the proceeds of a fresh issue of shares and as to redemption on such date or dates (to be fixed prior to the issue of such shares) and terms and in such manner as may be determined at any time or times by the Directors, provided nevertheless that the amount to be paid on redemption shall be fixed on, and by the terms of, the issue of the shares;

provided always that any shares purchased or redeemed by the Company shall be treated as cancelled and that within one month of the redemption of any redeemable shares the Company gives notice to the registrar specifying the shares redeemed.

8. Purchase of own shares

Subject to the provisions of the Act and, if applicable, subject to any approval by means of a special resolution at a separate class meeting of the holders of any class of convertible shares, the Company shall have power to purchase its own shares, including any redeemable shares.

9. Commission and brokerage

In addition to all other powers of paying commission, the Company may exercise the powers conferred by the Act in paying commission to persons subscribing or procuring subscriptions for shares in the Company, or agreeing so to do, whether absolutely or conditionally; provided that the rate or the amount of the commission paid or agreed to be paid shall be disclosed in the manner required by the Act and shall not exceed ten per cent of the price which the shares in respect of which the commission is paid are issued or an amount equivalent thereto. Subject to the provisions of the Act any such commission may be satisfied by the payment of cash or by the allotment or fully or partly paid shares or partly in one way and partly in the other. The Company may also, on any issue of shares, pay such brokerage as is lawful.

10. Trusts not to be recognised

Except as required by law (including, without limitation, the Regulations), no person shall be recognised by the Company as holding any share upon any trust, and the Company shall not be bound by or compelled in any way to recognise (even when having notice) any equitable, contingent, future or partial interest in any share, or (except only as provided by these Articles or as required by law) any interest in any fractional part of a share or any other right in respect of any share, except an absolute right to the entirety of it in the registered holder.

SHARE CERTIFICATES

11. Share certificates

11.1 Every person whose name is entered as a member in the register of members (except a recognised clearing house or a nominee thereof or other person in respect of whom the Company is not by law required to complete and have ready for delivery a certificate) shall be entitled without payment to one certificate for all his shares of each class. Every certificate shall be issued within two months after allotment or the lodgement

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with the Company of the transfer of the shares, not being a transfer which the Company is for any reason entitled to refuse to register and does not register (unless the conditions of issue of such shares otherwise provide), and shall specify the number and class and distinguishing numbers (if any) of the shares to which it relates and the amount paid up thereon. The Company shall not be bound to register more than four persons as the joint holders of any share or shares and, in the case of a share held jointly by several persons, the Company shall not be bound to issue more than one certificate therefor, and delivery of a certificate for shares to the first named joint holders shall be sufficient delivery to all. Where a member transfers part of the shares comprised in his holding he shall be entitled to a certificate for the balance of his holding without charge.

11.2 Any share certificate and any certificate for debentures of the Company which has been approved for sealing by the Directors or a committee of the Directors need not (save to the extent that the terms and conditions for the time being relating to any debentures of the Company otherwise require) be signed or countersigned by any person. Subject as aforesaid, any such certificate may, if the Directors so determine, bear signatures affixed by some mechanical system or process or printed on them or the names of the Company’s issuing agents and need not be signed by any person.

12. Replacement of share certificate

If a share certificate is defaced, worn out, lost or destroyed, it may be replaced on such terms (if any) as to evidence and indemnity and the payment of any exceptional out of pocket expenses incurred by the Company in investigating evidence as the Directors think fit but otherwise free of charge and (in case of defacement or wearing out) on delivery up of the old certificate.

ISSUE OF SHARES

13. Uncertificated shares

13.1 In these Articles references to a share (or to holding of shares) being in uncertificated form or in certificated form are references, respectively, to that share being an uncertificated unit of a security or a certificated unit of a security.

13.2 The Directors shall have power to implement such arrangements as they may, in their absolute discretion, think fit in order for any class of shares to be a participating security (subject always to the Regulations and the facilities and requirements of the relevant system concerned). Where they do so the following Article shall commence to have effect immediately prior to the time at which the Operator concerned permits the class of shares concerned to be a participating security.

13.3 In relation to any class of shares which is, for the time being, a participating security, and for so long as such class remains a participating security, no provision of these Articles shall apply or have effect to the extent that it is in any respect inconsistent with:

(a) the holding of shares of that class in uncertificated form;

(b) the transfer of title to shares of that class by means of a relevant system; or

(c) the Regulations.

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14. Relevant Class

14.1 Without prejudice to the generality of the preceding Article and notwithstanding anything contained in these Articles, where any class of shares is, for the time being, a participating security (such class being referred to hereinafter as the “Relevant Class”):

(a) the register relating to the Relevant Class shall be maintained at all times in the United Kingdom;

(b) shares of the Relevant Class may be issued in uncertificated form in accordance with and subject as provided in the Regulations;

(c) unless the Directors otherwise determine, shares of the Relevant Class held by the same holder or joint holder in certificated form and uncertificated form shall be treated as separate holdings;

(d) shares of the Relevant Class may be changed from uncertificated to certificated form, and from certificated to uncertificated form, in accordance with and subject as provided in the Regulations;

(e) title to shares of the Relevant Class which are recorded on the register as being held in uncertificated form may be transferred by means of the relevant system concerned and accordingly none of the provisions of these Articles shall apply in respect of such shares to the extent that any provision requires or contemplates the effecting of a transfer by an instrument in writing and the production of a certificate for the share to be transferred;

(f) the Company shall comply with the provisions of Regulations 27 and 28 in relation to the Relevant Class and all provisions in these Articles shall be read as subject to Regulation 28;

(g) the provisions of these Articles with respect to meetings of or including holders of the Relevant Class, including notices of such meetings, shall have effect subject to the provisions of Regulation 41; and

(h) no provision of these Articles shall apply so as to require the Company to issue a certificate to any person holding shares of the Relevant Class in uncertificated form.

CALLS ON SHARES

15. Calls

The Directors may, subject to the provisions of these Articles and to any conditions of allotment, from time to time make calls upon the members in respect of any monies unpaid on their shares (whether on account of the nominal amount of the shares or by way of premium) and each member shall (subject to being given at least 14 days’ notice specifying the time or times and place of payment) pay to the Company at the time or times and place so specified the amount called on his shares.

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16. Payment

A call may be made payable by instalments. A call may be postponed and a call may be wholly or in part revoked as the Directors may determine. A call shall be deemed to have been made at the time when the resolution of the Directors authorising the call was passed. The joint holders of a share shall be jointly and severally liable to pay all calls in respect of it. A person upon whom a call is made shall remain liable for calls made upon him notwithstanding the subsequent transfer of the shares in respect of which the call was made.

17. Interest on calls

If a sum called in respect of a share is not paid before or on the day appointed for its payment, the person from whom the sum is due shall pay interest on the sum from the day appointed for payment to the time of actual payment at the rate fixed by the terms of allotment of the share or in the notice of the call or, if no rate is so fixed, at the base rate from time to time of the Bank or at such lower rate as the Directors may agree to accept, but the Directors shall be at liberty to waive payment of such interest wholly or in part.

18. Sums treated as calls

Any sum which by the terms of issue of a share becomes payable upon allotment or at any fixed date, whether on account of the nominal amount of the share or by way of premium or as an instalment of a call, shall, for all the purposes of these Articles, be deemed to be a call duly made and payable on the date on which by the terms of issue the same becomes payable, and, in case of non-payment, all the relevant provisions of these Articles as to payment of interest, forfeiture or otherwise shall apply as if such sum had become payable by virtue of a call duly made and notified.

19. Power to differentiate

Subject to the terms of allotment, the Directors may, on the issue of shares, differentiate between the holders in the amount of calls to be paid and in the times of payment.

20. Payment in advance of calls

The Directors may, if they think fit, receive from any member willing to advance the same all or any part of the money unpaid upon the shares held by him beyond the sums actually called upon as a payment in advance of calls, and any such payment in advance of calls shall extinguish, so far as the same shall extend but subject as in these Articles provided, the liability upon the shares in respect of which it is advanced; and upon the money so received, or so much of it as from time to time exceeds the amount of the calls then made upon the shares in respect of which it has been received, the Company may pay interest at such rate not exceeding the base rate from time to time of the Bank as the member paying such sum and the Directors agree.

FORFEITURE, SURRENDER, LIEN AND UNTRACED MEMBERS

21. Notice if call not paid

If a member fails to pay any call or instalment of a call on the day appointed for its

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payment, the Directors may at any time after such date, during such time as any part of such call or instalment remains unpaid, serve a notice on him requiring payment of so much of the call or instalment as is unpaid, together with any interest which may have accrued. The notice shall name a further day (not earlier than 7 days from the date of service of such notice) on or before which and the place where the payment required by the notice is to be made, and shall state that in the event of non-payment at or before the time and at the place appointed the shares on which the call was made will be liable to be forfeited.

22. Forfeiture for non-compliance

If the requirements of any such notice as referred to are not complied with, any share in respect of which such notice has been given may at any time after service of such notice, before payment of all calls and interest due has been made, be forfeited by a resolution of the Directors to that effect, and such forfeiture shall include all dividends which shall have been declared on the forfeited shares and not actually paid before the forfeiture. The Directors may accept a surrender of any shares liable to be forfeited under this Article.

23. Disposal of forfeited shares

Subject to the provisions of the Act, a share so forfeited or surrendered shall become the property of the Company and may be sold, re-allotted or otherwise disposed of either to the person who was before such forfeiture or surrender the holder or entitled to such shares, or to any other person, upon such terms and in such manner as the Directors shall think fit. At any time before a sale, re-allotment or disposal, the forfeiture or surrender may be cancelled on such terms as the Directors think fit. The Directors may, if they think fit, authorise some person to execute an instrument or transfer of a forfeited or surrendered share to such any other person.

24. Effect of forfeiture

A member whose shares have been forfeited or surrendered shall cease to be a member in respect of the forfeited or surrendered shares and shall surrender to the Company for cancellation the certificate for the share forfeited, but shall notwithstanding such forfeiture or surrender remain liable to pay to the Company all monies which at the date of forfeiture or surrender were presently payable by him to the Company in respect of the shares, with interest, unless and to the extent that the Directors resolve to waive interest, at the rate at which interest was payable on those monies before the forfeiture or, if no interest was so payable, at the base rate from time to time of the Bank or at such lower rate as the Directors may agree to accept from the date of forfeiture or surrender until payment, and the Directors may enforce payment without any allowance for the value of the shares at the time of forfeiture or surrender or for any consideration received on their disposal.

25. Lien

Subject to the provisions of Section 670 of the Act the Company shall have a first and paramount lien on every share (not being a fully paid share) for all monies, whether presently payable or not, called or payable at a fixed time in respect of such share. The Company’s lien (if any) on a share shall extend to all dividends or other monies payable

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on or in respect of such shares together with any interest or expenses which may have accrued. The Directors may resolve that any share shall for some specified period be wholly or in part exempt from the provisions of this Article.

26. Enforcement of lien by sale

The Company may sell, in such manner as the Directors think fit, any shares on which the Company has a lien, but no sale shall be made unless some sum in respect of which the lien exists is presently payable, nor until the expiration of 14 days after a notice in writing, stating and demanding payment of the sum presently payable, and giving notice of intention to sell in default, shall have been served on the holder for the time being of the shares or the person entitled by reason of his death or bankruptcy to the shares.

27. Application of proceeds of sale

The net proceeds of such sale, after payment of the relevant costs, shall be applied in or towards payment or satisfaction of the debt or liability in respect of which the lien exists, so far as the same is presently payable, and any residue shall (upon surrender to the Company for cancellation of the certificate for the shares sold and subject to a like lien for debts or liabilities not presently payable as existed upon the shares prior to the sale) be paid to the person entitled to the shares at the time of the sale. For giving effect to any such sale, the Directors may authorise some person to execute an instrument of transfer of the shares sold to, or in accordance with, the directions of the purchaser of such shares. The purchaser shall be registered as the holder of the shares and he shall not be bound to see the application of the purchase money and his title to the shares shall not be affected by any irregularity or invalidity in the proceedings in reference to the sale.

28. Untraced members

28.1 The Company shall be entitled to sell at the best price reasonably obtainable in such manner and for such price as the Directors think fit any share of a member, or any share to which a person is entitled by transmission on death or bankruptcy, if and provided that:

(a) during the period of 12 years prior to the date of the publication of the advertisements referred to in sub-paragraph (b) (or, if published on different dates, the earlier or earliest date) no cheque, order or warrant in respect of such share sent by the Company through the post in a pre-paid envelope addressed to the member or to the person entitled by transmission to the share, at his address on the register or other last known address given by the member or person to which cheques, orders or warrants in respect of such share are to be sent has been cashed and the Company has received no communications in respect of such share from such member or person, provided that during such period of 12 years the Company has paid at least three cash dividends (whether interim or final) and no such dividend has been claimed by the person entitled to it;

(b) on or after expiry of the said period of 12 years the Company has given notice of its intention to sell such share by advertisements in two newspapers of which one shall be a national newspaper published in the United Kingdom and the other shall be a newspaper circulating in the area of the address on the register

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or other last known address of the member or the person entitled by transmission to the share or the address for the service of notices otherwise notified by a member or transferee to the Company;

(c) the said advertisements, if not published on the same day, shall have been published within thirty days of each other; and

(d) during the further period of three months following the date of publication of the said advertisements (or, if published on different dates, the later or latest date) and prior to the exercise of the power of sale the Company has not received any communication in respect of such share from the member or person entitled by transmission.

28.2 To give effect to any sale of shares pursuant to this Article, the Directors may authorise some person to transfer the shares in question and may enter the name of the transferee in respect of the transferred shares in the register notwithstanding the absence of any share certificate being lodged in respect thereof and may issue a new certificate to the transferee. An instrument of transfer executed by (or a dematerialised instruction given by) that person shall be as effective as if it had been executed or effected by the holder of, or the person entitled by transmission to, the shares. The purchaser shall not be bound to see to the application of the purchase monies, nor shall his title to the shares be affected by any irregularity or invalidity in the proceedings relating to the sale.

28.3 If during the period of 12 years referred to in Article 28.1, or during any period ending on the date when all the requirements of sub-paragraphs 28.1(a) to 28.1(d) have been satisfied, any additional shares have been issued in respect of those held at the beginning of, or previously so issued during, any such period and all the requirements of sub-paragraphs 28.1(a) to 28.1(d) have been satisfied in regard to such additional shares, the Company shall also be entitled to sell the additional shares.

28.4 The Company shall account to the member or other person entitled to such share for the net proceeds of such sale by carrying all monies in respect of such sale to a separate account. The Company shall be deemed to be a debtor to, and not a trustee for, such member or other person in respect of such monies. Monies carried to such separate account may either be employed in the business of the Company or invested in such investments as the Directors may from time to time think fit. No interest shall be payable to such member or other person in respect of such monies and the Company shall not be required to account for any money earned on them.

29. Evidence of forfeiture

A statutory declaration in writing that the declarant is a director of the Company or the Secretary and that a share has been duly forfeited, surrendered or sold, whether to satisfy a lien of the Company or otherwise on a date stated in the declaration, shall be conclusive evidence of the facts stated in such declaration as against all persons claiming to be entitled to the share. Such declaration and the receipt of the Company for the consideration (if any) given for the share on its sale, re-allotment or disposal, together with the share certificate delivered to the relevant purchaser or allottee, shall (subject to the execution of an instrument of transfer if the same be required) constitute a good title to the share and the person to whom the share is sold, re-allotted or disposed of shall be registered as the holder of the share and shall not be bound to see to the

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application of the consideration (if any) nor shall his title to the share be affected by any irregularity or invalidity in the proceedings in reference to the forfeiture, surrender, sale, re-allotment or disposal of the share.

TRANSFER OF SHARES

30. Transfer of title and interest

Title to and interest in shares may be transferred without a written instrument in accordance with statutory regulations from time to time made under the Act.

31. Transfer of shares

Transfer of shares may be effected by transfer in writing in any usual or common form or in any other form acceptable to the Directors or as required by any rules from time to time made by the Operator. The instrument of transfer shall be signed by or on behalf of the transferor and (except in the case of fully paid shares) by or on behalf of the transferee. The transferor shall be deemed to remain the holder of the share until the name of the transferee is entered on the register of members in respect of it.

32. Right to refuse registration

32.1 The Directors may decline to recognise any instrument of transfer, unless:

(a) the instrument of transfer duly stamped is deposited at the Office or such other place as the Directors may appoint, accompanied by the certificate for the shares to which it relates and such other evidence as the Directors may reasonably require to show the right of the transferor to make the transfer, provided that, in the case of a transfer by a nominee of a recognised clearing house or of a recognised investment exchange, the lodgement of a share certificate will only be necessary if a certificate has been issued in respect of the share in question;

(b) the instrument of transfer is in respect of only one class of share;

(c) the instrument of transfer is in favour of not more than four transferees; and

(d) the instrument of transfer is in respect of a share in respect of which all sums presently payable to the Company have been paid;

provided that the Directors shall not refuse to register any transfer or renunciation of partly paid shares in breach of the AIM Rules for Companies published by the London Stock Exchange plc from time to time (if applicable).

33. Notice of refusal

If the Directors refuse to register a transfer of any shares, they shall, within 2 months after the date on which the transfer was lodged with the Company or the Operator as the case may be, send to the transferor and the transferee notice of the refusal.

34. Closing of register

The registration of transfers of shares or of any class of shares may be suspended at

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such time and for such periods as the Directors may from time to time determine, provided always that the register of members shall not be closed for more than 30 days in any year.

35. Fees on registration

No fee will be charged by the Company in respect of the registration of any instrument of transfer, probate, letters of administration, certificate of marriage or death, stop notice or power of attorney or other document relating to or affecting the title to any shares or otherwise for making any entry in the register of members relating to or affecting the title to any shares.

36. Retention

All instruments of transfer which shall be registered may be retained by the Company, but any instrument of transfer which the Directors refuse to register shall (except in any case of fraud) be returned to the person depositing the same.

37. Transfer by renunciation

Nothing in these Articles shall preclude the Directors from recognising a renunciation of the allotment of any share by the allottee in favour of some other persons.

TRANSMISSION OF SHARES

38. On death

In the case of the death of a member the survivors or survivor where the deceased was a joint holder, and the legal personal representatives of the deceased where he was a sole or only surviving holder, shall be the only persons recognised by the Company as having any title to his interest in the shares, but nothing in this Article shall release the estate of a deceased holder (whether sole or joint) from any liability in respect of any share held by him.

39. Election of person entitled by transmission

Any person becoming entitled to a share in consequence of the death or bankruptcy of a member may, upon such evidence as to his title being produced as may from time to time be required by the Directors and subject as provided in these Articles, elect either to be registered himself as a holder of the share or to have some person nominated by him registered as the transferee.

40. Transfer notice

If the person so becoming entitled shall elect to be registered himself, he shall deliver or send to the Company a notice in writing signed by him stating that he so elects. If he shall elect to have another person registered, he shall testify his election by executing a transfer of the share in favour of that person. All the limitations, restrictions and provisions of these Articles relating to the right to transfer and the registration of transfers of shares shall be applicable to any such notice or transfer as if the death or bankruptcy of the member had not occurred and the notice or transfer were a transfer signed by such member.

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41. Rights on transmission

Save as otherwise provided by or in accordance with these Articles, a person becoming entitled to a share in consequence of the death or bankruptcy of a member shall (upon supplying to the Company such evidence as the Directors may reasonably require as to his title to the share) be entitled to receive and may give a discharge for all benefits arising or accruing on or in respect of the share, but he shall not be entitled in respect of that share to receive notices of or to attend or vote at general meetings of the Company or at any separate meeting of the holders of any class of shares in the Company nor to any of the rights or privileges of a member, until he shall have become a member in respect of the share provided always that the Directors may at any time give notice requiring any such person to elect either to be registered himself or to transfer the share, and if within 60 days the notice is not complied with such person shall (but only in the case of a share which is fully paid up) be deemed to have elected to be registered as a member in respect of such share and may be registered accordingly.

INCREASE OF CAPITAL

42. Increase of Capital

The Company may from time to time by ordinary resolution increase its capital by such sum to be divided into shares of such amounts and carrying such rights as the resolution may prescribe.

43. New Shares

All new shares shall (unless the Company shall in general meeting otherwise determine) be subject to the provisions of these Articles with reference to payment of calls, forfeiture, surrender, lien, transfer, transmission and otherwise, and unless otherwise provided by or pursuant to these Articles or by the conditions of issue the new shares shall upon issue be ordinary shares.

ALTERATION OF CAPITAL

44. Alteration

44.1 The Company may by ordinary resolution:

(a) consolidate and divide all or any of its share capital into shares of larger amount than its existing shares; whenever as a result of any consolidation of shares any member would become entitled to a fraction of a share, the Directors may for the purpose of eliminating such fractions sell the shares representing the fractions for the best price reasonably obtainable to any person including, subject to the provisions of the Act, the Company and distribute the proceeds of sale in due proportion among the members who would have been entitled to the fractions of shares, or retain such proceeds for the benefit of the Company and for the purpose of any such sale the Directors may authorise some person to transfer the shares representing the fractions to the purchaser thereof whose name shall then be entered in the register of members as the holder of the shares, and who shall not be bound to see to the application of the purchase money nor

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shall his title to the shares be affected by any irregularity or invalidity in the proceedings relating to the sale;

(b) cancel any shares which, at the date of the passing of the resolution, have not been taken, or agreed to be taken, by any person, and diminish the amount of share capital by the amount of the shares so cancelled;

(c) sub-divide its shares, or any of them, into shares of smaller amount than is fixed by the Memorandum of Association (subject nevertheless to the provisions of the Act) and so that the resolution by which any share is subdivided may determine that, as regards each share so subdivided, one or more of the shares resulting from such subdivision may have any such preferred or other special rights over, or may have such deferred rights, or be subject to any such restrictions as compared with the others, as the Company has power to attach to unissued or new shares.

44.2 The Company may by special resolution reduce its share capital and any capital redemption reserve and any share premium account in any manner subject to the provisions of the Act.

GENERAL MEETINGS

45. Annual general meetings

The Company shall in each year hold a general meeting as its annual general meeting in addition to any other meetings in that year and within six months of the end of any financial period provided that not more than 15 months shall elapse between the date of one annual general meeting of the Company and that of the next. Subject as aforesaid and to the provisions of the Act, the annual general meeting shall be held at such time and place as the Directors may determine.

46. General meetings

The Directors may whenever they think fit, and shall on requisition in accordance with the Act, proceed to convene a general meeting.

47. Notice of general meetings

47.1 Subject to the provisions of the Act, an annual general meeting shall be called by 21 days’ notice at the least, and all other general meetings shall be called by 14 days’ notice at the least (exclusive in each case of the day on which the notice is served or deemed to be served and of the day for which the notice is given).

47.2 Every notice shall be in writing and shall specify the place, the day and the time of meeting, the general nature of such business, and in the case of an annual general meeting shall specify the meeting as such. Notice in writing includes notices given by electronic communication to an address notified for that purpose to the Company and/or making such notices available on the Company’s website subject to notifying the address of such website to members who have agreed that notices of meetings may be accessed by them on a website and then in accordance with the manner agreed by such members and the Company as to such notification.

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47.3 Notices shall be given in accordance with these Articles to all the members, other than those who under the provisions of these Articles or the conditions of issue of the shares held by them are not entitled to receive the notice, to the Directors (including the alternate directors) and to the auditors for the time being and (where required by the Act) former auditors of the Company.

48. Statement

In every notice calling a meeting of the Company there shall appear with reasonable prominence a statement that a member entitled to attend and vote is entitled to appoint one or more proxies to attend and vote instead of him and that a proxy need not also be a member.

49. Omission of notice

The accidental omission to give notice of a meeting to (or to send a form of proxy with such notice where required), or the non-receipt of notice or form of proxy by, any person entitled to receive notice shall not invalidate the proceedings at that meeting.

PROCEEDINGS AT GENERAL MEETINGS

50. Business of meetings

All business shall be deemed special that is transacted at a general meeting, and also all business that is transacted at an annual general meeting, with the exception of the declaration of dividends, the consideration of accounts and of the reports of the directors and of the auditors and other documents annexed to the accounts, the appointment or reappointment of directors in the place of those retiring by rotation or otherwise, the reappointment of the auditors (save where special notice of such reappointment is required by the Act) and the fixing of the remuneration of the auditors or of the manner in which such remuneration is to be fixed and the giving, varying, revoking or renewing of any authority or person for the purposes of sections 549, 551 and 559 of the Act.

51. Notice of resolution

Where, by any provision contained in the Act, special notice is required of a resolution, the resolution shall not be effective unless notice of the intention to move it has been given to the Company not less than 28 days (or such shorter period as the Act permit) before the meeting at which it is moved, and the Company shall give to the members notice of any such resolution as required by and in accordance with the provisions of the Act.

52. Quorum

No business shall be transacted at any general meeting unless a quorum is present when the meeting proceeds to business. Save as otherwise provided in these Articles, two members present in person or by proxy and entitled to vote at the meeting shall be a quorum for all purposes.

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53. Quorum not present

If within 30 minutes from the time appointed for the meeting a quorum is not present, the meeting, if convened on the requisition of or by members, shall be dissolved. In any other case it shall stand adjourned to the same day in the next week at the same time and place or to such other day, and at such time and place, as the Directors may determine, and if at such adjourned meeting a quorum is not present within 30 minutes from the time appointed for holding the meeting, the meeting shall be dissolved.

54. Chairman

The chairman (if any) of the board of directors, or in his absence the deputy chairman (to be chosen, if there be more than one, by agreement amongst them or, failing agreement, by lot) or in the absence of any deputy chairman the vice-chairman (to be chosen, if there be more than one, by agreement amongst them or, failing agreement, by lot) shall preside as chairman at every general meeting of the Company, but if at any meeting neither such chairman nor such deputy chairman nor such vice-chairman be present within five minutes after the time appointed for holding the meeting, or if none of them be willing to act as chairman, the Directors present shall choose some Director present to be chairman, or if no Director be present, or if all the Directors present decline to take the chair, the members present shall choose some other member present to be chairman.

55. Power to adjourn

The chairman of any meeting at which a quorum is present may, with the consent of such meeting (and shall if so directed by the meeting) adjourn the meeting from time to time and from place to place, but no business shall be transacted at any adjourned meeting except business which might lawfully have been transacted at the meeting from which the adjournment took place. However, without prejudice to any other power which the chairman may have under these Articles or at common law, he may, without the need for the consent of the meeting, interrupt or adjourn any meeting from time to time and from place to place or for an indefinite period if he is of the opinion that it has become necessary to do so in order to secure the comfort, safety and security of those attending and the proper and orderly conduct of the meeting or to give all persons entitled to do so a reasonable opportunity of attending, speaking and voting at the meeting. When a meeting is adjourned for 30 days or more, notice of the adjourned meeting shall be given as in the case of an original meeting, but it shall not be necessary to specify in such notice the nature of the business to be transacted at the adjourned meeting. Save as set out in this Article it shall not be necessary to give any notice of an adjournment.

56. Directors may attend and speak

A Director and an alternate director shall, notwithstanding that he is not a member, be entitled to attend and speak at any general meeting and at any separate meeting of the holders of any class or group of shares of the Company.

57. Amendment

If an amendment shall be proposed to any resolution under consideration but shall in

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good faith be ruled out of order by the chairman of the meeting the proceedings on any substantive resolution shall not be invalidated by any error in such ruling.

VOTES OF MEMBERS

58. Votes

Subject to any special rights or restrictions as to voting attached to any shares by or in accordance with these Articles, on a show of hands every member who (being an individual) is present in person or by proxy not being himself a member or (being a corporation) is present by a representative or by proxy not being himself a member shall have one vote and on a poll every member who is present in person or by proxy shall have one vote for every share of which he is the holder.

59. Joint holders

In the case of joint holders of a share, the vote of the senior who tenders a vote, whether in person or by proxy, shall be accepted to the exclusion of the votes of the other joint holders, and for this purpose seniority shall be determined by the order in which the names stand in the register of members in respect of the share.

60. Vote by proxy

A member suffering from mental disorder in respect of whom an order has been made or a direction or authority given by a court of competent jurisdiction may vote, whether on a show of hands or on a poll, by his receiver, curator bonis or other person authorised in that behalf appointed by such court and such receiver, curator bonis or other person may on a poll vote by proxy, provided that such evidence as the Directors may require of the authority of the person claiming to vote shall have been deposited at the place at which proxies for the meeting in question are to be deposited under Article 74 not less than 48 hours before the time for holding the meeting or adjourned meeting at which such person claims to vote and in default the right to vote shall not be exercisable.

61. Restriction on voting rights

No member shall, unless the Directors otherwise determine, be entitled to vote at any general meeting or at any separate meeting of the holders of any class of shares in the Company either personally or by proxy, or to exercise any privilege as a member, unless all calls or, other sums presently payable by him in respect of shares in the Company have been paid.

62. Objection to error in voting

No objection shall be raised to the qualification of any voter except at the meeting or adjourned meeting at which the vote objected to is given or tendered, and every vote not disallowed at such meeting shall be valid for all purposes. Any such objection made in due time shall be referred to the chairman of the meeting whose decision shall be final and conclusive.

63. Votes on a poll

On a poll, votes may be given either personally or by proxy. On a poll, a member

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entitled to more than one vote need not, if he votes, use all his votes or cast all the votes he uses in the same way.

POLLS

64. Method of voting

64.1 At any general meeting a resolution put to the vote of the meeting shall be decided on a show of hands, unless before or upon the declaration of the result of the show of hands a poll is demanded:

(a) by the chairman of the meeting;

(b) by not less than 5 members having the right to vote at the meeting;

(c) by a member or members representing not less than one tenth of the total voting rights of all the members having the right to vote at the meeting; or

(d) by a member or members holding shares in the Company conferring a right to vote at the meeting, being shares on which an aggregate sum has been paid up equal to not less than one tenth of the total sum paid up on all the shares conferring that right.

64.2 Unless a poll be so demanded a declaration by the chairman of the meeting that a resolution has been carried, or carried unanimously, or by a particular majority, or lost, or not carried by a particular majority, and an entry to that effect in the book containing the minutes of the proceedings of general meetings of the Company, shall be conclusive evidence of the fact without proof of the number or proportion of the votes recorded in favour of or against such resolution.

65. Proxy

The instrument appointing a proxy to vote at a meeting shall be deemed also to confer authority to demand or join in demanding a poll, and for the purposes of the last preceding Article a demand by a person as proxy for a member shall be the same as a demand by the member.

66. Error

If any votes shall be counted which ought not to have been counted, or might have been rejected, or if any votes shall not be counted which ought to have been counted, or might have been allowed, the error shall not vitiate the result of the voting unless it be pointed out at the same meeting, or at any adjournment thereof, and not in that case unless it shall in the opinion of the chairman of the meeting be of sufficient magnitude to vitiate the result of the voting.

67. Procedure on a poll

If a poll is duly demanded, it shall be taken in such manner as the chairman of the meeting may direct (including the use of ballot or voting papers or forms), and the result of a poll shall be deemed to be the resolution of the meeting at which the poll was demanded. The chairman of the meeting may, in the event of a poll, appoint scrutineers

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(who need not be members) and may fix some place and time for the purpose of declaring the result of the poll.

68. Poll to be taken forthwith

A poll demanded on the election of a chairman or on a question of adjournment shall be taken forthwith. A poll demanded on any other question shall be taken at such time and place as the chairman of the meeting shall direct not being more than 30 days from the date of the meeting or the adjourned meeting at which the poll was demanded. No notice need be given of a poll not taken forthwith if the time and place at which it is to be taken are announced at the meeting at which it is demanded. In any other case at least seven days’ notice shall be given specifying the time and place at which the poll is to be taken.

69. Casting vote

In the case of an equality of votes, whether on a show of hands or on a poll, the chairman of the meeting at which the show of hands takes place or at which the poll is demanded shall be entitled to a casting vote in addition to any other vote he may have.

70. Demand for poll

The demand for a poll shall not prevent the continuance of a meeting for the transaction of any business other than the question on which the poll has been demanded.

71. Withdrawal

A demand for a poll may, before the poll is taken, be withdrawn but only with the consent of the chairman and a demand so withdrawn shall not be taken to have invalidated the result of a show of hands declared before the demand was made. If a poll is demanded before the declaration of the result of a show of hands and the demand is duly withdrawn with the consent of the chairman, the meeting shall continue as if the demand had not been made.

PROXY

72. Form of Proxy

Any person (whether a member or not) may be appointed to act as a proxy. A member may appoint more than one proxy to attend on the same occasion. When two or more valid but differing instruments of proxy are delivered in respect of the same share for use at the same meeting and in respect of the same matter, the one which is last validly delivered (regardless of its date or the date of its execution) shall be treated as replacing and revoking the other or others as regards that share. If the Company is unable to determine which instrument was last validly delivered, none of them shall be treated as valid in respect of that share.

73. Appointment of proxy

The appointment of a proxy shall be in writing in the usual common form, or such other form as may be approved by the Directors, and shall be signed by the appointor or by his attorney duly authorised in writing, or if the appointor is a corporation, shall be

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either under its common seal or under the hand of a duly authorised officer or attorney of the corporation. The Directors may, but shall not be bound to, require evidence of authority of such officer or attorney. An instrument of proxy need not be witnessed.

74. Deposit of proxy

74.1 The appointment of a proxy together with (unless the Directors waive such requirement) the power of attorney or other authority (if any) under which it is signed, or a notarially certified or office copy of such power or authority, shall:

(a) in the case of an instrument in writing be deposited at the Office, or at such other place in the United Kingdom as is specified for that purpose in the notice calling the meeting, or in any instrument of proxy sent out by the Company in relation to the meeting, not less than 48 hours (excluding weekends and bank holidays) before the time appointed for holding the meeting or adjourned meeting at which the person named in the instrument proposes to vote; or

(b) in the case of an appointment contained in an electronic communication, where an address has been specified for the purpose of receiving electronic communications, in the notice convening the meeting, or in any instrument of proxy sent out by the Company in relation to the meeting, or in any invitation contained in an electronic communication to appoint a proxy issued by the Company in relation to the meeting, to be received at such address not less than 48 hours (excluding weekends and bank holidays) before the time for holding the meeting or adjourned meeting at which the person named in the appointment proposes to vote,

and in default, the appointment shall not be treated as valid. An appointment of proxy to vote at any meeting and deposited, delivered or received as set out in this Article shall be valid to empower the proxy so appointed to vote on any poll taken or demanded at such meeting or at any adjournment of such meeting.

74.2 No appointment of a proxy shall be valid after the expiration of 12 months from the date named in it as the date of its execution, except on a poll demanded at a meeting or an adjourned meeting in cases where the meeting was originally held within 12 months from such date. In this Article and the next, “address” in relation to electronic communications, includes any number or address used for the purposes of such communications.

75. Validity

A vote given in accordance with the terms of an instrument of proxy or by the duly authorised representative of a corporate member or poll demanded by proxy or by the duly authorised representative of a corporate member shall be valid notwithstanding (in the case of a proxy) the previous death or mental disorder of the principal or the revocation of the instrument of proxy or of the authority under which the instrument of proxy was executed or (in the case of a duly authorised representative of a corporate member) the revocation of his appointment, provided that no intimation in writing of such death, mental disorder or revocation shall have been received by the Company at the Office or (in the case of an instrument of proxy) such other place or address at which it was required to be deposited or received under Article 74 at least 48 hours (excluding

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weekends and bank holidays) before the commencement of the meeting or adjourned meeting at which the vote is given or the poll demanded or (in the case of a poll taken otherwise than on the same day as the meeting or adjourned meeting) the time appointed for taking the poll.

76. Supply of proxy cards

The Directors may at the expense of the Company send, by post or otherwise, to the members instruments of proxy (with or without provision for their return prepaid) for use at any general meeting or at any meeting of any class of members of the Company either in blank or nominating in the alternative any one or more of the Directors or the chairman of the meeting or any other person or persons. If for the purpose of any meeting invitations to appoint as proxy a person, or one of a number of persons, specified in the invitations are issued at the Company’s expense they shall be issued to all (and not to some only) of the members entitled to be sent a notice of the meeting and to vote at such meeting by proxy.

77. Corporate representative

Any corporation which is a member of the Company may, by resolution of its Directors or other governing body, authorise such person as it thinks fit to act as its representative at any meeting of the Company, or at any meeting of any class of members of the Company, and on presentation of a certified copy of such resolution the person so authorised shall be entitled to exercise the same powers on behalf of the corporation which he represents as that corporation could exercise if it were an individual member of the Company and such corporation shall, for the purpose of these Articles, be deemed to be present in person at such meeting if a person so authorised is present at it.

DISCLOSURE OF INTERESTS

78. Section 793

Section 793 of the Act (“Section 793”) shall be deemed to be incorporated into these Articles and accordingly to apply as between the Company and each member. If a notice is given under Section 793 (“a Section 793 notice”) to a person who appears to be interested in any shares a copy shall at the same time be given to the holder of those shares but the accidental omission to do so or the non-receipt by the member shall not prejudice the operation or the following provisions of Articles 79 to 83. The following provisions of Articles 79 to 83 shall be without prejudice to the provisions of Sections 793 and 796 of the Act, and in particular, the Company shall be entitled to apply to the court under Section 794 of the Act whether or not these provisions apply or have been applied.

79. Default

79.1 If a member or any person appearing to be interested in any shares held by a member has been duly served with a Section 793 notice and is in default for the relevant period (as defined in Article 82) from such service in supplying to the Company the information thereby required, the following provisions shall apply:

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(a) if a member has a holding of less than 0.25% of any class of shares, then, subject to Article 80 and unless the Directors otherwise determine, a member shall not be entitled in respect of those shares held by him (whether or not referred to in the Section 793 Notice) to vote at a general meeting either personally or by proxy, or to exercise any other right conferred by membership in relation to meetings of the Company; or

(b) if a member has a holding of at least 0.25% of any class of shares, then, subject to Article 80 and unless the Directors otherwise determine, the member shall not be entitled in respect of the shares held by him (whether or not referred to in the Section 793 notice):

(i) to vote at a general meeting either personally or by proxy, or to exercise any other right conferred by membership in relation to meetings of the Company; or

(ii) to receive any dividend payable in respect of such shares; or

(iii) to transfer or agree to transfer any of such shares, or any rights therein.

79.2 The restrictions imposed by Article 79.1 in relation to any shares shall continue until a relevant event occurs in relation to those shares and shall lapse when it does so. For this purpose, a “relevant event” is either of the following:

(a) the default is remedied; and

(b) the shares are registered in the name of the purchaser or offeror (or that of his nominee) pursuant to an arm’s length transfer (as defined in Article 81).

79.3 Any dividends withheld pursuant to Article 79 shall be paid to the member as soon as practicable after the restrictions contained in Article 79.1(b) lapse.

80. Restrictions

The restrictions in Article 79 shall be without prejudice to the right of either the member holding the shares concerned or, if different, the beneficial owner of those shares to effect or agree to sell under an arm’s length transfer of those shares.

81. Arms length transfer

81.1 For the purposes of Articles 78 to 83, an “arm’s length” transfer in relation to any shares is a transfer pursuant to:

(a) a sale of those shares on a recognised investment exchange (as defined in the Financial Services and Markets Act 2000) or on any stock exchange outside the United Kingdom on which the shares are normally traded; or

(b) a sale to an offeror following acceptance of an offer made to all the holders (or all the holders other than the person making the offer and his nominees) of shares of the same class as those shares to acquire all the shares of that class or a specified proportion of them.

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82. Relevant period

For the purposes of Articles 78 to 83, the “relevant period” shall, in a case falling within Article 79.1(a), be 28 days and, in a case falling within Article 79.1(b), be 14 days.

83. Interest in shares

83.1 For the purposes of Articles 78 to 83, the Company shall be entitled to treat any person as appearing to be interested in any shares if:

(a) the member holding such shares or any person who is or may be interested in such shares either fails to respond to a section 793 notice or has given to the Company a notification pursuant to a Section 793 notice which in the opinion of the Directors fails to establish the identities of those interested in the shares and if (after taking into account the said notification and any other relevant notification pursuant to a Section 793 notice) the Company knows or has reasonable cause to believe that the person in question is or may be interested in the shares; or

(b) that person (not being the member) is interested in those shares for the purposes of Section 793.

APPOINTMENT OF DIRECTORS

84. Power of Company to appoint Directors

Subject to the provisions of these Articles, the Company may by ordinary resolution appoint a person who is willing to act to be a Director, either to fill a vacancy or as an addition to the existing Directors, but the total number of Directors shall not exceed the maximum number fixed in accordance with these Articles (if any).

85. Power of Board to appoint Directors

85.1 Without prejudice to the power of the Company to appoint any person to be a Director pursuant to these Articles, the Directors shall have power at any time to appoint any person who is willing to act as a Director, either to fill a vacancy or as an addition to the existing Directors but so that the total number of Directors shall not exceed at any time the maximum number (if any) fixed by or in accordance with these Articles.

85.2 Any Director so appointed shall retire at the conclusion of the annual general meeting of the Company next following such appointment and shall be eligible for reappointment at that meeting. If not reappointed at such annual general meeting, such Director shall vacate office at the conclusion of such annual general meeting.

86. Number of Directors

Subject as provided in these Articles, the Directors shall be not less than two in number and no more than seven in number but the Company may by special resolution from time to time vary the minimum and maximum number of directors.

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87. Additional remuneration

87.1 Any Director who serves on any committee or who devotes special attention to the business of the Company, or who otherwise performs services which in the opinion of the Directors are outside the scope of the ordinary duties of a Director, may be paid such remuneration by way of salary, lump sum, percentage of profits or otherwise as the Directors may determine.

87.2 The Directors shall also be entitled to be paid all travelling, hotel and other expenses properly incurred by them in connection with the business of the Company, or in attending and returning from meetings of the Directors or of committees of the Directors or general meetings or separate meetings of the holders of any class of shares or of debentures of the Company or otherwise in connection with the discharge of their duties.

ALTERNATE DIRECTORS

88. Appointment

Each Director (other than an alternate director) may at any time appoint another Director or (subject to the approval of a majority of the Directors for the time being) any other person to be an alternate director of the Company, and may at any time remove any alternate director so appointed by him from office and, subject to such requisite approval, appoint another person in his place. An alternate director shall (subject to his giving to the Company an address within the United Kingdom at which notices may be served upon him) be entitled to receive notices of all meetings of the Directors and of all meetings of committees of the Directors of which his appointor is a member and to attend and vote as a Director at any such meeting at which the Director appointing him is not personally present, and generally to perform all the functions of his appointor as a Director in the absence of such appointor. An alternate director shall ipso facto cease to be an alternate director if his appointor ceases for any reason to be a Director; provided that if any Director retires, whether by rotation or otherwise, but is re-appointed or is deemed to have been re-appointed by the meeting at which such retirement took effect, any appointment made by him pursuant to this Article which was in force immediately prior to his retirement shall continue to operate after his reappointment as if he had not so retired. All appointments and removals of alternate directors shall be effected by instrument in writing signed by the appointor Director and authenticated in such manner as the other Directors may accept. The appointor Director shall deposit the original signed instrument at the office as soon as reasonably practicable, but failure or delay in doing so shall not prejudice the validity of the appointment.

89. Remuneration

Save as otherwise provided in these Articles, an alternate director shall be deemed for all purposes to be a Director of the Company and shall alone be responsible to the Company for his own acts and defaults, and he shall not be deemed to be the agent of or for the Director appointing him. An alternate director shall not be entitled to receive any remuneration from the Company for his services as an alternate director but his remuneration shall be payable out of the remuneration payable to the Director appointing him, and shall consist of such part (if any) of the latter’s remuneration as

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shall be agreed between them.

INTERESTS OF DIRECTORS

90. Other office of Director

A Director, including an alternate director, may hold any other office or place of profit under the Company (other than the office of auditor) in conjunction with his office of Director on such terms as to tenure of office, remuneration and otherwise as the Directors may determine. Any Director may act by himself or his firm in a professional capacity (other than that of auditor) for the Company and he or his firm shall be entitled to remuneration for such professional services.

91. Disqualification

91.1 No Director or proposed Director, including an alternate director, shall be disqualified by his office from contracting with the Company either with regard to his tenure of any other office or place of profit, or as vendor, purchaser or otherwise, nor shall any such contract, or any contract or arrangement entered into by or on behalf of the Company in which any Director is in any way, whether directly or indirectly, interested, be liable to be avoided, nor shall any Director so contracting or being so interested, be liable to account to the Company for any profit realised by any such contract or arrangement, by reason of such Director holding that office or of the fiduciary relationship thereby established.

91.2 Any Director, including an alternate director, may continue to be or become a director or other officer or member of or otherwise interested in any other company promoted by the Company or any subsidiary of the Company or in which the Company or any subsidiary of the Company may be interested, as a member or otherwise, or in which the Company or any subsidiary of the Company thereof has decided not to take any shareholding or other interest whatsoever, and no such Director shall be accountable for any remuneration or other benefits whatsoever received by him or as a director or other officer or member of or from his interest in any such other company. The Directors may exercise the voting power conferred by the shares of any other company held or owned by the Company, or exercisable by them as directors of such other company, in such manner in all respects as they think fit but subject to the restrictions contained in these Articles.

92. Declaration of interest

A Director who is in any way, whether directly or indirectly, interested or deemed by the Act to be interested in a contract, transaction or arrangement or a proposed contract, transaction or arrangement with the Company shall declare the nature of his interest at a meeting of the Directors in accordance with Section 177 of the Act.

93. Material interest

Save as provided in these Articles, a Director (including an alternate director) shall not vote in respect of any contract or arrangement or any other proposal in which he has any material interest otherwise than by virtue of his interests in shares or debentures or other securities or rights of the Company. However a Director shall be entitled to vote

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in respect of any contract or arrangement or any other proposal in which he has any interest which is not material. A Director shall not be counted in the quorum at a meeting in relation to any resolution on which he is debarred from voting.

94. Voting

94.1 A Director (including an alternate director) shall (in the absence of some material interest other than as indicated below) be entitled to vote (and be counted in the quorum) in respect of any resolution concerning any of the following matters, namely:

(a) the giving of any security, guarantee or indemnity to him in respect of money lent or obligations incurred by him at the request of or for the benefit of the Company or any of its subsidiaries;

(b) the giving of any security, guarantee or indemnity to a third party in respect of a debt or obligation of the Company or any of its subsidiaries for which he himself has assumed responsibility in whole or in part by the giving of security or under a guarantee of indemnity;

(c) any proposal concerning an offer for subscription or purchase of shares or debentures or other securities or rights of or by the Company or any of its subsidiaries or of any company which the Company may promote or in which it may be interested in which offer he is or is to be interested directly or as a participant in the underwriting or associated sub-underwriting;

(d) any proposal concerning any other company in which he is interested directly or indirectly and whether in any one or more of the capacities of officer, creditor, employee or holder of shares, debentures, securities or rights of that other company, but where he is not the holder (otherwise than as a nominee for the Company or any of its subsidiaries) of or beneficially interested in one per cent or more of the issued shares of any class of such company or of any third company through which his interest is derived or of the voting rights available to members of the relevant company (any such interest being deemed for the purpose of this Article to be a material interest in all circumstances);

(e) any proposal concerning the adoption, modification or operation of a superannuation fund, retirement benefits scheme, share option scheme or share incentive scheme under which he may benefit; or

(f) any proposal concerning the purchase and/or maintenance of any insurance policy under which he may benefit.

95. Two Directors

Where proposals are under consideration concerning the appointment (including fixing or varying the terms of appointment) of two or more Directors to offices or employment with the Company or any company in which the Company is interested, such proposals may be divided and considered in relation to each Director separately and in such case each of the Directors concerned (if not otherwise debarred from voting by the terms of these Articles) shall be entitled to vote (and be counted in the quorum) in respect of each resolution except that concerning his own appointment.

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96. Directors interests

If any question shall arise at any meeting as to the materiality of the entitlement of any Director to vote and such question is not resolved by his involuntary agreeing to abstain from voting, such question shall (subject to the Act) be referred to the chairman of the meeting (or, where such question shall arise concerning such chairman, to such other Director present at the meeting as the Directors present, other than such chairman, shall by majority vote appoint) and his ruling in relation to any other Director shall be final and conclusive except in a case where the nature or extent of the interests of the Director concerned have not been fairly disclosed.

97. Interest of connected person

For the purposes of these Articles, the interest of any person who is connected with a Director (within the meaning of Section 252 of the Act) shall be taken to be the interest of that Director.

98. Suspension of provisions

The Company may by ordinary resolution suspend or relax the provisions of Articles 93 to 97 to any extent either generally or in respect of any particular matter, or ratify any transaction not duly authorised by reason of a contravention of those Articles.

99. Directors’ conflict of interest

99.1 The Board may, in accordance with the requirements set out in this Article, authorise any matter or situation proposed to them by any Director which would, if not authorised, involve a Director (an “Interested Director”) breaching his duty under the Act to avoid conflicts of interest (“Conflict”).

99.2 A Director seeking authorisation in respect of a Conflict shall declare to the Board the nature and extent of his interest in a Conflict as soon as is reasonably practicable. The Director shall provide the Board with such details of the matter as are reasonably necessary for the Board to decide how to address the Conflict together with such additional information as may be reasonably requested by the Board and provided that such additional information is requested no less than five business days before the consideration of the relevant matter at a meeting of the Board.

99.3 Any authorisation under this Article will be effective only if:

(a) any requirement as to the quorum for consideration of the relevant matter is met without counting the Interested Director and any other Interested Director; and

(b) the matter is agreed to without the Interested Director voting or would be agreed to if the Interested Director’s and any other Interested Director’s vote is not counted.

99.4 Any authorisation of Conflict under this Article will be effective if given at the same meeting of the Board as that at which the relevant matter is considered or any other meeting of the Board.

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99.5 Any authorisation of a Conflict under this Article must be recorded in writing (but the authority shall be effective whether or not the terms are so recorded) and may (whether at the time of giving the authorisation or subsequently):

(a) extend to any actual or potential Conflict of interest which may reasonably be expected to arise out of the matter or situation so authorised;

(b) provide that the Interested Director be excluded from the receipt of documents and information and the participation in discussions (whether at meetings of the Directors or otherwise) related to the Conflict;

(c) impose upon the Interested Director such other reasonable terms for the purposes of dealing with the Conflict as the Directors think fit;

(d) permit the Interested Director to absent himself from the discussion of matters relating to the Conflict at any meeting of the Directors and be excused from reviewing papers prepared by, or for, the Directors to the extent they relate to such matters.

99.6 Where a Director obtains, or has obtained whether through his involvement in a Conflict, through his position as a Director or howsoever otherwise, information that is confidential to a third party, he may, at his sole discretion, decide whether or not to disclose that information to the Company, or to use it in relation to the Company’s affairs and shall not be under any obligation to disclose such information to the Company.

99.7 Where the Directors authorise a Conflict, the Interested Director will be obliged to conduct himself in accordance with any reasonable terms and conditions imposed by the Directors in relation to the Conflict.

99.8 Subject to Article 99.3, where the Directors authorise a Conflict, the Interested Director shall be entitled to vote at any meeting of the Board at which the relevant matter related to the Conflict is considered and/or approved.

99.9 The Directors may revoke or vary such authorisation by written notice to the Interested Director, but this will not affect anything done by the Interested Director, prior to such revocation or variation, in accordance with the terms of such authorisation.

99.10 A Director is not required, by reason of being a Director (or because of the fiduciary relationship established by reason of being a director), to account to the Company for any remuneration, profit or other benefit which he derives from or in connection with a relationship involving a Conflict which has been authorised by the directors or by the Company in general meeting (subject in each case to any terms, limits or conditions attaching to that authorisation) and no contract shall be liable to be avoided on such grounds.

100. Benefits

The Directors may establish, maintain, participate in or contribute to or procure the establishment and maintenance of, participation in or contribution to any pension, annuities, superannuation, benevolent or life assurance fund, scheme or arrangement (whether contributory or otherwise) for the benefit of, and give or procure the giving of

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donations, gratuities, pensions, allowances, benefits and emoluments to, any persons who are or were at any time in the employment or service of the Company, or any of its predecessors in business, or of any company which is a subsidiary of the Company or is allied to or associated with the Company, or with any such subsidiary, or who may be or have been Directors or officers of the Company, or of any such other company, and the spouses, widows/widowers, families and dependants of any such persons, and also establish, subsidise and subscribe to any institutions, associations, societies, clubs, trusts or firms calculated to be for the benefit of or to advance the interests and well-being of the Company or of such other company, or of any such persons, and make payments for or award the insurance of any such persons as aforesaid, and (subject to the provisions of the Act) establish and contribute to any scheme for the acquisition of shares in the Company or its holding company (whether or not an employee share scheme within the meaning of the Act) and (subject as set out in this Article) lend money to the Company’s employees to enable them to acquire such shares, and subscribe or guarantee money for charitable or benevolent objects, or for any exhibition or for any public, general or useful object, and do any of such matters either alone or in conjunction with others. Subject always, if the Act shall so require, to particulars with respect to the proposed payment being disclosed to the members of the Company and to the proposal being approved by the Company by an ordinary resolution, any Director shall be entitled to participate in and retain for his own benefit any such donation, gratuity, pension, allowance, benefit or emolument.

101. Exercise of power

The Company shall exercise the power conferred upon it by Section 247 of the Act to make provision for the benefit of persons employed or formerly employed by the Company or any of its subsidiaries, in connection with the cessation or the transfer to any person of the whole or part of the undertaking of the Company or any subsidiary only with the prior sanction of a special resolution. However the Directors are entitled to exercise the power contained in Section 247 of the Act by means of a resolution of the Directors but this shall be limited to a maximum payment to any individual employee of fifty per cent of the employee’s gross annual salary.

GENERAL POWERS OF DIRECTORS

102. Management

The business of the Company shall be managed by the Directors, who may exercise all such powers of the Company as are not by the Act or by these Articles required to be exercised by the Company in general meeting, subject nevertheless to any provisions of these Articles, to the provisions of the Act and to such regulations (being not inconsistent with the aforesaid regulations or provisions) as may be prescribed by the Company by special resolution but no regulation made by the Company by special resolution shall invalidate any prior act of the Directors which would have been valid if such regulation had not been made. The general powers given to the Directors by this Article shall not be limited or restricted by any special authority or power given to the Directors by any other Article.

103. Delegation of Authority

The Directors may establish any local boards or agencies for managing any of the

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affairs of the Company, either in the United Kingdom or elsewhere, and may appoint any persons to be members of such local boards, or any managers or agents, and may fix their remuneration, and may delegate to any local board, manager or agent any of the powers, authorities and discretions vested in the Directors (other than the power of making calls), with power to sub-delegate, and may authorise the members of any local board, or any of them, to fill any vacancies in such local board, and to act notwithstanding filling vacancies, and any such appointment or delegation may be made upon such terms and subject to such conditions as the Directors may think fit, and the Directors may remove any person so appointed, and may annul or vary any such delegation, but no person dealing in good faith and without notice of any such annulment or variation shall be affected by it.

104. Power of Attorney

The Directors may, by power of attorney, appoint any person or persons to be the agent of the Company and may delegate to any such person or persons any of the powers, authorities and discretions of the Directors (with power to sub-delegate), in each case for such purposes and for such time, on such terms (including as to remuneration) and subject to such conditions as the Directors think fit. The Directors may confer such powers either collaterally with, or to the exclusion of and in substitution for, all or any of the powers of the Directors in that respect and may from time to time revoke, withdraw, alter or vary any of such powers.

105. Overseas registers

Subject to the provisions of the Statutes, the Directors may exercise the powers conferred on the Company with regard to the keeping of an overseas branch, local or other register and may make and vary such regulations as they think fit as regards the keeping of any such register.

106. Uncalled capital

If any uncalled capital of the Company is included in or charged by any mortgage or other security, the Directors may delegate to the person in whose favour such mortgage or security is executed, or to any other person in trust for him, the power to make calls on the members in respect of such uncalled capital, and to sue in the name of the Company or otherwise for the recovery of monies becoming due in respect of calls so made and to give valid receipts for such monies, and the power so delegated shall subsist during the continuance of the mortgage or security, notwithstanding any change of Directors, and shall be assignable if expressed so to be.

DIRECTORS HOLDING EXECUTIVE OFFICE

107. Office

The Directors may from time to time appoint any one or more of their body to be holder of any executive office for such period and on such terms and with or without such title or titles (including but not limited to chairman, deputy chairman, vice chairman, managing director, chief executive and joint, deputy or assistant managing director or chief executive) as they think fit. A Director holding any such office (whether appointed by the Directors or otherwise) shall, whilst holding such office, be subject to retirement

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by rotation, and shall (subject to the terms of any contract between him and the Company) be subject to the same provisions as to resignation and removal as the other Directors of the Company and if he shall vacate the office of director or (subject as aforesaid) if the Directors resolve that his term of office as holder of such executive office as aforesaid be determined, his appointment as such shall ipso facto determine.

108. Remuneration

A Director appointed to any executive office as referred to in Article 107 shall receive such remuneration (whether specifically by way of salary, bonus, commission, participation in profits, provision for retirement or insurance benefit, or partly in one way and partly in another, or otherwise) as may be determined by the Board who may delegate their authority.

109. Powers

109.1 The Directors may from time to time:

(a) delegate or entrust to and confer on any Director holding executive office (including a chief executive or managing director) such of the powers, authorities and discretions of the Directors (with power to sub-delegate) for such time, on such terms and subject to such conditions as the Directors think fit; and

(b) revoke, withdraw, alter or vary all or any of such powers.

RETIREMENT OF DIRECTORS

110. Retirement

Any provision of the Statutes which, subject to the provisions of these Articles, would have the effect of rendering any person ineligible for appointment or election as a Director or liable to vacate office as a director on account of such person having reached any specified age or of requiring special notice or any other special formality in connection with the appointment or election of any Director over a specified age, shall not apply to the Company.

111. Vacation of office

111.1 The office of a Director shall be vacated in any of the following events, namely:

(a) if (but in the case of a Director holding any executive office subject to the terms of any contract between him and the Company) he resigns his office by instrument in writing signed by the resigning Director and authenticated in such manner as the other Directors or Director may accept (provided that the resigning Director shall deposit the original signed instrument at the office as soon as reasonably practicable but failure or delay in his doing so shall not prejudice the validity of the resignation);

(b) if he becomes bankrupt or has a receiving order made against him or makes any arrangement or composition with his creditors generally;

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(c) if, in the opinion of the majority of Directors other than the Director vacating office and in the written opinion of a suitably qualified medical expert, he becomes of unsound mind;

(d) if he is absent from meetings of the Directors for six successive months without leave, and his alternate director (if any) shall not during such period have attended in his stead, and the Directors resolve that his office be vacated;

(e) if he ceases to be a Director by virtue of any provision of the Statutes or becomes prohibited by law from being a director;

(f) he is removed from office by notice in writing signed by all of the other Directors (without prejudice to any claim for damages which he may have for breach of any contract between him and the Company) and, for this purpose, a set of like notices each signed by, one or more of the Directors shall be as effective as a single notice signed by the requisite number of Directors;

(g) he ceases to hold the number of shares required to qualify him for office (if any) or does not acquire the same within two months after election or appointment; or

(h) he is removed as a Director by ordinary resolution of the members provided that such removal shall be without prejudice to any claim he may have for breach of contract between him and the Company.

112. Resolution as to a vacancy conclusive

A resolution of the Directors declaring a Director to have vacated office under the terms of Article 111 shall be conclusive as to the fact and grounds of vacation stated in the resolution.

ROTATION OF DIRECTORS

113. Retirement by rotation

At each annual general meeting of the Company that occurs after 25 September 2021, one-third of the Directors (or, if they are not a multiple of three, the number nearest to but not greater than one third subject to a minimum of one) shall retire from office by rotation provided always that any Director retiring pursuant to Article 111 shall not be taken into account in determining the Directors who are to retire by rotation at that meeting. A Director retiring by rotation shall be eligible for re-election.

114. Retirement in every year

Subject to the provisions of the Act and of these Articles, the Directors to retire in every year shall include (so far as necessary to obtain the number required) any Director who wishes to retire and not to offer himself for re-election. Any further Directors so to retire shall be those who have been longest in office since their last appointment or reappointment. Subject as aforesaid, a retiring Director shall be eligible for reappointment.

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115. Vacated office

The Company at the meeting at which a Director retires in accordance with these Articles may fill the vacated office by appointing a person to fill such vacancy, and in default the retiring Director, if willing to act, shall be deemed to have been re-appointed, unless at such meeting it is expressly resolved not to fill the vacancy, or a resolution for the reappointment of such Director shall have been put to the meeting and lost.

116. Appointment

No person other than a Director retiring at the meeting shall, unless recommended by the Directors for appointment, be eligible for appointment to the office of director at any general meeting unless, not less than seven nor more than 28 days before the day appointed for the meeting, there shall have been given to the Company notice in writing by some member duly qualified to attend and vote at the meeting for which such notice is given of his intention to propose such person for appointment stating the particulars which would, if he were so appointed, be required to be included in the Company’s register of directors, and also notice in writing signed by the person to be proposed of his willingness to be appointed.

117. Motion

At a general meeting a motion for the appointment of two or more persons as Directors by a single resolution shall not be made unless a resolution that it shall be so made has been first agreed to by the meeting without any vote being given against it, and for the purposes of this Article a motion for approving a person’s appointment or for nominating a person for appointment shall be treated as a motion for his appointment.

PROCEEDINGS OF DIRECTORS

118. Meetings

118.1 Subject to the provisions of these Articles, the Directors may meet together for the dispatch of business, adjourn and otherwise regulate their meetings as they think fit. Questions arising at any meeting shall be determined by a majority of votes. In case of an equality of votes the chairman of the meeting shall have a second or casting vote. A Director who is also an alternate director shall be entitled in the absence of his appointor to a separate vote on behalf of the Director he is representing in addition to his own vote. A Director may, and the Secretary on the requisition of a Director shall, at any time summon a meeting of the Directors. Notices in respect of such meetings may be sent by facsimile or by electronic communication sent to an address notified to the Company for that purpose or by word of mouth including to any Director for the time being absent from the United Kingdom to such address (whether inside or outside the United Kingdom) notified by him to the Directors for this purpose. A Director may waive the requirement that notices of meetings of the Directors must be given to him either prospectively or retrospectively.

118.2 A meeting of the Directors may be validly held notwithstanding that all of the Directors are not present at the same place provided that:

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(a) the Directors at the time of the meeting are in direct communication with each other whether by way of telephone, audio link or other form of telecommunications (and such a meeting shall be deemed to take place where the largest group of those participating is assembled or, if there is no such group, where the chairman of the meeting then is); and

(b) all of the Directors entitled to notice of a meeting of the Directors agree to the holding of the meeting in the manner described in this Article.

119. Authorisation to vote

A Director who is unable to attend any meeting of the Directors and has not appointed an alternate director may authorise any other Director to vote for him at the meeting, and in that event the Director so authorised shall have a vote for each Director by whom he is so authorised in addition to his own vote. Any such authority must be by instrument signed by the authorising Director and authenticated in such manner as the other Directors may accept. The authorising Director shall deposit the original signed instrument at the office as soon as reasonably practicable but failure or delay in his doing so shall not prejudice the validity of the authorisation.

120. Quorum

The quorum necessary for the transaction of the business of the Directors may be fixed by the Directors, and unless otherwise determined shall be two. For the purposes of this Article a person who holds office only as an alternate director shall, if his appointor is not present, be counted in a quorum, but so that not less than two individuals shall constitute the quorum. Any Director or alternate director who attends a meeting of Directors by telephone or other conference facility shall be deemed to be personally present at such meeting for all purposes of these Articles and shall be counted in the quorum accordingly. A meeting of the Directors for the time being at which a quorum is present shall be competent to exercise all powers and discretions for the time being exercisable by the Directors.

121. Minimum number of directors

The continuing Directors or a sole continuing Director may act notwithstanding any vacancies in their body, but if and so long as the number of Directors is reduced below the minimum number fixed by or in accordance with these Articles, or below the number fixed by or pursuant to these Articles as the quorum of Directors, the continuing Directors or Director may act for the purpose of filling vacancies in their body or of summoning general meetings of the Company, but not for any other purpose. If there be no Directors or Director able or willing to act, then any two members may summon a general meeting for the purpose of appointing Directors.

122. Chairman

The Directors may, from their number, from time to time elect and remove a chairman and, if thought fit, one or more deputy chairmen or vice-chairmen and determine the period for which they are to hold office. The chairman, or in his absence the deputy chairman (to be chosen, if there be more than one, by agreement amongst themselves or, failing agreement, by lot), or in the absence of any deputy chairman the vice

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chairman (to be chosen, if there be more than one, by agreement amongst themselves or, failing agreement, by lot), shall preside at all meetings of the Directors, but if no such chairman, deputy chairman or vice chairman be elected, or if at any meeting neither the chairman nor deputy chairman or vice chairman be willing to preside or none of them be present within five minutes after the time appointed for holding the same, the Directors present may choose one of their number to be chairman of the meeting.

123. Resolutions

A resolution in writing, signed by all the Directors for the time being entitled to receive notice of a meeting of Directors or of a committee of Directors, shall be as effective as a resolution passed at a meeting of the Directors or (as the case may be) a committee of Directors duly convened and held, and may consist of several documents in the like form each signed by one or more of the Directors and so that any such resolution or document signed by an alternate director shall be deemed to have been signed by the Director who appointed such alternate director and, if it is signed by a Director who has appointed an alternate director, it need not be signed by the alternate director in that capacity.

124. Committees

The Directors may delegate any of their powers to committees consisting of or including at least one member of their body as they think fit, provided that at least a majority of the members of any such committee shall be Directors of the Company and no resolution of a committee may be effective unless a majority of those present either in person or by proxy when the resolution was passed are Directors or alternate directors. Any committee so formed shall in the exercise of the powers so delegated conform to any regulations that may be imposed on it by the Directors. The meetings and proceedings of any such committee consisting of two or more Directors shall be governed by the provisions of these Articles regulating the meetings and proceedings of the Directors, so far as the same are applicable and are not superseded by any regulations imposed by the Directors under this Article.

125. Validity

All acts done by any meeting of Directors, or of a committee of Directors, or by any person acting as a Director, shall, notwithstanding that it be afterwards discovered that there was some defect in the appointment of any such Director, or person acting as a Director, or that they or any of them were disqualified from holding office, or had vacated office, or were not entitled to vote, be as valid as if every such person had been duly appointed, and was qualified and had continued to be a Director and had been entitled to vote.

BORROWING POWERS

126. Powers

The Directors may exercise all the powers of the Company to borrow money, to guarantee and to mortgage or charge its undertaking, property, assets and uncalled capital, and (subject to the Act) to issue debentures and other securities, whether

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outright or as collateral security for any debt, liability or obligation of the Company or of any third party.

OTHER DIRECTORS

127. Appointment

Subject to the provisions of the Act the Directors may from time to time, and at any time pursuant to this Article appoint any other persons to any post with such descriptive title including that of director (whether as executive, group, divisional, departmental, deputy, assistance, local, advisory director or otherwise) as the Directors may determine and may define, limit, vary and restrict the powers, authorities and discretions of persons so appointed and may fix and determine their remuneration and duties and, subject to any contract between him and the Company, may remove from such post any person so appointed. A person so appointed shall not be a Director of the Company for any of the purposes of these Articles or of the Act and accordingly shall not be a member of the board of Directors or any committee of the Directors, nor shall he be entitled to be present at any meeting of the directors or of any such committee, except at the request of the Directors or of such committee, and if present at such request he shall not be entitled to vote at such meeting.

MINUTES AND BOOKS

128. Minutes

128.1 The Directors shall cause minutes to be made:

(a) of all appointments of officers made by the Directors;

(b) of the names of the Directors present at each meeting of Directors and of any committee of Directors;

(c) of all resolutions and proceedings at all meetings of the Company and of any class of members of the Company and of the Directors and of committees of Directors.

128.2 Any such minutes if purporting to be signed by the chairman of the meeting at which the proceedings took place, or by the chairman of the next following meeting, shall be evidence of the proceedings.

129. Records

Subject as required by law, any register, index, minute book or accounting records required by these Articles or by law to be kept by or on behalf of the Company may be kept either by making entries in bound books or by recording them in any other manner. In any case in which bound books are not used, the Directors shall take adequate precautions for guarding against, and for facilitating the discovery of, falsification.

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SECRETARY

130. Appointment

Subject to the Act the Secretary shall be appointed by the Directors on such terms and for such period as they may think fit and the Directors may also appoint one or more assistant or deputy secretaries. Any Secretary or assistant or deputy secretary so appointed may at any time be removed from office by the Directors without prejudice to any claim for damages for breach of any contract of service between him and the Company.

131. Office

Anything by the Act required or authorised to be done by or to the Secretary may, if the office is vacant or such Secretary is absent or there is for any other reason no such Secretary capable of acting, be done by or to any assistant or deputy secretary or, if there is no assistant or deputy secretary, or if such assistant or deputy secretary is absent or for any other reason not capable of acting by or to any officer of the Company authorised generally or specially in that behalf by the Directors provided that any provision of the Act or of these Articles required or authorising a thing to be done by or to a Director and the Secretary shall not be satisfied by its being done by or to the same person acting both as Director and as, or in place of, the Secretary.

THE SEAL

132. Safe custody

The Directors shall provide for the safe custody of the Seal and the Securities Seal and neither shall be used except by the authority of a resolution of the Directors or of a committee of the Directors authorised in that behalf by the Directors.

133. Application

The Directors may from time to time make such regulations as they see fit (subject to the provisions of these Articles in relation to share certificates and debenture certificates) determining the persons and the number of such persons who shall sign every instrument to which the Seal or the Securities Seal is affixed, and until otherwise so determined (and subject to the provisions of this Article) every such instrument shall be signed by one Director and shall be countersigned by the Secretary or by a second Director.

134. Seal for use abroad

The Company may have an official seal for use abroad under the provisions of the Act where and as the Directors shall determine, and the Company may by writing under the Seal appoint any agent or committee abroad to be the duly authorised agent of the Company for the purpose of affixing and using such official seal, and may impose such restrictions on its use as shall be thought fit. Wherever in these Articles reference is made to the Seal, the reference shall, when and so far as may be applicable, be deemed to include any such official seal as aforesaid.

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135. Issue

135.1 Every certificate or share warrant shall be issued either:

(a) by affixing the Securities Seal to it, by mechanical, electronic or other means;

(b) by printing a representation of the Securities Seal on it, by mechanical, electronic or other means, including laser printing; or

(c) in such other manner as the Board, having regard to the Statutes and any regulations which may apply to the Company from time to time.

136. Seal

The Company need not have a company seal and pursuant to the Act may execute and deliver any document as a deed under the signature of any two Directors or of one Director and the Secretary or any Director in the presence of a witness who attests his signature. A certificate in respect of any shares or other securities in the Company shall be validly issued if it is executed as a deed in accordance with this Article.

AUTHENTICATION OF DOCUMENTS

137. Authentication

Any Director or the Secretary or any person appointed by the Directors for the purpose shall have power to authenticate any documents affecting the constitution of the Company and any resolutions passed by the Company or the Directors or any committee of the Directors, and any books, records, documents and accounts relating to the business of the Company, and to certify copies of such documents or extracts from them as true copies or extracts. A document purporting to be a copy of a resolution, or a copy of or an extract from the minutes of a meeting of the Company or of the Directors or any committee of the Directors, which is so certified shall be conclusive evidence in favour of all persons dealing with the Company that such resolution has been duly passed or, as the case may be, that such copy or extract is a true and accurate record of proceedings at a duly constituted meeting.

DIVIDENDS

138. Declaration of dividends

The profits of the Company available for distribution and resolved to be distributed shall be applied in the payment of dividends to the members in accordance with their respective rights and priorities. The Company in general meeting may declare dividends accordingly.

139. Dividends payable

No dividends shall be payable otherwise than in accordance with the Act and out of the profits of the Company available for that purpose and no dividend shall exceed the amount recommended by the Directors.

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140. Payment of dividends

Subject to the rights of persons, if any, entitled to shares with special rights as to dividends, all dividends shall be declared and paid according to the amounts paid-up on the shares in respect whereof the dividend is paid, but no amount paid up on a share in advance of calls shall be treated for the purposes of this Article as paid up on the share. All dividends shall be apportioned and paid pro rata according to the amount paid up on the shares during any portion or portions of the period in respect of which the dividend is paid, except that if any share is issued on terms providing that it shall carry any particular rights as to dividend, such share shall rank for dividend accordingly.

141. Interim dividends

The Directors may if they think fit from time to time pay to the members such interim dividends as appear to the Directors to be justified by the profits of the Company and are permitted by the Act. If at any time the share capital of the Company is divided into different classes, the Directors may (subject to the provisions of the Act) pay such interim dividends in respect of those shares in the capital of the Company which confer on their holders deferred or non preferred rights as well as in respect of those shares which confer on their holders preferential rights with regard to divided but no interim dividend shall be paid on shares carrying deferred or non preferred rights if, at any time of payment, any preferential dividend is in arrears. The Directors may also pay half yearly, or at other suitable intervals to be settled by them, any dividend which may be payable at a fixed rate if they are of the opinion that the profits justify the payment and if and to the extent that such payment is permitted by the Act. Provided the Directors act bona fide they shall not incur any responsibility to the holders of shares conferring a preference for any damage that they may suffer by reason of the payment of any interim dividend on any shares having deferred or non preferred rights.

142. Profits and losses

Subject to the provisions of the Act or as otherwise required by law, where any asset, business or property is bought by the Company as from a past date, whether such date be before or after the incorporation of the Company, the profits and losses thereof as from such date may at the discretion of the Directors in whole or in part be carried to a revenue account and treated for all purposes as profits or losses of the Company. Subject to that, if any shares or securities are purchased cum dividend or interest, such dividend or interest may at the discretion of the Directors be treated as revenue and it shall not be obligatory to capitalise the same or any part of the same.

143. Calls or debts deducted from dividends

The Directors may deduct from any dividend or other monies payable to any member on or in respect of a share all sums of money (if any) presently payable by him to the Company on account of calls or otherwise in relation to shares of the Company.

144. Retention of dividends

The Directors may retain the dividends payable upon shares in respect of which any person is, under the provisions as to the transmission of shares contained in these Articles, entitled to become a member, or which any person is under those provisions

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entitled to transfer, until such person shall become a member in respect of such shares or shall transfer the same.

145. Unclaimed dividends

All unclaimed dividends may be invested or otherwise made use of by the Directors for the benefit of the Company until claimed and the payment of any such dividend into a separate account or the investment of such dividend shall not constitute the Company a trustee in respect of such dividend. No dividend or other monies payable in respect of a share shall bear interest as against the Company unless otherwise provided by the rights attached to the share. Any dividend which has remained unclaimed for a period of 12 years from the date of its declaration shall at the expiration of that period be forfeited and cease to remain owing by the Company and shall then belong to the Company absolutely.

146. Payment of dividends

146.1 Any dividend or other monies payable on or in respect of a share may be paid by cheque or warrant sent through the post to the registered address of the member or person entitled to it and in the case of joint holders to the first named of such joint holders, or to such person and such address as the holder or joint holders may in writing direct. Every such cheque or warrant shall be made payable to the order of the person to whom it is sent or to such other person as the holder or joint holders may in writing direct, and payment of the cheque or warrant shall be a good discharge to the Company. Every such cheque or warrant shall be sent at the risk of the person entitled to the money represented by such cheque or warrant.

146.2 The Company may cease to send any cheque or warrant through the post for any dividend payable on any shares in the Company which is normally paid in that manner on those shares if in respect of at least two consecutive dividends payable on those shares the cheques or warrants have been returned undelivered or remain uncashed but, subject to the provisions of these Articles, shall recommence sending cheques or warrants in respect of dividends payable on those shares if the holder or person entitled by transmission claims the arrears of dividend and does not instruct the Company to pay future dividends in some other way.

147. Receipts for dividends

If several persons are registered as joint holder of any share, any one of them may give effectual receipts for any dividend or other monies payable on or in respect of the share.

148. Scrip dividends

148.1 The Directors may subject as provided in these Articles declare that each Ordinary Shareholder may elect to forego his right to participate in such dividend (or such part of it as the Directors may determine) and to receive instead an allotment of Ordinary Shares to the extent and within the limits and on the terms and conditions set out below. The Directors shall announce any such decision in conjunction with any announcement of the relevant dividend and shall send to the relevant Ordinary Shareholders notices of election as soon as practicable.

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148.2 If the Directors make a declaration pursuant to Article 148.1 each holder of Ordinary Shares may (by notice in writing to the Company given in such form and within such period as the Directors may from time to time determine) elect to forego the dividend which otherwise would have been paid on all or so many of his Ordinary Shares as he shall specify in notice of election and to receive in lieu such number of Ordinary Shares to be allotted to him credited as fully paid as is equal to the number resulting from resolving the following fraction (but ignoring any fraction of an additional Ordinary Share):

A x B

C

where:

(a) A equals the number of Ordinary Shares in respect of which such election has been made;

(b) B equals the amount of the dividend per share foregone (expressed in terms of pence and fractions of a penny); and

(c) C equals the price at which each Ordinary Share in respect of which such election has been made is to be allotted as determined by the Directors.

148.3 Following the receipt of a notice or notices of election the Directors shall appropriate out of the undistributed profits or reserves of the Company an amount equal to the aggregate nominal value of the number of Ordinary Shares required to be allotted to the holders of Ordinary Shares who have given notice of election and shall apply such amount in paying up in full such number of Ordinary Shares.

148.4 The Ordinary Shares so allotted credited as fully paid shall not be entitled to participate in the dividend then being declared or paid but shall in all other respects rank pari passu with the existing Ordinary Shares of the Company.

148.5 The Directors shall not make any such decision under this Article unless the Company has sufficient unissued Ordinary Shares and undistributed profits or reserves to give effect to any elections which could be made as a consequence of such decision.

148.6 The Directors shall not make any such decision under this Article unless the Company shall by ordinary resolution approve the exercise by the Directors of their powers so to do in respect of the dividend in question or in respect of any dividends declare or paid in respect of each specified financial year or period of the Company which dividends include the dividend in question.

148.7 The Directors may do all acts and things considered necessary or expedient to give effect to any such capitalisation, with full power to the Directors to make such provisions as they think fit for the case of shares becoming distributable in fractions (including provisions whereby, in whole or in part, fractional entitlements are disregarded or rounded up or the benefit of fractional entitlements accrue to the Company rather than to the members concerned). The Directors may authorise any person to enter, on behalf of all the members interested, into an agreement with the Company providing for such capitalisation and matters incidental thereto and any agreement made under such authority shall be effective and binding on all concerned.

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148.8 This Article shall have effect without prejudice to the provisions of Article 146 any other provisions of these Articles and such provisions shall also have effect without prejudice to the provisions of this Article.

149. General meeting to declare dividend

A general meeting declaring a dividend may, upon the recommendation of the Directors, direct payment of such dividend wholly or in part by the distribution of specific assets, and in particular of paid up shares, debentures or other securities or rights of any other Company, and the Directors shall give effect to such resolution and where any difficulty arises in regard to the distribution the Directors may settle the same as they think expedient, and in particular may issue fractional certificates, and may fix the value for distribution of such specific assets, or any part of them, and may determine that cash payments shall be made to any members upon the footing of the value so fixed in order to adjust the rights of members, and may vest any specific assets in trustees upon trusts for the persons entitled to the dividend as may seem expedient to the Directors, and generally may make such arrangements for the allotment, acceptance and sale of such specific assets or fractional certificates or any part of them and otherwise as they think fit.

150. Reserves

The Directors may before recommending any dividend, whether preferential or otherwise, carry to reserve out of the profits of the Company, (including any premiums received upon the issue of debentures or other securities or rights of the Company) such sums as they think proper as a reserve or reserves which shall, at the discretion of the Directors, be applicable for any purpose to which the profits of the Company may properly be applied and pending such applications may at the like discretion either be employed in the business of the Company or be invested in such investments (including, but subject to the provisions of the Act shares of the Company or its holding company, if any) as the Directors may from time to time think fit. The Directors may also without placing the same to reserve carry forward any profits which they may think it prudent not to divide.

151. Capitalisation

151.1 The Company in general meeting may upon the recommendation of the Directors resolve that it is desirable to capitalise any undivided profits of the Company standing to the credit of the profit and loss account or otherwise and available for distribution (not being required for the payment of fixed dividends on any shares entitled to fixed preferential dividends with or without further participation in profits) and accordingly that the Directors be authorised and directed to appropriate the profits resolved to be capitalised to the members who would have been entitled to such profits if distributed by way of dividend and in the same proportions on condition that the same be not paid in cash but be applied either in or towards paying up any amounts for the time being unpaid on any shares held by such members respectively or paying up in full unissued shares or debentures of the Company to be allotted and distributed credited as fully paid up to and amongst such members in the same proportions, or partly in the one way and partly in the other, and the Directors shall give effect to such resolution.

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151.2 The Company in general meeting may, subject to the provisions of the Act upon the recommendation of the Directors, resolve that it is desirable to capitalise any part of the amount for the time being standing to the credit of any reserve account of the Company (including its share premium account and capital redemption reserve) of its profit and loss account and whether or not available for distribution by applying such sum in paying up in full unissued shares to be allotted as fully paid to those members of the Company who would have been entitled to that sum if it were distributed by way of dividend (and in the same proportions), and the Directors shall give effect to such resolution.

152. Authority

Whenever such a resolution as referred to in Article 151 shall have been passed, the Directors shall make all appropriations and applications of the profits or sum so resolved to be capitalised, and (subject to the provisions of the Act) all allotments and issues of fully paid shares or debentures, if any, and generally shall do all acts and things required to give effect to such capitalisation with full power to the Directors to make such provision by the issue of fractional certificates or by payment in cash or otherwise as they think fit for the case of shares or debentures becoming distributable in fractions, or to make provision whereby the benefit of fractional entitlements accrues to the Company rather than to the members concerned, and also to authorise any person to enter on behalf of all the members entitled to the benefit of such appropriations and applications into an agreement with the Company providing for the allotment to them respectively, credited as fully paid up, of any further shares to which they may be entitled upon such capitalisation, and any agreement made under such authority shall be effective and binding on all such members.

153. Record Dates

Notwithstanding any other provision of these Articles but without prejudice to the rights attached to any shares and subject always to the Statutes, the Company or the Directors may by resolution specify any date (the “record date”) as the date at the close of business (or such other time as the Directors may determine) on which persons registered as the holders of shares or other securities shall be entitled to receipt of any dividend, distribution, interest, allotment, issue, notice, information, document or circular and such record date may be on or at any time before the date on which the same is paid, made, given or served or (in the case of any dividend, distribution, interest, allotment or issue) at any time after the same is recommended, resolved, declared or announced, but without prejudice to the rights inter se in respect of the same of transferors and transferees of any such shares or other securities. No change in the register of such holders after the record date shall invalidate the same.

ACCOUNTS

154. Accounting records

The Directors shall cause true accounting records to be kept and preserved in accordance with the Act. The accounting records shall be kept at the Office, or (subject to the provisions of the Act) at such other place as the Directors think fit, and shall always be open to inspection by the officers of the Company. No member (other than an officer of the Company) shall have any right of inspecting any account or book or

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document of the Company except as conferred by Statutes or authorised by the Directors or by the Company in general meeting.

155. Preparation of accounts

The Directors shall from time to time, in accordance with the provisions of the Act, cause to be prepared and to be laid before the Company in general meeting such profit and loss accounts, balance sheets, group accounts (if any) and reports as are specified in the Act.

156. Accounts to members

156.1 A copy of every balance sheet and profit and loss account (including every document required by law to be annexed to them) which is to be laid before the Company in general meeting and of the directors’ and auditors’ reports shall not less than 21 days before the date of the meeting be sent to every member and to every holder of debentures of the Company, provided that:

(a) this Article shall not require copies of such documents to be sent to any person to whom, by virtue of section 423(2) of the Act, the Company is not required to send the same, nor to any person of whose address the Company is not aware nor to more than one of the joint holders of any shares or debentures; and

(b) instead of these documents there may be sent a copy of such summary financial statement as may be permitted, in such form as may be specified and subject to such conditions as may be required, by law to be sent to the members of, and holders of debentures of, the Company.

157. Electronic means

In accordance with Article 163.1, if and to the extent permitted by the Statutes, the summary financial statement as referred to in Article 156.1(b) may be delivered by means of electronic mail and/or through making it available on a website.

AUDITORS

158. Appointment

Auditors shall be appointed and their duties, rights and remuneration regulated in accordance with the provisions of the Act. Subject to the provisions of the Act, all acts done by any person acting as an auditor shall, as regards all persons dealing in good faith with the Company, be valid, notwithstanding that there was some defect in his appointment or that he was at the time of his appointment not qualified for appointment.

159. Correctness

In respect of each financial year of the Company the accounts of the Company shall be examined and the correctness of the balance sheet, profit and loss account and group accounts (if any) ascertained by an auditor or auditors.

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160. Auditors to attend meetings

The auditor or auditors shall be entitled to attend any general meeting and to receive notices of and other communications relating to any general meeting which a member is entitled to receive, and to be heard at any general meeting on any part of the business of the meeting which concerns him or them as auditor or auditors.

161. Change of auditors

The Company shall comply with the provisions of the Act relating to the sending of copies of special notices of certain resolutions concerning changes of auditors and to the giving notice of, and circulating to members, representations made by auditors retiring or proposed to be removed.

SERVICE OF NOTICE ON MEMBERS

162. Notices to be in writing

Any notice to be given to or by any person pursuant to these Articles shall be in writing, except that a notice convening a Directors’ or Directors’ committee meetings need not be in writing.

163. Service of notice on members

163.1 The Company may give any notice or document (including a share certificate, annual report, annual financial statements and/or a summary of financial statements) to a member either:

(a) personally; or

(b) by sending it by post or other delivery service in a prepaid envelope addressed to the member at his registered address; or

(c) by leaving it at that address; or

(d) by sending it in electronic form to such address (if any) as may for the time being be notified to the Company by or on behalf of the member for that purpose; or

(e) by making it available on a website and notifying the member of its availability in accordance with the Act. A member shall be deemed to have agreed that the Company may send or supply a document or information by means of a website if the conditions set out in the Act have been satisfied; or

(f) by any other means authorised in writing by the member concerned.

163.2 In the case of a member registered on an overseas branch register any such notice or document which is posted may be posted either in the United Kingdom or in the territory in which such branch register is maintained.

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163.3 In the case of joint holders of a share, all notices or documents shall be given to the joint holder whose name stands first in the register in respect of the joint holding. Notice so given shall be sufficient notice to all the joint holders.

163.4 Where a member (or, in the case of joint holders, the person first named in the register) has a registered address outside the United Kingdom but has notified the Company of an address within the United Kingdom at which notices or other documents may be given to him, he shall be entitled to have notices given to him at that address, but otherwise, no such member shall be entitled to receive any notice or document from the Company.

163.5 If on three consecutive occasions, notices or other documents have been sent through the post to any member at his registered address or his address for the service of notices but have been returned undelivered, such member shall not thereafter be entitled to receive notices or other documents from the Company until he shall have communicated with the Company and supplied in writing a new registered address or address within the United Kingdom for the service of notices.

163.6 The Directors may from time to time make such arrangements or regulations (if any) as they may from time to time in their absolute discretion think fit in relation to the giving of notices or other documents or information by electronic means by or to the Company and otherwise for the purpose of implementing and/or supplementing the provisions of these Articles and the Statutes in relation to electronic means; and such arrangements and regulations (as the case may be) shall have the same effect as if set out in this Article.

163.7 Nothing in this Article shall affect any provision of any of the Statutes requiring notices or documents to be delivered in a particular way.

164. Notice in case of death, bankruptcy or mental disorder

The Company may give notice to the person entitled to a share in consequence of the death or bankruptcy of a member or otherwise by operation of law, by sending or delivering it in any manner authorised by these Articles for the giving of notice to a member, addressed to that person by name, or by the title of representative of the deceased or trustee of the bankrupt or representative by operation of law or by any like description, at the address (if any) within the United Kingdom supplied for the purpose by the person claiming to be so entitled. Until such an address has been so supplied, a notice may be given in any manner in which it might have been given if the death or bankruptcy or operation of law had not occurred.

165. Evidence of service

165.1 Any member present, in person or by proxy, at any meeting of the Company or of the holders of any class of shares of the Company shall be deemed to have received due notice of such meeting, and, where requisite, of the purposes for which such meeting was called.

165.2 Any notice, certificate or other document addressed to a member at his registered address or address for service in the United Kingdom shall, if sent by post, be deemed to have been served or delivered on the day after the day when it was put in the post

49

(or, where second-class mail is employed, on the second day after the day when it was put in the post). Proof that an envelope containing the notice or document was properly addressed and put into the post as a prepaid letter shall be conclusive evidence that the notice was given. Any notice, certificate or other document not sent by post, but delivered or left at a registered address or address for service in the United Kingdom shall be deemed to have been served or delivered on the day on which it was so delivered or left. Any notice, certificate or other document sent by electronic communication shall, subject to the Statutes and these Articles, be deemed to have been served or delivered at the expiration of 24 hours from the time at which it was sent. Any notice or other document sent by a website shall, subject to the Statutes and these Articles, be deemed to have been served or delivered when first made available on the website or, if later, when the recipient received (or is deemed to have received) notice of the fact that such notice or document was available on the website.

166. Notice binding on transferees

Every person who, by operation of law, transfers or by any other means becomes entitled to a share shall be bound by a notice in respect of that share (other than a notice given by the Company under Section 793 of the Act) which, before his name is entered in the register, has been duly given to a person from whom he derives his title.

167. Notice by advertisement

Any notice to be given by the Company to the members or any of them, and not otherwise provided for by these Articles, shall be sufficiently given if given by advertisement in at least one national newspaper published in the United Kingdom and, where the Company keeps an overseas branch register, in at least one daily newspaper published in the territory in which such register is maintained. Any notice given by advertisement shall be deemed to have been served at noon on the day on which the advertisement first appears.

168. Suspension of the postal services

If, at any time by reason of the general suspension, interruption or curtailment of postal services or electronic communication or threat of such suspension, interruption or curtailment within the United Kingdom the Company is or would be unable effectively to convene a general meeting by notices sent through the post or by electronic communication, a general meeting may be convened by a notice advertised in at least one national newspaper published in the United Kingdom and, where the Company keeps an overseas branch register, in at least one daily newspaper published in the territory in which such register is maintained. Such notice shall be deemed to have been duly served on all members entitled thereto at noon on the day on which the first of such advertisement appears. In any such case the Company shall send confirmatory copies of the notice by post or by electronic communication if, at least seven days prior to the meeting, the posting of notices to addresses throughout the United Kingdom or, as the case may be, the sending of such notices by electronic communication, again becomes practicable.

169. Service of notices on the Company

Subject to the Statutes, Article 163.1 shall apply mutatis mutandis to the service by

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members of notices and documents on the Company, save that any notice, certificate (but not a share certificate) or document sent by electronic communication to the Company shall be deemed to have been served or delivered at the time it is received by the Company.

ELECTRONIC COMMUNICATION

170. Electronic Communication

170.1 Notwithstanding anything in these Articles to the contrary, any document or information to be given, sent, supplied, delivered or provided to any person by the Company, whether pursuant to these Articles, the Statutes or otherwise, is also to be treated as given, sent, supplied, delivered or provided where it is made available on a website, or is sent in electronic form, in the manner provided by the 2006 Act for the purposes of the Act (subject to the provisions of these Articles).

For the purposes of paragraph 10(2)(b) of schedule 5 to the Act, the Company may give, send, supply, deliver or provide documents or information to Members by making them available on a website.

For the purposes of paragraph 6.1.8R(1) of the Disclosure Guidance and Transparency Rules of the Financial Conduct Authority, the Company may use electronic means (as defined therein) to convey information or documents to members or holders of debt securities (as defined therein).

170.2 Notwithstanding anything in these Articles to the contrary, the Directors may from time to time make such arrangements or regulations (if any) as they may from time to time in their absolute discretion think fit in relation to the giving of notices or other documents or information by electronic means by or to the Company and otherwise for the purpose of implementing and/or supplementing the provisions of these Articles and the Statutes in relation to electronic means; and such arrangements and regulations (as the case may be) shall have the same effect as if set out in this Article 170.2.

DESTRUCTION OF DOCUMENTS

171. Destruction

171.1 The Company may destroy:

(a) any instrument of transfer, after six years from the date on which it is registered;

(b) any dividend mandate or any variation or cancellation of it or any notification of change of name or address, after two years from the date on which it is recorded;

(c) any share certificate, after one year from the date on which it is cancelled;

(d) any proxy form which has been used for a poll, after one year from the date of use;

(e) any proxy form which has not been used for a poll, after one month from the general meeting to which it relates and at which the poll was demanded; and

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(f) any other document on the basis of which any entry in the register is made, after six years from the date on which an entry was first made in the register in respect of it,

provided that the Company may destroy any such type of document at a date earlier than that authorised by this Article if a copy of such document is retained on microfilm or by other similar means on which such copy is retained until the expiration of the period applicable to the destruction of the original of such document.

172. Correct entries

172.1 It shall be conclusively presumed in favour of the Company that every entry in the register purporting to have been made on the basis of a document so destroyed was duly and properly made, that every instrument of transfer so destroyed was duly registered, that every share certificate so destroyed was duly cancelled, that every other document so destroyed had been properly dealt with in accordance with its terms and was valid and effective in accordance with the particulars in the records of the Company, provided that:

(a) this Article shall apply only to the destruction of a document in good faith and without notice of any claim (regardless of the parties to it) to which the document might be relevant;

(b) nothing in this Article shall be construed as imposing on the Company any liability in respect of the destruction of any such document otherwise than as provided for in this Article which would not attach to the Company in the absence of this Article; and

(c) references in this Article to the destruction of any document include references to the disposal of it in any manner.

WINDING UP

173. Authority to divide assets

If the Company shall be wound up (whether the liquidation is altogether voluntary, under supervision or by the court) the liquidator may, with the authority of a special resolution and any other sanction required by the Act, divide among the members in specie the whole or any part of the assets of the Company, and whether or not the assets shall consist of property of one kind or shall consist of properties of different kinds, and may for such purposes set such value as he deems fair upon any one or more class or classes of property, and may determine how such divisions shall be carried out as between the members or different classes of members. The liquidator may, with the like authority, vest any part of the assets in trustees upon such trusts for the benefit of members as the liquidator, with the like authority, shall think fit, and the liquidation of the Company may be closed and the Company dissolved, but so that no member shall be compelled to accept any shares in respect of which there is a liability.

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INDEMNITY

174. Right to indemnity

So far as the law allows, but without prejudice to any indemnity to which he may otherwise be entitled, any person who is or was at any time a Director, alternate director, officer or employee of the Company shall be entitled to be indemnified and, if the directors so determine, any other Relevant Person shall be entitled to be indemnified, out of the assets of the Company against any Relevant Liability.

175. Power to Insure

So far as the law allows, the Directors may take out, maintain, renew, establish, participate in and/or contribute to the cost of, insurance for, or for the benefit of any Relevant Person or any person who is or was at any time a trustee of any pension fund in which any employee or former employee of the Company or any of the other bodies corporate which are referred to in the definition of “Relevant Person” are interested, including insurance against any Relevant Liability and, so far as the law allows, may indemnify or exempt any such person from or against any such Relevant Liability.

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EX-4.1

Exhibit 4.1

EXECUTION VERSION

DEPOSIT AGREEMENT

by and among

AMRYT PHARMA HOLDINGS PLC

and

CITIBANK, N.A.,

as Depositary,

and

THE HOLDERS AND BENEFICIAL OWNERS OF

AMERICAN DEPOSITARY SHARES
ISSUED HEREUNDER

Dated as of September 24, 2019

TABLE OF CONTENTS

ARTICLE I
DEFINITIONS			1
	Section 1.1	“ADS Record Date”	1
	Section 1.2	“Affiliate”	2
	Section 1.3	“American Depositary Receipt(s)”, “ADR(s)” and “Receipt(s)”	2
	Section 1.4	“American Depositary Share(s)” and “ADS(s)”	2
	Section 1.5	“Articles of Association”	2
	Section 1.6	“Beneficial Owner”	2
	Section 1.7	“Certificated ADS(s)”	3
	Section 1.8	“Citibank”	3
	Section 1.9	“Commission”	3
	Section 1.10	“Company”	3
	Section 1.11	“CREST”	3
	Section 1.12	“Custodian”	3
	Section 1.13	“Deliver” and “Delivery”	3
	Section 1.14	“Deposit Agreement”	4
	Section 1.15	“Depositary”	4
	Section 1.16	“Deposited Property”	4
	Section 1.17	“Deposited Securities”	4
	Section 1.18	“Dollars” and “$”	4
	Section 1.19	“DTC”	4
	Section 1.20	“DTC Participant”	4
	Section 1.21	“Exchange Act”	4
	Section 1.22	“Foreign Currency”	4
	Section 1.23	“Full Entitlement ADR(s)”, “Full Entitlement ADS(s)” and “Full Entitlement Share(s)”	5
	Section 1.24	“Holder(s)”	5
	Section 1.25	“Partial Entitlement ADR(s)”, “Partial Entitlement ADS(s)” and “Partial Entitlement Share(s)”	5
	Section 1.26	“Principal Office”	5
	Section 1.27	“Registrar”	5
	Section 1.28	“Restricted Securities”	5
	Section 1.29	“Restricted ADR(s)”, “Restricted ADS(s)” and “Restricted Shares”	5
	Section 1.30	“Securities Act”	6
	Section 1.31	“Share Registrar”	6
	Section 1.32	“Shares”	6
	Section 1.33	“Uncertificated ADS(s)”	6
	Section 1.34	“United States” and “U.S.”	6
ARTICLE II
APPOINTMENT OF DEPOSITARY; FORM OF RECEIPTS; DEPOSIT OF SHARES; EXECUTION AND DELIVERY, TRANSFER AND SURRENDER OF RECEIPTS			6
	Section 2.1	Appointment of Depositary	6
	Section 2.2	Form and Transferability of ADSs	7
	Section 2.3	Deposit of Shares	8

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	Section 2.4	Registration and Safekeeping of Deposited Securities	10
	Section 2.5	Issuance of ADSs	10
	Section 2.6	Transfer, Combination and Split-up of ADRs	11
	Section 2.7	Surrender of ADSs and Withdrawal of Deposited Securities	12
	Section 2.8	Limitations on Execution and Delivery, Transfer, etc. of ADSs; Suspension of Delivery, Transfer, etc	13
	Section 2.9	Lost ADRs, etc	14
	Section 2.10	Cancellation and Destruction of Surrendered ADRs; Maintenance of Records	14
	Section 2.11	Escheatment	14
	Section 2.12	Partial Entitlement ADSs	14
	Section 2.13	Certificated/Uncertificated ADSs	15
	Section 2.14	Restricted ADSs	16
ARTICLE III
CERTAIN OBLIGATIONS OF HOLDERS AND BENEFICIAL OWNERS OF ADSs			18
	Section 3.1	Proofs, Certificates and Other Information	18
	Section 3.2	Liability for Taxes and Other Charges	19
	Section 3.3	Representations and Warranties on Deposit of Shares	19
	Section 3.4	Compliance with Information Requests	19
	Section 3.5	Ownership Restrictions	20
	Section 3.6	Reporting Obligations and Regulatory Approvals	20
ARTICLE IV
THE DEPOSITED SECURITIES			20
	Section 4.1	Cash Distributions	20
	Section 4.2	Distribution in Shares	21
	Section 4.3	Elective Distributions in Cash or Shares	22
	Section 4.4	Distribution of Rights to Purchase Additional ADSs	23
	Section 4.5	Distributions Other Than Cash, Shares or Rights to Purchase Shares	25
	Section 4.6	Distributions with Respect to Deposited Securities in Bearer Form	25
	Section 4.7	Redemption	26
	Section 4.8	Conversion of Foreign Currency	26
	Section 4.9	Fixing of ADS Record Date	27
	Section 4.10	Voting of Deposited Securities	28
	Section 4.11	Changes Affecting Deposited Securities	30
	Section 4.12	Available Information	31
	Section 4.13	Reports	31
	Section 4.14	List of Holders	31
	Section 4.15	Taxation	31
ARTICLE V
THE DEPOSITARY, THE CUSTODIAN AND THE COMPANY			32
	Section 5.1	Maintenance of Office and Transfer Books by the Registrar	32
	Section 5.2	Exoneration	33
	Section 5.3	Standard of Care	34

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	Section 5.4	Resignation and Removal of the Depositary; Appointment of Successor Depositary	35
	Section 5.5	The Custodian	35
	Section 5.6	Notices and Reports	36
	Section 5.7	Issuance of Additional Shares, ADSs etc	37
	Section 5.8	Indemnification	38
	Section 5.9	ADS Fees and Charges	39
	Section 5.10	Restricted Securities Owners	40
ARTICLE VI
AMENDMENT AND TERMINATION			40
	Section 6.1	Amendment/Supplement	40
	Section 6.2	Termination	41
ARTICLE VII
MISCELLANEOUS			42
	Section 7.1	Counterparts	42
	Section 7.2	No Third‑Party Beneficiaries/Acknowledgments	42
	Section 7.3	Severability	43
	Section 7.4	Holders and Beneficial Owners as Parties; Binding Effect	43
	Section 7.5	Notices	43
	Section 7.6	Governing Law and Jurisdiction	44
	Section 7.7	Assignment	46
	Section 7.8	Compliance with, and No Disclaimer under, U.S. Securities Laws	46
	Section 7.9	English Law References	46
	Section 7.10	Titles and References	46
EXHIBITS
		Form of ADR.	A-1
		Fee Schedule.	B-1

iii

DEPOSIT AGREEMENT

DEPOSIT AGREEMENT, dated as of September 24, 2019, by and among (i) Amryt Pharma Holdings plc, a public limited company incorporated under the laws of England and Wales, and its successors (the “Company”), (ii) CITIBANK, N.A., a national banking association organized under the laws of the United States of America, and its successors (“Citibank”) acting in its capacity as depositary under the terms of this Deposit Agreement, and any successor depositary hereunder (Citibank in such capacity, the “Depositary”), and (iii) all Holders and Beneficial Owners of American Depositary Shares issued hereunder (all such capitalized terms as hereinafter defined).

W I T N E S S E T H   T H A T:

WHEREAS, the Company desires to establish with the Depositary an ADR facility to provide inter alia for the deposit of the Shares (as hereinafter defined) and the creation of American Depositary Shares representing the Shares so deposited and for the execution and Delivery (as hereinafter defined) of American Depositary Receipts (as hereinafter defined) evidencing such American Depositary Shares; and

WHEREAS, the Depositary is willing to act as the Depositary for such ADR facility upon the terms set forth in the Deposit Agreement (as hereinafter defined); and

WHEREAS, any American Depositary Receipts issued pursuant to the terms of the Deposit Agreement are to be substantially in the form of Exhibit A attached hereto, with appropriate insertions, modifications and omissions, as hereinafter provided in the Deposit Agreement; and

WHEREAS, the board of directors of the Company (or an authorized committee thereof) has duly approved the establishment of such ADR facility on the terms set forth in the Deposit Agreement, the execution and delivery of the Deposit Agreement on behalf of the Company, and the actions of the Company and the transactions contemplated herein.

NOW, THEREFORE, for good and valuable consideration, the receipt and sufficiency of which are hereby acknowledged, the parties hereto agree as follows:

ARTICLE I

DEFINITIONS

All capitalized terms used, but not otherwise defined, herein shall have the meanings set forth below, unless otherwise clearly indicated:

Section 1.1          “ADS Record Date” shall have the meaning given to such term in Section 4.9.

Section 1.2          “Affiliate” shall have the meaning assigned to such term by the Commission (as hereinafter defined) under Regulation C promulgated under the Securities Act (as hereinafter defined), or under any successor regulation thereto.

Section 1.3          “American Depositary Receipt(s)”, “ADR(s)” and “Receipt(s)” shall mean the certificate(s) issued by the Depositary to evidence the American Depositary Shares issued under the terms of the Deposit Agreement in the form of Certificated ADS(s) (as hereinafter defined), as such ADRs may be amended from time to time in accordance with the provisions of the Deposit Agreement.  An ADR may evidence any number of ADSs and may, in the case of ADSs held through a central depository such as DTC, be in the form of a “Balance Certificate.”

Section 1.4          “American Depositary Share(s)” and “ADS(s)” shall mean the rights and interests in the Deposited Property (as hereinafter defined) granted to the Holders and Beneficial Owners pursuant to the terms and conditions of the Deposit Agreement and, if issued as Certificated ADS(s) (as hereinafter defined), the ADR(s) issued to evidence such ADSs.  ADS(s) may be issued under the terms of the Deposit Agreement in the form of (a) Certificated ADS(s) (as hereinafter defined), in which case the ADS(s) are evidenced by ADR(s), or (b) Uncertificated ADS(s) (as hereinafter defined), in which case the ADS(s) are not evidenced by ADR(s) but are reflected on the direct registration system maintained by the Depositary for such purposes under the terms of Section 2.13.  Unless otherwise specified in the Deposit Agreement or in any ADR, or unless the context otherwise requires, any reference to ADS(s) shall include Certificated ADS(s) and Uncertificated ADS(s), individually or collectively, as the context may require.  Each ADS shall represent the right to receive, and to exercise the beneficial ownership interests in, the number of Shares specified in the form of ADR attached hereto as Exhibit A (as amended from time to time) that are on deposit with the Depositary and/or the Custodian, subject, in each case, to the terms and conditions of the Deposit Agreement and the applicable ADR (if issued as a Certificated ADS), until there shall occur a distribution upon Deposited Securities referred to in Section 4.2 or a change in Deposited Securities referred to in Section 4.11 with respect to which additional ADSs are not issued, and thereafter each ADS shall represent the right to receive, and to exercise the beneficial ownership interests in, the applicable Deposited Property on deposit with the Depositary and the Custodian determined in accordance with the terms of such Sections, subject, in each case, to the terms and conditions of the Deposit Agreement and the applicable ADR (if issued as a Certificated ADS).  In addition, the ADS(s)-to-Share(s) ratio is subject to amendment as provided in Articles IV and VI of the Deposit Agreement (which may give rise to Depositary fees).

Section 1.5          “Articles of Association” shall mean the Articles of Association of the Company, as amended and restated from time to time.

Section 1.6          “Beneficial Owner” shall mean, as to any ADS, any person or entity having a beneficial interest deriving from the ownership of such ADS.  Notwithstanding anything to the contrary contained in the Deposit Agreement, any ADR(s) or any other instruments or agreements relating to the ADSs and the corresponding Deposited Property, the Depositary, the Custodian and their respective nominees are intended to be, and shall at all times during the term of the Deposit Agreement be, the record holders only of the Deposited Property represented by the ADSs for the benefit of the Holders and Beneficial Owners of the corresponding ADSs.  The Depositary, on its own behalf and on behalf of the Custodian and their respective nominees, disclaims any beneficial ownership interest in the Deposited Property held on behalf of the Holders and Beneficial Owners of ADSs.  The beneficial ownership interests in the Deposited Property are intended to be, and shall at all times during the term of the Deposit Agreement continue to be, vested in the Beneficial Owners of the ADSs representing the Deposited Property.  The beneficial ownership interests in the Deposited Property shall, unless otherwise agreed by the Depositary, be exercisable by the Beneficial Owners of the ADSs only through the Holders of such ADSs, by the Holders of the ADSs (on behalf of the applicable Beneficial Owners) only through the Depositary, and by the Depositary (on behalf of the Holders and Beneficial Owners of the corresponding ADSs) directly, or indirectly through the Custodian or their respective nominees, in each case upon the terms of the Deposit Agreement and, if applicable, the terms of the ADR(s) evidencing the ADSs.A Beneficial Owner of ADSs may or may not be the Holder of such ADSs.  A Beneficial Owner shall be able to exercise any right or receive any benefit hereunder solely through the person who is the Holder of the ADSs owned by such Beneficial Owner.  Unless otherwise identified to the Depositary, a Holder shall be deemed to be the Beneficial Owner of all the ADSs registered in his/her/its name.The manner in which a Beneficial Owner holds ADSs (e.g., in a brokerage account vs. as registered holder) may affect the rights and obligations of, the manner in which, and the extent to which, services are made available to, Beneficial Owners pursuant to the terms of the Deposit Agreement.

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Section 1.7          “Certificated ADS(s)” shall have the meaning set forth in Section 2.13.

Section 1.8          “Citibank” shall have the meaning set forth in the preamble.

Section 1.9          “Commission” shall mean the Securities and Exchange Commission of the United States or any successor governmental agency thereto in the United States.

Section 1.10        “Company” shall have the meaning set forth in the preamble.

Section 1.11        “CREST” shall mean the system for the paperless settlement of trades in securities and the holding of uncertificated securities operated by CREST Limited in accordance with the Uncertificated Securities Regulations 2001 (SI 2001 No. 3755), as amended from time to time, or any successor thereto.

Section 1.12        “Custodian” shall mean (i) as of the date hereof, Citibank, N.A.(London), having its principal office at Citigroup Centre, Canary Wharf, London, E14 5LB, United Kingdom, as the custodian of Deposited Property for the purposes of the Deposit Agreement, (ii) Citibank, N.A., acting as custodian of Deposited Property pursuant to the Deposit Agreement, and (iii) any other entity that may be appointed by the Depositary pursuant to the terms of Section 5.5 as successor, substitute or additional custodian hereunder.  The term “Custodian” shall mean any Custodian individually or all Custodians collectively, as the context requires.

Section 1.13        “Deliver” and “Delivery” shall mean (x) when used in respect of Shares and other Deposited Securities, either (i) the physical delivery of the certificate(s) representing such securities, or (ii) the book-entry transfer and recordation of such securities on the books of the Share Registrar (as hereinafter defined), in the book-entry settlement of CREST or in any other applicable book-entry settlement system, if available, and (y) when used in respect of ADSs, either (i) the physical delivery of ADR(s) evidencing the ADSs, or (ii) the book-entry transfer and recordation of ADSs on the books of the Depositary or any book-entry settlement system in which the ADSs are settlement-eligible.

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Section 1.14        “Deposit Agreement” shall mean this Deposit Agreement and all exhibits hereto, as the same may from time to time be amended and supplemented from time to time in accordance with the terms of the Deposit Agreement.

Section 1.15        “Depositary” shall have the meaning set forth in the preamble.

Section 1.16        “Deposited Property” shall mean the Deposited Securities and any cash and other property held on deposit by the Depositary and the Custodian in respect of the ADSs under the terms of the Deposit Agreement, subject, in the case of cash, to the provisions of Section 4.8.  All Deposited Property shall be held by the Custodian, the Depositary and their respective nominees for the benefit of the Holders and Beneficial Owners of the ADSs representing the Deposited Property.  The Deposited Property is not intended to, and shall not, constitute proprietary assets of the Depositary, the Custodian or their nominees. Beneficial ownership in the Deposited Property is intended to be, and shall at all times during the term of the Deposit Agreement continue to be, vested in the Beneficial Owners of the ADSs representing the Deposited Property.

Section 1.17       “Deposited Securities” shall mean the Shares and any other securities held on deposit by the Custodian from time to time in respect of the ADSs under the Deposit Agreement and constituting Deposited Property.

Section 1.18        “Dollars” and “$” shall refer to the lawful currency of the United States.

Section 1.19        “DTC” shall mean The Depository Trust Company, a national clearinghouse and the central book-entry settlement system for securities traded in the United States and, as such, the custodian for the securities of DTC Participants (as hereinafter defined) maintained in DTC, and any successor thereto.

Section 1.20        “DTC Participant” shall mean any financial institution (or any nominee of such institution) having one or more participant accounts with DTC for receiving, holding and delivering the securities and cash held in DTC.  A DTC Participant may or may not be a Beneficial Owner.  If a DTC Participant is not the Beneficial Owner of the ADSs credited to its account at DTC, or of the ADSs in respect of which the DTC Participant is otherwise acting, such DTC Participant shall be deemed, for all purposes hereunder, to have all requisite authority to act on behalf of the Beneficial Owner(s) of the ADSs credited to its account at DTC or in respect of which the DTC Participant is so acting.  A DTC Participant,  upon acceptance in any one of its DTC accounts of any ADSs (or any interest therein) issued in accordance with the terms and conditions of the Deposit Agreement, shall (notwithstanding any explicit or implicit disclosure that it may be acting on behalf of another party) be deemed for all purposes to be a party to, and bound by, the terms of the Deposit Agreement and the applicable ADR(s) to the same extent as, and as if the DTC Participant were, the Holder of such ADSs.

Section 1.21        “Exchange Act” shall mean the United States Securities Exchange Act of 1934, as amended from time to time.

Section 1.22        “Foreign Currency” shall mean any currency other than Dollars.

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Section 1.23        “Full Entitlement ADR(s)”, “Full Entitlement ADS(s)” and “Full Entitlement Share(s)” shall have the respective meanings set forth in Section 2.12.

Section 1.24        “Holder(s)” shall mean the person(s) in whose name the ADSs are registered on the books of the Depositary (or the Registrar, if any) maintained for such purpose.  A Holder may or may not be a Beneficial Owner.  If a Holder is not the Beneficial Owner of the ADS(s) registered in its name, such person shall be deemed, for all purposes hereunder, to have all requisite authority to act on behalf of the Beneficial Owners of the ADSs registered in its name.  The manner in which a Holder holds ADSs (e.g., in certificated vs. uncertificated form) may affect the rights and obligations of, and the manner in which, and the extent to which, the services are made available to, Holders pursuant to the terms of the Deposit Agreement.

Section 1.25        “Partial Entitlement ADR(s)”, “Partial Entitlement ADS(s)” and “Partial Entitlement Share(s)” shall have the respective meanings set forth in Section 2.12.

Section 1.26        “Principal Office” shall mean, when used with respect to the Depositary, the principal office of the Depositary at which at any particular time its depositary receipts business shall be administered, which, at the date of the Deposit Agreement, is located at 388 Greenwich Street, New York, New York 10013, U.S.A.

Section 1.27        “Registrar” shall mean the Depositary or any bank or trust company having an office in the Borough of Manhattan, The City of New York, which shall be appointed by the Depositary to register issuances, transfers and cancellations of ADSs as herein provided, and shall include any co-registrar appointed by the Depositary for such purposes.  Registrars (other than the Depositary) may be removed and substitutes appointed by the Depositary in accordance with Section 5.1 hereof.  Each Registrar (other than the Depositary) appointed pursuant to the Deposit Agreement shall be required to give notice in writing to the Depositary accepting such appointment and agreeing to be bound by the applicable terms of the Deposit Agreement.

Section 1.28        “Restricted Securities” shall mean Shares, Deposited Securities or ADSs which (i) have been acquired directly or indirectly from the Company or any of its Affiliates in a transaction or chain of transactions not involving any public offering and are subject to resale limitations under the Securities Act or the rules issued thereunder, or (ii) are held by an executive officer or director (or persons performing similar functions) or other Affiliate of the Company, or (iii) are subject to other restrictions on sale or deposit under the laws of the United States, England and Wales, or under a shareholder agreement or the Articles of Association or under the regulations of an applicable securities exchange unless, in each case, such Shares, Deposited Securities or ADSs are being transferred or sold to persons other than an Affiliate of the Company in a transaction (a) covered by an effective resale registration statement, or (b) exempt from the registration requirements of the Securities Act (as hereinafter defined), and the Shares, Deposited Securities or ADSs are not, when held by such person(s), Restricted Securities.

Section 1.29        “Restricted ADR(s)”, “Restricted ADS(s)” and “Restricted Shares” shall have the respective meanings set forth in Section 2.14.

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Section 1.30        “Securities Act” shall mean the United States Securities Act of 1933, as amended from time to time.

Section 1.31        “Share Registrar” shall mean Link Asset Services, a company registered in England and Wales, or any other institution organized under the laws of England and Wales appointed by the Company from time to time to carry out the duties of registrar for the Shares, and any successor thereto as the Company appoints from time to time.

Section 1.32        “Shares” shall mean the Company’s ordinary shares, having a par value of £0.06 per share, validly issued and outstanding and fully paid and may, if the Depositary so agrees after consultation with the Company, include evidence of the right to receive Shares; provided that in no event shall Shares include evidence of the right to receive Shares with respect to which the full purchase price has not been paid or Shares as to which preemptive rights have theretofore not been validly waived or exercised; provided further, however, that, if there shall occur any change in par value, split‑up, consolidation, reclassification, exchange, conversion or any other event described in Section 4.11 in respect of the Shares of the Company, the term “Shares” shall thereafter, to the maximum extent permitted by law, represent the successor securities resulting from such event.

Section 1.33        “Uncertificated ADS(s)” shall have the meaning set forth in Section 2.13.

Section 1.34        “United States” and “U.S.” shall have the meaning assigned to it in Regulation S as promulgated by the Commission under the Securities Act.

ARTICLE II

APPOINTMENT OF DEPOSITARY; FORM OF RECEIPTS; DEPOSIT OF SHARES; EXECUTION AND DELIVERY, TRANSFER AND SURRENDER OF RECEIPTS

Section 2.1          Appointment of Depositary.  The Company hereby appoints the Depositary as depositary for the Deposited Property and hereby authorizes and directs the Depositary to act in accordance with the terms and conditions set forth in the Deposit Agreement and the applicable ADRs.  Each Holder and each Beneficial Owner, upon acceptance of any ADSs (or any interest therein) issued in accordance with the terms and conditions of the Deposit Agreement shall be deemed for all purposes to (a) be a party to and bound by the terms of the Deposit Agreement and the applicable ADR(s), and (b) appoint the Depositary its attorney-in-fact, with full power to delegate, to act on its behalf and to take any and all actions contemplated in the Deposit Agreement and the applicable ADR(s), to adopt any and all procedures necessary to comply with applicable law and to take such action as the Depositary in its sole discretion may deem necessary or appropriate to carry out the purposes of the Deposit Agreement and the applicable ADR(s), the taking of such actions to be the conclusive determinant of the necessity and appropriateness thereof.

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Section 2.2          Form and Transferability of ADSs.

(a)          Form.  Certificated ADSs shall be evidenced by definitive ADRs which shall be engraved, printed, lithographed or produced in such other manner as may be agreed upon by the Company and the Depositary.  ADRs may be issued under the Deposit Agreement in denominations of any whole number of ADSs.  The ADRs shall be substantially in the form set forth in Exhibit A to the Deposit Agreement, with any appropriate insertions, modifications and omissions, in each case as otherwise contemplated in the Deposit Agreement or required by law.  ADRs shall be (i) dated, (ii) signed by the manual or facsimile signature of a duly authorized signatory of the Depositary, (iii) countersigned by the manual or facsimile signature of a duly authorized signatory of the Registrar, and (iv) registered in the books maintained by the Registrar for the registration of issuances and transfers of ADSs.  No ADR and no CertificatedADS evidenced thereby shall be entitled to any benefits under the Deposit Agreement or be valid or enforceable for any purpose against the Depositary or the Company, unless such ADR shall have been so dated, signed, countersigned and registered.ADRs bearing the facsimile signature of a duly-authorized signatory of the Depositary or the Registrar, who at the time of signature was a duly-authorized signatory of the Depositary or the Registrar, as the case may be, shall bind the Depositary, notwithstanding the fact that such signatory has ceased to be so authorized prior to the Delivery of such ADR by the Depositary.  The ADRs shall bear a CUSIP number that is different from any CUSIP number that was, is or may be assigned to any depositary receipts previously or subsequently issued pursuant to any other arrangement between the Depositary (or any other depositary) and the Company and which are not ADRs outstanding hereunder.

(b)          Legends.  The ADRs may be endorsed with, or have incorporated in the text thereof, such legends or recitals not inconsistent with the provisions of the Deposit Agreement as may be (i) necessary to enable the Depositary and the Company to perform their respective obligations hereunder, (ii) required to comply with any applicable laws or regulations, or with the rules and regulations of any securities exchange or market upon which ADSs may be traded, listed or quoted, or to conform with any usage with respect thereto, (iii) necessary to indicate any special limitations or restrictions to which any particular ADRs or ADSs are subject by reason of the date of issuance of the Deposited Securities or otherwise, or (iv) required by any book-entry system in which the ADSs are held.  Holders and Beneficial Owners shall be deemed, for all purposes, to have notice of, and to be bound by, the terms and conditions of the legends set forth, in the case of Holders, on the ADR registered in the name of the applicable Holders or, in the case of Beneficial Owners, on the ADR representing the ADSs owned by such Beneficial Owners.

(c)          Title.  Subject to the limitations contained herein and in the ADR, title to an ADR (and to each Certificated ADS evidenced thereby) shall be transferable upon the same terms as a certificated security under the laws of the State of New York, provided that, in the case of Certificated ADSs, such ADR has been properly endorsed or is accompanied by proper instruments of transfer.  Notwithstanding any notice to the contrary, the Depositary and the Company may deem and treat the Holder of an ADS (that is, the person in whose name an ADS is registered on the books of the Depositary) as the absolute owner thereof for all purposes.  Neither the Depositary nor the Company shall have any obligation nor be subject to any liability under the Deposit Agreement or any ADR to any holder or any Beneficial Owner unless, in the case of a holder of ADSs, such holder is the Holder registered on the books of the Depositary or, in the case of a Beneficial Owner, such Beneficial Owner, or the Beneficial Owner’s representative, is the Holder registered on the books of the Depositary.

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(d)          Book‑Entry Systems.  The Depositary shall make arrangements for the acceptance of the ADSs into DTC.  All ADSs held through DTC will be registered in the name of the nominee for DTC (currently “Cede & Co.”).  As such, the nominee for DTC will be the only “Holder” of all ADSs held through DTC.  Unless issued by the Depositary as Uncertificated ADSs, the ADSs registered in the name of Cede & Co. will be evidenced by one or more ADR(s) in the form of a “Balance Certificate,” which will provide that it represents the aggregate number of ADSs from time to time indicated in the records of the Depositary as being issued hereunder and that the aggregate number of ADSs represented thereby may from time to time be increased or decreased by making adjustments on such records of the Depositary and of DTC or its nominee as hereinafter provided.  Citibank, N.A. (or such other entity as is appointed by DTC or its nominee) may hold the “Balance Certificate” as custodian for DTC.  Each Beneficial Owner of ADSs held through DTC must rely upon the procedures of DTC and the DTC Participants to exercise or be entitled to any rights attributable to such ADSs.  The DTC Participants shall for all purposes be deemed to have all requisite power and authority to act on behalf of the Beneficial Owners of the ADSs held in the DTC Participants’ respective accounts in DTC and the Depositary shall for all purposes be authorized to rely upon any instructions and information given to it by DTC Participants.  So long as ADSs are held through DTC or unless otherwise required by law, ownership of beneficial interests in the ADSs registered in the name of the nominee for DTC will be shown on, and transfers of such ownership will be effected only through, records maintained by (i) DTC or its nominee (with respect to the interests of DTC Participants), or (ii) DTC Participants or their nominees (with respect to the interests of clients of DTC Participants).  Any distributions made, and any notices given, by the Depositary to DTC under the terms of the Deposit Agreement shall (unless otherwise specified by the Depositary) satisfy the Depositary’s obligations under the Deposit Agreement to make such distributions, and give such notices, in respect of the ADSs held in DTC (including, for avoidance of doubt, to the DTC Participants holding the ADSs in their DTC accounts and to the Beneficial Owners of such ADSs).

Section 2.3          Deposit of Shares.  Subject to the terms and conditions of the Deposit Agreement and applicable law, Shares or evidence of rights to receive Shares (other than Restricted Securities) may be deposited by any person (including the Depositary in its individual capacity but subject, however, in the case of the Company or any Affiliate of the Company, to Section 5.7) at any time, whether or not the transfer books of the Company or the Share Registrar, if any, are closed, by Delivery of the Shares to the Custodian.  Every deposit of Shares shall be accompanied by the following:  (A) (i) in the case of Shares represented by certificates issued in registered form, appropriate instruments of transfer or endorsement, in a form satisfactory to the Custodian, (ii) in the case of Shares represented by certificates in bearer form, the requisite coupons and talons pertaining thereto, and (iii) in the case of Shares delivered by book-entry transfer and recordation, confirmation of such book-entry transfer and recordation in the books of the Share Registrar or of CREST or any other book-entry settlement system, as applicable, to the Custodian or that irrevocable instructions have been given to cause such Shares to be so transferred and recorded, (B) such certifications and payments (including, without limitation, the Depositary’s fees and related charges) and evidence of such payments (including, without limitation, stamping or otherwise marking such Shares by way of receipt) as may be required by the Depositary or the Custodian in accordance with the provisions of the Deposit Agreement and applicable law, (C) if the Depositary so requires, a written order directing the Depositary to issue and deliver to, or upon the written order of, the person(s) stated in such order the number of ADSs representing the Shares so deposited, (D) evidence reasonably satisfactory to the Depositary (which may be an opinion of counsel;  provided that, if the Company is not the depositor, it shall not be obligated to pay the costs of obtaining any such opinion) that all necessary approvals have been granted by, or there has been compliance with the rules and regulations of, any applicable governmental agency of England and Wales, and (E) if the Depositary so requires, (i) an agreement, assignment or instrument satisfactory to the Depositary or the Custodian which provides for the prompt transfer by any person in whose name the Shares are or have been recorded to the Custodian of any distribution, or right to subscribe for additional Shares or to receive other property in respect of any such deposited Shares or, in lieu thereof, such indemnity or other agreement as shall be satisfactory to the Depositary or the Custodian and (ii) if the Shares are registered in the name of the person on whose behalf they are presented for deposit, a proxy or proxies entitling the Custodian to exercise voting rights in respect of the Shares for any and all purposes until the Shares so deposited are registered in the name of the Depositary, the Custodian or any nominee.

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Without limiting any other provision of the Deposit Agreement, the Depositary shall instruct the Custodian not to, and the Depositary shall not knowingly, accept for deposit (a) any Restricted Securities (except as contemplated by Section 2.14) nor (b) any fractional Shares or fractional Deposited Securities nor (c) a number of Shares or Deposited Securities which upon application of the ADS to Shares ratio would give rise to fractional ADSs.  No Shares shall be accepted for deposit unless accompanied by evidence, if any is required by the Depositary, that is reasonably satisfactory to the Depositary or the Custodian that all conditions to such deposit have been satisfied by the person depositing such Shares under the laws and regulations of England and Wales and any necessary approval has been granted by any applicable governmental body in England and Wales, if any.  The Depositary may issue ADSs against evidence of rights to receive Shares from the Company, any agent of the Company or any custodian, registrar, transfer agent, clearing agency or other entity involved in ownership or transaction records in respect of the Shares.  Such evidence of rights shall consist of written blanket or specific guarantees of ownership of Shares furnished by the Company or any such custodian, registrar, transfer agent, clearing agency or other entity involved in ownership or transaction records in respect of the Shares.

Without limitation of the foregoing, the Depositary shall not knowingly accept for deposit under the Deposit Agreement (A) any Shares or other securities required to be registered under the provisions of the Securities Act, unless (i) a registration statement is in effect as to such Shares or other securities or (ii) the deposit is made upon terms contemplated in Section 2.14, or (B) any Shares or other securities the deposit of which would violate any provisions of the Articles of Association or any applicable laws.  For purposes of the foregoing sentence, the Depositary shall be entitled to rely upon representations and warranties made or deemed made pursuant to the Deposit Agreement and shall not be required to make any further investigation.  The Depositary will comply with written instructions of the Company (received by the Depositary reasonably in advance) not to accept for deposit hereunder any Shares identified in such instructions at such times and under such circumstances as may reasonably be specified in such instructions in order to facilitate the Company’s compliance with the securities laws of the United States.

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Section 2.4          Registration and Safekeeping of Deposited Securities.  The Depositary shall instruct the Custodian upon each Delivery of registered Shares being deposited hereunder with the Custodian (or other Deposited Securities pursuant to Article IV hereof), together with the other documents above specified, to present such Shares, together with the appropriate instrument(s) of transfer or endorsement, duly stamped (if applicable), to the Share Registrar for transfer and registration of the Shares or, to the extent applicable, other Deposited Securities (as soon as transfer and registration can be accomplished and at the expense of the person for whom the deposit is made) in the name of the Depositary, the Custodian or a nominee of either.  Deposited Securities shall be held by the Depositary, or by a Custodian for the account and to the order of the Depositary or a nominee of the Depositary, in each case, on behalf of the Holders and Beneficial Owners, at such place(s) as the Depositary or the Custodian shall determine.  Notwithstanding anything to the contrary contained in the Deposit Agreement, any ADR(s), or any other instruments or agreements relating to the ADSs and the corresponding Deposited Property, the registration of the Deposited Securities in the name of the Depositary, the Custodian or any of their respective nominees, shall, to the maximum extent permitted by applicable law, vest in the Depositary, the Custodian or the applicable nominee the record ownership in the applicable Deposited Securities with the beneficial ownership rights and interests in such Deposited Securities being at all times vested with the Beneficial Owners of the ADSs representing the Deposited Securities.  Notwithstanding the foregoing, the Depositary, the Custodian and the applicable nominee shall at all times be entitled to exercise the beneficial ownership rights in all Deposited Property, in each case only on behalf of the Holders and Beneficial Owners of the ADSs representing the Deposited Property, upon the terms set forth in the Deposit Agreement and, if applicable, the ADR(s) representing the ADSs.  The Depositary, the Custodian and their respective nominees shall for all purposes be deemed to have all requisite power and authority to act in respect of Deposited Property on behalf of the Holders and Beneficial Owners of ADSs representing the Deposited Property, and upon making payments to, or acting upon instructions from, or information provided by, the Depositary, the Custodian or their respective nominees all persons shall be authorized to rely upon such power and authority.

Section 2.5          Issuance of ADSs.  The Depositary has made arrangements with the Custodian for the Custodian to confirm to the Depositary upon receipt of a deposit of Shares (i) that a deposit of Shares has been made pursuant to Section 2.3, (ii) that such Deposited Securities have been recorded in the name of the Depositary, the Custodian or a nominee of either on the shareholders’ register maintained by or on behalf of the Company by the Share Registrar, on the books of CREST or any other applicable book-entry settlement entity, if available, (iii) that all required documents have been received, and (iv) the person(s) to whom or upon whose order ADSs are deliverable in respect thereof and the number of ADSs to be so delivered.  Such notification may be made by letter, cable, telex, SWIFT message or, at the risk and expense of the person making the deposit, by facsimile or other means of electronic transmission.  Upon receiving such notice from the Custodian, the Depositary, subject to the terms and conditions of the Deposit Agreement and applicable law, shall issue the ADSs representing the Shares so deposited to or upon the order of the person(s) named in the notice delivered to the Depositary and, if applicable, shall execute and deliver at its Principal Office Receipt(s) registered in the name(s) requested by such person(s) and evidencing the aggregate number of ADSs to which such person(s) are entitled, but, in each case, only upon payment to the Depositary of the charges of the Depositary for accepting a deposit of Shares and issuing ADSs (as set forth in Section 5.9 and Exhibit B hereto) and all taxes and governmental charges and fees payable in connection with such deposit and the transfer of the Shares and the issuance of the ADS(s).  The Depositary shall only issue ADSs in whole numbers and deliver, if applicable, ADR(s) evidencing whole numbers of ADSs.

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Section 2.6          Transfer, Combination and Split-up of ADRs.

(a)          Transfer.  The Registrar shall register the transfer of ADRs (and of the ADSs represented thereby) on the books maintained for such purpose and the Depositary shall (x) cancel such ADRs and execute new ADRs evidencing the same aggregate number of ADSs as those evidenced by the ADRs canceled by the Depositary, (y) cause the Registrar to countersign such new ADRs and (z) Deliver such new  ADRs to or upon the order of the person entitled thereto, if each of the following conditions has been satisfied:  (i) the ADRs have been duly Delivered by the Holder (or by a duly authorized attorney of the Holder) to the Depositary at its Principal Office for the purpose of effecting a transfer thereof, (ii) the surrendered ADRs have been properly endorsed or are accompanied by proper instruments of transfer (including signature guarantees in accordance with standard securities industry practice), (iii) the surrendered ADRs have been duly stamped (if required by the laws of the State of New York or of the United States), and (iv) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 and Exhibit B hereto) have been paid, subject, however, in each case, to the terms and conditions of the applicable ADRs, of the Deposit Agreement and of applicable law, in each case as in effect at the time thereof.

(b)          Combination & Split-Up.  The Registrar shall register the split-up or combination of ADRs (and of the ADSs represented thereby) on the books maintained for such purpose and the Depositary shall (x) cancel such ADRs and execute new ADRs for the number of ADSs requested, but in the aggregate not exceeding the number of ADSs evidenced by the ADRs canceled by the Depositary, (y) cause the Registrar to countersign such new ADRs and (z) Deliver such new ADRs to or upon the order of the Holder thereof, if each of the following conditions has been satisfied:  (i) the ADRs have been duly Delivered by the Holder (or by a duly authorized attorney of the Holder) to the Depositary at its Principal Office for the purpose of effecting a split-up or combination thereof, and (ii) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 and Exhibit B hereto) have been paid, subject, however, in each case, to the terms and conditions of the applicable ADRs, of the Deposit Agreement and of applicable law, in each case as in effect at the time thereof.

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Section 2.7          Surrender of ADSs and Withdrawal of Deposited Securities.  The Holder of ADSs shall be entitled to Delivery (at the Custodian’s designated office) of the Deposited Securities at the time represented by the ADSs upon satisfaction of each of the following conditions: (i) the Holder (or a duly-authorized attorney of the Holder) has duly Delivered ADSs to the Depositary at its Principal Office (and if applicable, the ADRs evidencing such ADSs) for the purpose of withdrawal of the Deposited Securities represented thereby, (ii) if applicable andso required by the Depositary, the ADRs Delivered to the Depositary for such purpose have been properly endorsed in blank or are accompanied by proper instruments of transfer in blank (including signature guarantees in accordance with standard securities industry practice), (iii) if so required by the Depositary, the Holder of the ADSs has executed and delivered to the Depositary a written order directing the Depositary to cause the Deposited Securities being withdrawn to be Delivered to or upon the written order of the person(s) designated in such order, and (iv) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 and Exhibit B) have been paid, subject, however, in each case, to the terms and conditions of the ADRs evidencing the surrendered ADSs, of the Deposit Agreement, of the Company’s Articles of Association and of any applicable laws and the rules of CREST or any other applicable book-entry settlement system, if available, and to any provisions of or governing the Deposited Securities , in each case as in effect at the time thereof.

Upon satisfaction of each of the conditions specified above, the Depositary (i) shall cancel the ADSs Delivered to it (and, if applicable, the ADR(s) evidencing the ADSs so Delivered), (ii) shall direct the Registrar to record the cancellation of the ADSs so Delivered on the books maintained for such purpose, and (iii) shall direct the Custodian to Deliver, or cause the Delivery of, in each case, without unreasonable delay, the Deposited Securities represented by the ADSs so canceled together with any certificate or other document of title for the Deposited Securities, or evidence of the electronic transfer thereof (if available), as the case may be, to or upon the written order of the person(s) designated in the order delivered to the Depositary for such purpose, subject however, in each case, to the terms and conditions of the Deposit Agreement, of the ADRs evidencing the ADSs so canceled, of the Articles of Association, of any applicable laws and of the rules of CREST or any other applicable book-entry settlement system, if available, and to the terms and conditions of or governing the Deposited Securities, in each case as in effect at the time thereof.

The Depositary shall not accept for surrender ADSs representing less than one (1) Share.  In the case of Delivery to it of ADSs representing a number other than a whole number of Shares, the Depositary shall cause ownership of the appropriate whole number of Shares to be Delivered in accordance with the terms hereof, and shall, at the discretion of the Depositary, either (i) return to the person surrendering such ADSs the number of ADSs representing any remaining fractional Share, or (ii) sell or cause to be sold the fractional Share represented by the ADSs so surrendered and remit the proceeds of such sale (net of (a) applicable fees and charges of, and expenses incurred by, the Depositary and (b) taxes withheld) to the person surrendering the ADSs.

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Notwithstanding anything to the contrary contained in any ADR or the Deposit Agreement, the Depositary may make delivery at the Principal Office of the Depositary of Deposited Property consisting of (i) any cash dividends or cash distributions, or (ii) any proceeds from the sale of any non-cash distributions, which are at the time held by the Depositary in respect of the Deposited Securities represented by the ADSs surrendered for cancellation and withdrawal.  At the request, risk and expense of any Holder so surrendering ADSs, and for the account of such Holder, the Depositary shall direct the Custodian to forward (to the extent permitted by law) any Deposited Property (other than Deposited Securities) held by the Custodian in respect of such ADSs to the Depositary for delivery at the Principal Office of the Depositary.  Such direction shall be given by letter or, at the request, risk and expense of such Holder, by cable, telex or facsimile transmission.

Section 2.8          Limitations on Execution and Delivery, Transfer, etc. of ADSs; Suspension of Delivery, Transfer, etc.

(a)          Additional Requirements.  As a condition precedent to the execution and Delivery, the registration of issuance, transfer, split-up, combination or surrender, of any ADS, the delivery of any distribution thereon, or the withdrawal of any Deposited Property, the Depositary or the Custodian may require (i) payment from the depositor of Shares or presenter of ADSs or of an ADR of a sum sufficient to reimburse it for any tax or other governmental charge and any stock transfer or registration fee with respect thereto (including any such tax or charge and fee with respect to Shares being deposited or withdrawn) and payment of any applicable fees and charges of the Depositary as provided in Section 5.9 and Exhibit B, (ii) the production of proof satisfactory to it as to the identity and genuineness of any signature or any other matter contemplated by Section 3.1, and (iii) compliance with (A) any laws or governmental regulations relating to the execution and Delivery of ADRs or ADSs or to the withdrawal of Deposited Securities and (B) such reasonable regulations as the Depositary and the Company may establish consistent with the provisions of the representative ADR, if applicable, the Deposit Agreement and applicable law.

(b)          Additional Limitations.  The issuance of ADSs against deposits of Shares generally or against deposits of particular Shares may be suspended, or the deposit of particular Shares may be refused, or the registration of transfer of ADSs in particular instances may be refused, or the registration of transfers of ADSs generally may be suspended, during any period when the transfer books of the Company, the Depositary, a Registrar or the Share Registrar are closed or if any such action is deemed necessary or advisable by the Depositary (whereupon the Depositary shall notify the Company) or the Company, in good faith, at any time or from time to time because of any requirement of law or regulation, any government or governmental body or commission or any securities exchange on which the ADSs or Shares are listed, or under any provision of the Deposit Agreement or the representative ADR(s), if applicable, or under any provision of, or governing, the Deposited Securities, or because of a meeting of shareholders of the Company or for any other reason, subject, in all cases, to Section 7.8.

(c)          Regulatory Restrictions.  Notwithstanding any provision of the Deposit Agreement or any ADR(s) to the contrary, Holders are entitled to surrender outstanding ADSs to withdraw the Deposited Securities associated herewith at any time subject only to (i) temporary delays caused by closing the transfer books of the Depositary or the Company or the deposit of Shares in connection with voting at a shareholders’ meeting or the payment of dividends, (ii) the payment of fees, taxes and similar charges, (iii) compliance with any U.S. or foreign laws or governmental regulations relating to the ADSs or to the withdrawal of the Deposited Securities, and (iv) other circumstances specifically contemplated by Instruction I.A.(l) of the General Instructions to Form F-6 (as such General Instructions may be amended from time to time) under the Securities Act.

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Section 2.9          Lost ADRs, etc.  In case any ADR shall be mutilated, destroyed, lost, or stolen, the Depositary shall execute and deliver a new ADR of like tenor at the expense of the Holder (a) in the case of a mutilated ADR, in exchange of and substitution for such mutilated ADR upon cancellation thereof, or (b) in the case of a destroyed, lost or stolen ADR, in lieu of and in substitution for such destroyed, lost, or stolen ADR, after the Holder thereof (i) has submitted to the Depositary a written request for such exchange and substitution before the Depositary has notice that the ADR has been acquired by a bona fide purchaser, (ii) has provided such security or indemnity (including an indemnity bond) as may be required by the Depositary to save it and any of its agents harmless, and (iii) has satisfied any other reasonable requirements imposed by the Depositary, including, without limitation, evidence satisfactory to the Depositary of such destruction, loss or theft of such ADR, the authenticity thereof and the Holder’s ownership thereof.

Section 2.10        Cancellation and Destruction of Surrendered ADRs; Maintenance of Records.  All ADRs surrendered to the Depositary shall be canceled by the Depositary.  Canceled ADRs shall not be entitled to any benefits under the Deposit Agreement or be valid or enforceable against the Depositary or the Company for any purpose.  The Depositary is authorized to destroy ADRs so canceled, provided the Depositary maintains a record of all destroyed ADRs.  Any ADSs held in book-entry form (e.g., through accounts at DTC) shall be deemed canceled when the Depositary causes the number of ADSs evidenced by the Balance Certificate to be reduced by the number of ADSs surrendered (without the need to physically destroy the Balance Certificate).  The Depositary agrees to maintain records of all ADRs surrendered and the Shares withdrawn, substitute ADRs, delivered and cancelled or destroyed ADRs as required by the regulations governing the stock transfer industry.  Upon the request of the Company, the Depositary shall provide a copy of such records to the Company.

Section 2.11        Escheatment.  In the event any unclaimed property relating to the ADSs, for any reason, is in the possession of Depositary and has not been claimed by the Holder thereof or cannot be delivered to the Holder thereof through usual channels, the Depositary shall, upon expiration of any applicable statutory period relating to abandoned property laws, escheat such unclaimed property to the relevant authorities in accordance with the laws of each of the relevant States of the United States.

Section 2.12        Partial Entitlement ADSs.  In the event any Shares are deposited which (i) entitle the holders thereof to receive a per-share distribution or other entitlement in an amount different from the Shares then on deposit or (ii) are not fully fungible (including, without limitation, as to settlement or trading) with the Shares then on deposit (the Shares then on deposit collectively, “Full Entitlement Shares” and the Shares with different entitlement, “Partial Entitlement Shares”), the Depositary shall (i) cause the Custodian to hold Partial Entitlement Shares separate and distinct from Full Entitlement Shares, and (ii) subject to the terms of the Deposit Agreement, issue ADSs representing Partial Entitlement Shares which are separate and distinct from the ADSs representing Full Entitlement Shares, by means of separate CUSIP numbering and legending (if necessary) and, if applicable, by issuing ADRs evidencing such ADSs with applicable notations thereon (“Partial Entitlement ADSs/ADRs” and “Full Entitlement ADSs/ADRs”, respectively).  If and when Partial Entitlement Shares become Full Entitlement Shares, the Depositary shall (a) give notice thereof to Holders of Partial Entitlement ADSs and give Holders of Partial Entitlement ADRs the opportunity to exchange such Partial Entitlement ADRs for Full Entitlement ADRs, (b) cause the Custodian to transfer the Partial Entitlement Shares into the account of the Full Entitlement Shares, and (c) take such actions as are necessary to remove the distinctions between (i) the Partial Entitlement ADRs and ADSs, on the one hand, and (ii) the Full Entitlement ADRs and ADSs on the other.  Holders and Beneficial Owners of Partial Entitlement ADSs shall only be entitled to the entitlements of Partial Entitlement Shares.  Holders and Beneficial Owners of Full Entitlement ADSs shall be entitled only to the entitlements of Full Entitlement Shares.  All provisions and conditions of the Deposit Agreement shall apply to Partial Entitlement ADRs and ADSs to the same extent as Full Entitlement ADRs and ADSs, except as contemplated by this Section 2.12.  The Depositary is authorized to take any and all other actions as may be necessary (including, without limitation, making the necessary notations on ADRs) to give effect to the terms of this Section 2.12.  The Company agrees to give timely written notice to the Depositary if any Shares issued or to be issued are Partial Entitlement Shares and shall provide reasonable assistance to the Depositary with the establishment of procedures enabling the identification of Partial Entitlement Shares upon Delivery to the Custodian.

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Section 2.13        Certificated/Uncertificated ADSs.  Notwithstanding any other provision of the Deposit Agreement, the Depositary may, at any time and from time to time, issue ADSs that are not evidenced by ADRs (such ADSs, the “Uncertificated ADS(s)” and the ADS(s) evidenced by ADR(s), the “Certificated ADS(s)”).  When issuing and maintaining Uncertificated ADS(s) under the Deposit Agreement, the Depositary shall at all times be subject to (i) the standards applicable to registrars and transfer agents maintaining direct registration systems for equity securities in New York and issuing uncertificated securities under New York law, and (ii) the terms of New York law applicable to uncertificated equity securities.  Uncertificated ADSs shall not be represented by any instruments but shall be evidenced by registration in the books of the Depositary maintained for such purpose.  Holders of Uncertificated ADSs, that are not subject to any registered pledges, liens, restrictions or adverse claims of which the Depositary has notice at such time, shall at all times have the right to exchange the Uncertificated ADS(s) for Certificated ADS(s) of the same type and class, subject in each case to (x) applicable laws and any rules and regulations the Depositary may have established in respect of the Uncertificated ADSs, and (y) the continued availability of Certificated ADSs in the U.S.  Holders of Certificated ADSs shall, if the Depositary maintains a direct registration system for the ADSs, have the right to exchange the Certificated ADSs for Uncertificated ADSs upon (i) the due surrender of the Certificated ADS(s) to the Depositary for such purpose and (ii) the presentation of a written request to that effect to the Depositary, subject in each case to (a) all liens and restrictions noted on the ADR evidencing the Certificated ADS(s) and all adverse claims of which the Depositary then has notice, (b) the terms of the Deposit Agreement and the rules and regulations that the Depositary may establish for such purposes hereunder, (c) applicable law, and (d) payment of the Depositary fees and expenses applicable to such exchange of Certificated ADS(s) for Uncertificated ADS(s).  Uncertificated ADSs shall in all material respects be identical to Certificated ADS(s) of the same type and class, except that (i) no ADR(s) shall be, or shall need to be, issued to evidence Uncertificated ADS(s), (ii) Uncertificated ADS(s) shall, subject to the terms of the Deposit Agreement, be transferable upon the same terms and conditions as uncertificated securities under New York law, (iii) the ownership of Uncertificated ADS(s) shall be recorded on the books of the Depositary maintained for such purpose and evidence of such ownership shall be reflected in periodic statements provided by the Depositary to the Holder(s) in accordance with applicable New York law, (iv) the Depositary may from time to time, upon notice to the Holders of Uncertificated ADSs affected thereby, establish rules and regulations, and amend or supplement existing rules and regulations, as may be deemed reasonably necessary to maintain Uncertificated ADS(s) on behalf of Holders, provided that (a) such rules and regulations do not conflict with the terms of the Deposit Agreement and applicable law, and (b) the terms of such rules and regulations are readily available to Holders upon request, (v) the Uncertificated ADS(s) shall not be entitled to any benefits under the Deposit Agreement or be valid or enforceable for any purpose against the Depositary or the Company unless such Uncertificated ADS(s) is/are registered on the books of the Depositary maintained for such purpose, (vi) the Depositary may, in connection with any deposit of Shares resulting in the issuance of Uncertificated ADSs and with any transfer, pledge, release and cancellation of Uncertificated ADSs, require the prior receipt of such documentation as the Depositary may deem reasonably appropriate, and (vii) upon termination of the Deposit Agreement, the Depositary shall not require Holders of Uncertificated ADSs to affirmatively instruct the Depositary before remitting proceeds from the sale of the Deposited Property represented by such Holders’ Uncertificated ADSs under the terms of Section 6.2 hereof.  When issuing ADSs under the terms of the Deposit Agreement, including, without limitation, issuances pursuant to Sections 2.5, 4.2, 4.3, 4.4, 4.5 and4.11 hereof, the Depositary may in its discretion determine to issue Uncertificated ADSs rather than Certificated ADSs, unless otherwise specifically instructed by the applicable Holder to issue Certificated ADSs.  All provisions and conditions of the Deposit Agreement shall apply to Uncertificated ADSs to the same extent as to Certificated ADSs, except as contemplated by this Section 2.13.  The Depositary is authorized and directed to take any and all actions and establish any and all procedures deemed reasonably necessary to give effect to the terms of this Section 2.13.  Any references in the Deposit Agreement or any ADR(s) to the terms “American Depositary Share(s)” or “ADS(s)” shall, unless the context otherwise requires, include Certificated ADS(s) and Uncertificated ADS(s).  Except as set forth in this Section 2.13 and except as required by applicable law, the Uncertificated ADSs shall be treated as ADSs issued and outstanding under the terms of the Deposit Agreement.  In the event that, in determining the rights and obligations of parties hereto with respect to any Uncertificated ADSs, any conflict arises between (a) the terms of the Deposit Agreement (other than this Section 2.13) and (b) the terms of this Section 2.13, the terms and conditions set forth in this Section 2.13 shall be controlling and shall govern the rights and obligations of the parties to the Deposit Agreement pertaining to the Uncertificated ADSs.

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Section 2.14        Restricted ADSs.  The Depositary shall, at the request and expense of the Company, establish procedures enabling the deposit hereunder of Shares that are Restricted Securities in order to enable the holder of such Shares to hold its ownership interests in such Restricted Securities in the form of ADSs issued under the terms hereof (such Shares, “Restricted Shares”).  Upon receipt of a written request from the Company to accept Restricted Shares for deposit hereunder, the Depositary agrees to establish procedures permitting the deposit of such Restricted Shares and the issuance of ADSs representing the right to receive, subject to the terms of the Deposit Agreement and the applicable ADR (if issued as a Certificated ADS), such deposited Restricted Shares (such ADSs, the “Restricted ADSs,” and the ADRs evidencing such Restricted ADSs, the “Restricted ADRs”).  Notwithstanding anything contained in this Section 2.14, the Depositary and the Company may, to the extent not prohibited by law, agree to issue the Restricted ADSs in uncertificated form (“Uncertificated Restricted ADSs”) upon such terms and conditions as the Company and the Depositary may deem necessary and appropriate.  The Company shall assist the Depositary in the establishment of such procedures and agrees that it shall take all steps necessary and satisfactory to the Depositary to ensure that the establishment of such procedures does not violate the provisions of the Securities Act or any other applicable laws.  The depositors of such Restricted Shares and the Holders of the Restricted ADSs may be required prior to the deposit of such Restricted Shares, the transfer of the Restricted ADRs and the Restricted ADSs evidenced thereby, or the withdrawal of the Restricted Shares represented by Restricted ADSs to provide such written certifications or agreements as the Depositary or the Company may require.  The Company shall provide to the Depositary in writing the legend(s) to be affixed to the Restricted ADRs (if the Restricted ADSs are to be issued as Certificated ADSs), or to be included in the statements issued from time to time to Holders of Uncertificated ADSs (if issued as Uncertificated Restricted ADSs), which legends shall (i) be in a form reasonably satisfactory to the Depositary and (ii) contain the specific circumstances under which the Restricted ADSs, and, if applicable, the Restricted ADRs evidencing the Restricted ADSs, may be transferred or the Restricted Shares withdrawn.  The Restricted ADSs issued upon the deposit of Restricted Shares shall be separately identified on the books of the Depositary and the Restricted Shares so deposited shall, to the extent required by law, be held separate and distinct from the other Deposited Securities held hereunder.  The Restricted ADSs shall not be eligible for inclusion in any book-entry settlement system, including, without limitation, DTC, and shall not in any way be fungible with the ADSs issued under the terms hereof that are not Restricted ADSs.  The Restricted ADSs, and, if applicable, the Restricted ADRs evidencing the Restricted ADSs, shall be transferable only by the Holder thereof upon delivery to the Depositary of (i) all documentation otherwise contemplated by the Deposit Agreement and (ii) an opinion of counsel reasonably satisfactory to the Depositary setting forth, inter alia, the conditions upon which the Restricted ADSs presented, and, if applicable, the Restricted ADRs evidencing the Restricted ADSs, are transferable by the Holder thereof under applicable securities laws and the transfer restrictions contained in the legend applicable to the Restricted ADSs presented for transfer.  Except as set forth in this Section 2.14 and except as required by applicable law, the Restricted ADSs and the Restricted ADRs evidencing Restricted ADSs shall be treated as ADSs and ADRs issued and outstanding under the terms of the Deposit Agreement.  In the event that, in determining the rights and obligations of parties hereto with respect to any Restricted ADSs, any conflict arises between (a) the terms of the Deposit Agreement (other than this Section 2.14) and (b) the terms of (i) this Section 2.14 or (ii) the applicable Restricted ADR, the terms and conditions set forth in this Section 2.14 and of the Restricted ADR shall be controlling and shall govern the rights and obligations of the parties to the Deposit Agreement pertaining to the deposited Restricted Shares, the Restricted ADSs and Restricted ADRs.

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If the Restricted ADRs, the Restricted ADSs and the Restricted Shares cease to be Restricted Securities, the Depositary, upon receipt of (x) an opinion of counsel reasonably satisfactory to the Depositary setting forth, inter alia, that the Restricted ADRs, the Restricted ADSs and the Restricted Shares are not as of such time Restricted Securities, and (y) instructions from the Company to remove the restrictions applicable to the Restricted ADRs, the Restricted ADSs and the Restricted Shares, shall (i) eliminate the distinctions and separations that may have been established between the applicable Restricted Shares held on deposit under this Section 2.14 and the other Shares held on deposit under the terms of the Deposit Agreement that are not Restricted Shares, (ii) treat the newly unrestricted ADRs and ADSs on the same terms as, and fully fungible with, the other ADRs and ADSs issued and outstanding under the terms of the Deposit Agreement that are not Restricted ADRs or Restricted ADSs, and (iii) take all actions necessary to remove any distinctions, limitations and restrictions previously existing under this Section 2.14 between the applicable Restricted ADRs and Restricted ADSs, respectively, on the one hand, and the other ADRs and ADSs that are not Restricted ADRs or Restricted ADSs, respectively, on the other hand, including, without limitation, by making the newly-unrestricted ADSs eligible for inclusion in the applicable book-entry settlement systems.

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ARTICLE III

CERTAIN OBLIGATIONS OF HOLDERS AND BENEFICIAL OWNERS OF ADSs

Section 3.1          Proofs, Certificates and Other Information.  Any person presenting Shares for deposit, any Holder and any Beneficial Owner may be required, and every Holder and Beneficial Owner agrees, from time to time to provide to the Company, the Depositary and the Custodian such proof of citizenship or residence, taxpayer status, payment of all applicable taxes or other governmental charges, exchange control approval, legal or beneficial ownership of ADSs and Deposited Property, compliance with applicable laws, the terms of the Deposit Agreement or the ADR(s) evidencing the ADSs and the provisions of, or governing, the Deposited Property, to execute such certifications and to make such representations and warranties, and to provide such other information and documentation (or, in the case of Shares in registered form presented for deposit, such information relating to the registration on the books of the Company or of the Share Registrar) as the Depositary or the Custodian may deem necessary or proper or as the Company may reasonably require by written request to the Depositary consistent with its obligations under the Deposit Agreement and the applicable ADR(s).  The Depositary and the Registrar, as applicable, and at the reasonable request of the Company, shall, to the extent practicable, may withhold the execution or delivery or registration of transfer of any ADR or ADS or the distribution or sale of any dividend or distribution of rights or of the proceeds thereof or, to the extent not limited by the terms of Section 7.8, the delivery of any Deposited Property until such proof or other information is filed or such certifications are executed, or such representations and warranties are made, or such other documentation or information is provided, in each case to the Depositary’s, the Registrar’s and the Company’s satisfaction.  The Depositary shall provide the Company, in a timely manner, with copies or originals if necessary and appropriate of (i) any such proofs of citizenship or residence, taxpayer status, or exchange control approval or copies of written representations and warranties which it receives from Holders and Beneficial Owners, and (ii) any other information or documents which the Company may reasonably request and which the Depositary shall request and receive from any Holder or Beneficial Owner or any person presenting Shares for deposit or ADSs for cancellation, transfer or withdrawal.  Nothing herein shall obligate the Depositary to (i) obtain any information for the Company if not provided by the Holders or Beneficial Owners, or (ii) verify or vouch for the accuracy of the information so provided by the Holders or Beneficial Owners.

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Section 3.2          Liability for Taxes and Other Charges.  Any tax or other governmental charge payable by the Custodian or by the Depositary with respect to any Deposited Property, ADSs or ADRs shall be payable by the Holders and Beneficial Owners to the Depositary.  The Company, the Custodian and/or the Depositary may withhold or deduct from any distributions made in respect of Deposited Property held on behalf of such Holder and/or Beneficial Owner, and may sell for the account of a Holder and/or Beneficial Owner any or all of such Deposited Property and apply such distributions and sale proceeds in payment of, any taxes (including applicable interest and penalties) or charges that are or may be payable by Holders or Beneficial Owners in respect of the ADSs, Deposited Property and ADRs, the Holder and the Beneficial Owner remaining liable for any deficiency.  The Custodian may refuse the deposit of Shares and the Depositary may refuse to issue ADSs, to deliver ADRs, register the transfer of ADSs, register the split‑up or combination of ADRs and (subject to Section 7.8) the withdrawal of Deposited Property until payment in full of such tax, charge, penalty or interest is received.  Every Holder and Beneficial Owner agrees to indemnify the Depositary, the Company, the Custodian, and any of their respective agents, officers, employees and Affiliates for, and to hold each of them harmless from, any claims (including, without limitation, by any governmental authority or other person or entity) with respect to taxes (including applicable interest and penalties thereon) arising from any tax benefit obtained for such Holder and/or Beneficial Owner.  Notwithstanding anything to the contrary contained in the Deposit Agreement or any ADR, the obligations of Holders and Beneficial Owners under this Section 3.2 shall survive any transfer of ADSs, any cancellation of ADSs and withdrawal of Deposited Securities, and the termination of the Deposit Agreement.

Section 3.3          Representations and Warranties on Deposit of Shares.  Each person depositing Shares under the Deposit Agreement shall be deemed thereby to represent and warrant that (i) such Shares and the certificates therefor are duly authorized, validly allotted and issued, fully paid, not subject to any call for the payment of further capital and legally obtained by such person, (ii) all preemptive (and similar) rights, if any, with respect to such Shares have been validly waived, disapplied or exercised, (iii) the person making such deposit is duly authorized so to do, (iv) the Shares presented for deposit are free and clear of any lien, encumbrance, security interest, charge, mortgage or adverse claim, (v) the Shares presented for deposit are not, and the ADSs issuable upon such deposit will not be, Restricted Securities (except as contemplated in Section 2.14), (vi) the Shares presented for deposit have not been stripped of any rights or entitlements, and (vii) the deposit of the Shares does not violate any applicable provisions of English law.  Such representations and warranties shall survive the deposit and withdrawal of Shares, the issuance and cancellation of ADSs in respect thereof and the transfer of such ADSs.  If any such representations or warranties are false in any way, the Company and the Depositary shall be authorized, at the cost and expense of the person depositing Shares, to take any and all actions necessary to correct the consequences thereof.

Section 3.4          Compliance with Information Requests.  Notwithstanding any other provision of the Deposit Agreement or any ADR(s), each Holder and Beneficial Owner agrees to comply with requests from the Company pursuant to applicable law, the rules and requirements of any stock exchange on which the Shares or ADSs are, or will be, registered, traded or listed or the Articles of Association, which are made to provide information, inter alia, as to the capacity in which such Holder or Beneficial Owner owns ADSs (and Shares as the case may be) and regarding the identity of any other person(s) interested in such ADSs and the nature of such interest and various other matters, whether or not they are Holders and/or Beneficial Owners at the time of such request.  The Depositary agrees to use its reasonable efforts to assist the Company in obtaining such information, including agreeing to forward, upon the request of the Company and at the Company’s expense, any such request from the Company to the Holders and to forward to the Company any such responses to such requests received by the Depositary.

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Section 3.5          Ownership Restrictions.  Notwithstanding any other provision contained in the Deposit Agreement or any ADR(s) to the contrary, the Company may restrict transfers of the Shares where such transfer might result in ownership of Shares exceeding limits imposed by applicable law or the Articles of Association.  The Company may also restrict, in such manner as it deems appropriate, transfers of the ADSs where such transfer may result in the total number of Shares represented by the ADSs owned by a single Holder or Beneficial Owner to exceed any such limits.  The Company may, in its sole discretion but subject to applicable law, instruct the Depositary to take action with respect to the ownership interest of any Holder or Beneficial Owner in excess of the limits set forth in the preceding sentence, including, but not limited to, the imposition of restrictions on the transfer of ADSs, the removal or limitation of voting rights or mandatory sale or disposition on behalf of a Holder or Beneficial Owner of the Shares represented by the ADSs held by such Holder or Beneficial Owner in excess of such limitations, if and to the extent such disposition is permitted by applicable law and the Articles of Association.  Nothing herein shall be interpreted as obligating the Depositary or the Company to ensure compliance with the ownership restrictions described in this Section 3.5.

Section 3.6          Reporting Obligations and Regulatory Approvals.  Applicable laws and regulations may require holders and beneficial owners of Shares, including the Holders and Beneficial Owners of ADSs, to satisfy reporting requirements and obtain regulatory approvals in certain circumstances.  Holders and Beneficial Owners of ADSs are solely responsible for determining and complying with such reporting requirements and obtaining such approvals.  Each Holder and each Beneficial Owner hereby agrees to make such determination, file such reports, and obtain such approvals to the extent and in the form required by applicable laws and regulations as in effect from time to time.  Neither the Depositary, the Custodian, the Company or any of their respective agents or affiliates shall be required to take any actions whatsoever on behalf of Holders or Beneficial Owners to determine or satisfy such reporting requirements or obtain such regulatory approvals under applicable laws and regulations.

ARTICLE IV

THE DEPOSITED SECURITIES

Section 4.1          Cash Distributions.  Whenever the Company intends to make a distribution of a cash dividend or other cash distribution in respect of any Deposited Securities, the Company shall give timely notice thereof to the Depositary at least twenty (20) days prior to the proposed distribution specifying, inter alia, the record date applicable for determining the holders of Deposited Securities entitled to receive such distribution.  Upon the timely receipt of such notice, the Depositary shall establish an ADS Record Date upon the terms described in Section 4.9 hereof.  Upon receipt of confirmation from the Custodian of the receipt of (x) any cash dividend or other cash distribution on any Deposited Securities, or (y) proceeds from the sale of any Deposited Property held in respect of the ADSs under the terms hereof, the Depositary will (i) if any amounts are received in a Foreign Currency, promptly convert or cause to be converted such cash dividend, distribution or proceeds into Dollars (subject to the terms and on the conditions of Section 4.8), (ii) if applicable and unless previously established, establish the ADS Record Date upon the terms described in Section 4.9, and (iii) distribute promptly the amount thus received from such conversion (net of (a) the applicable fees and charges set forth in the Fee Schedule attached hereto as Exhibit B, and (b) applicable taxes withheld) to the Holders entitled thereto as of the ADS Record Date in proportion to the number of ADSs held as of the ADS Record Date.  The Depositary shall distribute only such amount, however, as can be distributed without attributing to any Holder a fraction of one cent, and any balance not so distributed shall be held by the Depositary (without liability for interest thereon) and shall be added to and become part of the next sum received by the Depositary for distribution to Holders of ADSs outstanding at the time of the next distribution.  If the Company, the Custodian or the Depositary is required to withhold and does withhold from any cash dividend or other cash distribution in respect of any Deposited Securities, or from any cash proceeds from the sales of Deposited Property, an amount on account of taxes, duties or other governmental charges, the amount distributed to Holders on the ADSs shall be reduced accordingly.  Such withheld amounts shall be forwarded by the Company, the Custodian or the Depositary to the relevant governmental authority.  Evidence of payment thereof by the Company shall be forwarded by the Company to the Depositary upon request.  The Depositary will hold any cash amounts it is unable to distribute in a non-interest bearing account for the benefit of the applicable Holders and Beneficial Owners of ADSs until the distribution can be effected or the funds that the Depositary holds must be escheated as unclaimed property in accordance with the laws of the relevant states of the United States.  Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for in this Section 4.1, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in this Section 4.1, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.1 where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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Section 4.2          Distribution in Shares.  Whenever the Company intends to make a distribution that consists of a dividend in, or free distribution of, Shares, the Company shall give timely notice thereof to the Depositary at least twenty (20) days prior to the proposed distribution, specifying, inter alia, the record date applicable to holders of Deposited Securities entitled to receive such distribution.  Upon the timely receipt of such notice from the Company, the Depositary shall establish the ADS Record Date upon the terms described in Section 4.9.  Upon receipt of confirmation from the Custodian of the receipt of the Shares so distributed by the Company, the Depositary shall either (i) subject to Section 5.9, distribute to the Holders as of the ADS Record Date in proportion to the number of ADSs held as of the ADS Record Date, additional ADSs, which represent in the aggregate the number of Shares received as such dividend, or free distribution, subject to the other terms of the Deposit Agreement (including, without limitation, (a) the applicable fees and charges of, and expenses incurred by, the Depositary and (b) taxes), or (ii) if additional ADSs are not so distributed, take all actions necessary so that each ADS issued and outstanding after the ADS Record Date shall, to the extent permissible by law, thenceforth also represent rights and interests in the additional integral number of Shares distributed upon the Deposited Securities represented thereby (net of (a) the applicable fees and charges of, and expenses incurred by, the Depositary and (b) taxes).  In lieu of delivering fractional ADSs, the Depositary shall sell the number of Shares or ADSs, as the case may be, represented by the aggregate of such fractions and distribute the net proceeds upon the terms described in Section 4.1.  In the event that the Depositary determines that any distribution in property (including Shares) is subject to any tax or other governmental charges which the Depositary is obligated to withhold, or, if the Company in the fulfillment of its obligation under Section 5.7, has furnished an opinion of U.S. counsel determining that Shares must be registered under the Securities Act or other laws in order to be distributed to Holders (and no such registration statement has been declared effective), the Depositary may dispose of all or a portion of such property (including Shares and rights to subscribe therefor) in such amounts and in such manner, including by public or private sale, as the Depositary deems necessary and practicable, and the Depositary shall distribute the net proceeds of any such sale (after deduction of (a) taxes and (b) fees and charges of, and expenses incurred by, the Depositary) to Holders entitled thereto upon the terms described in Section 4.1.  The Depositary shall hold and/or distribute any unsold balance of such property in accordance with the provisions of the Deposit Agreement.  Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for in this Section 4.2, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in this Section 4.2, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.2 where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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Section 4.3          Elective Distributions in Cash or Shares.  Whenever the Company intends to make a distribution payable at the election of the holders of Deposited Securities in cash or in additional Shares, the Company shall give timely notice thereof to the Depositary at least sixty (60) days prior to the proposed distributionspecifying, inter alia, the record date applicable to holders of Deposited Securities entitled to receive such elective distribution and whether or not it wishes such elective distribution to be made available to Holders of ADSs.  Upon the timely receipt of a notice indicating that the Company wishes such elective distribution to be made available to Holders of ADSs, the Depositary shall consult with the Company to determine, and the Company shall assist the Depositary in its determination, whether it is lawful and reasonably practicable to make such elective distribution available to the Holders of ADSs.  The Depositary shall make such elective distribution available to Holders only if (i) the Company shall have timely requested that the elective distribution be made available to Holders, (ii) the Depositary shall have determined, upon consultation with the Company, that such distribution is reasonably practicable and (iii) the Depositary shall have received satisfactory documentation within the terms of Section 5.7.  If the above conditions are not satisfied or if the Company requests such elective distribution not to be made available to Holders of ADSs, the Depositary shall establish an ADS Record Date on the terms described in Section 4.9 and, to the extent permitted by law, distribute to the Holders, on the basis of the same determination as is made in England and Wales in respect of the Shares for which no election is made, either (X) cash upon the terms described in Section 4.1 or (Y) additional ADSs representing such additional Shares upon the terms described in Section 4.2.  If the above conditions are satisfied, the Depositary shall establish an ADS Record Date on the terms described in Section 4.9 and establish procedures to enable Holders to elect the receipt of the proposed distribution in cash or in additional ADSs.  The Company shall assist the Depositary in establishing such procedures to the extent necessary.  If a Holder elects to receive the proposed distribution (X) in cash, the distribution shall be made upon the terms described in Section 4.1, or (Y) in ADSs, the distribution shall be made upon the terms described in Section 4.2.  Nothing herein shall obligate the Depositary to make available to Holders a method to receive the elective distribution in Shares (rather than ADSs).  There can be no assurance that Holders generally, or any Holder in particular, will be given the opportunity to receive elective distributions on the same terms and conditions as the holders of Shares.  Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for in this Section 4.3, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in this Section 4.3, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.3 where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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Section 4.4          Distribution of Rights to Purchase Additional ADSs.

(a)          Distribution to ADS Holders.  Whenever the Company intends to distribute to the holders of the Deposited Securities rights to subscribe for additional Shares, the Company shall give timely notice thereof to the Depositary at least sixty (60) days prior to the proposed distributionspecifying, inter alia, the record date applicable to holders of Deposited Securities entitled to receive such distribution and whether or not it wishes such rights to be made available to Holders of ADSs.  Upon the timely receipt of a notice indicating that the Company wishes such rights to be made available to Holders of ADSs, the Depositary shall consult with the Company to determine, and the Company shall assist the Depositary in its determination, whether it is lawful and reasonably practicable to make such rights available to the Holders.  The Depositary shall make such rights available to Holders only if (i) the Company shall have timely requested that such rights be made available to Holders, (ii) the Depositary shall have received satisfactory documentation within the terms of Section 5.7, and (iii) the Depositary shall have determined that such distribution of rights is reasonably practicable.  In the event any of the conditions set forth above are not satisfied or if the Company requests that the rights not be made available to Holders of ADSs, the Depositary shall proceed with the sale of the rights as contemplated in Section 4.4(b) below.  In the event all conditions set forth above are satisfied, the Depositary shall establish an ADS Record Date (upon the terms described in Section 4.9) and establish procedures to (x) distribute rights to purchase additional ADSs (by means of warrants or otherwise), (y) enable the Holders to exercise such rights (upon payment of the subscription price and of the applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes), and (z) deliver ADSs upon the valid exercise of such rights.  The Company shall assist the Depositary to the extent necessary in establishing such procedures.  Nothing herein shall obligate the Depositary to make available to the Holders a method to exercise rights to subscribe for Shares (rather than ADSs).

(b)          Sale of Rights.  If (i) the Company does not timely request the Depositary to make the rights available to Holders or requests that the rights not be made available to Holders, (ii) the Depositary fails to receive satisfactory documentation within the terms of Section 5.7, or determines, upon consultation with the Company, it is not reasonably practicable to make the rights available to Holders, or (iii) any rights made available are not exercised and appear to be about to lapse, the Depositary shall, upon consultation with the Company, determine whether it is lawful and reasonably practicable to sell such rights, in a riskless principal capacity, at such place and upon such terms (including public or private sale) as it may deem practicable.  The Company shall assist the Depositary to the extent necessary to determine such legality and practicability.  The Depositary shall, upon such sale, convert and distribute proceeds of such sale (net of applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) upon the terms set forth in Section 4.1.

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(c)          Lapse of Rights.  If the Depositary is unable to make any rights available to Holders upon the terms described in Section 4.4(a) or to arrange for the sale of the rights upon the terms described in Section 4.4(b), the Depositary shall allow such rights to lapse.

The Depositary shall not be liable for (i) any failure to accurately determine whether it may be lawful or practicable to make such rights available to Holders in general or any Holders in particular, (ii) any foreign exchange exposure or loss incurred in connection with such sale, or exercise, or (iii) the content of any materials forwarded to the Holders on behalf of the Company in connection with the rights distribution.

Notwithstanding anything to the contrary in this Section 4.4, if registration (under the Securities Act or any other applicable law) of the rights or the securities to which any rights relate may be required in order for the Company to offer such rights or such securities to Holders and to sell the securities represented by such rights, the Depositary will not distribute such rights to the Holders (i) unless and until a registration statement under the Securities Act (or other applicable law) covering such offering is in effect or (ii) unless the Company furnishes the Depositary opinion(s) of counsel for the Company in the United States and counsel to the Company in any other applicable country in which rights would be distributed, in each case reasonably satisfactory to the Depositary, to the effect that the offering and sale of such securities to Holders and Beneficial Owners are exempt from, or do not require registration under, the provisions of the Securities Act or any other applicable laws.

In the event that the Company, the Depositary or the Custodian shall be required to withhold and does withhold from any distribution of Deposited Property (including rights) an amount on account of taxes or other governmental charges, the amount distributed to the Holders of ADSs representing such Deposited Property shall be reduced accordingly.  In the event that the Depositary determines that any distribution of Deposited Property (including Shares and rights to subscribe therefor) is subject to any tax or other governmental charges which the Depositary is obligated to withhold, the Depositary may dispose of all or a portion of such Deposited Property (including Shares and rights to subscribe therefor) in such amounts and in such manner, including by public or private sale, as the Depositary deems necessary and practicable to pay any such taxes or charges.

There can be no assurance that Holders generally, or any Holder in particular, will be given the opportunity to receive or exercise rights on the same terms and conditions as the holders of Shares or be able to exercise such rights.  Nothing herein shall obligate the Company to file any registration statement in respect of any rights or Shares or other securities to be acquired upon the exercise of such rights.

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Section 4.5          Distributions Other Than Cash, Shares or Rights to Purchase Shares.

(a)          Whenever the Company intends to distribute to the holders of Deposited Securities property other than cash, Shares or rights to purchase additional Shares, the Company shall give timely notice thereof to the Depositary and shall indicate whether or not it wishes such distribution to be made to Holders of ADSs.  Upon receipt of a notice indicating that the Company wishes such distribution to be made to Holders of ADSs, the Depositary shall consult with the Company, and the Company shall assist the Depositary, to determine whether such distribution to Holders is lawful and reasonably practicable.  The Depositary shall not make such distribution unless (i) the Company shall have requested the Depositary to make such distribution to Holders, (ii) the Depositary shall have received satisfactory documentation within the terms of Section 5.7, and (iii) the Depositary shall have determined, after consultation with the Company, that such distribution is reasonably practicable.

(b)          Upon receipt of reasonably satisfactory documentation and the request of the Company to distribute property to Holders of ADSs and after making the requisite determinations set forth in (a) above, the Depositary shall distribute the property so received to the Holders of record, as of the ADS Record Date, in proportion to the number of ADSs held by them respectively and in such manner as the Depositary may deem practicable for accomplishing such distribution (i) upon receipt of payment or net of the applicable fees and charges of, and expenses incurred by, the Depositary, and (ii) net of any taxes withheld.  The Depositary may dispose of all or a portion of the property so distributed and deposited, in such amounts and in such manner (including public or private sale) as the Depositary may deem practicable or necessary to satisfy any taxes (including applicable interest and penalties) or other governmental charges applicable to the distribution.

(c)          If (i) the Company does not request the Depositary to make such distribution to Holders or requests the Depositary not to make such distribution to Holders, (ii) the Depositary does not receive reasonably satisfactory documentation within the terms of Section 5.7, or (iii) the Depositary determines that all or a portion of such distribution is not reasonably practicable, the Depositary shall sell or cause such property to be sold in a public or private sale, at such place or places and upon such terms as it may deem practicable and shall (i) cause the proceeds of such sale, if any, to be converted into Dollars and (ii) distribute the proceeds of such conversion received by the Depositary (net of applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) to the Holders as of the ADS Record Date upon the terms of Section 4.1.  If the Depositary is unable to sell such property, the Depositary may dispose of such property for the account of the Holders in any way it deems reasonably practicable under the circumstances.

(d)          Neither the Depositary nor the Company shall be liable for (i) any failure to accurately determine whether it is lawful or practicable to make the property described in this Section 4.5 available to Holders in general or any Holders in particular, nor (ii) any loss incurred in connection with the sale or disposal of such property.

Section 4.6          Distributions with Respect to Deposited Securities in Bearer Form.Subject to the terms of this Article IV, distributions in respect of Deposited Securities that are held by the Depositary or the Custodian in bearer form shall be made to the Depositary for the account of the respective Holders of ADS(s) with respect to which any such distribution is made upon due presentation by the Depositary or the Custodian to the Company of any relevant coupons, talons, or certificates.  The Company shall promptly notify the Depositary of such distributions.  The Depositary or the Custodian shall promptly present such coupons, talons or certificates, as the case may be, in connection with any such distribution.

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Section 4.7          Redemption.  If the Company intends to exercise any right of redemption in respect of any of the Deposited Securities, the Company shall give timely notice thereof to the Depositary at least sixty (60) days prior to the intended date of redemptionwhich notice shall set forth the particulars of the proposed redemption.  Upon timely receipt of (i) such notice and (ii) satisfactory documentation given by the Company to the Depositary within the terms of Section 5.7, and only if, after consultation with the Company, the Depositary shall have determined that such proposed redemption is practicable, the Depositary shall provide to each Holder a notice setting forth the intended exercise by the Company of the redemption rights and any other particulars set forth in the Company’s notice to the Depositary.  The Depositary shall instruct the Custodian to present to the Company the Deposited Securities in respect of which redemption rights are being exercised against payment of the applicable redemption price.  Upon receipt of confirmation from the Custodian that the redemption has taken place and that funds representing the redemption price have been received, the Depositary shall convert, transfer, and distribute the proceeds (net of applicable (a) fees and charges of, and the expenses incurred by, the Depositary, and (b) taxes), retire ADSs and cancel ADRs, if applicable, upon delivery of such ADSs by Holders thereof and the terms set forth in Sections 4.1 and 6.2.  If less than all outstanding Deposited Securities are redeemed, the ADSs to be retired will be selected by lot or on a pro rata basis, as may be determined by the Depositary, after consultation with the Company.  The redemption price per ADS shall be the dollar equivalent of the per share amount received by the Depositary (adjusted to reflect the ratio of ADS(s) to Share(s)) upon the redemption of the Deposited Securities represented by ADSs (subject to the terms of Section 4.8 and the applicable fees and charges of, and expenses incurred by, the Depositary, and taxes) multiplied by the number of Deposited Securities represented by each ADS redeemed.

Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed redemption provided for in this Section 4.7, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in this Section 4.7, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.7 where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

Section 4.8          Conversion of Foreign Currency.  Whenever the Depositary or the Custodian shall receive Foreign Currency, by way of dividends or other distributions or the net proceeds from the sale of Deposited Property, which in the judgment of the Depositary can at such time be converted on a practicable basis, by sale or in any other manner that it may determine in accordance with applicable law, into Dollars transferable to the United States and distributable to the Holders entitled thereto, the Depositary shall convert or cause to be converted, by sale or in any other manner that it may reasonably determine, such Foreign Currency into Dollars, and shall distribute such Dollars (net of the fees and charges set forth in the Fee Schedule attached hereto as Exhibit B, and applicable taxes withheld) in accordance with the terms of the applicable sections of the Deposit Agreement.  The Depositary and/or its agent (which may be a division, branch or Affiliate of the Depositary) may act as principal for any conversion of Foreign Currency.  If the Depositary shall have distributed warrants or other instruments that entitle the holders thereof to such Dollars, the Depositary shall distribute such Dollars to the holders of such warrants and/or instruments upon surrender thereof for cancellation, in either case without liability for interest thereon.  Such distribution may be made upon an averaged or other practicable basis without regard to any distinctions among Holders on account of any application of exchange restrictions or otherwise.

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If such conversion or distribution generally or with regard to a particular Holder can be effected only with the approval or license of any government or agency thereof, the Depositary shall have authority to file such application for approval or license, if any, as it may deem desirable.  In no event, however, shall the Depositary be obligated to make such a filing.

If at any time the Depositary shall determine that in its judgment the conversion of any Foreign Currency and the transfer and distribution of proceeds of such conversion received by the Depositary is not practicable or lawful, or if any approval or license of any governmental authority or agency thereof that is required for such conversion, transfer and distribution is denied or, in the opinion of the Depositary, not obtainable at a reasonable cost or within a reasonable period, the Depositary may, in its discretion, (i) make such conversion and distribution in Dollars to the Holders for whom such conversion, transfer and distribution is lawful and practicable, (ii) distribute the Foreign Currency (or an appropriate document evidencing the right to receive such Foreign Currency) to Holders for whom this is lawful and practicable, or (iii) hold (or cause the Custodian to hold) such Foreign Currency (without liability for interest thereon) for the respective accounts of the Holders entitled to receive the same.

Section 4.9          Fixing of ADS Record Date.  Whenever (a) the Depositary shall receive notice of the fixing of a record date by the Company for the determination of holders of Deposited Securities entitled to receive any distribution (whether in cash, Shares, rights, or other distribution), (b) for any reason the Depositary causes a change in the number of Shares that are represented by each ADS, (c) the Depositary shall receive notice of any meeting of, or solicitation of consents or proxies of, holders of Shares or other Deposited Securities, or (d) the Depositary shall find it necessary or convenient in connection with the giving of any notice, solicitation of any consent or any other matter, the Depositary shall fix the record date (the “ADS Record Date”) for the determination of the Holders of ADS(s) who shall be entitled to receive such distribution, to give instructions for the exercise of voting rights at any such meeting, to give or withhold such consent, to receive such notice or solicitation or to otherwise take action, or to exercise the rights of Holders with respect to such changed number of Shares represented by each ADS.  The Depositary shall make reasonable efforts to establish the ADS Record Date as closely as possible to the applicable record date for the Deposited Securities (if any) set by the Company in England and Walesand shall not announce the establishment of any ADS Record Date prior to the relevant corporate action having been made public by the Company (if such corporate action affects the Deposited Securities).  Subject to applicable law and the provisions of Section 4.1 through 4.8 and to the other terms and conditions of the Deposit Agreement, only the Holders of ADSs at the close of business in New York on such ADS Record Date shall be entitled to receive such distribution, to give such voting instructions, to receive such notice or solicitation, or otherwise take action.

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Section 4.10        Voting of Deposited Securities.  As soon as practicable after receipt of notice of any meeting at which the holders of Deposited Securities are entitled to vote, or of solicitation of consents or proxies from holders of Deposited Securities, the Depositary shall fix the ADS Record Date in respect of such meeting or solicitation of consent or proxy in accordance with Section 4.9.  The Depositary shall, if requested by the Company in writing in a timely manner (except as otherwise contemplated in this Section 4.10, the Depositary having no obligation to take any further action if the request shall not have been received by the Depositary at least thirty (30) days prior to the date of such vote or meeting), at the Company’s expense and provided no U.S. legal prohibitions exist, distribute as soon as practicable after receipt thereof to Holders as of the ADS Record Date: (a) such notice of meeting or solicitation of consent or proxy, (b) a statement that the Holders at the close of business on the ADS Record Date will be entitled, subject to any applicable law, the provisions of the Deposit Agreement, the Articles of Association and the provisions of or governing the Deposited Securities (which provisions, if any, shall be summarized in pertinent part by the Company), to instruct the Depositary as to the exercise of the voting rights, if any, pertaining to the Deposited Securities represented by such Holder’s ADSs, and (c) a brief statement as to the manner in which such voting instructions may be given or deemed to have been given in accordance with this Section 4.10, it being acknowledged that if no instructions are received prior to the deadline set for such purposes to the Depositary to give a discretionary proxy to a person designated by the Company.

The Company shall use its reasonable endeavors to notify the Depositary in writing of its intention to convene a meeting where the holders of Deposited Securities are entitled to vote, at least thirty (30) days prior to the date of the proposed meeting.

Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to timely request that the Depositary distribute the information as provided for in this Section 4.10, if the Depositary so agrees after consultation with the Company, the Depositary will use commercially reasonable efforts to perform the actions contemplated in this Section 4.10, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.10, where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

Notwithstanding anything contained in the Deposit Agreement or any ADR, the Depositary may, to the extent not prohibited by law or regulations, or by the requirements of the stock exchange on which the ADSs are listed, in lieu of distribution of the materials provided to the Depositary in connection with any meeting of, or solicitation of consents or proxies from, holders of Deposited Securities, distribute to the Holders a notice that provides Holders with, or otherwise publicizes to Holders, instructions on how to retrieve such materials or receive such materials upon request (e.g., by reference to a website containing the materials for retrieval or a contact for requesting copies of the materials).

The Depositary has been advised by the Company that under the Articles of Association as in effect on the date of the Deposit Agreement, voting at any meeting of shareholders of the Company is by show of hands unless (before or on the declaration of the result of the show of hands) a poll is demanded.  The Depositary will not join in demanding a poll, whether or not requested to do so by Holders of ADSs.  Under the Articles of Association as in effect on the date of the Deposit Agreement, at any meeting a resolution put to the vote of the meeting shall be decided on a show of hands, unless before or upon the declaration of the result of the show of hands a poll is demanded by: (a) the chairman of the meeting; (b) not less than five (5) members of the Company having the right to vote at the meeting; (c) a member or members of the Company representing not less than one tenth of the total voting rights of all the members of the Company having the right to vote at the meeting; or (d) a member or members of the Company holding shares in the Company conferring a right to vote at the meeting, being shares on which an aggregate sum has been paid up equal to not less than one tenth of the total sum paid up on all the shares conferring that right..

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Voting instructions may be given only in respect of a number of ADSs representing an integral number of Deposited Securities.  Upon the timely receipt from a Holder of ADSs as of the ADS Record Date of voting instructions in the manner specified by the Depositary, the Depositary shall endeavor, insofar as practicable and permitted under applicable law, the provisions of the Deposit Agreement, Articles of Association and the provisions of the Deposited Securities, to vote, or cause the Custodian to vote, the Deposited Securities (in person or by proxy) represented by such Holder’s ADSs as follows: (a) in the event voting takes place at a shareholders’ meeting by a show of hands, the Depositary will instruct the Custodian to vote all Deposited Securities in accordance with the voting instructions received timely from a majority of Holders of ADSs who provided voting instructions, and (b) in the event voting takes place at a shareholders’ meeting by poll, the Depositary will instruct the Custodian to vote the Deposited Securities in accordance with the voting instructions timely received from the Holders of ADSs.  If voting is by poll and the Depositary does not receive voting instructions from a Holder as of the ADS Record Date on or before the date established by the Depositary for such purpose, such Holder shall be deemed, and the Depositary shall (unless otherwise specified in the notice distributed to Holders) deem such Holder, to have instructed the Depositary to give a discretionary proxy to a person designated by the Company to vote the Deposited Securities; provided, however, that no such discretionary proxy shall be given by the Depositary with respect to any matter to be voted upon as to which the Company informs the Depositary that (a) the Company does not wish such proxy to be given, (b) substantial opposition exists, or (c) the rights of holders of Deposited Securities may be adversely affected.

Deposited Securities represented by ADSs for which no timely voting instructions are received by the Depositary from the Holder shall not be voted (except (a) in the case voting is by show of hands, in which case the Depositary will instruct the Custodian to vote all Deposited Securities in accordance with the voting instructions received from a majority of Holders of ADSs who provided timely voting instructions, and (b) as contemplated in this Section 4.10).  Neither the Depositary nor the Custodian shall under any circumstances exercise any discretion as to voting and neither the Depositary nor the Custodian shall vote, attempt to exercise the right to vote, or in any way make use of, for purposes of establishing a quorum or otherwise, the Deposited Securities represented by ADSs, except pursuant to and in accordance with the voting instructions timely received from Holders or as otherwise contemplated herein.  If the Depositary timely receives voting instructions from a Holder which fail to specify the manner in which the Depositary is to vote the Deposited Securities represented by such Holder’s ADSs, the Depositary will deem such Holder (unless otherwise specified in the notice distributed to Holders) to have instructed the Depositary to vote in favor of the items set forth in such voting instructions.

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Notwithstanding anything to the contrary contained herein, the Depositary shall, if so requested in writing by the Company, represent all Deposited Securities (whether or not voting instructions have been received in respect of such Deposited Securities from Holders as of the ADS Record Date) for the sole purpose of establishing quorum at a meeting of shareholders.

Notwithstanding anything to the contrary contained in the Deposit Agreement or any ADR, the Depositary shall not have any obligation to take any action with respect to any meeting, or solicitation of consents or proxies, of holders of Deposited Securities if the taking of such action would violate U.S. or English laws.  The Company agrees to take any and all actions reasonably necessary and as permitted by any applicable law to enable Holders and Beneficial Owners to exercise the voting rights accruing to the Deposited Securities and to deliver to the Depositary an opinion of U.S. counsel addressing any actions requested to be taken if so requested by the Depositary.

There can be no assurance that Holders generally or any Holder in particular will receive the notice described above with sufficient time to enable the Holder to return voting instructions to the Depositary in a timely manner.

Section 4.11        Changes Affecting Deposited Securities.  Upon any change in nominal or par value, split‑up, cancellation, consolidation or any other reclassification of Deposited Securities, or upon any recapitalization, reorganization, merger, consolidation or sale of assets affecting the Company or to which it is a party, any property which shall be received by the Depositary or the Custodian in exchange for, or in conversion of, or replacement of, or otherwise in respect of, such Deposited Securities shall, to the extent permitted by law, be treated as new Deposited Property under the Deposit Agreement, and the ADSs shall, subject to the provisions of the Deposit Agreement, any ADR(s) evidencing such ADSs and applicable law, represent the right to receive such additional or replacement Deposited Property.  In giving effect to such change, split-up, cancellation, consolidation or other reclassification of Deposited Securities, recapitalization, reorganization, merger, consolidation or sale of assets, the Depositary may, with the Company’s approval, and shall, if the Company shall so request, subject to the terms of the Deposit Agreement (including, without limitation, (a) the applicable fees and charges of, and expenses incurred by, the Depositary, and (b) taxes) and receipt of an opinion of counsel to the Company reasonably satisfactory to the Depositary that such actions are not in violation of any applicable laws or regulations, (i) issue and deliver additional ADSs as in the case of a stock dividend on the Shares, (ii) amend the Deposit Agreement and the applicable ADRs, (iii) amend the applicable Registration Statement(s) on Form F-6 as filed with the Commission in respect of the ADSs, (iv) call for the surrender of outstanding ADRs to be exchanged for new ADRs, and (v) take such other actions as the Depositary, in consultation with the Company, considers appropriate to reflect the transaction with respect to the ADSs.  The Company agrees to, jointly with the Depositary, amend the Registration Statement on Form F-6 as filed with the Commission to permit the issuance of such new form of ADRs.  Notwithstanding the foregoing, in the event that any Deposited Property so received may not be, upon consultation with the Company, lawfully distributed to some or all Holders, the Depositary may, with the Company’s approval, and shall, if the Company requests, subject to receipt of an opinion of Company’s counsel reasonably satisfactory to the Depositary that such action is not in violation of any applicable laws or regulations, sell such Deposited Property at public or private sale, at such place or places and upon such terms as it may deem proper and may allocate the net proceeds of such sales (net of (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) for the account of the Holders otherwise entitled to such Deposited Property upon an averaged or other practicable basis without regard to any distinctions among such Holders and distribute the net proceeds so allocated to the extent practicable as in the case of a distribution received in cash pursuant to Section 4.1.  The Depositary shall not be responsible for (i) any failure to determine whether it may be lawful or practicable to make such Deposited Property available to Holders in general or to any Holder in particular, (ii) any foreign exchange exposure or loss incurred in connection with such sale or (iii) any liability to the purchaser of such Deposited Property.

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Section 4.12        Available Information.  The Company publishes the information contemplated in Rule 12g3-2(b)(2)(i) under the Exchange Act on its internet website or through an electronic information delivery system generally available to the public in the Company’s primary trading market.  As of the date hereof the Company’s internet website is www.amrytpharma.com.  The information so published by the Company may not be in English, except that the Company is required, in order to maintain its exemption from the Exchange Act reporting obligations pursuant to Rule 12g3-2(b), to translate such information into English to the extent contemplated in the instructions to Rule 12g3-2(b).  The information so published by the Company cannot be retrieved from the Commission’s internet website, and cannot be inspected or copied at the public reference facilities maintained by the Commission located (as of the date of the Deposit Agreement) at 100 F Street, N.E., Washington, D.C. 20549.

Section 4.13        Reports.  The Depositary shall make available for inspection by Holders at its Principal Office, this Deposit Agreement, the provisions of or governing Deposited Securities and any reports and communications, including any proxy soliciting materials, received from the Company which are both (a) received by the Depositary, the Custodian, or the nominee of either of them as the holder of the Deposited Property and (b) made generally available to the holders of such Deposited Property by the Company.  The Depositary shall also provide or make available to Holders copies of such reports when furnished by the Company pursuant to Section 5.6.

Section 4.14        List of Holders.  Promptly upon written request by the Company, the Depositary shall furnish to the Company a list, as of a recent date, of the names, addresses and holdings of ADSs of all Holders.  Upon the written request of the Company, the Depositary shall, to the extent practicable, obtain and deliver to the Company a list of non-objecting Beneficial Owners.

Section 4.15        Taxation.  The Depositary will, and will instruct the Custodian to, forward to the Company or its agents such information from its records as the Company may reasonably request to enable the Company or its agents to file the necessary tax reports with governmental authorities or agencies.  The Depositary, the Custodian or the Company and its agents may file such reports as are necessary to reduce or eliminate applicable taxes on dividends and on other distributions in respect of Deposited Property under applicable tax treaties or laws for the Holders and Beneficial Owners.  In accordance with instructions from the Company and to the extent practicable, the Depositary or the Custodian will take reasonable administrative actions to obtain tax refunds, reduced withholding of tax at source on dividends and other benefits under applicable tax treaties or laws with respect to dividends and other distributions on the Deposited Property.  As a condition to receiving such benefits, Holders and Beneficial Owners of ADSs may be required from time to time, and in a timely manner, to file such proof of taxpayer status, residence and beneficial ownership (as applicable), to execute such certificates and to make such representations and warranties, or to provide any other information or documents, as the Company, the Depositary or the Custodian may deem necessary or proper to fulfill the Depositary’s or the Custodian’s obligations under applicable law.  The Depositary, the Custodian and the Company shall not have any obligation or liability to any person if any Holder or Beneficial Owner fails to provide such information or if such information does not reach the relevant tax authorities in time for any Holder or Beneficial Owner to obtain the benefits of any tax treatment.  The Holders and Beneficial Owners shall indemnify the Depositary, the Company, the Custodian and any of their respective directors, employees, agents and Affiliates against, and hold each of them harmless from, any claims by any governmental authority with respect to taxes, additions to tax, penalties or interest arising out of any refund of taxes, reduced rate of withholding at source or other tax benefit obtained.

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If the Company (or any of its agents) withholds from any distribution any amount on account of taxes or governmental charges, or pays any other tax in respect of such distribution (e.g., stamp duty tax, capital gains or other similar tax), the Company shall (and shall cause such agent to) remit promptly to the Depositary information about such taxes or governmental charges withheld or paid, and, if so requested, the tax receipt (or other proof of payment to the applicable governmental authority) therefor, in each case, in a form satisfactory to the Depositary.  The Depositary shall, to the extent required by U.S. law, report to Holders any taxes withheld by it or the Custodian, and, if such information is provided to it by the Company, any taxes withheld by the Company.  The Depositary and the Custodian shall not be required to provide the Holders with any evidence of the remittance by the Company (or its agents) of any taxes withheld, or of the payment of taxes by the Company, except to the extent the evidence is provided by the Company to the Depositary or the Custodian, as applicable.  Neither the Depositary or the Custodian shall be liable for the failure by any Holder or Beneficial Owner to obtain the benefits of credits on the basis of non‑U.S. tax paid against such Holder’s or Beneficial Owner’s income tax liability.  The Depositary is under no obligation to provide the Holders and Beneficial Owners with any information about the tax status of the Company.  The Depositary shall not incur any liability for any tax consequences that may be incurred by Holders and Beneficial Owners on account of their ownership of the ADSs, including without limitation, tax consequences resulting from the Company (or any of its subsidiaries) being treated as a “Passive Foreign Investment Company” (in each case as defined in the U.S. Internal Revenue Code of 1986, as amended, and the regulations issued thereunder) or otherwise.

ARTICLE V

THE DEPOSITARY, THE CUSTODIAN AND THE COMPANY

Section 5.1          Maintenance of Office and Transfer Books by the Registrar.  Until termination of the Deposit Agreement in accordance with its terms, the Registrar shall maintain in the Borough of Manhattan, the City of New York, an office and facilities for the issuance and delivery of ADSs, the acceptance for surrender of ADS(s) for the purpose of withdrawal of Deposited Securities, the registration of issuances, cancellations, transfers, combinations and split‑ups of ADS(s) and, if applicable, to countersign ADRs evidencing the ADSs so issued, transferred, combined or split-up, in each case in accordance with the provisions of the Deposit Agreement.

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The Registrar shall keep books for the registration of ADSs which at all reasonable times shall be open for inspection by the Company and by the Holders of such ADSs, provided that such inspection shall not be, to the Registrar’s knowledge, for the purpose of communicating with Holders of such ADSs in the interest of a business or object other than the business of the Company or other than a matter related to the Deposit Agreement or the ADSs.  The Company shall have the right to examine and copy the transfer and registration records of the Depositary or its agent, take copies thereof and require the Depositary or its agent, the Registrar and any co-transfer agents or co-registrars to supply copies of such portions of such records, to the extent practicable, as the Company may request from time to time.

The Registrar may close the transfer books with respect to the ADSs, at any time or from time to time, when deemed necessary or advisable by it in good faith in connection with the performance of its duties hereunder, or at the reasonable written request of the Company subject, in all cases, to Section 7.8.

If any ADSs are listed on one or more stock exchanges or automated quotation systems in the United States, the Depositary shall act as Registrar or appoint a Registrar or one or more co‑registrars for registration of issuances, cancellations, transfers, combinations and split-ups of ADSs and, if applicable, to countersign ADRs evidencing the ADSs so issued, transferred, combined or split-up,in accordance with any requirements of such exchanges or systems.  Such Registrar or co-registrars may, with notice to the Company as promptly as practicable, be removed and a substitute or substitutes appointed by the Depositary.

Section 5.2          Exoneration.  Notwithstanding any other provision of the Deposit Agreement or any ADR(s) to the contrary, neither the Depositary nor the Company shall be obligated to do or perform any act which is inconsistent with the provisions of the Deposit Agreement or incur any liability (to the extent not limited by Section 7.8(b)) (i) if the Depositary, the Custodian, the Company or their respective agents shall be prevented or forbidden from, or delayed in, doing or performing any act or thing required or contemplated by the terms of the Deposit Agreement, by reason of any provision of any present or future law or regulation of the United States, England and Wales, or any other country, or of any other governmental authority or regulatory authority or stock exchange, or on account of the potential criminal or civil penalties or restraint, or by reason of any provision, present or future, of the Articles of Association or any provision of or governing any Deposited Securities, or by reason of any act of God or war or other circumstances beyond its control (including, without limitation, nationalization, expropriation, currency restrictions, work stoppage, strikes, civil unrest, acts of terrorism, revolutions, rebellions, explosions and computer failure), (ii) by reason of any exercise of, or failure to exercise, any discretion provided for in the Deposit Agreement or in the Articles of Association or provisions of or governing Deposited Securities, (iii) for any action or inaction in reliance upon the advice of or information from legal counsel, accountants, any person presenting Shares for deposit, any Holder, any Beneficial Owner or authorized representative thereof, or any other person believed by it in good faith to be competent to give such advice or information, (iv) for the inability by a Holder or Beneficial Owner to benefit from any distribution, offering, right or other benefit which is made available to holders of Deposited Securities but is not, under the terms of the Deposit Agreement, made available to Holders of ADSs, (v) for any action or inaction of any clearing or settlement system (and any participant thereof) for the Deposited Property or the ADSs, or (vi) for any consequential or punitive damages (including lost profits) for any breach of the terms of the Deposit Agreement.

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The Depositary, its controlling persons, its agents, any Custodian and the Company, its Affiliates, its controlling persons and its agents may rely and shall be protected in acting upon any written notice, request or other document believed by it to be genuine and to have been signed or presented by the proper party or parties.

Section 5.3          Standard of Care.  The Company and the Depositary and their respective Affiliates, directors, officers, employees and agents assume no obligation and shall not be subject to any liability under the Deposit Agreement or any ADRs to any Holder(s) or Beneficial Owner(s), except that the Company and the Depositary agree to perform their respective obligations specifically set forth in the Deposit Agreement or the applicable ADRs without negligence or bad faith.

Without limitation of the foregoing, neither the Depositary, nor the Company, nor any of their respective Affiliates, directors, officers, controlling persons, employees or agents, shall be under any obligation to appear in, prosecute or defend any action, suit or other proceeding in respect of any Deposited Property or in respect of the ADSs, which in its opinion may involve it in expense or liability, unless indemnity satisfactory to it against all expense (including fees and disbursements of counsel) and liability be furnished as often as may be required (and no Custodian shall be under any obligation whatsoever with respect to such proceedings, the responsibility of the Custodian being solely to the Depositary).

The Depositary and its agents shall not be liable for any failure to carry out any instructions to vote any of the Deposited Securities, or for the manner in which any vote is cast or the effect of any vote, provided that any such action or omission is in good faith and without negligence and in accordance with the terms of the Deposit Agreement.  The Depositary shall not incur any liability for any failure to accurately determine whether any distribution or action may be lawful or reasonably practicable, for any investment risk associated with acquiring an interest in the Deposited Property, for the validity or worth of the Deposited Property, for the value of any Deposited Property or any distribution thereon, for any interest on Deposited Property, for any tax consequences that may result from the ownership of ADSs, Shares or other Deposited Property, for the credit‑worthiness of any third party. The Depositary shall not incur any liability for the content of any information submitted to it by the Company for distribution to the Holders or for any inaccuracy of any translation thereof, for allowing any rights to lapse upon the terms of the Deposit Agreement, for the failure or timeliness of any notice from the Company, or for any action of or failure to act by, or any information provided or not provided by, DTC or any DTC Participant.

The Depositary shall not be liable for any acts or omissions made by a successor depositary whether in connection with a previous act or omission of the Depositary or in connection with any matter arising wholly after the removal or resignation of the Depositary, provided that in connection with the issue out of which such potential liability arises the Depositary performed its obligations without negligence or bad faith while it acted as Depositary.

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Section 5.4          Resignation and Removal of the Depositary; Appointment of Successor Depositary.  The Depositary may at any time resign as Depositary hereunder by written notice of resignation delivered to the Company, such resignation to be effective on the earlier of (i) the 90th day after delivery thereof to the Company (whereupon the Depositary shall be entitled to take the actions contemplated in Section 6.2), or (ii) the appointment by the Company of a successor depositary and its acceptance of such appointment as hereinafter provided.

The Depositary, in such capacity, may at any time be removed by the Company by written notice of such removal, which removal shall be effective on the later of (i) the 90th day after delivery thereof to the Depositary (whereupon the Depositary shall be entitled to take the actions contemplated in Section 6.2), or (ii) the appointment by the Company of a successor depositary and its acceptance of such appointment as hereinafter provided.

In case at any time the Depositary acting hereunder shall resign or be removed, the Company shall use its best efforts to appoint a successor depositary, which shall be a bank or trust company having an office in the Borough of Manhattan, the City of New York.  Every successor depositary shall be required by the Company to execute and deliver to its predecessor and to the Company an instrument in writing accepting its appointment hereunder, and thereupon such successor depositary, without any further act or deed (except as required by applicable law), shall become fully vested with all the rights, powers, duties and obligations of its predecessor (other than as contemplated in Sections 5.8 and 5.9).  The predecessor depositary, upon payment of all sums due it and on the written request of the Company, shall, (i) execute and deliver an instrument transferring to such successor all rights and powers of such predecessor hereunder (other than as contemplated in Sections 5.8 and 5.9), (ii) duly assign, transfer and deliver all of the Depositary’s right, title and interest to the Deposited Property to such successor, and (iii) deliver to such successor a list of the Holders of all outstanding ADSs and such other information relating to ADSs and Holders thereof as the successor may reasonably request.  Any such successor depositary shall promptly provide notice of its appointment to such Holders.

Any entity into or with which the Depositary may be merged or consolidated shall be the successor of the Depositary without the execution or filing of any document or any further act.

Section 5.5          The Custodian.  The Depositary has initially appointed Citibank, N.A. (London) as Custodian for the purpose of the Deposit Agreement.  The Custodian or its successors in acting hereunder shall be authorized to act as custodian in England and Wales and shall be subject at all times and in all respects to the direction of the Depositary for the Deposited Property for which the Custodian acts as custodian and shall be responsible solely to it.  If any Custodian resigns or is discharged from its duties hereunder with respect to any Deposited Property and no other Custodian has previously been appointed hereunder, the Depositary shall, with notice to the Company as soon as practicable, promptly appoint a substitute custodian.  The Depositary shall require such resigning or discharged Custodian to Deliver, or cause the Delivery of, the Deposited Property held by it, together with all such records maintained by it as Custodian with respect to such Deposited Property as the Depositary may request, to the Custodian designated by the Depositary.  Whenever the Depositary determines, in its discretion, that it is appropriate to do so, it may appoint an additional custodian with respect to any Deposited Property, or discharge the Custodian with respect to any Deposited Property and appoint a substitute custodian, which shall thereafter be Custodian hereunder with respect to the Deposited Property.  Immediately upon any such change, the Depositary shall give notice thereof in writing to all Holders of ADSs, each other Custodian and the Company.

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Citibank may at any time act as Custodian of the Deposited Property pursuant to the Deposit Agreement, in which case any reference to Custodian shall mean Citibank solely in its capacity as Custodian pursuant to the Deposit Agreement.  Notwithstanding anything contained in the Deposit Agreement or any ADR to the contrary, the Depositary shall not be obligated to give notice to the Company, any Holders of ADSs or any other Custodian of its acting as Custodian pursuant to the Deposit Agreement.

Upon the appointment of any successor depositary, any Custodian then acting hereunder shall, unless otherwise instructed by the Depositary, continue to be the Custodian of the Deposited Property without any further act or writing, and shall be subject to the direction of the successor depositary.  The successor depositary so appointed shall, nevertheless, on the written request of any Custodian, execute and deliver to such Custodian all such instruments as may be proper to give to such Custodian full and complete power and authority to act on the direction of such successor depositary.

Section 5.6          Notices and Reports.  On or before the first date on which the Company gives notice, by publication or otherwise, of any meeting of holders of Shares or other Deposited Securities, or of any adjourned meeting of such holders, or of the taking of any action by such holders other than at a meeting, or of the taking of any action in respect of any cash or other distributions or the offering of any rights in respect of Deposited Securities, the Company shall transmit to the Depositary and the Custodian a copy of the notice thereof in the English language but otherwise in the form given or to be given to holders of Shares or other Deposited Securities. The Company shall also furnish to the Custodian and the Depositary a summary, in English, of any applicable provisions or proposed provisions of the Articles of Association that may be relevant or pertain to such notice of meeting or be the subject of a vote thereat.

The Company will also transmit to the Depositary English‑language versions of the other notices, reports and communications which are made generally available by the Company to holders of its Shares or other Deposited Securities.  The Depositary shall arrange, at the request of the Company and at the Company’s expense, to provide copies thereof to all Holders or make such notices, reports and other communications available to all Holders on a basis similar to that for holders of Shares or other Deposited Securities or on such other basis as the Company may advise the Depositary or as may be required by any applicable law, regulation or stock exchange requirement.  The Company has delivered to the Depositary and the Custodian a copy of the Company’s Articles of Association along with the provisions of or governing the Shares and any other Deposited Securities issued by the Company in connection with such Shares, and promptly upon any amendment thereto or change therein, the Company shall deliver to the Depositary and the Custodian a copy of such amendment thereto or change therein.  The Depositary may rely upon such copy for all purposes of the Deposit Agreement.

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The Depositary will, at the expense of the Company, make available a copy of any such notices, reports or communications issued by the Company and delivered to the Depositary for inspection by the Holders of the ADSs at the Depositary’s Principal Office, at the office of the Custodian and at any other designated transfer office.

Section 5.7          Issuance of Additional Shares, ADSs etc.  The Company agrees that in the event it or any of its Affiliates proposes (i) an issuance, sale or distribution of additional Shares, (ii) an offering of rights to subscribe for Shares or other Deposited Securities, (iii) an issuance or assumption of securities convertible into or exchangeable for Shares, (iv) an issuance of rights to subscribe for securities convertible into or exchangeable for Shares, (v) an elective dividend of cash or Shares, (vi) a redemption of Deposited Securities, (vii) a meeting of holders of Deposited Securities, or solicitation of consents or proxies, relating to any reclassification of securities, merger or consolidation or transfer of assets, (viii) any assumption, reclassification, recapitalization, reorganization, merger, consolidation or sale of assets which affects the Deposited Securities, or (ix) a distribution of securities other than Shares, it will obtain U.S. legal advice and take all steps necessary to ensure that the application of the proposed transaction to Holders and Beneficial Owners does not violate the registration provisions of the Securities Act, or any other applicable laws (including, without limitation, the Investment Company Act of 1940, as amended, the Exchange Act and the securities laws of the states of the U.S.).  In support of the foregoing and in connection with any such transactions, the Company will furnish to the Depositary, if applicable, (a) a written opinion of U.S. counsel (reasonably satisfactory to the Depositary) stating whether such transaction (1) requires a registration statement under the Securities Act to be in effect or (2) is exempt from the registration requirements of the Securities Act and (b) an opinion of English counsel stating that (1) making the transaction available to Holders and Beneficial Owners does not violate the laws or regulations of England and Wales and (2) all requisite regulatory consents and approvals, if any, have been obtained in England and Wales.  If the filing of a registration statement is required, the Depositary shall not have any obligation to proceed with the transaction unless it shall have received evidence reasonably satisfactory to it that such registration statement has been declared, or has otherwise become, effective.  If, being advised by counsel, the Company determines that a transaction is required to be registered under the Securities Act, the Company will either (i) register such transaction to the extent necessary, (ii) alter the terms of the transaction to avoid the registration requirements of the Securities Act or (iii) direct the Depositary to take specific measures, in each case as contemplated in the Deposit Agreement, to prevent such transaction from violating the registration requirements of the Securities Act.  The Company agrees with the Depositary that neither the Company nor any of its Affiliates will at any time (i) deposit any Shares or other Deposited Securities, either upon original issuance or upon a sale of Shares or other Deposited Securities previously issued and reacquired by the Company or by any such Affiliate, or (ii) issue additional Shares, rights to subscribe for such Shares, securities convertible into or exchangeable for Shares or rights to subscribe for such securities or distribute securities other than Shares, unless such transaction and the securities issuable in such transaction do not violate the registration provisions of the Securities Act, or any other applicable laws (including, without limitation, the Investment Company Act of 1940, as amended, the Exchange Act and the securities laws of the states of the U.S.).

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Notwithstanding anything to the contrary contained in the Deposit Agreement, nothing in the Deposit Agreement shall be deemed to obligate the Company to file any registration statement in respect of any proposed transaction.

Section 5.8          Indemnification.  The Depositary agrees to indemnify the Company and its directors, officers, employees, agents and Affiliates against, and hold each of them harmless from, any direct loss, liability, tax, charge or expense of any kind whatsoever (including, but not limited to, the reasonable fees and expenses of counsel) which may arise out of acts performed or omitted by the Depositary or the Custodian (for so long as the Custodian is a branch of Citibank N.A.) under the terms hereof due to the negligence or bad faith of the Depositary or the Custodian (for so long as the Custodian is a branch of Citibank N.A.), as applicable.

The Company agrees to indemnify the Depositary, the Custodian and any of their respective directors, officers, employees, agents and Affiliates against, and hold each of them harmless from, any direct loss, liability, tax, charge or expense of any kind whatsoever (including, but not limited to, the reasonable fees and expenses of counsel) that may arise (a) out of, or in connection with, any offer, issuance, sale, resale, transfer, deposit or withdrawal of ADRs, ADSs, the Shares, or other Deposited Securities, as the case may be, (b) out of, or as a result of, any offering documents in respect thereof or (c) out of acts performed or omitted, including, but not limited to, any delivery by the Depositary on behalf of the Company of information regarding the Company, in connection with the Deposit Agreement, any ancillary or supplemental agreement entered into between the Company and the Depositary, the ADRs, the ADSs, the Shares, or any Deposited Property, in any such case (i) by the Depositary, the Custodian or any of their respective directors, officers, employees, agents and Affiliates, except to the extent such loss, liability, tax, charge or expense is due to the negligence or bad faith of any of them, or (ii) by the Company or any of its directors, officers, employees, agents and Affiliates.  The Company shall not indemnify the Depositary, the Custodian and any of their respective directors, officers, employees, agents and Affiliates against any liability or expense arising out of information relating to the Depositary or such Custodian, as the case may be, furnished to the Company by the Depositary or such Custodian for use in any registration statement, prospectus or preliminary prospectus relating to any Deposited Securities represented by the ADSs.

The obligations set forth in this Section shall survive the termination of the Deposit Agreement and the succession or substitution of any party hereto.

Any person seeking indemnification hereunder (an “indemnified person”) shall notify the person from whom it is seeking indemnification (the “indemnifying person”) of the commencement of any indemnifiable action or claim promptly after such indemnified person becomes aware of such commencement (provided that the failure to make such notification shall not affect such indemnified person’s rights to seek indemnification except to the extent the indemnifying person is materially prejudiced by such failure) and shall consult in good faith with the indemnifying person as to the conduct of the defense of such action or claim that may give rise to an indemnity hereunder, which defense shall be reasonable in the circumstances.  No indemnified person shall compromise or settle any action or claim that may give rise to an indemnity hereunder without the consent of the indemnifying person, which consent shall not be unreasonably withheld.

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Section 5.9           ADS Fees and Charges.  The Company, the Holders, the Beneficial Owners, persons depositing Shares or withdrawing Deposited Securities in connection with the issuance and cancellation of ADSs, and persons receiving ADSs upon issuance or whose ADSs are being cancelled shall be required to pay to the Depositary the Depositary’s fees and related charges identified as payable by them respectively in the Fee Schedule attached hereto as Exhibit B.  All ADS fees and charges so payable may be deducted from distributions or must be remitted to the Depositary, or its designee,and may, at any time and from time to time, be changed by agreement between the Depositary and the Company, but, in the case of ADS fees and charges payable by Holders and Beneficial Owners, only in the manner contemplated in Section 6.1.  The Depositary shall provide, without charge, a copy of its latest ADS fee schedule to anyone upon request.

ADS fees and charges for (i) the issuance of ADSs and (ii) the cancellation of ADSs will be payable by the person for whom the ADSs are so issued by the Depositary (in the case of ADS issuances) and by the person for whom ADSs are being cancelled (in the case of ADS cancellations).  In the case of ADSs issued by the Depositary into DTC or presented to the Depositary via DTC, the ADS issuance and cancellation fees and charges will be payable by the DTC Participant(s) receiving the ADSs from the Depositary or the DTC Participant(s) holding the ADSs being cancelled, as the case may be, on behalf of the Beneficial Owner(s) and will be charged by the DTC Participant(s) to the account(s) of the applicable Beneficial Owner(s) in accordance with the procedures and practices of the DTC participant(s) as in effect at the time.  ADS fees and charges in respect of distributions and the ADS service fee are payable by Holders as of the applicable ADS Record Date established by the Depositary.  In the case of distributions of cash, the amount of the applicable ADS fees and charges is deducted from the funds being distributed.  In the case of (i) distributions other than cash and (ii) the ADS service fee, the applicable Holders as of the ADS Record Date established by the Depositary will be invoiced for the amount of the ADS fees and charges and such ADS fees may be deducted from distributions made to Holders.  For ADSs held through DTC, the ADS fees and charges for distributions other than cash and the ADS service fee may be deducted from distributions made through DTC, and may be charged to the DTC Participants in accordance with the procedures and practices prescribed by DTC from time to time and the DTC Participants in turn charge the amount of such ADS fees and charges to the Beneficial Owners for whom they hold ADSs.  In the case of (i) registration of ADS transfers, the ADS transfer fee will be payable by the ADS Holder whose ADSs are being transferred or by the person to whom the ADSs are transferred, and (ii) conversion of ADSs of one series for ADSs of another series, the ADS conversion fee will be payable by the Holder whose ADSs are converted or by the person to whom the converted ADSs are delivered.

The Depositary may reimburse the Company for certain expenses incurred by the Company in respect of the ADR program established pursuant to the Deposit Agreement, by making available a portion of the ADS fees charged in respect of the ADR program or otherwise, upon such terms and conditions as the Company and the Depositary agree from time to time.  The Company shall pay to the Depositary such fees and charges, and reimburse the Depositary for such out-of-pocket expenses, as the Depositary and the Company may agree from time to time.  Responsibility for payment of such fees, charges and reimbursements may from time to time be changed by agreement between the Company and the Depositary.  Unless otherwise agreed, the Depositary shall present its statement for such fees, charges and reimbursements to the Company once every three months.  The charges and expenses of the Custodian are for the sole account of the Depositary.

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The obligation of Holders and Beneficial Owners to pay ADS fees and charges shall survive the termination of the Deposit Agreement.  As to any Depositary, upon the resignation or removal of such Depositary as described in Section 5.4, the right to collect ADS fees and charges shall extend for those ADS fees and charges incurred prior to the effectiveness of such resignation or removal.

Section 5.10        Restricted Securities Owners.  The Company agrees to advise in writing each of the persons or entities who, to the knowledge of the Company, holds Restricted Securities that such Restricted Securities are ineligible for deposit hereunder (except under the circumstances contemplated in Section 2.14) and, to the extent practicable, shall require each of such persons to represent in writing that such person will not deposit Restricted Securities hereunder (except under the circumstances contemplated in Section 2.14).

ARTICLE VI

AMENDMENT AND TERMINATION

Section 6.1          Amendment/Supplement.  Subject to the terms and conditions of this Section 6.1 and applicable law, the ADRs outstanding at any time, the provisions of the Deposit Agreement and the form of ADR attached hereto and to be issued under the terms hereof may at any time and from time to time be amended or supplemented by written agreement between the Company and the Depositary in any respect which they may deem necessary or desirable without the prior written consent of the Holders or Beneficial Owners.  Any amendment or supplement which shall impose or increase any fees or charges (other than charges in connection with foreign exchange control regulations, and taxes and other governmental charges, delivery and other such expenses), or which shall otherwise materially prejudice any substantial existing right of Holders or Beneficial Owners, shall not, however, become effective as to outstanding ADSs until the expiration of thirty (30) days after notice of such amendment or supplement shall have been given to the Holders of outstanding ADSs.  Notice of any amendment to the Deposit Agreement or any ADR shall not need to describe in detail the specific amendments effectuated thereby, and failure to describe the specific amendments in any such notice shall not render such notice invalid, provided, however, that, in each such case, the notice given to the Holders identifies a means for Holders and Beneficial Owners to retrieve or receive the text of such amendment (e.g., upon retrieval from the Commission’s, the Depositary’s or the Company’s website or upon request from the Depositary).  The parties hereto agree that any amendments or supplements which (i) are reasonably necessary (as agreed by the Company and the Depositary) in order for (a) the ADSs to be registered on Form F-6 under the Securities Act or (b) the ADSs to be settled solely in electronic book‑entry form and (ii) do not in either such case impose or increase any fees or charges to be borne by Holders, shall be deemed not to materially prejudice any substantial existing rights of Holders or Beneficial Owners.  Every Holder and Beneficial Owner at the time any amendment or supplement so becomes effective shall be deemed, by continuing to hold such ADSs, to consent and agree to such amendment or supplement and to be bound by the Deposit Agreement and the ADR, if applicable, as amended or supplemented thereby.  In no event shall any amendment or supplement impair the right of the Holder to surrender such ADS and receive therefor the Deposited Securities represented thereby, except in order to comply with mandatory provisions of applicable law.  Notwithstanding the foregoing, if any governmental body should adopt new laws, rules or regulations which would require an amendment of, or supplement to, the Deposit Agreement to ensure compliance therewith, the Company and the Depositary may amend or supplement the Deposit Agreement and any ADRs at any time in accordance with such changed laws, rules or regulations.  Such amendment or supplement to the Deposit Agreement and any ADRs in such circumstances may become effective before a notice of such amendment or supplement is given to Holders or within any other period of time as required for compliance with such laws, rules or regulations.

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Section 6.2          Termination.  The Depositary shall, at any time at the written direction of the Company, terminate the Deposit Agreement by distributing notice of such termination to the Holders of all ADSs then outstanding at least thirty (30) days prior to the date fixed in such notice for such termination.  If (i) ninety (90) days shall have expired after the Depositary shall have delivered to the Company a written notice of its election to resign, or (ii) ninety (90) days shall have expired after the Company shall have delivered to the Depositary a written notice of the removal of the Depositary, and, in either case, a successor depositary shall not have been appointed and accepted its appointment as provided in Section 5.4 of the Deposit Agreement, the Depositary may terminate the Deposit Agreement by distributing notice of such termination to the Holders of all ADSs then outstanding at least thirty (30) days prior to the date fixed in such notice for such termination.  The date so fixed for termination of the Deposit Agreement in any termination notice so distributed by the Depositary to the Holders of ADSs is referred to as the “Termination Date”.  Until the Termination Date, the Depositary shall continue to perform all of its obligations under the Deposit Agreement, and the Holders and Beneficial Owners will be entitled to all of their rights under the Deposit Agreement.

If any ADSs shall remain outstanding after the Termination Date, the Registrar and the Depositary shall not, after the Termination Date, have any obligation to perform any further acts under the Deposit Agreement, except that the Depositary shall, subject, in each case, to the terms and conditions of the Deposit Agreement, continue to (i) collect dividends and other distributions pertaining to Deposited Securities, (ii) sell Deposited Property received in respect of Deposited Securities, (iii) deliver Deposited Securities, together with any dividends or other distributions received with respect thereto and the net proceeds of the sale of any other Deposited Property, in exchange for ADSs surrendered to the Depositary (after deducting, or charging, as the case may be, in each case, the fees and charges of, and expenses incurred by, the Depositary, and all applicable taxes or governmental charges for the account of the Holders and Beneficial Owners, in each case upon the terms set forth in Section 5.9 of the Deposit Agreement), and (iv) take such actions as may be required under applicable law in connection with its role as Depositary under the Deposit Agreement.

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At any time after the Termination Date, the Depositary may sell the Deposited Property then held under the Deposit Agreement and shall after such sale hold un-invested the net proceeds of such sale, together with any other cash then held by it under the Deposit Agreement, in an un-segregated account and without liability for interest, for the pro rata benefit of the Holders whose ADSs have not theretofore been surrendered.  After making such sale, the Depositary shall be discharged from all obligations under the Deposit Agreement except (i) to account for such net proceeds and other cash (after deducting, or charging, as the case may be, in each case, the fees and charges of, and expenses incurred by, the Depositary, and all applicable taxes or governmental charges for the account of the Holders and Beneficial Owners, in each case upon the terms set forth in Section 5.9 of the Deposit Agreement), and (ii) as may be required at law in connection with the termination of the Deposit Agreement.  After the Termination Date, the Company shall be discharged from all obligations under the Deposit Agreement, except for its obligations to the Depositary under Sections 5.8, 5.9 and 7.6 of the Deposit Agreement.  The obligations under the terms of the Deposit Agreement of Holders and Beneficial Owners of ADSs outstanding as of the Termination Date shall survive the Termination Date and shall be discharged only when the applicable ADSs are presented by their Holders to the Depositary for cancellation under the terms of the Deposit Agreement (except as specifically provided in the Deposit Agreement).

ARTICLE VII

MISCELLANEOUS

Section 7.1          Counterparts.  The Deposit Agreement may be executed in any number of counterparts, each of which shall be deemed an original and all of such counterparts together shall constitute one and the same agreement.  Copies of the Deposit Agreement shall be maintained with the Depositary and shall be open to inspection by any Holder during business hours.

Section 7.2          No Third‑Party Beneficiaries/Acknowledgments.  The Deposit Agreement is for the exclusive benefit of the parties hereto (and their successors) and shall not be deemed to give any legal or equitable right, remedy or claim whatsoever to any other person, except to the extent specifically set forth in the Deposit Agreement.  Nothing in the Deposit Agreement shall be deemed to give rise to a partnership or joint venture among the parties nor establish a fiduciary or similar relationship among the parties.  The parties hereto acknowledge and agree that (i) Citibank and its Affiliates may at any time have multiple banking relationships with the Company,  the Holders, the Beneficial Owners, and their respective Affiliates,  (ii) Citibank and its Affiliates may own and deal in any class of securities of the Company and its Affiliates and in ADSs, and may be engaged at any time in transactions in which parties adverse to the Company, the Holders, the Beneficial Owners or their respective Affiliates may have interests, (iii) the Depositary and its Affiliates may from time to time have in their possession non-public information about the Company, the Holders, the Beneficial Owners, and their respective Affiliates, (iv) nothing contained in the Deposit Agreement shall (a) preclude Citibank or any of its Affiliates from engaging in such transactions or establishing or maintaining such relationships, or (b) obligate Citibank or any of its Affiliates to disclose such information, transactions or relationships, or to account for any profit made or payment received in such transactions or relationships,  (v) the Depositary shall not be deemed to have knowledge of any information any other division of Citibank or any of its Affiliates may have about the Company, the Holders, the Beneficial Owners, or any of their respective Affiliates, and (vi) the Company, the Depositary, the Custodian and their respective agents and controlling persons may be subject to the laws and regulations of jurisdictions other than the U.S. and England and Wales, and the authority of courts and regulatory authorities of such other jurisdictions, and, consequently, the requirements and the limitations of such other laws and regulations, and the decisions and orders of such other courts and regulatory authorities, may affect the rights and obligations of the parties to the Deposit Agreement.

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Section 7.3          Severability.  In case any one or more of the provisions contained in the Deposit Agreement or in the ADRs should be or become invalid, illegal or unenforceable in any respect, the validity, legality and enforceability of the remaining provisions contained herein or therein shall in no way be affected, prejudiced or disturbed thereby.

Section 7.4          Holders and Beneficial Owners as Parties; Binding Effect.  The Holders and Beneficial Owners from time to time of ADSs issued hereunder shall be parties to the Deposit Agreement and shall be bound by all of the terms and conditions hereof and of any ADR evidencing their ADSs by acceptance thereof or any beneficial interest therein.

Section 7.5          Notices.  Any and all notices to be given to the Company shall be in writing and shall be deemed to have been duly given if personally delivered or sent by mail, air courier or email as follows:

Address:	90 Harcourt Street, Dublin 2, Ireland
Attention:	Chief Financial Officer
Email:	rory.nealon@amrytpharma.com,

or to any other address which the Company may specify in writing to the Depositary.

Any and all notices to be given to the Depositary shall be in writing and deemed to have been duly given if personally delivered or sent by mail, air courier or cable, telex or facsimile transmission, confirmed by letter personally delivered or sent by mail or air courier, addressed to Citibank, N.A., 388 Greenwich Street, New York, New York 10013, U.S.A., Attention:  Depositary Receipts Department, or to any other address which the Depositary may specify in writing to the Company.

Any and all notices to be given to any Holder shall be deemed to have been duly given

(a) if personally delivered or sent by mail or cable, telex or facsimile transmission, confirmed by letter, addressed to such Holder at the address of such Holder as it appears on the books of the Depositary or, if such Holder shall have filed with the Depositary a request that notices intended for such Holder be mailed to some other address, at the address specified in such request, or

(b) if a Holder shall have designated such means of notification as an acceptable means of notification under the terms of the Deposit Agreement, by means of electronic messaging addressed for delivery to the e-mail address designated by the Holder for such purpose.  Notice to Holders shall be deemed to be notice to Beneficial Owners for all purposes of the Deposit Agreement.  Failure to notify a Holder or any defect in the notification to a Holder shall not affect the sufficiency of notification to other Holders or to the Beneficial Owners of ADSs held by such other Holders.  Any notices given to DTC under the terms of the Deposit Agreement shall (unless otherwise specified by the Depositary) constitute notice to the DTC Participants who hold the ADSs in their DTC accounts and to the Beneficial Owners of such ADSs.

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Delivery of a notice sent by mail, air courier or cable, telex or facsimile transmission shall be deemed to be effective at the time when a duly addressed letter containing the same (or a confirmation thereof in the case of a cable, telex or facsimile transmission) is deposited, postage prepaid, in a post-office letter box or delivered to an air courier service, without regard for the actual receipt or time of actual receipt thereof by a Holder.  The Depositary or the Company may, however, act upon any cable, telex or facsimile transmission received by it from any Holder, the Custodian, the Depositary, or the Company, notwithstanding that such cable, telex or facsimile transmission shall not be subsequently confirmed by letter.

Delivery of a notice by means of email or electronic messaging shall be deemed to be effective at the time of the initiation of the transmission by the sender (as shown on the sender’s records), notwithstanding that the intended recipient retrieves the message at a later date, fails to retrieve such message, or fails to receive such notice on account of its failure to maintain the designated e-mail address, its failure to designate a substitute e-mail address or for any other reason.

Section 7.6          Governing Law and Jurisdiction.  The Deposit Agreement, the ADRs and the ADSs shall be interpreted in accordance with, and all rights hereunder and thereunder and provisions hereof and thereof shall be governed by, the laws of the State of New York without reference to the principles of choice of law thereof.  Notwithstanding any other provision of the Deposit Agreement to the contrary, any ADR or any present or future provisions of the laws of the State of New York, the rights of holders of Shares and of any other Deposited Securities and the obligations and duties of the Company in respect of the holders of Shares and other Deposited Securities, as such, shall be governed by the laws of England and Wales (or, if applicable, such other laws as may govern the Deposited Securities).

Except as set forth in the following paragraph of this Section 7.6, the Company and the Depositary agree that the federal or state courts in the City of New York shall have jurisdiction to hear and determine any suit, action or proceeding and to settle any dispute between them that may arise out of or in connection with the Deposit Agreement and, for such purposes, each irrevocably submits to the non-exclusive jurisdiction of such courts.  The Company hereby irrevocably designates, appoints and empowers Aegerion Pharmaceuticals, Inc. (the “Agent”) now at 53 State Street, Suite 500, Boston, MA 02109 as its authorized agent to receive and accept for and on its behalf, and on behalf of its properties, assets and revenues, service by mail of any and all legal process, summons, notices and documents that may be served in any suit, action or proceeding brought against the Company in any federal or state court as described in the preceding sentence or in the next paragraph of this Section 7.6.  If for any reason the Agent shall cease to be available to act as such, the Company agrees to designate a new agent in New York on the terms and for the purposes of this Section 7.6 reasonably satisfactory to the Depositary.  The Company further hereby irrevocably consents and agrees to the service of any and all legal process, summons, notices and documents in any suit, action or proceeding against the Company, by service by mail of a copy thereof upon the Agent (whether or not the appointment of such Agent shall for any reason prove to be ineffective or such Agent shall fail to accept or acknowledge such service), with a copy mailed to the Company by registered or certified air mail, postage prepaid, to its address provided in Section 7.5.  The Company agrees that the failure of the Agent to give any notice of such service to it shall not impair or affect in any way the validity of such service or any judgment rendered in any action or proceeding based thereon.

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Notwithstanding the foregoing, the Depositary and the Company unconditionally agree that in the event of any suit, action or proceeding against (a) the Company, (b) the Depositary in its capacity as Depositary under the Deposit Agreement, or (c) against both the Company and the Depositary, in any such case, in any state or federal court of the United States, and the Depositary or the Company have any claim, for indemnification or otherwise, against each other arising out of the subject matter of such suit, action or proceeding, then the Company and the Depositary may pursue such claim against each other in the state or federal court in the United States in which such suit, action, or proceeding is pending and, for such purposes, the Company and the Depositary irrevocably submit to the non-exclusive jurisdiction of such courts.  The Company agrees that service of process upon the Agent in the manner set forth in the preceding paragraph shall be effective service upon it for any suit, action or proceeding brought against it as described in this paragraph.

The Company irrevocably and unconditionally waives, to the fullest extent permitted by law, any objection that it may now or hereafter have to the laying of venue of any actions, suits or proceedings brought in any court as provided in this Section 7.6, and hereby further irrevocably and unconditionally waives and agrees not to plead or claim in any such court that any such action, suit or proceeding brought in any such court has been brought in an inconvenient forum.

The Company irrevocably and unconditionally waives, to the fullest extent permitted by law, and agrees not to plead or claim, any right of immunity from legal action, suit or proceeding, from setoff or counterclaim, from the jurisdiction of any court, from service of process, from attachment upon or prior to judgment, from attachment in aid of execution or judgment, from execution of judgment, or from any other legal process or proceeding for the giving of any relief or for the enforcement of any judgment, and consents to such relief and enforcement against it, its assets and its revenues in any jurisdiction, in each case with respect to any matter arising out of, or in connection with, the Deposit Agreement, any ADR or the Deposited Property.

EACH OF THE PARTIES TO THE DEPOSIT AGREEMENT (INCLUDING, WITHOUT LIMITATION, EACH HOLDER AND BENEFICIAL OWNER) IRREVOCABLY WAIVES, TO THE FULLEST EXTENT PERMITTED BY APPLICABLE LAW, ANY AND ALL RIGHT TO TRIAL BY JURY IN ANY LEGAL PROCEEDING AGAINST THE COMPANY AND/OR THE DEPOSITARY ARISING OUT OF, OR RELATING TO, THE DEPOSIT AGREEMENT, ANY ADR AND ANY TRANSACTIONS CONTEMPLATED THEREIN (WHETHER BASED ON CONTRACT, TORT, COMMON LAW OR OTHERWISE).

The provisions of this Section 7.6 shall survive any termination of the Deposit Agreement, in whole or in part.

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Section 7.7          Assignment.  Subject to the provisions of Section 5.4, the Deposit Agreement may not be assigned by either the Company or the Depositary.

Section 7.8          Compliance with, and No Disclaimer under, U.S. Securities Laws.

(a)          Notwithstanding anything in the Deposit Agreement to the contrary, the withdrawal or delivery of Deposited Securities will not be suspended by the Company or the Depositary except as would be permitted by Instruction I.A.(1) of the General Instructions to Form F-6 Registration Statement, as amended from time to time, under the Securities Act.

(b)          Each of the parties to the Deposit Agreement (including, without limitation, each Holder and Beneficial Owner) acknowledges and agrees that no provision of the Deposit Agreement or any ADR shall, or shall be deemed to, disclaim any liability under the Securities Act or the Exchange Act, in each case to the extent established under applicable U.S. laws.

Section 7.9          English Law References.  Any summary of English laws and regulations and of the terms of the Company’s Articles of Association set forth in the Deposit Agreement have been provided by the Company solely for the convenience of Holders, Beneficial Owners and the Depositary.  While such summaries are believed by the Company to be accurate as of the date of the Deposit Agreement, (i) they are summaries and as such may not include all aspects of the materials summarized applicable to a Holder or Beneficial Owner, and (ii) these laws and regulations and the Company’s Articles of Association may change after the date of the Deposit Agreement.  Neither the Depositary nor the Company has any obligation under the terms of the Deposit Agreement to update any such summaries.

Section 7.10        Titles and References.

(a)          Deposit Agreement.  All references in the Deposit Agreement to exhibits, articles, sections, subsections, and other subdivisions refer to the exhibits, articles, sections, subsections and other subdivisions of the Deposit Agreement unless expressly provided otherwise.  The words “the Deposit Agreement”, “herein”, “hereof”, “hereby”, “hereunder”, and words of similar import refer to the Deposit Agreement as a whole as in effect at the relevant time between the Company, the Depositary and the Holders and Beneficial Owners of ADSs and not to any particular subdivision unless expressly so limited.  Pronouns in masculine, feminine and neuter gender shall be construed to include any other gender, and words in the singular form shall be construed to include the plural and vice versa unless the context otherwise requires.  Titles to sections of the Deposit Agreement are included for convenience only and shall be disregarded in construing the language contained in the Deposit Agreement.  References to “applicable laws and regulations” shall refer to laws and regulations applicable to ADRs, ADSs or Deposited Property as in effect at the relevant time of determination, unless otherwise required by law or regulation.

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(b)          ADRs.  All references in any ADR(s) to paragraphs, exhibits, articles, sections, subsections, and other subdivisions refer to the paragraphs, exhibits, articles, sections, subsections and other subdivisions of the ADR(s) in question unless expressly provided otherwise.  The words “the Receipt”, “the ADR”, “herein”, “hereof”, “hereby”, “hereunder”, and words of similar import used in any ADR refer to the ADR as a whole and as in effect at the relevant time, and not to any particular subdivision unless expressly so limited.  Pronouns in masculine, feminine and neuter gender in any ADR shall be construed to include any other gender, and words in the singular form shall be construed to include the plural and vice versa unless the context otherwise requires.  Titles to paragraphs of any ADR are included for convenience only and shall be disregarded in construing the language contained in the ADR.  References to “applicable laws and regulations” shall refer to laws and regulations applicable to the Company, the Depositary, the Custodian, their agents and controlling persons, the ADRs, the ADSs and the Deposited Property as in effect at the relevant time of determination, unless otherwise required by law or regulation.

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IN WITNESS WHEREOF, Amryt Pharma Holdings plc and CITIBANK, N.A. have duly executed the Deposit Agreement as of the day and year first above set forth and all Holders and Beneficial Owners shall become parties hereto upon acceptance by them of ADSs issued in accordance with the terms hereof, or upon acquisition of any beneficial interest therein.

AMRYT PHARMA HOLDINGS PLC
By:	/s/ Rory Nealon
	Name:  Rory Nealon
	Title:  Director

CITIBANK, N.A.
By:	/s/ Leslie DeLuca
	Name:  Leslie DeLuca
	Title:  Attorney-in-Fact

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EXHIBIT A

[FORM OF ADR]

Number CUSIP NUMBER:

American Depositary Shares (each

American Depositary Share

representing the right to receive five

(5) fully paid ordinaryshares)

AMERICAN DEPOSITARY RECEIPT

for

AMERICAN DEPOSITARY SHARES

representing

DEPOSITED ORDINARY SHARES

of

AMRYT PHARMA HOLDINGS PLC

(Incorporated under the laws of England and Wales)

CITIBANK, N.A., a national banking association organized and existing under the laws of the United States of America, as depositary (the “Depositary”), hereby certifies that _____________is the owner of ______________ American Depositary Shares (hereinafter “ADS”) representing deposited ordinary shares, including evidence of rights to receive such ordinary shares (the “Shares”), of Amryt Pharma Holdings plc, a public limited company incorporated under the laws of England and Wales, and its successors (the “Company”).  As of the date of issuance of this ADR, each ADS represents the right to receive five (5) Shares deposited under the Deposit Agreement (as hereinafter defined) with the Custodian, which at the date of issuance of this ADR is Citibank, N.A. (London) (the “Custodian”).  The ADS(s)-to-Share(s) ratio is subject to amendment as provided in Articles IV and VI of the Deposit Agreement.  The Depositary’s Principal Office is located at 388 Greenwich Street, New York, New York 10013, U.S.A.

A-1

(1)          The Deposit Agreement.  This American Depositary Receipt is one of an issue of American Depositary Receipts (“ADRs”), all issued and to be issued upon the terms and conditions set forth in the Deposit Agreement, dated as of September 24, 2019 (as amended and supplemented from time to time, the “Deposit Agreement”), by and among the Company, the Depositary, and all Holders and Beneficial Owners from time to time of ADSs issued thereunder.  The Deposit Agreement sets forth the rights and obligations of Holders and Beneficial Owners of ADSs and the rights and duties of the Depositary in respect of the Shares deposited thereunder and any and all other Deposited Property (as defined in the Deposit Agreement) from time to time received and held on deposit in respect of the ADSs.  Copies of the Deposit Agreement are on file at the Principal Office of the Depositary and with the Custodian.  Each Holder and each Beneficial Owner, upon acceptance of any ADSs (or any interest therein) issued in accordance with the terms and conditions of the Deposit Agreement, shall be deemed for all purposes to (a) be a party to and bound by the terms of the Deposit Agreement and the applicable ADR(s), and (b) appoint the Depositary its attorney‑in‑fact, with full power to delegate, to act on its behalf and to take any and all actions contemplated in the Deposit Agreement and the applicable ADR(s), to adopt any and all procedures necessary to comply with applicable law and to take such action as the Depositary in its sole discretion may deem necessary or appropriate to carry out the purposes of the Deposit Agreement and the applicable ADR(s), the taking of such actions to be the conclusive determinant of the necessity and appropriateness thereof.  The manner in which a Beneficial Owner holds ADSs (e.g., in a brokerage account vs. as registered holder) may affect the rights and obligations of, the manner in which, and the extent to which, services are made available to, Beneficial Owners pursuant to the terms of the Deposit Agreement.

The statements made on the face and reverse of this ADR are summaries of certain provisions of the Deposit Agreement and the Articles of Association (as in effect on the date of the signing of the Deposit Agreement) and are qualified by and subject to the detailed provisions of the Deposit Agreement and the Articles of Association, to which reference is hereby made.

All capitalized terms not defined herein shall have the meanings ascribed thereto in the Deposit Agreement.

The Depositary makes no representation or warranty as to the validity or worth of the Deposited Property.  The Depositary has made arrangements for the acceptance of the ADSs into DTC.  Each Beneficial Owner of ADSs held through DTC must rely on the procedures of DTC and the DTC Participants to exercise and be entitled to any rights attributable to such ADSs.  The Depositary may issue Uncertificated ADSs subject, however, to the terms and conditions of Section 2.13 of the Deposit Agreement.

(2)          Surrender of ADSs and Withdrawal of Deposited Securities.  The Holder of this ADR (and of the ADSs evidenced hereby) shall be entitled to Delivery (at the Custodian’s designated office) of the Deposited Securities at the time represented by the ADSs evidenced hereby upon satisfaction of each of the following conditions: (i) the Holder (or a duly-authorized attorney of the Holder) has duly Delivered ADSs to the Depositary at its Principal Office the ADSs evidenced hereby (and, if applicable, this ADR evidencing such ADSs) for the purpose of withdrawal of the Deposited Securities represented thereby, (ii) if applicable and so required by the Depositary, this ADR Delivered to the Depositary for such purpose has been properly endorsed in blank or is accompanied by proper instruments of transfer in blank (including signature guarantees in accordance with standard securities industry practice), (iii) if so required by the Depositary, the Holder of the ADSs has executed and delivered to the Depositary a written order directing the Depositary to cause the Deposited Securities being withdrawn to be Delivered to or upon the written order of the person(s) designated in such order, and (iv) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 of, and Exhibit B to, the Deposit Agreement) have been paid,subject, however, in each case, to the terms and conditions of this ADR evidencing the surrendered ADSs, of the Deposit Agreement, of the Company’s Articles of Associationand of any applicable laws and the rules of CREST or any other applicable book-entry settlement system, if available, and to any provisions of or governing the Deposited Securities, in each case as in effect at the time thereof.

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Upon satisfaction of each of the conditions specified above, the Depositary (i) shall cancel the ADSs Delivered to it (and, if applicable, this ADR(s) evidencing the ADSs so Delivered), (ii) shall direct the Registrar to record the cancellation of the ADSs so Delivered on the books maintained for such purpose, and (iii) shall direct the Custodian to Deliver, or cause the Delivery of, in each case,  without unreasonable delay, the Deposited Securities represented by the ADSs so canceled together with any certificate or other document of title for the Deposited Securities, or evidence of the electronic transfer thereof (if available), as the case may be, to or upon the written order of the person(s) designated in the order delivered to the Depositary for such purpose, subject however, in each case, to the terms and conditions of the Deposit Agreement, of this ADR evidencing the ADS so canceled, of the Articles of Association, of any applicable laws and of the rules of CREST or any other applicable book-entry settlement system, if available, and to the terms and conditions of  or governing the Deposited Securities, in each case as in effect at the time thereof.

The Depositary shall not accept for surrender ADSs representing less than one (1) Share.  In the case of Delivery to it of ADSs representing a number other than a whole number of Shares, the Depositary shall cause ownership of the appropriate whole number of Shares to be Delivered in accordance with the terms hereof, and shall, at the discretion of the Depositary, either (i) return to the person surrendering such ADSs the number of ADSs representing any remaining fractional Share, or (ii) sell or cause to be sold the fractional Share represented by the ADSs so surrendered and remit the proceeds of such sale (net of (a) applicable fees and charges of, and expenses incurred by, the Depositary and (b) taxes withheld) to the person surrendering the ADSs.

Notwithstanding anything to the contrary contained in this ADR or the Deposit Agreement, the Depositary may make delivery at the Principal Office of the Depositary of Deposited Property consisting of (i) any cash dividends or cash distributions, or (ii) any proceeds from the sale of any non-cash distributions, which are at the time held by the Depositary in respect of the Deposited Securities represented by the ADSs surrendered for cancellation and withdrawal.  At the request, risk and expense of any Holder so surrendering ADSs represented by this ADR, and for the account of such Holder, the Depositary shall direct the Custodian to forward (to the extent permitted by law) any Deposited Property (other than Deposited Securities) held by the Custodian in respect of such ADSs to the Depositary for delivery at the Principal Office of the Depositary.  Such direction shall be given by letter or, at the request, risk and expense of such Holder, by cable, telex or facsimile transmission.

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(3)          Transfer, Combination and Split-up of ADRs.  The Registrar shall register the transfer of this ADR (and of the ADSs represented hereby) on the books maintained for such purpose and the Depositary shall (x) cancel this ADR and execute new ADRs evidencing the same aggregate number of ADSs as those evidenced by this ADR canceled by the Depositary, (y) cause the Registrar to countersign such new ADRs, and (z) Deliver such new ADRs to or upon the order of the person entitled thereto, if each of the following conditions has been satisfied:  (i) this ADR has been duly Delivered by the Holder (or by a duly authorized attorney of the Holder) to the Depositary at its Principal Office for the purpose of effecting a transfer thereof, (ii) this surrendered ADR has been properly endorsed or is accompanied by proper instruments of transfer (including signature guarantees in accordance with standard securities industry practice), (iii) this surrendered ADR has been duly stamped (if required by the laws of the State of New York or of the United States), and (iv) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 of, and Exhibit B to, the Deposit Agreement) have been paid, subject, however, in each case, to the terms and conditions of this ADR, of the Deposit Agreement and of applicable law, in each case as in effect at the time thereof.

The Registrar shall register the split-up or combination of this ADR (and of the ADSs represented hereby) on the books maintained for such purpose and the Depositary shall (x) cancel this ADR and execute new ADRs for the number of ADSs requested, but in the aggregate not exceeding the number of ADSs evidenced by this ADR canceled by the Depositary, (y) cause the Registrar to countersign such new ADRs, and (z) Deliver such new ADRs to or upon the order of the Holder thereof, if each of the following conditions has been satisfied:  (i) this ADR has been duly Delivered by the Holder (or by a duly authorized attorney of the Holder) to the Depositary at its Principal Office for the purpose of effecting a split‑up or combination hereof, and (ii) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 of, and Exhibit B to, the Deposit Agreement) have been paid, subject, however, in each case, to the terms and conditions of this ADR, of the Deposit Agreement and of applicable law, in each case as in effect at the time thereof.

(4)          Pre-Conditions to Registration, Transfer, Etc.  As a condition precedent to the execution and Delivery, the registration of issuance, transfer, split‑up, combination or surrender, of any ADS, the delivery of any distribution thereon, or the withdrawal of any Deposited Property, the Depositary or the Custodian may require (i) payment from the depositor of Shares or presenter of ADSs or of this ADR of a sum sufficient to reimburse it for any tax or other governmental charge and any stock transfer or registration fee with respect thereto (including any such tax or charge and fee with respect to Shares being deposited or withdrawn) and payment of any applicable fees and charges of the Depositary as provided in Section 5.9 and Exhibit B to the Deposit Agreement and in this ADR, (ii) the production of proof satisfactory to it as to the identity and genuineness of any signature or any other matter contemplated by Section 3.1 of the Deposit Agreement, and (iii) compliance with (A) any laws or governmental regulations relating to the execution and Delivery of this ADR or ADSs or to the withdrawal of Deposited Securities and (B) such reasonable regulations as the Depositary and the Company may establish consistent with the provisions of this ADR, if applicable, the Deposit Agreement and applicable law.

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The issuance of ADSs against deposits of Shares generally or against deposits of particular Shares may be suspended, or the deposit of particular Shares may be refused, or the registration of transfer of ADSs in particular instances may be refused, or the registration of transfer of ADSs generally may be suspended, during any period when the transfer books of the Company, the Depositary, a Registrar or the Share Registrar are closed or if any such action is deemed necessary or advisable by the Depositary (whereupon the Depositary shall notify the Company) or the Company, in good faith, at any time or from time to time because of any requirement of law or regulation, any government or governmental body or commission or any securities exchange on which the ADSs or Shares are listed, or under any provision of the Deposit Agreement or this ADR, if applicable, or under any provision of, or governing, the Deposited Securities, or because of a meeting of shareholders of the Company or for any other reason, subject, in all cases to Section 7.8 of the Deposit Agreement and paragraph (25) of this ADR.  Notwithstanding any provision of the Deposit Agreement or this ADR to the contrary, Holders are entitled to surrender outstanding ADSs to withdraw the Deposited Securities associated therewith at any time subject only to (i) temporary delays caused by closing the transfer books of the Depositary or the Company or the deposit of Shares in connection with voting at a shareholders’ meeting or the payment of dividends, (ii) the payment of fees, taxes and similar charges, (iii) compliance with any U.S. or foreign laws or governmental regulations relating to the ADSs or to the withdrawal of the Deposited Securities, and (iv) other circumstances specifically contemplated by Instruction I.A.(l) of the General Instructions to Form F-6 (as such General Instructions may be amended from time to time) under the Securities Act.

(5)          Compliance with Information Requests.  Notwithstanding any other provision of the Deposit Agreement or this ADR, each Holder and Beneficial Owner of the ADSs represented hereby agrees to comply with requests from the Company pursuant to applicable law, the rules and requirements of any stock exchange on which the Shares or ADSs are, or will be, registered, traded or listed, or the Articles of Association, which are made to provide information, inter alia, as to the capacity in which such Holder or Beneficial Owner owns ADSs (and the Shares represented by such ADSs, as the case may be) and regarding the identity of any other person(s) interested in such ADSs (and the Shares represented by such ADSs, as the case may be) and the nature of such interest and various other matters, whether or not they are Holders and/or Beneficial Owners at the time of such request.

(6)          Ownership Restrictions.  Notwithstanding any other provision contained in this ADR or of the Deposit Agreement to the contrary, the Company may restrict transfers of the Shares where such transfer might result in ownership of Shares exceeding limits imposed by applicable law or the Articles of Association.  The Company may also restrict, in such manner as it deems appropriate, transfers of the ADSs where such transfer may result in the total number of Shares represented by the ADSs owned by a single Holder or Beneficial Owner to exceed any such limits.  The Company may, in its sole discretion but subject to applicable law, instruct the Depositary to take action with respect to the ownership interest of any Holder or Beneficial Owner in excess of the limits set forth in the preceding sentence, including but not limited to, the imposition of restrictions on the transfer of ADSs, the removal or limitation of voting rights or the mandatory sale or disposition on behalf of a Holder or Beneficial Owner of the Shares represented by the ADSs held by such Holder or Beneficial Owner in excess of such limitations, if and to the extent such disposition is permitted by applicable law and the Articles of Association.  Nothing herein or in the Deposit Agreement shall be interpreted as obligating the Depositary or the Company to ensure compliance with the ownership restrictions described herein or in Section 3.5 of the Deposit Agreement.

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(7)          Reporting Obligations and Regulatory Approvals.  Applicable laws and regulations may require holders and beneficial owners of Shares, including the Holders and Beneficial Owners of ADSs, to satisfy reporting requirements and obtain regulatory approvals in certain circumstances.  Holders and Beneficial Owners of ADSs are solely responsible for determining and complying with such reporting requirements and obtaining such approvals.  Each Holder and each Beneficial Owner hereby agrees to make such determination, file such reports, and obtain such approvals to the extent and in the form required by applicable laws and regulations as in effect from time to time.  Neither the Depositary, the Custodian, the Company or any of their respective agents or affiliates shall be required to take any actions whatsoever on behalf of Holders or Beneficial Owners to determine or satisfy such reporting requirements or obtain such regulatory approvals under applicable laws and regulations.

(8)          Liability for Taxes and Other Charges.  Any tax or other governmental charge payable by the Custodian or by the Depositary with respect to any Deposited Property, ADSs or this ADR shall be payable by the Holders and Beneficial Owners to the Depositary.  The Company, the Custodian and/or the Depositary may withhold or deduct from any distributions made in respect of Deposited Property held on behalf of such Holder and/or Beneficial Owner, and may sell for the account of a Holder and/or Beneficial Owner any or all of such Deposited Property and apply such distributions and sale proceeds in payment of, any taxes (including applicable interest and penalties) or charges that are or may be payable by Holders or Beneficial Owners in respect of the ADSs, Deposited Property and this ADR, the Holder and the Beneficial Owner hereof remaining liable for any deficiency.  The Custodian may refuse the deposit of Shares and the Depositary may refuse to issue ADSs, to deliver ADRs, register the transfer of ADSs, register the split-up or combination of ADRs and (subject to paragraph (25) of this ADR and Section 7.8 of the Deposit Agreement) the withdrawal of Deposited Property until payment in full of such tax, charge, penalty or interest is received.  Every Holder and Beneficial Owner agrees to indemnify the Depositary, the Company, the Custodian, and any of their respective agents, officers, employees and Affiliates for, and to hold each of them harmless from, any claims (including, without limitation, by any governmental authority or other person or entity) with respect to taxes (including applicable interest and penalties thereon) arising from any tax benefit obtained for such Holder and/or Beneficial Owner.  Notwithstanding anything to the contrary contained in the Deposit Agreement or this ADR, the obligations of Holders and Beneficial Owners under Section 3.2 of the Deposit Agreement shall survive any transfer of ADSs, any cancellation of ADSs and withdrawal of Deposited Securities, and the termination of the Deposit Agreement.

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(9)          Representations and Warranties on Deposit of Shares.  Each person depositing Shares under the Deposit Agreement shall be deemed thereby to represent and warrant that (i) such Shares and the certificates therefor are duly authorized, validly allotted and issued, fully paid, not subject to any call for the payment of further capital and legally obtained by such person, (ii) all preemptive (and similar) rights, if any, with respect to such Shares have been validly waived, disapplied or exercised, (iii) the person making such deposit is duly authorized so to do, (iv) the Shares presented for deposit are free and clear of any lien, encumbrance, security interest, charge, mortgage or adverse claim, (v) the Shares presented for deposit are not, and the ADSs issuable upon such deposit will not be, Restricted Securities (except as contemplated in Section 2.14 of the Deposit Agreement), (vi) the Shares presented for deposit have not been stripped of any rights or entitlements and (vii) the deposit of the Shares does not violate any applicable provisions of English law.  Such representations and warranties shall survive the deposit and withdrawal of Shares, the issuance and cancellation of ADSs in respect thereof and the transfer of such ADSs.  If any such representations or warranties are false in any way, the Company and the Depositary shall be authorized, at the cost and expense of the person depositing Shares, to take any and all actions necessary to correct the consequences thereof.

(10)        Proofs, Certificates and Other Information.  Any person presenting Shares for deposit, any Holder and any Beneficial Owner may be required, and every Holder and Beneficial Owner agrees, from time to time to provide to the Company, the Depositary and the Custodian such proof of citizenship or residence, taxpayer status, payment of all applicable taxes or other governmental charges, exchange control approval, legal or beneficial ownership of ADSs and Deposited Property, compliance with applicable laws, the terms of the Deposit Agreement or this ADR evidencing the ADSs and the provisions of, or governing, the Deposited Property, to execute such certifications and to make such representations and warranties, and to provide such other information and documentation (or, in the case of Shares in registered form presented for deposit, such information relating to the registration on the books of the Company or of the Share Registrar) as the Depositary or the Custodian may deem necessary or proper or as the Company may reasonably require by written request to the Depositary consistent with its obligations under the Deposit Agreement and this ADR.  The Depositary and the Registrar, as applicable, and at the reasonable request of the Company, shall, to the extent practicable, may withhold the execution or delivery or registration of transfer of any ADR or ADS or the distribution or sale of any dividend or distribution of rights or of the proceeds thereof or, to the extent not limited by paragraph (25) and Section 7.8 of the Deposit Agreement, the delivery of any Deposited Property until such proof or other information is filed or such certifications are executed, or such representations and warranties are made or such other documentation or information is provided, in each case to the Depositary’s, the Registrar’s and the Company’s satisfaction.

(11)        ADS Fees and Charges.  The following ADS fees are payable under the terms of the Deposit Agreement:

(i) ADS Issuance Fee:  by any person for whom ADSs are issued (e.g., an issuance upon a deposit of Shares, upon a change in the ADS(s)-to-Share(s) ratio, or for any other reason), excluding issuances as a result of distributions described in paragraph (iv) below, a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) issued under the terms of the Deposit Agreement;

(ii) ADS Cancellation Fee:  by any person for whom ADSs are being cancelled (e.g., a cancellation of ADSs for Delivery of deposited Shares, upon a change in the ADS(s)-to-Share(s) ratio, or for any other reason), a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) cancelled;

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(iii) Cash Distribution Fee:  by any Holder of ADSs, a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) held for the distribution of cash dividends or other cash distributions (e.g., upon a sale of rights and other entitlements);

(iv) Stock Distribution /Rights Exercise Fee:  by any Holder of ADS(s), a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) held for the distribution of ADSs pursuant to (a) stock dividends or other free stock distributions, or (b) an exercise of rights to purchase additional ADSs;

(v) Other Distribution Fee:  by any Holder of ADS(s), a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) held for the distribution of securities other than ADSs or rights to purchase additional ADSs (e.g., spin-off shares);

(vi) Depositary Services Fee:  by any Holder of ADS(s), a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) held on the applicable record date(s) established by the Depositary;

(vii) Registration of ADS Transfer Fee:  by any Holder of ADS(s) being transferred or by any person to whom ADSs are transferred, a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) transferred (e.g., upon a registration of the transfer of registered ownership of ADSs, upon a transfer of ADSs into DTC and vice versa, or for any other reason); and

(viii) ADS Conversion Fee:  by any Holder of ADS(s) being converted or by any person to whom the converted ADSs are delivered, a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) converted from one ADS series to another ADS series (e.g., upon conversion of Partial Entitlement ADSs for Full Entitlement ADSs, or upon conversion of Restricted ADSs into freely transferrable ADSs, and vice versa).

The Company, Holders, Beneficial Owners, persons depositing Shares or withdrawing Deposited Securities in connection with ADS issuances and cancellations, and persons for whom ADSs are issued or cancelled shall be responsible for the following ADS charges under the terms of the Deposit Agreement:

(a) taxes (including applicable interest and penalties) and other governmental charges;

(b) such registration fees as may from time to time be in effect for the registration of Shares or other Deposited Securities on the share register and applicable to transfers of Shares or other Deposited Securities to or from the name of the Custodian, the Depositary or any nominees upon the making of deposits and withdrawals, respectively;

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(c) such cable, telex and facsimile transmission and delivery expenses as are expressly provided in the Deposit Agreement to be at the expense of the person depositing Shares or withdrawing Deposited Securities or of the Holders and Beneficial Owners of ADSs;

(d) in connection with the conversion of Foreign Currency, the fees, expenses, spreads, taxes and other charges of the Depositary and/or conversion service providers (which may be a division, branch or Affiliate of the Depositary).  Such fees, expenses, spreads, taxes and other charges shall be deducted from the Foreign Currency;

(e) any reasonable and customary out-of-pocket expenses incurred in such conversion and/or on behalf of the Holders and Beneficial Owners in complying with currency exchange control or other governmental requirements; and

(f) the fees, charges, costs and expenses incurred by the Depositary, the Custodian, or any nominee in connection with the ADR program.

All ADS fees and charges so payable may be deducted from distributions or must be remitted to the Depositary, or its designee, and may, at any time and from time to time, be changed by agreement between the Depositary and Company but, in the case of ADS fees and charges payable by Holders and Beneficial Owners, only in the manner contemplated by paragraph (23) of this ADR and as contemplated in Section 6.1 of the Deposit Agreement.  The Depositary shall provide, without charge, a copy of its latest ADS fee schedule to anyone upon request.

ADS fees and charges for (i) the issuance of ADSs and (ii) the cancellation of ADSs will be payable by the person for whom the ADSs are so issued by the Depositary (in the case of ADS issuances) and by the person for whom ADSs are being cancelled (in the case of ADS cancellations).  In the case of ADSs issued by the Depositary into DTC or presented to the Depositary via DTC, the ADS issuance and cancellation fees and charges will be payable by the DTC Participant(s) receiving the ADSs from the Depositary or the DTC Participant(s) holding the ADSs being cancelled, as the case may be, on behalf of the Beneficial Owner(s) and will be charged by the DTC Participant(s) to the account(s) of the applicable Beneficial Owner(s) in accordance with the procedures and practices of the DTC Participant(s) as in effect at the time.  ADS fees and charges in respect of distributions and the ADS service fee are payable by Holders as of the applicable ADS Record Date established by the Depositary.  In the case of distributions of cash, the amount of the applicable ADS fees and charges is deducted from the funds being distributed.  In the case of (i) distributions other than cash and (ii) the ADS service fee, the applicable Holders as of the ADS Record Date established by the Depositary will be invoiced for the amount of the ADS fees and charges and such ADS fees may be deducted from distributions made to Holders.  For ADSs held through DTC, the ADS fees and charges for distributions other than cash and the ADS service fee may be deducted from distributions made through DTC, and may be charged to the DTC Participants in accordance with the procedures and practices prescribed by DTC from time to time and the DTC Participants in turn charge the amount of such ADS fees and charges to the Beneficial Owners for whom they hold ADSs.  In the case of (i) registration of ADS transfers, the ADS transfer fee will be payable by the ADS Holder whose ADSs are being transferred or by the person to whom the ADSs are transferred, and (ii) conversion of ADSs of one series for ADSs of another series, the ADS conversion fee will be payable by the Holder whose ADSs are converted or by the person to whom the converted ADSs are delivered.

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The Depositary may reimburse the Company for certain expenses incurred by the Company in respect of the ADR program established pursuant to the Deposit Agreement, by making available a portion of the ADS fees charged in respect of the ADR program or otherwise, upon such terms and conditions as the Company and the Depositary agree from time to time.  The Company shall pay to the Depositary such fees and charges, and reimburse the Depositary for such out-of-pocket expenses, as the Depositary and the Company may agree from time to time.  Responsibility for payment of such fees, charges and reimbursements may from time to time be changed by agreement between the Company and the Depositary.  Unless otherwise agreed, the Depositary shall present its statement for such fees, charges and reimbursements to the Company once every three months.  The charges and expenses of the Custodian are for the sole account of the Depositary.

The obligation of Holders and Beneficial Owners to pay ADS fees and charges shall survive the termination of the Deposit Agreement.  As to any Depositary, upon the resignation or removal of such Depositary as described in Section 5.4 of the Deposit Agreement, the right to collect ADS fees and charges shall extend for those ADS fees and charges incurred prior to the effectiveness of such resignation or removal.

(12)         Title to ADRs.  Subject to the limitations contained in the Deposit Agreement and in this ADR, it is a condition of this ADR, and every successive Holder of this ADR by accepting or holding the same consents and agrees, that title to this ADR (and to each Certificated ADS evidenced hereby) shall be transferable upon the same terms as a certificated security under the laws of the State of New York, provided that, in the case of Certificated ADSs, this ADR has been properly endorsed or is accompanied by proper instruments of transfer.  Notwithstanding any notice to the contrary, the Depositary and the Company may deem and treat the Holder of this ADR (that is, the person in whose name this ADR is registered on the books of the Depositary) as the absolute owner thereof for all purposes.  Neither the Depositary nor the Company shall have any obligation nor be subject to any liability under the Deposit Agreement or this ADR to any holder of this ADR or any Beneficial Owner unless, in the case of a holder of ADSs, such holder is the Holder of this ADR registered on the books of the Depositary or, in the case of a Beneficial Owner, such Beneficial Owner, or the Beneficial Owner’s representative, is the Holder registered on the books of the Depositary.

(13)         Validity of ADR.  The Holder(s) of this ADR (and the ADSs represented hereby) shall not be entitled to any benefits under the Deposit Agreement or be valid or enforceable for any purpose against the Depositary or the Company unless this ADR has been (i) dated, (ii) signed by the manual or facsimile signature of a duly-authorized signatory of the Depositary, (iii) countersigned by the manual or facsimile signature of a duly-authorized signatory of the Registrar, and (iv) registered in the books maintained by the Registrar for the registration of issuances and transfers of ADRs.  An ADR bearing the facsimile signature of a duly-authorized signatory of the Depositary or the Registrar, who at the time of signature was a duly authorized signatory of the Depositary or the Registrar, as the case may be, shall bind the Depositary, notwithstanding the fact that such signatory has ceased to be so authorized prior to the delivery of such ADR by the Depositary.

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(14)         Available Information; Reports; Inspection of Transfer Books.

The Company publishes the information contemplated in Rule 12g3-2(b)(2)(i) under the Exchange Act on its internet website or through an electronic information delivery system generally available to the public in the Company’s primary trading market.  As of the date hereof the Company’s internet website is www.amrytpharma.com.  The information so published by the Company may not be in English, except that the Company is required, in order to maintain its exemption from the Exchange Act reporting obligations pursuant to Rule 12g3-2(b), to translate such information into English to the extent contemplated in the instructions to Rule 12g3-2(b).  The information so published by the Company cannot be retrieved from the Commission’s internet website, and cannot be inspected or copied at the public reference facilities maintained by the Commission located (as of the date of the Deposit Agreement) at 100 F Street, N.E., Washington, D.C. 20549.

The Registrar shall keep books for the registration of ADSs which at all reasonable times shall be open for inspection by the Company and by the Holders of such ADSs, provided that such inspection shall not be, to the Registrar’s knowledge, for the purpose of communicating with Holders of such ADSs in the interest of a business or object other than the business of the Company or other than a matter related to the Deposit Agreement or the ADSs.

The Registrar may close the transfer books with respect to the ADSs, at any time or from time to time, when deemed necessary or advisable by it in good faith in connection with the performance of its duties hereunder, or at the reasonable written request of the Company subject, in all cases, to paragraph (25) and Section 7.8 of the Deposit Agreement.

Dated:

CITIBANK, N.A. Transfer Agent and Registrar		CITIBANK, N.A. as Depositary
By:		By:
	Authorized Signatory		Authorized Signatory

The address of the Principal Office of the Depositary is 388 Greenwich Street, New York, New York 10013, U.S.A.

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[FORM OF REVERSE OF ADR]

SUMMARY OF CERTAIN ADDITIONAL PROVISIONS

OF THE DEPOSIT AGREEMENT

(15)         Dividends and Distributions in Cash, Shares, etc.  (a) Cash Distributions: Upon the timely receipt by the Depositary of a notice from the Company that it intends to make a distribution of a cash dividend or other cash distribution, the Depositary shall establish the ADS Record Date upon the terms described in Section 4.9 of the Deposit Agreement.  Upon receipt of confirmation from the Custodian of receipt of (x) any cash dividend or other cash distribution on any Deposited Securities, or (y) proceeds from the sale of any Deposited Property held in respect of the ADSs under the terms of the Deposit Agreement, the Depositary will (i) if any amounts are received in a Foreign Currency, promptly convert or cause to be converted such cash dividend, distribution or proceeds into Dollars (subject to the terms and on the conditions of Section 4.8 of the Deposit Agreement), (ii) if applicable and unless previously established, establish the ADS Record Date upon the terms described in Section 4.9 of the Deposit Agreement, and (iii) distribute promptly the amount thus received from such conversion (net of (a) the applicable fees and charges described in the Fee Schedule attached as Exhibit B to the Deposit Agreement and (b) applicable taxes withheld) to the Holders entitled thereto as of the ADS Record Date in proportion to the number of ADSs held as of the ADS Record Date.  The Depositary shall distribute only such amount, however, as can be distributed without attributing to any Holder a fraction of one cent, and any balance not so distributed shall be held by the Depositary (without liability for interest thereon) and shall be added to and become part of the next sum received by the Depositary for distribution to Holders of ADSs outstanding at the time of the next distribution.  If the Company, the Custodian or the Depositary is required to withhold and does withhold from any cash dividend or other cash distribution in respect of any Deposited Securities, or from any cash proceeds from the sales of Deposited Property, an amount on account of taxes, duties or other governmental charges, the amount distributed to Holders on the ADSs shall be reduced accordingly.  Such withheld amounts shall be forwarded by the Company, the Custodian or the Depositary to the relevant governmental authority.  Evidence of payment thereof by the Company shall be forwarded by the Company to the Depositary upon request.  The Depositary will hold any cash amounts it is unable to distribute in a non-interest bearing account for the benefit of the applicable Holders and Beneficial Owners of ADSs until the distribution can be effected or the funds that the Depositary holds must be escheated as unclaimed property in accordance with the laws of the relevant states of the United States. Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for above, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in Section 4.1 of the Deposit Agreement, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in Section 4.1 of the Deposit Agreement where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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(b)  Share Distributions: Upon the timely receipt by the Depositary of a notice from the Company that it intends to make a distribution that consists of a dividend in, or free distribution of Shares, the Depositary shall establish the ADS Record Date upon the terms described in Section 4.9 of the Deposit Agreement.  Upon receipt of confirmation from the Custodian of the receipt of the Shares so distributed by the Company, the Depositary shall either (i) subject to Section 5.9 of the Deposit Agreement, distribute to the Holders as of the ADS Record Date in proportion to the number of ADSs held as of the ADS Record Date, additional ADSs, which represent in the aggregate the number of Shares received as such dividend, or free distribution, subject to the other terms of the Deposit Agreement (including, without limitation, (a) the applicable fees and charges of, and expenses incurred by, the Depositary and (b) taxes), or (ii) if additional ADSs are not so distributed, take all actions necessary so that each ADS issued and outstanding after the ADS Record Date shall, to the extent permissible by law, thenceforth also represent rights and interests in the additional integral number of Shares distributed upon the Deposited Securities represented thereby (net of (a) the applicable fees and charges of, and expenses incurred by, the Depositary, and (b) taxes).  In lieu of delivering fractional ADSs, the Depositary shall sell the number of Shares or ADSs, as the case may be, represented by the aggregate of such fractions and distribute the net proceeds upon the terms described in Section 4.1 of the Deposit Agreement.

In the event that the Depositary determines that any distribution in property (including Shares) is subject to any tax or other governmental charges which the Depositary is obligated to withhold, or, if the Company in the fulfillment of its obligations under Section 5.7 of the Deposit Agreement, has furnished an opinion of U.S. counsel determining that Shares must be registered under the Securities Act or other laws in order to be distributed to Holders (and no such registration statement has been declared effective), the Depositary may dispose of all or a portion of such property (including Shares and rights to subscribe therefor) in such amounts and in such manner, including by public or private sale, as the Depositary deems necessary and practicable, and the Depositary shall distribute the net proceeds of any such sale (after deduction of (a) taxes and (b) fees and charges of, and the expenses incurred by, the Depositary) to Holders entitled thereto upon the terms of Section 4.1 of the Deposit Agreement.  The Depositary shall hold and/or distribute any unsold balance of such property in accordance with the provisions of the Deposit Agreement. Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for above, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in Section 4.2 of the Deposit Agreement, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in Section 4.2 of the Deposit Agreement where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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(c)  Elective Distributions in Cash or Shares: Upon the timely receipt of a notice indicating that the Company wishes an elective distribution in cash or Shares to be made available to Holders of ADSs upon the terms described in the Deposit Agreement, the Company and the Depositary shall determine in accordance with the Deposit Agreement whether such distribution is lawful and reasonably practicable.  The Depositary shall make such elective distribution available to Holders only if (i) the Company shall have timely requested that the elective distribution be made available to Holders, (ii) the Depositary shall have determined, upon consultation with the Company, that such distribution is reasonably practicable and (iii) the Depositary shall have received satisfactory documentation within the terms of Section 5.7 of the Deposit Agreement. If the above conditions are satisfied, the Depositary shall, subject to the terms and conditions of the Deposit Agreement, establish an ADS Record Date according to paragraph (17) and Section 4.9 of the Deposit Agreement and establish procedures to enable the Holder hereof to elect to receive the proposed distribution in cash or in additional ADSs.  If a Holder elects to receive the distribution in cash, the distribution shall be made as in the case of a distribution in cash.  If the Holder hereof elects to receive the distribution in additional ADSs, the distribution shall be made as in the case of a distribution in Shares upon the terms described in the Deposit Agreement.  If such elective distribution is not reasonably practicable or if the Depositary did not receive satisfactory documentation set forth in the Deposit Agreement, the Depositary shall establish an ADS Record Date upon the terms of Section 4.9 of the Deposit Agreement and, to the extent permitted by law, distribute to Holders, on the basis of the same determination as is made in England and Wales in respect of the Shares for which no election is made, either (x) cash, upon the terms described in Section 4.1 of the Deposit Agreement or (y) additional ADSs representing such additional Shares, in each case, upon the terms described in Section 4.2 of the Deposit Agreement.  Nothing herein or in the Deposit Agreement shall obligate the Depositary to make available to the Holder hereof a method to receive the elective distribution in Shares (rather than ADSs).  There can be no assurance that the Holder hereof will be given the opportunity to receive elective distributions on the same terms and conditions as the holders of Shares. Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for above, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in Section 4.3 of the Deposit Agreement, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in Section 4.3 of the Deposit Agreement where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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(d)  Distribution of Rights to Purchase Additional ADSs: Upon the timely receipt by the Depositary of a notice indicating that the Company wishes rights to subscribe for additional Shares to be made available to Holders of ADSs, the Depositary upon consultation with the Company, shall determine, whether it is lawful and reasonably practicable to make such rights available to the Holders.  The Depositary shall make such rights available to any Holders only if (i) the Company shall have timely requested that such rights be made available to Holders, (ii) the Depositary shall have received satisfactory documentation within the terms of Section 5.7 of the Deposit Agreement, and (iii) the Depositary shall have determined that such distribution of rights is reasonably practicable.  If such conditions are not satisfied or if the Company requests that the rights not be made available to Holders of ADSs, the Depositary shall sell the rights as described below.  In the event all conditions set forth above are satisfied, the Depositary shall establish an ADS Record Date (upon the terms described in Section 4.9 of the Deposit Agreement) and establish procedures to (x) distribute rights to purchase additional ADSs (by means of warrants or otherwise), (y) enable the Holders to exercise such rights (upon payment of the subscription price and of the applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes), and (z) deliver ADSs upon the valid exercise of such rights.  Nothing herein or in the Deposit Agreement shall obligate the Depositary to make available to the Holders a method to exercise rights to subscribe for Shares (rather than ADSs).  If (i) the Company does not timely request the Depositary to make the rights available to Holders or requests that the rights not be made available to Holders, (ii) the Depositary fails to receive satisfactory documentation within the terms of Section 5,7 of the Deposit Agreement or determines, upon consultation with the Company, it is not reasonably practicable to make the rights available to Holders, or (iii) any rights made available are not exercised and appear to be about to lapse, the Depositary shall, upon consultation with the Company, determine whether it is lawful and reasonably practicable to sell such rights, in a riskless principal capacity, at such place and upon such terms (including public and private sale) as it may deem practicable.  The Depositary shall, upon such sale, convert and distribute proceeds of such sale (net of applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) upon the terms hereof and of Section 4.1 of the Deposit Agreement.  If the Depositary is unable to make any rights available to Holders upon the terms described in Section 4.4(a) of the Deposit Agreement or to arrange for the sale of the rights upon the terms described in Section 4.4(b) of the Deposit Agreement, the Depositary shall allow such rights to lapse.  The Depositary shall not be liable for (i) any failure to accurately determine whether it may be lawful or practicable to make such rights available to Holders in general or any Holders in particular, (ii) any foreign exchange exposure or loss incurred in connection with such sale or exercise, or (iii) the content of any materials forwarded to the Holders on behalf of the Company in connection with the rights distribution.

Notwithstanding anything herein or in Section 4.4 of the Deposit Agreement to the contrary, if registration (under the Securities Act or any other applicable law) of the rights or the securities to which any rights relate may be required in order for the Company to offer such rights or such securities to Holders and to sell the securities represented by such rights, the Depositary will not distribute such rights to the Holders (i) unless and until a registration statement under the Securities Act (or other applicable law) covering such offering is in effect or (ii) unless the Company furnishes the Depositary opinion(s) of counsel for the Company in the United States and counsel to the Company in any other applicable country in which rights would be distributed, in each case reasonably satisfactory to the Depositary, to the effect that the offering and sale of such securities to Holders and Beneficial Owners are exempt from, or do not require registration under, the provisions of the Securities Act or any other applicable laws.  In the event that the Company, the Depositary or the Custodian shall be required to withhold and does withhold from any distribution of Deposited Property (including rights) an amount on account of taxes or other governmental charges, the amount distributed to the Holders of ADSs representing such Deposited Property shall be reduced accordingly.  In the event that the Depositary determines that any distribution of Deposited Property (including Shares and rights to subscribe therefor) is subject to any tax or other governmental charges which the Depositary is obligated to withhold, the Depositary may dispose of all or a portion of such Deposited Property (including Shares and rights to subscribe therefor) in such amounts and in such manner, including by public or private sale, as the Depositary deems necessary and practicable to pay any such taxes or charges.

There can be no assurance that Holders generally, or any Holder in particular, will be given the opportunity to receive or exercise rights on the same terms and conditions as the holders of Shares or be able to exercise such rights.  Nothing herein or in the Deposit Agreement shall obligate the Company to file any registration statement in respect of any rights or Shares or other securities to be acquired upon the exercise of such rights.

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(e)  Distributions other than Cash, Shares or Rights to Purchase Shares:  Upon receipt of a notice indicating that the Company wishes property other than cash, Shares or rights to purchase additional Shares to be made to Holders of ADSs, the Depositary shall determine whether such distribution to Holders is lawful and reasonably practicable.  The Depositary shall not make such distribution unless (i) the Company shall have requested the Depositary to make such distribution to Holders, (ii) the Depositary shall have received satisfactory documentation contemplated in Section 5.7 of the Deposit Agreement, and (iii) the Depositary shall have determined, after consultation with the Company, that such distribution is reasonably practicable.  Upon satisfaction of such conditions, the Depositary shall distribute the property so received to the Holders of record, as of the ADS Record Date, in proportion to the number of ADSs held by them respectively and in such manner as the Depositary may deem practicable for accomplishing such distribution (i) upon receipt of payment or net of the applicable fees and charges of, and expenses incurred by, the Depositary, and (ii) net of any taxes withheld.  The Depositary may dispose of all or a portion of the property so distributed and deposited, in such amounts and in such manner (including public or private sale) as the Depositary may deem practicable or necessary to satisfy any taxes (including applicable interest and penalties) or other governmental charges applicable to the distribution.

If the conditions above are not satisfied, the Depositary shall sell or cause such property to be sold in a public or private sale, at such place or places and upon such terms as it may deem practicable and shall (i) cause the proceeds of such sale, if any, to be converted into Dollars and (ii) distribute the proceeds of such conversion received by the Depositary (net of applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) to the Holders as of the ADS Record Date upon the terms hereof and of Section 4.1 of the Deposit Agreement.  If the Depositary is unable to sell such property, the Depositary may dispose of such property for the account of the Holders in any way it deems reasonably practicable under the circumstances.

Neither the Depositary nor the Company shall be responsible for (i) any failure to determine whether it is lawful or practicable to make the property described in Section 4.5 of the Deposit Agreement available to Holders in general or any Holders in particular, nor (ii) any loss incurred in connection with the sale or disposal of such property.

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(16)         Redemption.  Upon timely receipt of notice from the Company that it intends to exercise its right of redemption in respect of any of the Deposited Securities, and satisfactory documentation, and only if, after consultation with the Company, the Depositary shall have determined that such proposed redemption is practicable, the Depositary shall (to the extent practicable) provide to each Holder a notice setting forth the Company’s intention to exercise the redemption rights and any other particulars set forth in the Company’s notice to the Depositary.  The Depositary shall instruct the Custodian to present to the Company the Deposited Securities in respect of which redemption rights are being exercised against payment of the applicable redemption price. Upon receipt of confirmation from the Custodian that the redemption has taken place and that funds representing the redemption price have been received, the Depositary shall convert, transfer, and distribute the proceeds (net of applicable (a) fees and charges of, and the expenses incurred by, the Depositary, and (b) taxes), retire ADSs and cancel ADRs, if applicable, upon delivery of such ADSs by Holders thereof and the terms set forth in Sections 4.1 and 6.2 of the Deposit Agreement.  If less than all outstanding Deposited Securities are redeemed, the ADSs to be retired will be selected by lot or on a pro rata basis, as may be determined by the Depositary after consultation with the Company.  The redemption price per ADS shall be the dollar equivalent of the per share amount received by the Depositary (adjusted to reflect the ratio of ADS(s) to Share(s)) upon the redemption of the Deposited Securities represented by ADSs (subject to the terms of Section 4.8 of the Deposit Agreement and the applicable fees and charges of, and expenses incurred by, the Depositary, and taxes) multiplied by the number of Deposited Securities represented by each ADS redeemed. Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed redemption provided for above, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in Section 4.7 of the Deposit Agreement, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in Section 4.7 of the Deposit Agreement where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

(17)         Fixing of ADS Record Date.  Whenever the Depositary shall receive notice of the fixing of a record date by the Company for the determination of holders of Deposited Securities entitled to receive any distribution (whether in cash, Shares, rights or other distribution), or whenever for any reason the Depositary causes a change in the number of Shares that are represented by each ADS, or whenever the Depositary shall receive notice of any meeting of, or solicitation of consents or proxies of, holders of Shares or other Deposited Securities, or whenever the Depositary shall find it necessary or convenient in connection with the giving of any notice, solicitation of any consent or any other matter, the Depositary shall fix the record date (the “ADS Record Date”) for the determination of the Holders of ADS(s) who shall be entitled to receive such distribution, to give instructions for the exercise of voting rights at any such meeting, to give or withhold such consent, to receive such notice or solicitation or to otherwise take action, or to exercise the rights of Holders with respect to such changed number of Shares represented by each ADS.  Subject to applicable law, the terms and conditions of this ADR and Sections 4.1 through 4.8 of the Deposit Agreement, only the Holders of ADSs at the close of business in New York on such ADS Record Date shall be entitled to receive such distribution, to give such voting instructions, to receive such notice or solicitation, or otherwise take action.

(18)         Voting of Deposited Securities.  As soon as practicable after receipt of notice of any meeting at which the holders of Deposited Securities are entitled to vote, or of solicitation of consents or proxies from holders of Deposited Securities, the Depositary shall fix the ADS Record Date in respect of such meeting or solicitation of consent or proxy in accordance with Section 4.9 of the Deposit Agreement.  The Depositary shall, if requested by the Company in writing in a timely manner (except as otherwise contemplated in Section 4.10 of the Deposit Agreement, the Depositary having no obligation to take any further action if the request shall not have been received by the Depositary at least thirty (30) days prior to the date of such vote or meeting), at the Company’s expense and provided no U.S. legal prohibitions exist, distribute as soon as practicable after receipt thereof to Holders as of the ADS Record Date: (a) such notice of meeting or solicitation of consent or proxy, (b) a statement that the Holders at the close of business on the ADS Record Date will be entitled, subject to any applicable law, the provisions of the Deposit Agreement, the Articles of Association and the provisions of or governing the Deposited Securities (which provisions, if any, shall be summarized in pertinent part by the Company), to instruct the Depositary as to the exercise of the voting rights, if any, pertaining to the Deposited Securities represented by such Holder’s ADSs, and (c) a brief statement as to the manner in which such voting instructions may be given or deemed to have been given in accordance with Section 4.10 of the Deposit Agreement, it being acknowledged that if no instructions are received prior to the deadline set for such purposes to the Depositary to give a discretionary proxy to a person designated by the Company.

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The Company shall use its reasonable endeavors to notify the Depositary in writing of its intention to convene a meeting where the holders of Deposited Securities are entitled to vote, at least thirty (30) days prior to the date of the proposed meeting.

Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to timely request that the Depositary distribute the information as provided for in this Section 4.10 of the Deposit Agreement, if the Depositary so agrees after consultation with the Company, the Depositary will use commercially reasonable efforts to perform the actions contemplated in this Section 4.10 of the Deposit Agreement, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.10 of the Deposit Agreement, where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

Notwithstanding anything contained in the Deposit Agreement or any ADR, the Depositary may, to the extent not prohibited by law or regulations, or by the requirements of the stock exchange on which the ADSs are listed, in lieu of distribution of the materials provided to the Depositary in connection with any meeting of, or solicitation of consents or proxies from, holders of Deposited Securities, distribute to the Holders a notice that provides Holders with, or otherwise publicizes to Holders, instructions on how to retrieve such materials or receive such materials upon request (e.g., by reference to a website containing the materials for retrieval or a contact for requesting copies of the materials).

The Depositary has been advised by the Company that under the Articles of Association as in effect on the date of the Deposit Agreement, voting at any meeting of shareholders of the Company is by show of hands unless (before or on the declaration of the result of the show of hands) a poll is demanded.  The Depositary will not join in demanding a poll, whether or not requested to do so by Holders of ADSs.  Under the Articles of Association as in effect on the date of the Deposit Agreement, at any meeting a resolution put to the vote of the meeting shall be decided on a show of hands, unless before or upon the declaration of the result of the show of hands a poll is demanded by: (a) the chairman of the meeting; (b) not less than five (5) members of the Company having the right to vote at the meeting; (c) a member or members of the Company representing not less than one tenth of the total voting rights of all the members of the Company having the right to vote at the meeting; or (d) a member or members of the Company holding shares in the Company conferring a right to vote at the meeting, being shares on which an aggregate sum has been paid up equal to not less than one tenth of the total sum paid up on all the shares conferring that right.

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Voting instructions may be given only in respect of a number of ADSs representing an integral number of Deposited Securities.  Upon the timely receipt from a Holder of ADSs as of the ADS Record Date of voting instructions in the manner specified by the Depositary, the Depositary shall endeavor, insofar as practicable and permitted under applicable law, the provisions of the Deposit Agreement, Articles of Association and the provisions of the Deposited Securities, to vote, or cause the Custodian to vote, the Deposited Securities (in person or by proxy) represented by such Holder’s ADSs as follows: (a) in the event voting takes place at a shareholders’ meeting by a show of hands, the Depositary will instruct the Custodian to vote all Deposited Securities in accordance with the voting instructions received timely from a majority of Holders of ADSs who provided voting instructions, and (b) in the event voting takes place at a shareholders’ meeting by poll, the Depositary will instruct the Custodian to vote the Deposited Securities in accordance with the voting instructions timely received from the Holders of ADSs.  If voting is by poll and the Depositary does not receive voting instructions from a Holder as of the ADS Record Date on or before the date established by the Depositary for such purpose, such Holder shall be deemed, and the Depositary shall (unless otherwise specified in the notice distributed to Holders) deem such Holder, to have instructed the Depositary to give a discretionary proxy to a person designated by the Company to vote the Deposited Securities; provided, however, that no such discretionary proxy shall be given by the Depositary with respect to any matter to be voted upon as to which the Company informs the Depositary that (a) the Company does not wish such proxy to be given, (b) substantial opposition exists, or (c) the rights of holders of Deposited Securities may be adversely affected.

Deposited Securities represented by ADSs for which no timely voting instructions are received by the Depositary from the Holder shall not be voted (except (a) in the case voting is by show of hands, in which case the Depositary will instruct the Custodian to vote all Deposited Securities in accordance with the voting instructions received from a majority of Holders of ADSs who provided timely voting instructions, and (b) as contemplated in this Section 4.10).  Neither the Depositary nor the Custodian shall under any circumstances exercise any discretion as to voting and neither the Depositary nor the Custodian shall vote, attempt to exercise the right to vote, or in any way make use of, for purposes of establishing a quorum or otherwise, the Deposited Securities represented by ADSs, except pursuant to and in accordance with the voting instructions timely received from Holders or as otherwise contemplated herein.  If the Depositary timely receives voting instructions from a Holder which fail to specify the manner in which the Depositary is to vote the Deposited Securities represented by such Holder’s ADSs, the Depositary will deem such Holder (unless otherwise specified in the notice distributed to Holders) to have instructed the Depositary to vote in favor of the items set forth in such voting instructions.

Notwithstanding anything to the contrary contained herein, the Depositary shall, if so requested in writing by the Company, represent all Deposited Securities (whether or not voting instructions have been received in respect of such Deposited Securities from Holders as of the ADS Record Date) for the sole purpose of establishing quorum at a meeting of shareholders.

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Notwithstanding anything to the contrary contained in the Deposit Agreement or any ADR, the Depositary shall not have any obligation to take any action with respect to any meeting, or solicitation of consents or proxies, of holders of Deposited Securities if the taking of such action would violate U.S. or English laws.  The Company agrees to take any and all actions reasonably necessary and as permitted by any applicable law to enable Holders and Beneficial Owners to exercise the voting rights accruing to the Deposited Securities and to deliver to the Depositary an opinion of U.S. counsel addressing any actions requested to be taken if so requested by the Depositary.

There can be no assurance that Holders generally or any Holder in particular will receive the notice described above with sufficient time to enable the Holder to return voting instructions to the Depositary in a timely manner.

(19)         Changes Affecting Deposited Securities.  Upon any change in nominal or par value, split‑up, cancellation, consolidation or any other reclassification of Deposited Securities, or upon any recapitalization, reorganization, merger, consolidation or sale of assets affecting the Company or to which it is a party, any property which shall be received by the Depositary or the Custodian in exchange for, or in conversion of, or replacement of, or otherwise in respect of, such Deposited Securities shall, to the extent permitted by law, be treated as new Deposited Property under the Deposit Agreement, and this ADR shall, subject to the provisions of the Deposit Agreement, this ADR evidencing such ADSs and applicable law, represent the right to receive such additional or replacement Deposited Property.  In giving effect to such change, split-up, cancellation, consolidation or other reclassification of Deposited Securities, recapitalization, reorganization, merger, consolidation or sale of assets, the Depositary may, with the Company’s approval, and shall, if the Company shall so request, subject to the terms of the Deposit Agreement (including, without limitation, (a) the applicable fees and charges of, and expenses incurred by, the Depositary, and (b) taxes) and receipt of an opinion of counsel to the Company reasonably satisfactory to the Depositary that such actions are not in violation of any applicable laws or regulations, (i) issue and deliver additional ADSs as in the case of a stock dividend on the Shares, (ii) amend the Deposit Agreement and the applicable ADRs, (iii) amend the applicable Registration Statement(s) on Form F-6 as filed with the Commission in respect of the ADSs, (iv) call for the surrender of outstanding ADRs to be exchanged for new ADRs, and (v) take such other actions as the Depositary, in consultation with the Company, considers appropriate to reflect the transaction with respect to the ADSs.  Notwithstanding the foregoing, in the event that any Deposited Property so received may not be, upon consultation with the Company, lawfully distributed to some or all Holders, the Depositary may, with the Company’s approval, and shall, if the Company requests, subject to receipt of an opinion of Company’s counsel reasonably satisfactory to the Depositary that such action is not in violation of any applicable laws or regulations, sell such Deposited Property at public or private sale, at such place or places and upon such terms as it may deem proper and may allocate the net proceeds of such sales (net of (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) for the account of the Holders otherwise entitled to such Deposited Property upon an averaged or other practicable basis without regard to any distinctions among such Holders and distribute the net proceeds so allocated to the extent practicable as in the case of a distribution received in cash pursuant to Section 4.1 of the Deposit Agreement.  The Depositary shall not be responsible for (i) any failure to determine whether it may be lawful or practicable to make such Deposited Property available to Holders in general or to any Holder in particular, (ii) any foreign exchange exposure or loss incurred in connection with such sale or (iii) any liability to the purchaser of such Deposited Property.

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(20)         Exoneration.  Notwithstanding anything contained in the Deposit Agreement or this ADR to the contrary, neither the Depositary nor the Company shall be obligated to do or perform any act which is inconsistent with the provisions of the Deposit Agreement or incur any liability (to the extent not limited by paragraph (25) hereof and Section 7.9 (b) of the Deposit Agreement) (i) if the Depositary, the Custodian, the Company or their respective agents shall be prevented or forbidden from, or delayed in, doing or performing any act or thing required or contemplated by the terms of the Deposit Agreement and this ADR, by reason of any provision of any present or future law or regulation of the United States, England and Wales or any other country, or of any other governmental authority or regulatory authority or stock exchange, or on account of the potential criminal or civil penalties or restraint, or by reason of any provision, present or future, of the Articles of Association or any provision of or governing any Deposited Securities, or by reason of any act of God or war or other circumstances beyond its control (including, without limitation, nationalization, expropriation, currency restrictions, work stoppage, strikes, civil unrest, acts of terrorism, revolutions, rebellions, explosions and computer failure), (ii) by reason of any exercise of, or failure to exercise, any discretion provided for in the Deposit Agreement or in the Articles of Association or provisions of or governing Deposited Securities, (iii) for any action or inaction in reliance upon the advice of or information from legal counsel, accountants, any person presenting Shares for deposit, any Holder, any Beneficial Owner or authorized representative thereof, or any other person believed by it in good faith to be competent to give such advice or information, (iv) for the inability by a Holder or Beneficial Owner to benefit from any distribution, offering, right or other benefit which is made available to holders of Deposited Securities but is not, under the terms of the Deposit Agreement, made available to Holders of ADSs, (v) for any action or inaction of any clearing or settlement system (any participant thereof) for the Deposited Property or the ADSs, or (vi) for any consequential or punitive damages (including lost profits) for any breach of the terms of the Deposit Agreement.  The Depositary, its controlling persons, its agents, any Custodian and the Company, its Affiliates, its controlling persons and its agents may rely and shall be protected in acting upon any written notice, request or other document believed by it to be genuine and to have been signed or presented by the proper party or parties.

(21)         Standard of Care.  The Company and the Depositary and their respective Affiliates, directors, officers, employees and agents assume no obligation and shall not be subject to any liability under the Deposit Agreement or this ADR to any Holder(s) or Beneficial Owner(s), except that the Company and the Depositary agree to perform their respective obligations specifically set forth in the Deposit Agreement or this ADR without negligence or bad faith.  Without limitation of the foregoing, neither the Depositary, nor the Company, nor any of their respective Affiliates, directors, officers, controlling persons, employees or agents, shall be under any obligation to appear in, prosecute or defend any action, suit or other proceeding in respect of any Deposited Property or in respect of the ADSs, which in its opinion may involve it in expense or liability, unless indemnity satisfactory to it against all expense (including fees and disbursements of counsel) and liability be furnished as often as may be required (and no Custodian shall be under any obligation whatsoever with respect to such proceedings, the responsibility of the Custodian being solely to the Depositary).

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The Depositary and its agents shall not be liable for any failure to carry out any instructions to vote any of the Deposited Securities, or for the manner in which any vote is cast or the effect of any vote, provided that any such action or omission is in good faith and without negligence and in accordance with the terms of the Deposit Agreement.  The Depositary shall not incur any liability for any failure to accurately determine whether any distribution or action may be lawful or reasonably practicable, for any investment risk associated with acquiring an interest in the Deposited Property, for the validity or worth of the Deposited Property, for the value of any Deposited Property or any distribution thereon, for any interest on Deposited Property, for any tax consequences that may result from the ownership of ADSs, Shares or other Deposited Property, for the credit‑worthiness of any third party. The Depositary shall not incur any liability for the content of any information submitted to it by the Company for distribution to the Holders or for any inaccuracy of any translation thereof, for allowing any rights to lapse upon the terms of the Deposit Agreement, for the failure or timeliness of any notice from the Company, or for any action of or failure to act by, or any information provided or not provided by, DTC or any DTC Participant.

The Depositary shall not be liable for any acts or omissions made by a successor depositary whether in connection with a previous act or omission of the Depositary or in connection with any matter arising wholly after the removal or resignation of the Depositary, provided that in connection with the issue out of which such potential liability arises the Depositary performed its obligations without negligence or bad faith while it acted as Depositary.

(22)         Resignation and Removal of the Depositary; Appointment of Successor Depositary.  The Depositary may at any time resign as Depositary under the Deposit Agreement by written notice of resignation delivered to the Company, such resignation to be effective on the earlier of (i) the 90th day after delivery thereof to the Company (whereupon the Depositary shall be entitled to take the actions contemplated in Section 6.2 of the Deposit Agreement), or (ii) the appointment by the Company of a successor depositary and its acceptance of such appointment as provided in the Deposit Agreement.  The Depositary, in such capacity, may at any time be removed by the Company by written notice of such removal, which removal shall be effective on the later of (i) the 90th day after delivery thereof to the Depositary (whereupon the Depositary shall be entitled to take the actions contemplated in Section 6.2 of the Deposit Agreement), or (ii) the appointment by the Company of a successor depositary and its acceptance of such appointment as provided in the Deposit Agreement.  In case at any time the Depositary acting hereunder shall resign or be removed, the Company shall use its best efforts to appoint a successor depositary, which shall be a bank or trust company having an office in the Borough of Manhattan, the City of New York.  Every successor depositary shall be required by the Company to execute and deliver to its predecessor and to the Company an instrument in writing accepting its appointment hereunder, and thereupon such successor depositary, without any further act or deed (except as required by applicable law), shall become fully vested with all the rights, powers, duties and obligations of its predecessor (other than as contemplated in Sections 5.8 and 5.9 of the Deposit Agreement).  The predecessor depositary, upon payment of all sums due it and on the written request of the Company shall (i) execute and deliver an instrument transferring to such successor all rights and powers of such predecessor hereunder (other than as contemplated in Sections 5.8 and 5.9 of the Deposit Agreement), (ii) duly assign, transfer and deliver all of the Depositary’s right, title and interest to the Deposited Property to such successor, and (iii) deliver to such successor a list of the Holders of all outstanding ADSs and such other information relating to ADSs and Holders thereof as the successor may reasonably request. Any such successor depositary shall promptly provide notice of its appointment to such Holders.  Any entity into or with which the Depositary may be merged or consolidated shall be the successor of the Depositary without the execution or filing of any document or any further act.

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(23)         Amendment/Supplement.  Subject to the terms and conditions of this paragraph 23, and Section 6.1 of the Deposit Agreement and applicable law, this ADR and any provisions of the Deposit Agreement may at any time and from time to time be amended or supplemented by written agreement between the Company and the Depositary in any respect which they may deem necessary or desirable without the prior written consent of the Holders or Beneficial Owners.  Any amendment or supplement which shall impose or increase any fees or charges (other than charges in connection with foreign exchange control regulations, and taxes and other governmental charges, delivery and other such expenses), or which shall otherwise materially prejudice any substantial existing right of Holders or Beneficial Owners, shall not, however, become effective as to outstanding ADSs until the expiration of thirty (30) days after notice of such amendment or supplement shall have been given to the Holders of outstanding ADSs.  Notice of any amendment to the Deposit Agreement or any ADR shall not need to describe in detail the specific amendments effectuated thereby, and failure to describe the specific amendments in any such notice shall not render such notice invalid, provided, however, that, in each such case, the notice given to the Holders identifies a means for Holders and Beneficial Owners to retrieve or receive the text of such amendment (e.g., upon retrieval from the Commission’s, the Depositary’s or the Company’s website or upon request from the Depositary).  The parties hereto agree that any amendments or supplements which (i) are reasonably necessary (as agreed by the Company and the Depositary) in order for (a) the ADSs to be registered on Form F-6 under the Securities Act or (b) the ADSs to be settled solely in electronic book-entry form and (ii) do not in either such case impose or increase any fees or charges to be borne by Holders, shall be deemed not to materially prejudice any substantial existing rights of Holders or Beneficial Owners.  Every Holder and Beneficial Owner at the time any amendment or supplement so becomes effective shall be deemed, by continuing to hold such ADSs, to consent and agree to such amendment or supplement and to be bound by the Deposit Agreement and this ADR, if applicable, as amended or supplemented thereby.  In no event shall any amendment or supplement impair the right of the Holder to surrender such ADS and receive therefor the Deposited Securities represented thereby, except in order to comply with mandatory provisions of applicable law.  Notwithstanding the foregoing, if any governmental body should adopt new laws, rules or regulations which would require an amendment of, or supplement to, the Deposit Agreement to ensure compliance therewith, the Company and the Depositary may amend or supplement the Deposit Agreement and this ADR at any time in accordance with such changed laws, rules or regulations.  Such amendment or supplement to the Deposit Agreement and this ADR in such circumstances may become effective before a notice of such amendment or supplement is given to Holders or within any other period of time as required for compliance with such laws, rules or regulations.

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(24)         Termination.  The Depositary shall, at any time at the written direction of the Company, terminate the Deposit Agreement by distributing notice of such termination to the Holders of all ADSs then outstanding at least thirty (30) days prior to the date fixed in such notice for such termination.  If (i) ninety (90) days shall have expired after the Depositary shall have delivered to the Company a written notice of its election to resign, or (ii) ninety (90) days shall have expired after the Company shall have delivered to the Depositary a written notice of the removal of the Depositary, and, in either case, a successor depositary shall not have been appointed and accepted its appointment as provided in Section 5.4 of the Deposit Agreement, the Depositary may terminate the Deposit Agreement by distributing notice of such termination to the Holders of all ADSs then outstanding at least thirty (30) days prior to the date fixed in such notice for such termination.  The date so fixed for termination of the Deposit Agreement in any termination notice so distributed by the Depositary to the Holders of ADSs is referred to as the “Termination Date”.  Until the Termination Date, the Depositary shall continue to perform all of its obligations under the Deposit Agreement, and the Holders and Beneficial Owners will be entitled to all of their rights under the Deposit Agreement.  If any ADSs shall remain outstanding after the Termination Date, the Registrar and the Depositary shall not, after the Termination Date, have any obligation to perform any further acts under the Deposit Agreement, except that the Depositary shall, subject, in each case, to the terms and conditions of the Deposit Agreement, continue to (i) collect dividends and other distributions pertaining to Deposited Securities, (ii) sell Deposited Property received in respect of Deposited Securities, (iii) deliver Deposited Securities, together with any dividends or other distributions received with respect thereto and the net proceeds of the sale of any other Deposited Property, in exchange for ADSs surrendered to the Depositary (after deducting, or charging, as the case may be, in each case, the fees and charges of, and expenses incurred by, the Depositary, and all applicable taxes or governmental charges for the account of the Holders and Beneficial Owners, in each case upon the terms set forth in Section 5.9 of the Deposit Agreement), and (iv) take such actions as may be required under applicable law in connection with its role as Depositary under the Deposit Agreement.  At any time after the Termination Date, the Depositary may sell the Deposited Property then held under the Deposit Agreement and shall after such sale hold un-invested the net proceeds of such sale, together with any other cash then held by it under the Deposit Agreement, in an un-segregated account and without liability for interest, for the   pro rata benefit of the Holders whose ADSs have not theretofore been surrendered.  After making such sale, the Depositary shall be discharged from all obligations under the Deposit Agreement except (i) to account for such net proceeds and other cash (after deducting, or charging, as the case may be, in each case, the fees and charges of, and expenses incurred by, the Depositary, and all applicable taxes or governmental charges for the account of the Holders and Beneficial Owners, in each case upon the terms set forth in Section 5.9 of the Deposit Agreement), and (ii) as may be required at law in connection with the termination of the Deposit Agreement.  After the Termination Date, the Company shall be discharged from all obligations under the Deposit Agreement, except for its obligations to the Depositary under Sections 5.8, 5.9 and 7.6 of the Deposit Agreement.  The obligations under the terms of the Deposit Agreement of Holders and Beneficial Owners of ADSs outstanding as of the Termination Date shall survive the Termination Date and shall be discharged only when the applicable ADSs are presented by their Holders to the Depositary for cancellation under the terms of the Deposit Agreement (except as specifically provided in the Deposit Agreement).

(25)         Compliance with, and No Disclaimer under, U.S. Securities Laws.  (a) Notwithstanding any provisions in this ADR or the Deposit Agreement to the contrary, the withdrawal or delivery of Deposited Securities will not be suspended by the Company or the Depositary except as would be permitted by Instruction I.A.(1) of the General Instructions to the Form F-6 Registration Statement, as amended from time to time, under the Securities Act.

(b)          Each of the parties to the Deposit Agreement (including, without limitation, each Holder and Beneficial Owner) acknowledges and agrees that no provision of the Deposit Agreement or any ADR shall, or shall be deemed to, disclaim any liability under the Securities Act or the Exchange Act, in each case to the extent established under applicable U.S. laws.

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(26)         No Third Party Beneficiaries/Acknowledgements.   The Deposit Agreement is for the exclusive benefit of the parties hereto (and their successors) and shall not be deemed to give any legal or equitable right, remedy or claim whatsoever to any other person, except to the extent specifically set forth in the Deposit Agreement.  Nothing in the Deposit Agreement shall be deemed to give rise to a partnership or joint venture among the parties nor establish a fiduciary or similar relationship among the parties.  The parties hereto acknowledge and agree that (i) Citibank and its Affiliates may at any time have multiple banking relationships with the Company,  the Holders, the Beneficial Owners, and their respective Affiliates,  (ii) Citibank and its Affiliates may own and deal in any class of securities of the Company and its Affiliates and in ADSs, and may be engaged at any time in transactions in which parties adverse to the Company, the Holders, the Beneficial Owners or their respective Affiliates may have interests, (iii) the Depositary and its Affiliates may from time to time have in their possession non-public information about the Company, the Holders, the Beneficial Owners, and their respective Affiliates, (iv) nothing contained in the Deposit Agreement shall (a) preclude Citibank or any of its Affiliates from engaging in such transactions or establishing or maintaining such relationships, or (b) obligate Citibank or any of its Affiliates to disclose such information, transactions or relationships, or to account for any profit made or payment received in such transactions or relationships, (v) the Depositary shall not be deemed to have knowledge of any information any other division of Citibank or any of its Affiliates may have about the Company, the Holders, the Beneficial Owners, or any of their respective Affiliates, and (vi) the Company, the Depositary, the Custodian and their respective agents and controlling persons may be subject to the laws and regulations of jurisdictions other than the U.S. and  England and Wales, and the authority of courts and regulatory authorities of such other jurisdictions, and, consequently, the requirements and the limitations of such other laws and regulations, and the decisions and orders of such other courts and regulatory authorities, may affect the rights and obligations of the parties to the Deposit Agreement.

(27)         Governing Law / Waiver of Jury Trial.  The Deposit Agreement, the ADRs and the ADSs shall be interpreted in accordance with, and all rights hereunder and thereunder and provisions hereof and thereof shall be governed by, the laws of the State of New York without reference to the principles of choice of law thereof.  Notwithstanding anything contained in the Deposit Agreement to the contrary, any ADR or any present or future provisions of the laws of the State of New York, the rights of holders of Shares and of any other Deposited Securities and the obligations and duties of the Company in respect of the holders of Shares and other Deposited Securities, as such, shall be governed by the laws of England and Wales (or, if applicable, such other laws as may govern the Deposited Securities).

EACH OF THE PARTIES TO THE DEPOSIT AGREEMENT (INCLUDING, WITHOUT LIMITATION, EACH HOLDER AND BENEFICIAL OWNER) IRREVOCABLY WAIVES, TO THE FULLEST EXTENT PERMITTED BY APPLICABLE LAW, ANY AND ALL RIGHT TO TRIAL BY JURY IN ANY LEGAL PROCEEDING AGAINST THE COMPANY AND/OR THE DEPOSITARY ARISING OUT OF, OR RELATING TO, THE DEPOSIT AGREEMENT, ANY ADR AND ANY TRANSACTIONS CONTEMPLATED THEREIN (WHETHER BASED ON CONTRACT, TORT, COMMON LAW OR OTHERWISE).

A-25

(ASSIGNMENT AND TRANSFER SIGNATURE LINES)

FOR VALUE RECEIVED, the undersigned Holder hereby sell(s), assign(s) and transfer(s) unto ______________________________ whose taxpayer identification number is _______________________ and whose address including postal zip code is ________________, the within ADR and all rights thereunder, hereby irrevocably constituting and appointing ________________________ attorney‑in‑fact to transfer said ADR on the books of the Depositary with full power of substitution in the premises.

Dated:	Name:
		By:
		Title:

	NOTICE: The signature of the Holder to this assignment must correspond with the name as written upon the face of the within instrument in every particular, without alteration or enlargement or any change whatsoever.
	If the endorsement be executed by an attorney, executor, administrator, trustee or guardian, the person executing the endorsement must give his/her full title in such capacity and proper evidence of authority to act in such capacity, if not on file with the Depositary, must be forwarded with this ADR.
SIGNATURE GUARANTEED
	All endorsements or assignments of ADRs must be guaranteed by a member of a Medallion Signature Program approved by the Securities Transfer Association, Inc.
	Legends

[The ADRs issued in respect of Partial Entitlement American Depositary Shares shall bear the following legend on the face of the ADR: “This ADR evidences ADSs representing 'partial entitlement' Shares of the Company and as such do not entitle the holders thereof to the same per-share entitlement as other Shares (which are ‘full entitlement’ Shares) issued and outstanding at such time.  The ADSs represented by this ADR shall entitle holders to distributions and entitlements identical to other ADSs when the Shares represented by such ADSs become ‘full entitlement’ Shares.”]

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EXHIBIT B

FEE SCHEDULE

ADS FEES AND RELATED CHARGES

All capitalized terms used but not otherwise defined herein shall have the meaning given to such terms in the Deposit Agreement.  Except as otherwise specified herein, any reference to ADSs herein includes Partial Entitlement ADSs, Full Entitlement ADSs, Certificated ADSs, Uncertificated ADSs, and Restricted ADSs.

I. ADS Fees

The following ADS fees are payable under the terms of the Deposit Agreement:

Service	Rate	By Whom Paid
(1)    Issuance of ADSs (e.g., an issuance upon a deposit of Shares, upon a change in the ADS(s)-to-Share(s) ratio, or for any other reason), excluding issuances as a result of distributions described in paragraph (4) below.	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) issued.	Person for whom ADSs are issued.
(2)    Cancellation of ADSs (e.g.,a cancellation of ADSs for Delivery of deposited Shares, upon a change in the ADS(s)-to-Share(s) ratio, or for any other reason).	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) cancelled.	Person for whom ADSs are being cancelled.
(3)    Distribution of cash dividends or other cash distributions (e.g., upon a sale of rights and other entitlements).	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) held.	Person to whom the distribution is made.
(4)    Distribution of ADSs pursuant to (i) stock dividends or other free stock distributions, or (ii) an exercise of rights to purchase additional ADSs.	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) held.	Person to whom the distribution is made.

B-1

(5)    Distribution of securities other than ADSs or rights to purchase additional ADSs (e.g., spin-off shares).	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) held.	Person to whom the distribution is made.
(6)    ADS Services.	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) held on the applicable record date(s) established by the Depositary.	Person holding ADSs on the applicable record date(s) established by the Depositary.
(7)    Registration of ADS Transfers (e.g., upon a registration of the transfer of registered ownership of ADSs, upon a transfer of ADSs into DTC and vice versa, or for any other reason).	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) transferred.	Person for whom or to whom ADSs are transferred.
(8)    Conversion of ADSs of one series for ADSs of another series (e.g., upon conversion of Partial Entitlement ADSs for Full Entitlement ADSs, or upon conversion of Restricted ADSs into freely transferable ADSs, and vice versa).	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) converted.	Person for whom ADSs are converted or to whom the converted ADSs are delivered.

II. Charges

The Company, Holders, Beneficial Owners, persons depositing Shares or withdrawing Deposited Securities in connection with ADS issuances and cancellations, and persons for whom ADSs are issued or cancelled shall be responsible for the following ADS charges under the terms of the Deposit Agreement:

(i) taxes (including applicable interest and penalties) and other governmental charges;

(ii) such registration fees as may from time to time be in effect for the registration of Shares or other Deposited Securities on the share register and applicable to transfers of Shares or other Deposited Securities to or from the name of the Custodian, the Depositary or any nominees upon the making of deposits and withdrawals, respectively;

(iii) such cable, telex and facsimile transmission and delivery expenses as are expressly provided in the Deposit Agreement to be at the expense of the person depositing Shares or withdrawing Deposited Property or of the Holders and Beneficial Owners of ADSs;

B-2

(iv) in connection with the conversion of Foreign Currency, the fees, expenses, spreads, taxes and other charges of the Depositary and/or conversion service providers (which may be a division, branch or Affiliate of the Depositary).  Such fees, expenses, spreads, taxes, and other charges shall be deducted from the Foreign Currency;

(v) any reasonable and customary out-of-pocket expenses incurred in such conversion and/or on behalf of the Holders and Beneficial Owners in complying with currency exchange control or other governmental requirements; and

(vi) the fees, charges, costs and expenses incurred by the Depositary, the Custodian, or any nominee in connection with the ADR program.

The above fees and charges may at any time and from time to time be changed by agreement between the Company and the Depositary.

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EX-4.1.1

Exhibit 4.1.1

EXECUTION VERSION

AMENDED AND RESTATED DEPOSIT AGREEMENT

by and among

AMRYT PHARMA PLC

and

CITIBANK, N.A.,

as Depositary,

and

THE HOLDERS AND BENEFICIAL OWNERS OF

AMERICAN DEPOSITARY SHARES
ISSUED HEREUNDER

Dated as of [●] [●], 2020

TABLE OF CONTENTS

ARTICLE I
DEFINITIONS		1
Section 1.1	“ADS Record Date”	2
Section 1.2	“Affiliate”	2
Section 1.3	“American Depositary Receipt(s)”, “ADR(s)” and “Receipt(s)”	2
Section 1.4	“American Depositary Share(s)” and “ADS(s)”	2
Section 1.5	“Articles of Association”	3
Section 1.6	“Beneficial Owner”	3
Section 1.7	“Certificated ADS(s)”	3
Section 1.8	“Citibank”	3
Section 1.9	“Commission”	3
Section 1.10	“Company”	4
Section 1.11	“CREST”	4
Section 1.12	“Custodian”	4
Section 1.13	“Deliver” and “Delivery”	4
Section 1.14	“Deposit Agreement”	4
Section 1.15	“Depositary”	4
Section 1.16	“Deposited Property”	4
Section 1.17	“Deposited Securities”	5
Section 1.18	“Dollars” and “$”	5
Section 1.19	“DTC”	5
Section 1.20	“DTC Participant”	5
Section 1.21	“Exchange Act”	5
Section 1.22	“Foreign Currency”	5
Section 1.23	“Full Entitlement ADR(s)”, “Full Entitlement ADS(s)” and “Full Entitlement Share(s)”	5
Section 1.24	“Holder(s)”	5
Section 1.25	“Original Deposit Agreement”	6
Section 1.26	“Partial Entitlement ADR(s)”, “Partial Entitlement ADS(s)” and “Partial Entitlement Share(s)”	6
Section 1.27	“Principal Office”	6
Section 1.28	“Registrar”	6
Section 1.29	“Restricted Securities”	6
Section 1.30	“Restricted ADR(s)”, “Restricted ADS(s)” and “Restricted Shares”	6
Section 1.31	“Securities Act”	6
Section 1.32	“Share Registrar”	6
Section 1.33	“Shares”	7
Section 1.34	“Uncertificated ADS(s)”	7
Section 1.35	“United States” and “U.S.”	7

i

ARTICLE II
APPOINTMENT OF DEPOSITARY; FORM OF RECEIPTS; DEPOSIT OF SHARES; EXECUTION AND DELIVERY, TRANSFER AND SURRENDER OF RECEIPTS		7
Section 2.1	Appointment of Depositary	7
Section 2.2	Form and Transferability of ADSs	7
Section 2.3	Deposit of Shares	9
Section 2.4	Registration and Safekeeping of Deposited Securities	11
Section 2.5	Issuance of ADSs	11
Section 2.6	Transfer, Combination and Split-up of ADRs	12
Section 2.7	Surrender of ADSs and Withdrawal of Deposited Securities	13
Section 2.8	Limitations on Execution and Delivery, Transfer, etc. of ADSs; Suspension of Delivery, Transfer, etc	14
Section 2.9	Lost ADRs, etc	15
Section 2.10	Cancellation and Destruction of Surrendered ADRs; Maintenance of Records	15
Section 2.11	Escheatment	15
Section 2.12	Partial Entitlement ADSs	16
Section 2.13	Certificated/Uncertificated ADSs	16
Section 2.14	Restricted ADSs	17
ARTICLE III
CERTAIN OBLIGATIONS OF HOLDERS AND BENEFICIAL OWNERS OF ADSs		18
Section 3.1	Proofs, Certificates and Other Information	18
Section 3.2	Liability for Taxes and Other Charges	19
Section 3.3	Representations and Warranties on Deposit of Shares	19
Section 3.4	Compliance with Information Requests	19
Section 3.5	Ownership Restrictions	20
Section 3.6	Reporting Obligations and Regulatory Approvals	20
ARTICLE IV
THE DEPOSITED SECURITIES		20
Section 4.1	Cash Distributions	20
Section 4.2	Distribution in Shares	21
Section 4.3	Elective Distributions in Cash or Shares	22
Section 4.4	Distribution of Rights to Purchase Additional ADSs	23
Section 4.5	Distributions Other Than Cash, Shares or Rights to Purchase Shares	25
Section 4.6	Distributions with Respect to Deposited Securities in Bearer Form	25
Section 4.7	Redemption	26
Section 4.8	Conversion of Foreign Currency	26
Section 4.9	Fixing of ADS Record Date	27
Section 4.10	Voting of Deposited Securities	28
Section 4.11	Changes Affecting Deposited Securities	30
Section 4.12	Available Information	31

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Section 4.13	Reports	31
Section 4.14	List of Holders	31
Section 4.15	Taxation	31
ARTICLE V
THE DEPOSITARY, THE CUSTODIAN AND THE COMPANY		32
Section 5.1	Maintenance of Office and Transfer Books by the Registrar	32
Section 5.2	Exoneration	33
Section 5.3	Standard of Care	34
Section 5.4	Resignation and Removal of the Depositary; Appointment of Successor Depositary	35
Section 5.5	The Custodian	35
Section 5.6	Notices and Reports	36
Section 5.7	Issuance of Additional Shares, ADSs etc	37
Section 5.8	Indemnification	38
Section 5.9	ADS Fees and Charges	39
Section 5.10	Restricted Securities Owners	40
ARTICLE VI
AMENDMENT AND TERMINATION		40
Section 6.1	Amendment/Supplement	40
Section 6.2	Termination	41
ARTICLE VII
MISCELLANEOUS		42
Section 7.1	Counterparts	42
Section 7.2	No Third‑Party Beneficiaries/Acknowledgments	42
Section 7.3	Severability	43
Section 7.4	Holders and Beneficial Owners as Parties; Binding Effect	43
Section 7.5	Notices	43
Section 7.6	Governing Law and Jurisdiction	44
Section 7.7	Assignment	46
Section 7.8	Compliance with, and No Disclaimer under, U.S. Securities Laws	46
Section 7.9	English Law References	46
Section 7.10	Titles and References	46
Section 7.11	Amendment and Restatement	47
EXHIBITS
	Form of ADR.	A-1
	Fee Schedule.	B-1

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AMENDED AND RESTATED DEPOSIT AGREEMENT

AMENDED AND RESTATED DEPOSIT AGREEMENT, dated as of [●] [●], 2020, by and among (i) Amryt Pharma plc, a public limited company incorporated under the laws of England and Wales, and its successors (the “Company”), (ii) CITIBANK, N.A., a national banking association organized under the laws of the United States of America, and its successors (“Citibank”) acting in its capacity as depositary under the terms of this Deposit Agreement, and any successor depositary hereunder (Citibank in such capacity, the “Depositary”), and (iii) all Holders and Beneficial Owners of American Depositary Shares issued hereunder (all such capitalized terms as hereinafter defined).

W I T N E S S E T H   T H A T:

WHEREAS, the Company and the Depositary previously entered into that certain Deposit Agreement, dated as of September 24, 2019 (the “Original Deposit Agreement”); and

WHEREAS, the Company and the Depositary now desire to amend and restate the terms and conditions of the Original Deposit Agreement; and

WHEREAS, the Company desires to amend and restated the Original Deposit Agreement and establish with the Depositary an ADR facility to provide inter alia for the deposit of the Shares (as hereinafter defined) and the creation of American Depositary Shares representing the Shares so deposited and for the execution and Delivery (as hereinafter defined) of American Depositary Receipts (as hereinafter defined) evidencing such American Depositary Shares; and

WHEREAS, the Depositary is willing to act as the Depositary for such ADR facility upon the terms set forth in the Deposit Agreement (as hereinafter defined); and

WHEREAS, any American Depositary Receipts issued pursuant to the terms of the Deposit Agreement are to be substantially in the form of Exhibit A attached hereto, with appropriate insertions, modifications and omissions, as hereinafter provided in the Deposit Agreement; and

WHEREAS, the board of directors of the Company (or an authorized committee thereof) has duly approved the establishment of such ADR facility on the terms set forth in the Deposit Agreement, the execution and delivery of the Deposit Agreement on behalf of the Company, and the actions of the Company and the transactions contemplated herein.

NOW, THEREFORE, for good and valuable consideration, the receipt and sufficiency of which are hereby acknowledged, the parties hereto agree as follows:

ARTICLE I

DEFINITIONS

All capitalized terms used, but not otherwise defined, herein shall have the meanings set forth below, unless otherwise clearly indicated:

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Section 1.1        “ADS Record Date” shall have the meaning given to such term in Section 4.9.

Section 1.2        “Affiliate”shall have the meaning assigned to such term by the Commission (as hereinafter defined) under Regulation C promulgated under the Securities Act (as hereinafter defined), or under any successor regulation thereto.

Section 1.3        “American Depositary Receipt(s)”, “ADR(s)” and “Receipt(s)” shall mean the certificate(s) issued by the Depositary to evidence the American Depositary Shares issued under the terms of the Deposit Agreement in the form of Certificated ADS(s) (as hereinafter defined), as such ADRs may be amended from time to time in accordance with the provisions of the Deposit Agreement.  An ADR may evidence any number of ADSs and may, in the case of ADSs held through a central depository such as DTC, be in the form of a “Balance Certificate.”  Notwithstanding anything else contained herein or therein to the contrary, the American depositary receipts issued and outstanding under the terms of the Original Deposit Agreement shall, from and after the date hereof, be treated as ADRs issued hereunder and shall, from and after the date hereof, be subject to the terms hereof in all respects.

Section 1.4        “American Depositary Share(s)” and “ADS(s)” shall mean the rights and interests in the Deposited Property (as hereinafter defined) granted to the Holders and Beneficial Owners pursuant to the terms and conditions of the Deposit Agreement and, if issued as Certificated ADS(s) (as hereinafter defined), the ADR(s) issued to evidence such ADSs.  ADS(s) may be issued under the terms of the Deposit Agreement in the form of (a) Certificated ADS(s) (as hereinafter defined), in which case the ADS(s) are evidenced by ADR(s), or (b) Uncertificated ADS(s) (as hereinafter defined), in which case the ADS(s) are not evidenced by ADR(s) but are reflected on the direct registration system maintained by the Depositary for such purposes under the terms of Section 2.13.  Unless otherwise specified in the Deposit Agreement or in any ADR, or unless the context otherwise requires, any reference to ADS(s) shall include Certificated ADS(s) and Uncertificated ADS(s), individually or collectively, as the context may require.  Each ADS shall represent the right to receive, and to exercise the beneficial ownership interests in, the number of Shares specified in the form of ADR attached hereto as Exhibit A (as amended from time to time) that are on deposit with the Depositary and/or the Custodian, subject, in each case, to the terms and conditions of the Deposit Agreement and the applicable ADR (if issued as a Certificated ADS), until there shall occur a distribution upon Deposited Securities referred to in Section 4.2 or a change in Deposited Securities referred to in Section 4.11 with respect to which additional ADSs are not issued, and thereafter each ADS shall represent the right to receive, and to exercise the beneficial ownership interests in, the applicable Deposited Property on deposit with the Depositary and the Custodian determined in accordance with the terms of such Sections, subject, in each case, to the terms and conditions of the Deposit Agreement and the applicable ADR (if issued as a Certificated ADS).  In addition, the ADS(s)-to-Share(s) ratio is subject to amendment as provided in Articles IV and VI of the Deposit Agreement (which may give rise to Depositary fees).  American depositary shares outstanding under the Original Deposit Agreement as of the date hereof shall, from and after the date hereof, for all purposes be treated as American Depositary Shares issued and outstanding hereunder and shall, from and after the date hereof, be subject to the terms and conditions of the Deposit Agreement in all respects, except that any amendment of the Original Deposit Agreement effected under the terms of the Deposit Agreement which prejudices any substantial existing right of “Holders” or “Owners” or “Beneficial Owners” (each as defined in the Original Deposit Agreement) shall not become effective as to “Owners” and “Beneficial Owners” of American depositary shares until the expiration of thirty (30) days after notice of the amendments effected by the Deposit Agreement shall have been given to the “Holders” or “Beneficial Owners” of American depositary shares outstanding under the Original Deposit Agreement as of the date hereof.

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Section 1.5        “Articles of Association” shall mean the Articles of Association of the Company, as amended and restated from time to time.

Section 1.6        “Beneficial Owner” shall mean, as to any ADS, any person or entity having a beneficial interest deriving from the ownership of such ADS.  Notwithstanding anything to the contrary contained in the Deposit Agreement, any ADR(s) or any other instruments or agreements relating to the ADSs and the corresponding Deposited Property, the Depositary, the Custodian and their respective nominees are intended to be, and shall at all times during the term of the Deposit Agreement be, the record holders only of the Deposited Property represented by the ADSs for the benefit of the Holders and Beneficial Owners of the corresponding ADSs.  The Depositary, on its own behalf and on behalf of the Custodian and their respective nominees, disclaims any beneficial ownership interest in the Deposited Property held on behalf of the Holders and Beneficial Owners of ADSs.  The beneficial ownership interests in the Deposited Property are intended to be, and shall at all times during the term of the Deposit Agreement continue to be, vested in the Beneficial Owners of the ADSs representing the Deposited Property.  The beneficial ownership interests in the Deposited Property shall, unless otherwise agreed by the Depositary, be exercisable by the Beneficial Owners of the ADSs only through the Holders of such ADSs, by the Holders of the ADSs (on behalf of the applicable Beneficial Owners) only through the Depositary, and by the Depositary (on behalf of the Holders and Beneficial Owners of the corresponding ADSs) directly, or indirectly through the Custodian or their respective nominees, in each case upon the terms of the Deposit Agreement and, if applicable, the terms of the ADR(s) evidencing the ADSs.A Beneficial Owner of ADSs may or may not be the Holder of such ADSs.  A Beneficial Owner shall be able to exercise any right or receive any benefit hereunder solely through the person who is the Holder of the ADSs owned by such Beneficial Owner.  Unless otherwise identified to the Depositary, a Holder shall be deemed to be the Beneficial Owner of all the ADSs registered in his/her/its name.The manner in which a Beneficial Owner holds ADSs (e.g., in a brokerage account vs. as registered holder) may affect the rights and obligations of, the manner in which, and the extent to which, services are made available to, Beneficial Owners pursuant to the terms of the Deposit Agreement. Persons who own beneficial interests in the American depositary shares issued under the terms of the Original Deposit Agreement and outstanding as of the date hereof shall, from and after the date hereof, be treated as Beneficial Owners of ADS(s) under the terms hereof.

Section 1.7        “Certificated ADS(s)”shall have the meaning set forth in Section 2.13.

Section 1.8        “Citibank”shall have the meaning set forth in the preamble.

Section 1.9        “Commission” shall mean the Securities and Exchange Commission of the United States or any successor governmental agency thereto in the United States.

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Section 1.10      “Company” shall have the meaning set forth in the preamble.

Section 1.11      “CREST” shall mean the system for the paperless settlement of trades in securities and the holding of uncertificated securities operated by CREST Limited in accordance with the Uncertificated Securities Regulations 2001 (SI 2001 No. 3755), as amended from time to time, or any successor thereto.

Section 1.12      “Custodian” shall mean (i) as of the date hereof, Citibank, N.A.(London), having its principal office at Citigroup Centre, Canary Wharf, London, E14 5LB, United Kingdom, as the custodian of Deposited Property for the purposes of the Deposit Agreement, (ii) Citibank, N.A., acting as custodian of Deposited Property pursuant to the Deposit Agreement, and (iii) any other entity that may be appointed by the Depositary pursuant to the terms of Section 5.5 as successor, substitute or additional custodian hereunder.  The term “Custodian” shall mean any Custodian individually or all Custodians collectively, as the context requires.

Section 1.13      “Deliver” and “Delivery” shall mean (x) when used in respect of Shares and other Deposited Securities, either (i) the physical delivery of the certificate(s) representing such securities, or (ii) the book-entry transfer and recordation of such securities on the books of the Share Registrar (as hereinafter defined), in the book-entry settlement of CREST or in any other applicable book-entry settlement system, if available, and (y) when used in respect of ADSs, either (i) the physical delivery of ADR(s) evidencing the ADSs, or (ii) the book-entry transfer and recordation of ADSs on the books of the Depositary or any book-entry settlement system in which the ADSs are settlement-eligible.

Section 1.14      “Deposit Agreement” shall mean this Amended and Restated Deposit Agreement and all exhibits hereto, as the same may from time to time be amended and supplemented from time to time in accordance with the terms of the Deposit Agreement.

Section 1.15      “Depositary” shall have the meaning set forth in the preamble.

Section 1.16      “Deposited Property”shall mean the Deposited Securities and any cash and other property held on deposit by the Depositary and the Custodian in respect of the ADSs under the terms of the Deposit Agreement, subject, in the case of cash, to the provisions of Section 4.8.  All Deposited Property shall be held by the Custodian, the Depositary and their respective nominees for the benefit of the Holders and Beneficial Owners of the ADSs representing the Deposited Property.  The Deposited Property is not intended to, and shall not, constitute proprietary assets of the Depositary, the Custodian or their nominees. Beneficial ownership in the Deposited Property is intended to be, and shall at all times during the term of the Deposit Agreement continue to be, vested in the Beneficial Owners of the ADSs representing the Deposited Property.

Notwithstanding anything else contained herein, the securities, cash and other property delivered to the Custodian and the Depositary in respect of American depositary shares outstanding as of the date hereof under the Original Deposit Agreement and defined as “Deposited Securities” thereunder shall, for all purposes from and after the date hereof, be considered to be, and treated as, Deposited Property hereunder in all respects.

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Section 1.17      “Deposited Securities”shall mean the Shares and any other securities held on deposit by the Custodian from time to time in respect of the ADSs under the Deposit Agreement and constituting Deposited Property.

Section 1.18      “Dollars” and “$”shall refer to the lawful currency of the United States.

Section 1.19      “DTC” shall mean The Depository Trust Company, a national clearinghouse and the central book-entry settlement system for securities traded in the United States and, as such, the custodian for the securities of DTC Participants (as hereinafter defined) maintained in DTC, and any successor thereto.

Section 1.20      “DTC Participant” shall mean any financial institution (or any nominee of such institution) having one or more participant accounts with DTC for receiving, holding and delivering the securities and cash held in DTC.  A DTC Participant may or may not be a Beneficial Owner.  If a DTC Participant is not the Beneficial Owner of the ADSs credited to its account at DTC, or of the ADSs in respect of which the DTC Participant is otherwise acting, such DTC Participant shall be deemed, for all purposes hereunder, to have all requisite authority to act on behalf of the Beneficial Owner(s) of the ADSs credited to its account at DTC or in respect of which the DTC Participant is so acting.  A DTC Participant,  upon acceptance in any one of its DTC accounts of any ADSs (or any interest therein) issued in accordance with the terms and conditions of the Deposit Agreement, shall (notwithstanding any explicit or implicit disclosure that it may be acting on behalf of another party) be deemed for all purposes to be a party to, and bound by, the terms of the Deposit Agreement and the applicable ADR(s) to the same extent as, and as if the DTC Participant were, the Holder of such ADSs.

Section 1.21      “Exchange Act” shall mean the United States Securities Exchange Act of 1934, as amended from time to time.

Section 1.22      “Foreign Currency” shall mean any currency other than Dollars.

Section 1.23      “Full Entitlement ADR(s)”, “Full Entitlement ADS(s)” and “Full Entitlement Share(s)”shall have the respective meanings set forth in Section 2.12.

Section 1.24      “Holder(s)” shall mean the person(s) in whose name the ADSs are registered on the books of the Depositary (or the Registrar, if any) maintained for such purpose.  A Holder may or may not be a Beneficial Owner.  If a Holder is not the Beneficial Owner of the ADS(s) registered in its name, such person shall be deemed, for all purposes hereunder, to have all requisite authority to act on behalf of the Beneficial Owners of the ADSs registered in its name.  The manner in which a Holder holds ADSs (e.g., in certificated vs. uncertificated form) may affect the rights and obligations of, and the manner in which, and the extent to which, the services are made available to, Holders pursuant to the terms of the Deposit Agreement.The “Holders” or “Beneficial Owners” (as defined in the Original Deposit Agreement) of American depositary shares issued under the terms of the Original Deposit Agreement and outstanding as of the date hereof shall from and after the date hereof, become Holders under the terms of the Deposit Agreement.

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Section 1.25      “Original Deposit Agreement”shall mean the deposit agreement, dated as of September 24, 2019, by and among the Company, the Depositary, and the Holders and Beneficial Owners (as defined therein) of American depositary shares issued thereunder.

Section 1.26      “Partial Entitlement ADR(s)”, “Partial Entitlement ADS(s)” and “Partial Entitlement Share(s)”shall have the respective meanings set forth in Section 2.12.

Section 1.27      “Principal Office” shall mean, when used with respect to the Depositary, the principal office of the Depositary at which at any particular time its depositary receipts business shall be administered, which, at the date of the Deposit Agreement, is located at 388 Greenwich Street, New York, New York 10013, U.S.A.

Section 1.28      “Registrar” shall mean the Depositary or any bank or trust company having an office in the Borough of Manhattan, The City of New York, which shall be appointed by the Depositary to register issuances, transfers and cancellations of ADSs as herein provided, and shall include any co-registrar appointed by the Depositary for such purposes.  Registrars (other than the Depositary) may be removed and substitutes appointed by the Depositary in accordance with Section 5.1 hereof.  Each Registrar (other than the Depositary) appointed pursuant to the Deposit Agreement shall be required to give notice in writing to the Depositary accepting such appointment and agreeing to be bound by the applicable terms of the Deposit Agreement.

Section 1.29      “Restricted Securities” shall mean Shares, Deposited Securities or ADSs which (i) have been acquired directly or indirectly from the Company or any of its Affiliates in a transaction or chain of transactions not involving any public offering and are subject to resale limitations under the Securities Act or the rules issued thereunder, or (ii) are held by an executive officer or director (or persons performing similar functions) or other Affiliate of the Company, or (iii) are subject to other restrictions on sale or deposit under the laws of the United States, England and Wales, or under a shareholder agreement or the Articles of Association or under the regulations of an applicable securities exchange unless, in each case, such Shares, Deposited Securities or ADSs are being transferred or sold to persons other than an Affiliate of the Company in a transaction (a) covered by an effective resale registration statement, or (b) exempt from the registration requirements of the Securities Act (as hereinafter defined), and the Shares, Deposited Securities or ADSs are not, when held by such person(s), Restricted Securities.

Section 1.30      “Restricted ADR(s)”, “Restricted ADS(s)” and “Restricted Shares” shall have the respective meanings set forth in Section 2.14.

Section 1.31      “Securities Act” shall mean the United States Securities Act of 1933, as amended from time to time.

Section 1.32      “Share Registrar” shall mean Link Asset Services, a company registered in England and Wales, or any other institution organized under the laws of England and Wales appointed by the Company from time to time to carry out the duties of registrar for the Shares, and any successor thereto as the Company appoints from time to time.

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Section 1.33      “Shares” shall mean the Company’s ordinary shares, having a par value of £0.06 per share, validly issued and outstanding and fully paid and may, if the Depositary so agrees after consultation with the Company, include evidence of the right to receive Shares; provided that in no event shall Shares include evidence of the right to receive Shares with respect to which the full purchase price has not been paid or Shares as to which preemptive rights have theretofore not been validly waived or exercised; provided further, however, that, if there shall occur any change in par value, split‑up, consolidation, reclassification, exchange, conversion or any other event described in Section 4.11 in respect of the Shares of the Company, the term “Shares” shall thereafter, to the maximum extent permitted by law, represent the successor securities resulting from such event.

Section 1.34      “Uncertificated ADS(s)”shall have the meaning set forth in Section 2.13.

Section 1.35      “United States” and “U.S.” shall have the meaning assigned to it in Regulation S as promulgated by the Commission under the Securities Act.

ARTICLE II

APPOINTMENT OF DEPOSITARY; FORM OF RECEIPTS;

DEPOSIT OF SHARES; EXECUTION AND DELIVERY,

TRANSFER AND SURRENDER OF RECEIPTS

Section 2.1        Appointment of Depositary.  The Company hereby appoints the Depositary as depositary for the Deposited Property and hereby authorizes and directs the Depositary to act in accordance with the terms and conditions set forth in the Deposit Agreement and the applicable ADRs.  Each Holder and each Beneficial Owner, upon acceptance of any ADSs (or any interest therein) issued in accordance with the terms and conditions of the Deposit Agreement or by continuing to hold, from and after the date hereof any American depositary shares issued and outstanding under the Original Deposit Agreement,shall be deemed for all purposes to (a) be a party to and bound by the terms of the Deposit Agreement and the applicable ADR(s), and (b) appoint the Depositary its attorney-in-fact, with full power to delegate, to act on its behalf and to take any and all actions contemplated in the Deposit Agreement and the applicable ADR(s), to adopt any and all procedures necessary to comply with applicable law and to take such action as the Depositary in its sole discretion may deem necessary or appropriate to carry out the purposes of the Deposit Agreement and the applicable ADR(s), the taking of such actions to be the conclusive determinant of the necessity and appropriateness thereof.

Section 2.2        Form and Transferability of ADSs.

(a)       Form.  Certificated ADSs shall be evidenced by definitive ADRs which shall be engraved, printed, lithographed or produced in such other manner as may be agreed upon by the Company and the Depositary.  ADRs may be issued under the Deposit Agreement in denominations of any whole number of ADSs.  The ADRs shall be substantially in the form set forth in Exhibit A to the Deposit Agreement, with any appropriate insertions, modifications and omissions, in each case as otherwise contemplated in the Deposit Agreement or required by law.  ADRs shall be (i) dated, (ii) signed by the manual or facsimile signature of a duly authorized signatory of the Depositary, (iii) countersigned by the manual or facsimile signature of a duly authorized signatory of the Registrar, and (iv) registered in the books maintained by the Registrar for the registration of issuances and transfers of ADSs.  No ADR and no CertificatedADS evidenced thereby shall be entitled to any benefits under the Deposit Agreement or be valid or enforceable for any purpose against the Depositary or the Company, unless such ADR shall have been so dated, signed, countersigned and registered (other than an American depositary receipt issued and outstanding as of the date hereof under the terms of the Original Deposit Agreement which from and after the date hereof becomes subject to the terms of the Deposit Agreement in all respects).ADRs bearing the facsimile signature of a duly-authorized signatory of the Depositary or the Registrar, who at the time of signature was a duly-authorized signatory of the Depositary or the Registrar, as the case may be, shall bind the Depositary, notwithstanding the fact that such signatory has ceased to be so authorized prior to the Delivery of such ADR by the Depositary.  The ADRs shall bear a CUSIP number that is different from any CUSIP number that was, is or may be assigned to any depositary receipts previously or subsequently issued pursuant to any other arrangement between the Depositary (or any other depositary) and the Company and which are not ADRs outstanding hereunder.

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(b)       Legends.  The ADRs may be endorsed with, or have incorporated in the text thereof, such legends or recitals not inconsistent with the provisions of the Deposit Agreement as may be (i) necessary to enable the Depositary and the Company to perform their respective obligations hereunder, (ii) required to comply with any applicable laws or regulations, or with the rules and regulations of any securities exchange or market upon which ADSs may be traded, listed or quoted, or to conform with any usage with respect thereto, (iii) necessary to indicate any special limitations or restrictions to which any particular ADRs or ADSs are subject by reason of the date of issuance of the Deposited Securities or otherwise, or (iv) required by any book-entry system in which the ADSs are held.  Holders and Beneficial Owners shall be deemed, for all purposes, to have notice of, and to be bound by, the terms and conditions of the legends set forth, in the case of Holders, on the ADR registered in the name of the applicable Holders or, in the case of Beneficial Owners, on the ADR representing the ADSs owned by such Beneficial Owners.

(c)        Title.  Subject to the limitations contained herein and in the ADR, title to an ADR (and to each Certificated ADS evidenced thereby) shall be transferable upon the same terms as a certificated security under the laws of the State of New York, provided that, in the case of Certificated ADSs, such ADR has been properly endorsed or is accompanied by proper instruments of transfer.  Notwithstanding any notice to the contrary, the Depositary and the Company may deem and treat the Holder of an ADS (that is, the person in whose name an ADS is registered on the books of the Depositary) as the absolute owner thereof for all purposes.  Neither the Depositary nor the Company shall have any obligation nor be subject to any liability under the Deposit Agreement or any ADR to any holder or any Beneficial Owner unless, in the case of a holder of ADSs, such holder is the Holder registered on the books of the Depositary or, in the case of a Beneficial Owner, such Beneficial Owner, or the Beneficial Owner’s representative, is the Holder registered on the books of the Depositary.

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(d)       Book‑Entry Systems.  The Depositary shall make arrangements for the acceptance of the ADSs into DTC.  All ADSs held through DTC will be registered in the name of the nominee for DTC (currently “Cede & Co.”).  As such, the nominee for DTC will be the only “Holder” of all ADSs held through DTC.  Unless issued by the Depositary as Uncertificated ADSs, the ADSs registered in the name of Cede & Co. will be evidenced by one or more ADR(s) in the form of a “Balance Certificate,” which will provide that it represents the aggregate number of ADSs from time to time indicated in the records of the Depositary as being issued hereunder and that the aggregate number of ADSs represented thereby may from time to time be increased or decreased by making adjustments on such records of the Depositary and of DTC or its nominee as hereinafter provided.  Citibank, N.A. (or such other entity as is appointed by DTC or its nominee) may hold the “Balance Certificate” as custodian for DTC.  Each Beneficial Owner of ADSs held through DTC must rely upon the procedures of DTC and the DTC Participants to exercise or be entitled to any rights attributable to such ADSs.  The DTC Participants shall for all purposes be deemed to have all requisite power and authority to act on behalf of the Beneficial Owners of the ADSs held in the DTC Participants’ respective accounts in DTC and the Depositary shall for all purposes be authorized to rely upon any instructions and information given to it by DTC Participants.  So long as ADSs are held through DTC or unless otherwise required by law, ownership of beneficial interests in the ADSs registered in the name of the nominee for DTC will be shown on, and transfers of such ownership will be effected only through, records maintained by (i) DTC or its nominee (with respect to the interests of DTC Participants), or (ii) DTC Participants or their nominees (with respect to the interests of clients of DTC Participants).  Any distributions made, and any notices given, by the Depositary to DTC under the terms of the Deposit Agreement shall (unless otherwise specified by the Depositary) satisfy the Depositary’s obligations under the Deposit Agreement to make such distributions, and give such notices, in respect of the ADSs held in DTC (including, for avoidance of doubt, to the DTC Participants holding the ADSs in their DTC accounts and to the Beneficial Owners of such ADSs).

Section 2.3        Deposit of Shares.  Subject to the terms and conditions of the Deposit Agreement and applicable law, Shares or evidence of rights to receive Shares (other than Restricted Securities) may be deposited by any person (including the Depositary in its individual capacity but subject, however, in the case of the Company or any Affiliate of the Company, to Section 5.7) at any time, whether or not the transfer books of the Company or the Share Registrar, if any, are closed, by Delivery of the Shares to the Custodian.  Every deposit of Shares shall be accompanied by the following:  (A) (i) in the case of Shares represented by certificates issued in registered form, appropriate instruments of transfer or endorsement, in a form satisfactory to the Custodian, (ii) in the case of Shares represented by certificates in bearer form, the requisite coupons and talons pertaining thereto, and (iii) in the case of Shares delivered by book-entry transfer and recordation, confirmation of such book-entry transfer and recordation in the books of the Share Registrar or of CREST or any other book-entry settlement system, as applicable, to the Custodian or that irrevocable instructions have been given to cause such Shares to be so transferred and recorded, (B) such certifications and payments (including, without limitation, the Depositary’s fees and related charges) and evidence of such payments (including, without limitation, stamping or otherwise marking such Shares by way of receipt) as may be required by the Depositary or the Custodian in accordance with the provisions of the Deposit Agreement and applicable law, (C) if the Depositary so requires, a written order directing the Depositary to issue and deliver to, or upon the written order of, the person(s) stated in such order the number of ADSs representing the Shares so deposited, (D) evidence reasonably satisfactory to the Depositary (which may be an opinion of counsel;  provided that, if the Company is not the depositor, it shall not be obligated to pay the costs of obtaining any such opinion) that all necessary approvals have been granted by, or there has been compliance with the rules and regulations of, any applicable governmental agency of England and Wales, and (E) if the Depositary so requires, (i) an agreement, assignment or instrument satisfactory to the Depositary or the Custodian which provides for the prompt transfer by any person in whose name the Shares are or have been recorded to the Custodian of any distribution, or right to subscribe for additional Shares or to receive other property in respect of any such deposited Shares or, in lieu thereof, such indemnity or other agreement as shall be satisfactory to the Depositary or the Custodian and (ii) if the Shares are registered in the name of the person on whose behalf they are presented for deposit, a proxy or proxies entitling the Custodian to exercise voting rights in respect of the Shares for any and all purposes until the Shares so deposited are registered in the name of the Depositary, the Custodian or any nominee.

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Without limiting any other provision of the Deposit Agreement, the Depositary shall instruct the Custodian not to, and the Depositary shall not knowingly, accept for deposit (a) any Restricted Securities (except as contemplated by Section 2.14) nor (b) any fractional Shares or fractional Deposited Securities nor (c) a number of Shares or Deposited Securities which upon application of the ADS to Shares ratio would give rise to fractional ADSs.  No Shares shall be accepted for deposit unless accompanied by evidence, if any is required by the Depositary, that is reasonably satisfactory to the Depositary or the Custodian that all conditions to such deposit have been satisfied by the person depositing such Shares under the laws and regulations of England and Wales and any necessary approval has been granted by any applicable governmental body in England and Wales, if any.  The Depositary may issue ADSs against evidence of rights to receive Shares from the Company, any agent of the Company or any custodian, registrar, transfer agent, clearing agency or other entity involved in ownership or transaction records in respect of the Shares.  Such evidence of rights shall consist of written blanket or specific guarantees of ownership of Shares furnished by the Company or any such custodian, registrar, transfer agent, clearing agency or other entity involved in ownership or transaction records in respect of the Shares.

Without limitation of the foregoing, the Depositary shall not knowingly accept for deposit under the Deposit Agreement (A) any Shares or other securities required to be registered under the provisions of the Securities Act, unless (i) a registration statement is in effect as to such Shares or other securities or (ii) the deposit is made upon terms contemplated in Section 2.14, or (B) any Shares or other securities the deposit of which would violate any provisions of the Articles of Association or any applicable laws.  For purposes of the foregoing sentence, the Depositary shall be entitled to rely upon representations and warranties made or deemed made pursuant to the Deposit Agreement and shall not be required to make any further investigation.  The Depositary will comply with written instructions of the Company (received by the Depositary reasonably in advance) not to accept for deposit hereunder any Shares identified in such instructions at such times and under such circumstances as may reasonably be specified in such instructions in order to facilitate the Company’s compliance with the securities laws of the United States.

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Section 2.4        Registration and Safekeeping of Deposited Securities.  The Depositary shall instruct the Custodian upon each Delivery of registered Shares being deposited hereunder with the Custodian (or other Deposited Securities pursuant to Article IV hereof), together with the other documents above specified, to present such Shares, together with the appropriate instrument(s) of transfer or endorsement, duly stamped (if applicable), to the Share Registrar for transfer and registration of the Shares or, to the extent applicable, other Deposited Securities (as soon as transfer and registration can be accomplished and at the expense of the person for whom the deposit is made) in the name of the Depositary, the Custodian or a nominee of either.  Deposited Securities shall be held by the Depositary, or by a Custodian for the account and to the order of the Depositary or a nominee of the Depositary, in each case, on behalf of the Holders and Beneficial Owners, at such place(s) as the Depositary or the Custodian shall determine.  Notwithstanding anything to the contrary contained in the Deposit Agreement, any ADR(s), or any other instruments or agreements relating to the ADSs and the corresponding Deposited Property, the registration of the Deposited Securities in the name of the Depositary, the Custodian or any of their respective nominees, shall, to the maximum extent permitted by applicable law, vest in the Depositary, the Custodian or the applicable nominee the record ownership in the applicable Deposited Securities with the beneficial ownership rights and interests in such Deposited Securities being at all times vested with the Beneficial Owners of the ADSs representing the Deposited Securities.  Notwithstanding the foregoing, the Depositary, the Custodian and the applicable nominee shall at all times be entitled to exercise the beneficial ownership rights in all Deposited Property, in each case only on behalf of the Holders and Beneficial Owners of the ADSs representing the Deposited Property, upon the terms set forth in the Deposit Agreement and, if applicable, the ADR(s) representing the ADSs.  The Depositary, the Custodian and their respective nominees shall for all purposes be deemed to have all requisite power and authority to act in respect of Deposited Property on behalf of the Holders and Beneficial Owners of ADSs representing the Deposited Property, and upon making payments to, or acting upon instructions from, or information provided by, the Depositary, the Custodian or their respective nominees all persons shall be authorized to rely upon such power and authority.

Section 2.5        Issuance of ADSs.  The Depositary has made arrangements with the Custodian for the Custodian to confirm to the Depositary upon receipt of a deposit of Shares (i) that a deposit of Shares has been made pursuant to Section 2.3, (ii) that such Deposited Securities have been recorded in the name of the Depositary, the Custodian or a nominee of either on the shareholders’ register maintained by or on behalf of the Company by the Share Registrar, on the books of CREST or any other applicable book-entry settlement entity, if available, (iii) that all required documents have been received, and (iv) the person(s) to whom or upon whose order ADSs are deliverable in respect thereof and the number of ADSs to be so delivered.  Such notification may be made by letter, cable, telex, SWIFT message or, at the risk and expense of the person making the deposit, by facsimile or other means of electronic transmission.  Upon receiving such notice from the Custodian, the Depositary, subject to the terms and conditions of the Deposit Agreement and applicable law, shall issue the ADSs representing the Shares so deposited to or upon the order of the person(s) named in the notice delivered to the Depositary and, if applicable, shall execute and deliver at its Principal Office Receipt(s) registered in the name(s) requested by such person(s) and evidencing the aggregate number of ADSs to which such person(s) are entitled, but, in each case, only upon payment to the Depositary of the charges of the Depositary for accepting a deposit of Shares and issuing ADSs (as set forth in Section 5.9 and Exhibit B hereto) and all taxes and governmental charges and fees payable in connection with such deposit and the transfer of the Shares and the issuance of the ADS(s).  The Depositary shall only issue ADSs in whole numbers and deliver, if applicable, ADR(s) evidencing whole numbers of ADSs.

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Section 2.6        Transfer, Combination and Split-up of ADRs.

(a)        Transfer.  The Registrar shall register the transfer of ADRs (and of the ADSs represented thereby) on the books maintained for such purpose and the Depositary shall (x) cancel such ADRs and execute new ADRs evidencing the same aggregate number of ADSs as those evidenced by the ADRs canceled by the Depositary, (y) cause the Registrar to countersign such new ADRs and (z) Deliver such new  ADRs to or upon the order of the person entitled thereto, if each of the following conditions has been satisfied:  (i) the ADRs have been duly Delivered by the Holder (or by a duly authorized attorney of the Holder) to the Depositary at its Principal Office for the purpose of effecting a transfer thereof, (ii) the surrendered ADRs have been properly endorsed or are accompanied by proper instruments of transfer (including signature guarantees in accordance with standard securities industry practice), (iii) the surrendered ADRs have been duly stamped (if required by the laws of the State of New York or of the United States), and (iv) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 and Exhibit B hereto) have been paid, subject, however, in each case, to the terms and conditions of the applicable ADRs, of the Deposit Agreement and of applicable law, in each case as in effect at the time thereof.

(b)       Combination & Split-Up.  The Registrar shall register the split-up or combination of ADRs (and of the ADSs represented thereby) on the books maintained for such purpose and the Depositary shall (x) cancel such ADRs and execute new ADRs for the number of ADSs requested, but in the aggregate not exceeding the number of ADSs evidenced by the ADRs canceled by the Depositary, (y) cause the Registrar to countersign such new ADRs and (z) Deliver such new ADRs to or upon the order of the Holder thereof, if each of the following conditions has been satisfied:  (i) the ADRs have been duly Delivered by the Holder (or by a duly authorized attorney of the Holder) to the Depositary at its Principal Office for the purpose of effecting a split-up or combination thereof, and (ii) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 and Exhibit B hereto) have been paid, subject, however, in each case, to the terms and conditions of the applicable ADRs, of the Deposit Agreement and of applicable law, in each case as in effect at the time thereof.

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Section 2.7        Surrender of ADSs and Withdrawal of Deposited Securities.  The Holder of ADSs shall be entitled to Delivery (at the Custodian’s designated office) of the Deposited Securities at the time represented by the ADSs upon satisfaction of each of the following conditions: (i) the Holder (or a duly-authorized attorney of the Holder) has duly Delivered ADSs to the Depositary at its Principal Office (and if applicable, the ADRs evidencing such ADSs) for the purpose of withdrawal of the Deposited Securities represented thereby, (ii) if applicable andso required by the Depositary, the ADRs Delivered to the Depositary for such purpose have been properly endorsed in blank or are accompanied by proper instruments of transfer in blank (including signature guarantees in accordance with standard securities industry practice), (iii) if so required by the Depositary, the Holder of the ADSs has executed and delivered to the Depositary a written order directing the Depositary to cause the Deposited Securities being withdrawn to be Delivered to or upon the written order of the person(s) designated in such order, and (iv) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 and Exhibit B) have been paid, subject, however, in each case, to the terms and conditions of the ADRs evidencing the surrendered ADSs, of the Deposit Agreement, of the Company’s Articles of Association and of any applicable laws and the rules of CREST or any other applicable book-entry settlement system, if available, and to any provisions of or governing the Deposited Securities , in each case as in effect at the time thereof.

Upon satisfaction of each of the conditions specified above, the Depositary (i) shall cancel the ADSs Delivered to it (and, if applicable, the ADR(s) evidencing the ADSs so Delivered), (ii) shall direct the Registrar to record the cancellation of the ADSs so Delivered on the books maintained for such purpose, and (iii) shall direct the Custodian to Deliver, or cause the Delivery of, in each case, without unreasonable delay, the Deposited Securities represented by the ADSs so canceled together with any certificate or other document of title for the Deposited Securities, or evidence of the electronic transfer thereof (if available), as the case may be, to or upon the written order of the person(s) designated in the order delivered to the Depositary for such purpose, subject however, in each case, to the terms and conditions of the Deposit Agreement, of the ADRs evidencing the ADSs so canceled, of the Articles of Association, of any applicable laws and of the rules of CREST or any other applicable book-entry settlement system, if available, and to the terms and conditions of or governing the Deposited Securities, in each case as in effect at the time thereof.

The Depositary shall not accept for surrender ADSs representing less than one (1) Share.  In the case of Delivery to it of ADSs representing a number other than a whole number of Shares, the Depositary shall cause ownership of the appropriate whole number of Shares to be Delivered in accordance with the terms hereof, and shall, at the discretion of the Depositary, either (i) return to the person surrendering such ADSs the number of ADSs representing any remaining fractional Share, or (ii) sell or cause to be sold the fractional Share represented by the ADSs so surrendered and remit the proceeds of such sale (net of (a) applicable fees and charges of, and expenses incurred by, the Depositary and (b) taxes withheld) to the person surrendering the ADSs.

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Notwithstanding anything to the contrary contained in any ADR or the Deposit Agreement, the Depositary may make delivery at the Principal Office of the Depositary of Deposited Property consisting of (i) any cash dividends or cash distributions, or (ii) any proceeds from the sale of any non-cash distributions, which are at the time held by the Depositary in respect of the Deposited Securities represented by the ADSs surrendered for cancellation and withdrawal.  At the request, risk and expense of any Holder so surrendering ADSs, and for the account of such Holder, the Depositary shall direct the Custodian to forward (to the extent permitted by law) any Deposited Property (other than Deposited Securities) held by the Custodian in respect of such ADSs to the Depositary for delivery at the Principal Office of the Depositary.  Such direction shall be given by letter or, at the request, risk and expense of such Holder, by cable, telex or facsimile transmission.

Section 2.8        Limitations on Execution and Delivery, Transfer, etc. of ADSs; Suspension of Delivery, Transfer, etc.

(a)       Additional Requirements.  As a condition precedent to the execution and Delivery, the registration of issuance, transfer, split-up, combination or surrender, of any ADS, the delivery of any distribution thereon, or the withdrawal of any Deposited Property, the Depositary or the Custodian may require (i) payment from the depositor of Shares or presenter of ADSs or of an ADR of a sum sufficient to reimburse it for any tax or other governmental charge and any stock transfer or registration fee with respect thereto (including any such tax or charge and fee with respect to Shares being deposited or withdrawn) and payment of any applicable fees and charges of the Depositary as provided in Section 5.9 and Exhibit B, (ii) the production of proof satisfactory to it as to the identity and genuineness of any signature or any other matter contemplated by Section 3.1, and (iii) compliance with (A) any laws or governmental regulations relating to the execution and Delivery of ADRs or ADSs or to the withdrawal of Deposited Securities and (B) such reasonable regulations as the Depositary and the Company may establish consistent with the provisions of the representative ADR, if applicable, the Deposit Agreement and applicable law.

(b)       Additional Limitations.  The issuance of ADSs against deposits of Shares generally or against deposits of particular Shares may be suspended, or the deposit of particular Shares may be refused, or the registration of transfer of ADSs in particular instances may be refused, or the registration of transfers of ADSs generally may be suspended, during any period when the transfer books of the Company, the Depositary, a Registrar or the Share Registrar are closed or if any such action is deemed necessary or advisable by the Depositary (whereupon the Depositary shall notify the Company) or the Company, in good faith, at any time or from time to time because of any requirement of law or regulation, any government or governmental body or commission or any securities exchange on which the ADSs or Shares are listed, or under any provision of the Deposit Agreement or the representative ADR(s), if applicable, or under any provision of, or governing, the Deposited Securities, or because of a meeting of shareholders of the Company or for any other reason, subject, in all cases, to Section 7.8.

(c)       Regulatory Restrictions.  Notwithstanding any provision of the Deposit Agreement or any ADR(s) to the contrary, Holders are entitled to surrender outstanding ADSs to withdraw the Deposited Securities associated herewith at any time subject only to (i) temporary delays caused by closing the transfer books of the Depositary or the Company or the deposit of Shares in connection with voting at a shareholders’ meeting or the payment of dividends, (ii) the payment of fees, taxes and similar charges, (iii) compliance with any U.S. or foreign laws or governmental regulations relating to the ADSs or to the withdrawal of the Deposited Securities, and (iv) other circumstances specifically contemplated by Instruction I.A.(l) of the General Instructions to Form F-6 (as such General Instructions may be amended from time to time) under the Securities Act.

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Section 2.9        Lost ADRs, etc.  In case any ADR shall be mutilated, destroyed, lost, or stolen, the Depositary shall execute and deliver a new ADR of like tenor at the expense of the Holder (a) in the case of a mutilated ADR, in exchange of and substitution for such mutilated ADR upon cancellation thereof, or (b) in the case of a destroyed, lost or stolen ADR, in lieu of and in substitution for such destroyed, lost, or stolen ADR, after the Holder thereof (i) has submitted to the Depositary a written request for such exchange and substitution before the Depositary has notice that the ADR has been acquired by a bona fide purchaser, (ii) has provided such security or indemnity (including an indemnity bond) as may be required by the Depositary to save it and any of its agents harmless, and (iii) has satisfied any other reasonable requirements imposed by the Depositary, including, without limitation, evidence satisfactory to the Depositary of such destruction, loss or theft of such ADR, the authenticity thereof and the Holder’s ownership thereof.

Section 2.10      Cancellation and Destruction of Surrendered ADRs; Maintenance of Records.  All ADRs surrendered to the Depositary shall be canceled by the Depositary.  Canceled ADRs shall not be entitled to any benefits under the Deposit Agreement or be valid or enforceable against the Depositary or the Company for any purpose.  The Depositary is authorized to destroy ADRs so canceled, provided the Depositary maintains a record of all destroyed ADRs.  Any ADSs held in book-entry form (e.g., through accounts at DTC) shall be deemed canceled when the Depositary causes the number of ADSs evidenced by the Balance Certificate to be reduced by the number of ADSs surrendered (without the need to physically destroy the Balance Certificate).  The Depositary agrees to maintain records of all ADRs surrendered and the Shares withdrawn, substitute ADRs, delivered and cancelled or destroyed ADRs as required by the regulations governing the stock transfer industry.  Upon the request of the Company, the Depositary shall provide a copy of such records to the Company.

Section 2.11      Escheatment.  In the event any unclaimed property relating to the ADSs, for any reason, is in the possession of Depositary and has not been claimed by the Holder thereof or cannot be delivered to the Holder thereof through usual channels, the Depositary shall, upon expiration of any applicable statutory period relating to abandoned property laws, escheat such unclaimed property to the relevant authorities in accordance with the laws of each of the relevant States of the United States.

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Section 2.12      Partial Entitlement ADSs.  In the event any Shares are deposited which (i) entitle the holders thereof to receive a per-share distribution or other entitlement in an amount different from the Shares then on deposit or (ii) are not fully fungible (including, without limitation, as to settlement or trading) with the Shares then on deposit (the Shares then on deposit collectively, “Full Entitlement Shares” and the Shares with different entitlement, “Partial Entitlement Shares”), the Depositary shall (i) cause the Custodian to hold Partial Entitlement Shares separate and distinct from Full Entitlement Shares, and (ii) subject to the terms of the Deposit Agreement, issue ADSs representing Partial Entitlement Shares which are separate and distinct from the ADSs representing Full Entitlement Shares, by means of separate CUSIP numbering and legending (if necessary) and, if applicable, by issuing ADRs evidencing such ADSs with applicable notations thereon (“Partial Entitlement ADSs/ADRs” and “Full Entitlement ADSs/ADRs”, respectively).  If and when Partial Entitlement Shares become Full Entitlement Shares, the Depositary shall (a) give notice thereof to Holders of Partial Entitlement ADSs and give Holders of Partial Entitlement ADRs the opportunity to exchange such Partial Entitlement ADRs for Full Entitlement ADRs, (b) cause the Custodian to transfer the Partial Entitlement Shares into the account of the Full Entitlement Shares, and (c) take such actions as are necessary to remove the distinctions between (i) the Partial Entitlement ADRs and ADSs, on the one hand, and (ii) the Full Entitlement ADRs and ADSs on the other.  Holders and Beneficial Owners of Partial Entitlement ADSs shall only be entitled to the entitlements of Partial Entitlement Shares.  Holders and Beneficial Owners of Full Entitlement ADSs shall be entitled only to the entitlements of Full Entitlement Shares.  All provisions and conditions of the Deposit Agreement shall apply to Partial Entitlement ADRs and ADSs to the same extent as Full Entitlement ADRs and ADSs, except as contemplated by this Section 2.12.  The Depositary is authorized to take any and all other actions as may be necessary (including, without limitation, making the necessary notations on ADRs) to give effect to the terms of this Section 2.12.  The Company agrees to give timely written notice to the Depositary if any Shares issued or to be issued are Partial Entitlement Shares and shall provide reasonable assistance to the Depositary with the establishment of procedures enabling the identification of Partial Entitlement Shares upon Delivery to the Custodian.

Section 2.13      Certificated/Uncertificated ADSs.  Notwithstanding any other provision of the Deposit Agreement, the Depositary may, at any time and from time to time, issue ADSs that are not evidenced by ADRs (such ADSs, the “Uncertificated ADS(s)” and the ADS(s) evidenced by ADR(s), the “Certificated ADS(s)”).  When issuing and maintaining Uncertificated ADS(s) under the Deposit Agreement, the Depositary shall at all times be subject to (i) the standards applicable to registrars and transfer agents maintaining direct registration systems for equity securities in New York and issuing uncertificated securities under New York law, and (ii) the terms of New York law applicable to uncertificated equity securities.  Uncertificated ADSs shall not be represented by any instruments but shall be evidenced by registration in the books of the Depositary maintained for such purpose.  Holders of Uncertificated ADSs, that are not subject to any registered pledges, liens, restrictions or adverse claims of which the Depositary has notice at such time, shall at all times have the right to exchange the Uncertificated ADS(s) for Certificated ADS(s) of the same type and class, subject in each case to (x) applicable laws and any rules and regulations the Depositary may have established in respect of the Uncertificated ADSs, and (y) the continued availability of Certificated ADSs in the U.S.  Holders of Certificated ADSs shall, if the Depositary maintains a direct registration system for the ADSs, have the right to exchange the Certificated ADSs for Uncertificated ADSs upon (i) the due surrender of the Certificated ADS(s) to the Depositary for such purpose and (ii) the presentation of a written request to that effect to the Depositary, subject in each case to (a) all liens and restrictions noted on the ADR evidencing the Certificated ADS(s) and all adverse claims of which the Depositary then has notice, (b) the terms of the Deposit Agreement and the rules and regulations that the Depositary may establish for such purposes hereunder, (c) applicable law, and (d) payment of the Depositary fees and expenses applicable to such exchange of Certificated ADS(s) for Uncertificated ADS(s).  Uncertificated ADSs shall in all material respects be identical to Certificated ADS(s) of the same type and class, except that (i) no ADR(s) shall be, or shall need to be, issued to evidence Uncertificated ADS(s), (ii) Uncertificated ADS(s) shall, subject to the terms of the Deposit Agreement, be transferable upon the same terms and conditions as uncertificated securities under New York law, (iii) the ownership of Uncertificated ADS(s) shall be recorded on the books of the Depositary maintained for such purpose and evidence of such ownership shall be reflected in periodic statements provided by the Depositary to the Holder(s) in accordance with applicable New York law, (iv) the Depositary may from time to time, upon notice to the Holders of Uncertificated ADSs affected thereby, establish rules and regulations, and amend or supplement existing rules and regulations, as may be deemed reasonably necessary to maintain Uncertificated ADS(s) on behalf of Holders, provided that (a) such rules and regulations do not conflict with the terms of the Deposit Agreement and applicable law, and (b) the terms of such rules and regulations are readily available to Holders upon request, (v) the Uncertificated ADS(s) shall not be entitled to any benefits under the Deposit Agreement or be valid or enforceable for any purpose against the Depositary or the Company unless such Uncertificated ADS(s) is/are registered on the books of the Depositary maintained for such purpose, (vi) the Depositary may, in connection with any deposit of Shares resulting in the issuance of Uncertificated ADSs and with any transfer, pledge, release and cancellation of Uncertificated ADSs, require the prior receipt of such documentation as the Depositary may deem reasonably appropriate, and (vii) upon termination of the Deposit Agreement, the Depositary shall not require Holders of Uncertificated ADSs to affirmatively instruct the Depositary before remitting proceeds from the sale of the Deposited Property represented by such Holders’ Uncertificated ADSs under the terms of Section 6.2 hereof.  When issuing ADSs under the terms of the Deposit Agreement, including, without limitation, issuances pursuant to Sections 2.5, 4.2, 4.3, 4.4, 4.5 and4.11 hereof, the Depositary may in its discretion determine to issue Uncertificated ADSs rather than Certificated ADSs, unless otherwise specifically instructed by the applicable Holder to issue Certificated ADSs.  All provisions and conditions of the Deposit Agreement shall apply to Uncertificated ADSs to the same extent as to Certificated ADSs, except as contemplated by this Section 2.13.  The Depositary is authorized and directed to take any and all actions and establish any and all procedures deemed reasonably necessary to give effect to the terms of this Section 2.13.  Any references in the Deposit Agreement or any ADR(s) to the terms “American Depositary Share(s)” or “ADS(s)” shall, unless the context otherwise requires, include Certificated ADS(s) and Uncertificated ADS(s).  Except as set forth in this Section 2.13 and except as required by applicable law, the Uncertificated ADSs shall be treated as ADSs issued and outstanding under the terms of the Deposit Agreement.  In the event that, in determining the rights and obligations of parties hereto with respect to any Uncertificated ADSs, any conflict arises between (a) the terms of the Deposit Agreement (other than this Section 2.13) and (b) the terms of this Section 2.13, the terms and conditions set forth in this Section 2.13 shall be controlling and shall govern the rights and obligations of the parties to the Deposit Agreement pertaining to the Uncertificated ADSs.

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Section 2.14      Restricted ADSs.  The Depositary shall, at the request and expense of the Company, establish procedures enabling the deposit hereunder of Shares that are Restricted Securities in order to enable the holder of such Shares to hold its ownership interests in such Restricted Securities in the form of ADSs issued under the terms hereof (such Shares, “Restricted Shares”).  Upon receipt of a written request from the Company to accept Restricted Shares for deposit hereunder, the Depositary agrees to establish procedures permitting the deposit of such Restricted Shares and the issuance of ADSs representing the right to receive, subject to the terms of the Deposit Agreement and the applicable ADR (if issued as a Certificated ADS), such deposited Restricted Shares (such ADSs, the “Restricted ADSs,” and the ADRs evidencing such Restricted ADSs, the “Restricted ADRs”).  Notwithstanding anything contained in this Section 2.14, the Depositary and the Company may, to the extent not prohibited by law, agree to issue the Restricted ADSs in uncertificated form (“Uncertificated Restricted ADSs”) upon such terms and conditions as the Company and the Depositary may deem necessary and appropriate.  The Company shall assist the Depositary in the establishment of such procedures and agrees that it shall take all steps necessary and satisfactory to the Depositary to ensure that the establishment of such procedures does not violate the provisions of the Securities Act or any other applicable laws.  The depositors of such Restricted Shares and the Holders of the Restricted ADSs may be required prior to the deposit of such Restricted Shares, the transfer of the Restricted ADRs and the Restricted ADSs evidenced thereby, or the withdrawal of the Restricted Shares represented by Restricted ADSs to provide such written certifications or agreements as the Depositary or the Company may require.  The Company shall provide to the Depositary in writing the legend(s) to be affixed to the Restricted ADRs (if the Restricted ADSs are to be issued as Certificated ADSs), or to be included in the statements issued from time to time to Holders of Uncertificated ADSs (if issued as Uncertificated Restricted ADSs), which legends shall (i) be in a form reasonably satisfactory to the Depositary and (ii) contain the specific circumstances under which the Restricted ADSs, and, if applicable, the Restricted ADRs evidencing the Restricted ADSs, may be transferred or the Restricted Shares withdrawn.  The Restricted ADSs issued upon the deposit of Restricted Shares shall be separately identified on the books of the Depositary and the Restricted Shares so deposited shall, to the extent required by law, be held separate and distinct from the other Deposited Securities held hereunder.  The Restricted ADSs shall not be eligible for inclusion in any book-entry settlement system, including, without limitation, DTC, and shall not in any way be fungible with the ADSs issued under the terms hereof that are not Restricted ADSs.  The Restricted ADSs, and, if applicable, the Restricted ADRs evidencing the Restricted ADSs, shall be transferable only by the Holder thereof upon delivery to the Depositary of (i) all documentation otherwise contemplated by the Deposit Agreement and (ii) an opinion of counsel reasonably satisfactory to the Depositary setting forth, inter alia, the conditions upon which the Restricted ADSs presented, and, if applicable, the Restricted ADRs evidencing the Restricted ADSs, are transferable by the Holder thereof under applicable securities laws and the transfer restrictions contained in the legend applicable to the Restricted ADSs presented for transfer.  Except as set forth in this Section 2.14 and except as required by applicable law, the Restricted ADSs and the Restricted ADRs evidencing Restricted ADSs shall be treated as ADSs and ADRs issued and outstanding under the terms of the Deposit Agreement.  In the event that, in determining the rights and obligations of parties hereto with respect to any Restricted ADSs, any conflict arises between (a) the terms of the Deposit Agreement (other than this Section 2.14) and (b) the terms of (i) this Section 2.14 or (ii) the applicable Restricted ADR, the terms and conditions set forth in this Section 2.14 and of the Restricted ADR shall be controlling and shall govern the rights and obligations of the parties to the Deposit Agreement pertaining to the deposited Restricted Shares, the Restricted ADSs and Restricted ADRs.

If the Restricted ADRs, the Restricted ADSs and the Restricted Shares cease to be Restricted Securities, the Depositary, upon receipt of (x) an opinion of counsel reasonably satisfactory to the Depositary setting forth, inter alia, that the Restricted ADRs, the Restricted ADSs and the Restricted Shares are not as of such time Restricted Securities, and (y) instructions from the Company to remove the restrictions applicable to the Restricted ADRs, the Restricted ADSs and the Restricted Shares, shall (i) eliminate the distinctions and separations that may have been established between the applicable Restricted Shares held on deposit under this Section 2.14 and the other Shares held on deposit under the terms of the Deposit Agreement that are not Restricted Shares, (ii) treat the newly unrestricted ADRs and ADSs on the same terms as, and fully fungible with, the other ADRs and ADSs issued and outstanding under the terms of the Deposit Agreement that are not Restricted ADRs or Restricted ADSs, and (iii) take all actions necessary to remove any distinctions, limitations and restrictions previously existing under this Section 2.14 between the applicable Restricted ADRs and Restricted ADSs, respectively, on the one hand, and the other ADRs and ADSs that are not Restricted ADRs or Restricted ADSs, respectively, on the other hand, including, without limitation, by making the newly-unrestricted ADSs eligible for inclusion in the applicable book-entry settlement systems.

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ARTICLE III

CERTAIN OBLIGATIONS OF HOLDERS AND

BENEFICIAL OWNERS OF ADSs

Section 3.1        Proofs, Certificates and Other Information.  Any person presenting Shares for deposit, any Holder and any Beneficial Owner may be required, and every Holder and Beneficial Owner agrees, from time to time to provide to the Company, the Depositary and the Custodian such proof of citizenship or residence, taxpayer status, payment of all applicable taxes or other governmental charges, exchange control approval, legal or beneficial ownership of ADSs and Deposited Property, compliance with applicable laws, the terms of the Deposit Agreement or the ADR(s) evidencing the ADSs and the provisions of, or governing, the Deposited Property, to execute such certifications and to make such representations and warranties, and to provide such other information and documentation (or, in the case of Shares in registered form presented for deposit, such information relating to the registration on the books of the Company or of the Share Registrar) as the Depositary or the Custodian may deem necessary or proper or as the Company may reasonably require by written request to the Depositary consistent with its obligations under the Deposit Agreement and the applicable ADR(s).  The Depositary and the Registrar, as applicable, and at the reasonable request of the Company, shall, to the extent practicable, may withhold the execution or delivery or registration of transfer of any ADR or ADS or the distribution or sale of any dividend or distribution of rights or of the proceeds thereof or, to the extent not limited by the terms of Section 7.8, the delivery of any Deposited Property until such proof or other information is filed or such certifications are executed, or such representations and warranties are made, or such other documentation or information is provided, in each case to the Depositary’s, the Registrar’s and the Company’s satisfaction.  The Depositary shall provide the Company, in a timely manner, with copies or originals if necessary and appropriate of (i) any such proofs of citizenship or residence, taxpayer status, or exchange control approval or copies of written representations and warranties which it receives from Holders and Beneficial Owners, and (ii) any other information or documents which the Company may reasonably request and which the Depositary shall request and receive from any Holder or Beneficial Owner or any person presenting Shares for deposit or ADSs for cancellation, transfer or withdrawal.  Nothing herein shall obligate the Depositary to (i) obtain any information for the Company if not provided by the Holders or Beneficial Owners, or (ii) verify or vouch for the accuracy of the information so provided by the Holders or Beneficial Owners.

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Section 3.2        Liability for Taxes and Other Charges.  Any tax or other governmental charge payable by the Custodian or by the Depositary with respect to any Deposited Property, ADSs or ADRs shall be payable by the Holders and Beneficial Owners to the Depositary.  The Company, the Custodian and/or the Depositary may withhold or deduct from any distributions made in respect of Deposited Property held on behalf of such Holder and/or Beneficial Owner, and may sell for the account of a Holder and/or Beneficial Owner any or all of such Deposited Property and apply such distributions and sale proceeds in payment of, any taxes (including applicable interest and penalties) or charges that are or may be payable by Holders or Beneficial Owners in respect of the ADSs, Deposited Property and ADRs, the Holder and the Beneficial Owner remaining liable for any deficiency.  The Custodian may refuse the deposit of Shares and the Depositary may refuse to issue ADSs, to deliver ADRs, register the transfer of ADSs, register the split‑up or combination of ADRs and (subject to Section 7.8) the withdrawal of Deposited Property until payment in full of such tax, charge, penalty or interest is received.  Every Holder and Beneficial Owner agrees to indemnify the Depositary, the Company, the Custodian, and any of their respective agents, officers, employees and Affiliates for, and to hold each of them harmless from, any claims (including, without limitation, by any governmental authority or other person or entity) with respect to taxes (including applicable interest and penalties thereon) arising from any tax benefit obtained for such Holder and/or Beneficial Owner.  Notwithstanding anything to the contrary contained in the Deposit Agreement or any ADR, the obligations of Holders and Beneficial Owners under this Section 3.2 shall survive any transfer of ADSs, any cancellation of ADSs and withdrawal of Deposited Securities, and the termination of the Deposit Agreement.

Section 3.3        Representations and Warranties on Deposit of Shares.  Each person depositing Shares under the Deposit Agreement shall be deemed thereby to represent and warrant that (i) such Shares and the certificates therefor are duly authorized, validly allotted and issued, fully paid, not subject to any call for the payment of further capital and legally obtained by such person, (ii) all preemptive (and similar) rights, if any, with respect to such Shares have been validly waived, disapplied or exercised, (iii) the person making such deposit is duly authorized so to do, (iv) the Shares presented for deposit are free and clear of any lien, encumbrance, security interest, charge, mortgage or adverse claim, (v) the Shares presented for deposit are not, and the ADSs issuable upon such deposit will not be, Restricted Securities (except as contemplated in Section 2.14), (vi) the Shares presented for deposit have not been stripped of any rights or entitlements, and (vii) the deposit of the Shares does not violate any applicable provisions of English law.  Such representations and warranties shall survive the deposit and withdrawal of Shares, the issuance and cancellation of ADSs in respect thereof and the transfer of such ADSs.  If any such representations or warranties are false in any way, the Company and the Depositary shall be authorized, at the cost and expense of the person depositing Shares, to take any and all actions necessary to correct the consequences thereof.

Section 3.4        Compliance with Information Requests.  Notwithstanding any other provision of the Deposit Agreement or any ADR(s), each Holder and Beneficial Owner agrees to comply with requests from the Company pursuant to applicable law, the rules and requirements of any stock exchange on which the Shares or ADSs are, or will be, registered, traded or listed or the Articles of Association, which are made to provide information, inter alia, as to the capacity in which such Holder or Beneficial Owner owns ADSs (and Shares as the case may be) and regarding the identity of any other person(s) interested in such ADSs and the nature of such interest and various other matters, whether or not they are Holders and/or Beneficial Owners at the time of such request.  The Depositary agrees to use its reasonable efforts to assist the Company in obtaining such information, including agreeing to forward, upon the request of the Company and at the Company’s expense, any such request from the Company to the Holders and to forward to the Company any such responses to such requests received by the Depositary.

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Section 3.5        Ownership Restrictions.  Notwithstanding any other provision contained in the Deposit Agreement or any ADR(s) to the contrary, the Company may restrict transfers of the Shares where such transfer might result in ownership of Shares exceeding limits imposed by applicable law or the Articles of Association.  The Company may also restrict, in such manner as it deems appropriate, transfers of the ADSs where such transfer may result in the total number of Shares represented by the ADSs owned by a single Holder or Beneficial Owner to exceed any such limits.  The Company may, in its sole discretion but subject to applicable law, instruct the Depositary to take action with respect to the ownership interest of any Holder or Beneficial Owner in excess of the limits set forth in the preceding sentence, including, but not limited to, the imposition of restrictions on the transfer of ADSs, the removal or limitation of voting rights or mandatory sale or disposition on behalf of a Holder or Beneficial Owner of the Shares represented by the ADSs held by such Holder or Beneficial Owner in excess of such limitations, if and to the extent such disposition is permitted by applicable law and the Articles of Association.  Nothing herein shall be interpreted as obligating the Depositary or the Company to ensure compliance with the ownership restrictions described in this Section 3.5.

Section 3.6        Reporting Obligations and Regulatory Approvals.  Applicable laws and regulations may require holders and beneficial owners of Shares, including the Holders and Beneficial Owners of ADSs, to satisfy reporting requirements and obtain regulatory approvals in certain circumstances.  Holders and Beneficial Owners of ADSs are solely responsible for determining and complying with such reporting requirements and obtaining such approvals.  Each Holder and each Beneficial Owner hereby agrees to make such determination, file such reports, and obtain such approvals to the extent and in the form required by applicable laws and regulations as in effect from time to time.  Neither the Depositary, the Custodian, the Company or any of their respective agents or affiliates shall be required to take any actions whatsoever on behalf of Holders or Beneficial Owners to determine or satisfy such reporting requirements or obtain such regulatory approvals under applicable laws and regulations.

ARTICLE IV

THE DEPOSITED SECURITIES

Section 4.1        Cash Distributions.  Whenever the Company intends to make a distribution of a cash dividend or other cash distribution in respect of any Deposited Securities, the Company shall give timely notice thereof to the Depositary at least twenty (20) days prior to the proposed distribution specifying, inter alia, the record date applicable for determining the holders of Deposited Securities entitled to receive such distribution.  Upon the timely receipt of such notice, the Depositary shall establish an ADS Record Date upon the terms described in Section 4.9 hereof.  Upon receipt of confirmation from the Custodian of the receipt of (x) any cash dividend or other cash distribution on any Deposited Securities, or (y) proceeds from the sale of any Deposited Property held in respect of the ADSs under the terms hereof, the Depositary will (i) if any amounts are received in a Foreign Currency, promptly convert or cause to be converted such cash dividend, distribution or proceeds into Dollars (subject to the terms and on the conditions of Section 4.8), (ii) if applicable and unless previously established, establish the ADS Record Date upon the terms described in Section 4.9, and (iii) distribute promptly the amount thus received from such conversion (net of (a) the applicable fees and charges set forth in the Fee Schedule attached hereto as Exhibit B, and (b) applicable taxes withheld) to the Holders entitled thereto as of the ADS Record Date in proportion to the number of ADSs held as of the ADS Record Date.  The Depositary shall distribute only such amount, however, as can be distributed without attributing to any Holder a fraction of one cent, and any balance not so distributed shall be held by the Depositary (without liability for interest thereon) and shall be added to and become part of the next sum received by the Depositary for distribution to Holders of ADSs outstanding at the time of the next distribution.  If the Company, the Custodian or the Depositary is required to withhold and does withhold from any cash dividend or other cash distribution in respect of any Deposited Securities, or from any cash proceeds from the sales of Deposited Property, an amount on account of taxes, duties or other governmental charges, the amount distributed to Holders on the ADSs shall be reduced accordingly.  Such withheld amounts shall be forwarded by the Company, the Custodian or the Depositary to the relevant governmental authority.  Evidence of payment thereof by the Company shall be forwarded by the Company to the Depositary upon request.  The Depositary will hold any cash amounts it is unable to distribute in a non-interest bearing account for the benefit of the applicable Holders and Beneficial Owners of ADSs until the distribution can be effected or the funds that the Depositary holds must be escheated as unclaimed property in accordance with the laws of the relevant states of the United States.  Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for in this Section 4.1, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in this Section 4.1, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.1 where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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Section 4.2        Distribution in Shares.  Whenever the Company intends to make a distribution that consists of a dividend in, or free distribution of, Shares, the Company shall give timely notice thereof to the Depositary at least twenty (20) days prior to the proposed distribution, specifying, inter alia, the record date applicable to holders of Deposited Securities entitled to receive such distribution.  Upon the timely receipt of such notice from the Company, the Depositary shall establish the ADS Record Date upon the terms described in Section 4.9.  Upon receipt of confirmation from the Custodian of the receipt of the Shares so distributed by the Company, the Depositary shall either (i) subject to Section 5.9, distribute to the Holders as of the ADS Record Date in proportion to the number of ADSs held as of the ADS Record Date, additional ADSs, which represent in the aggregate the number of Shares received as such dividend, or free distribution, subject to the other terms of the Deposit Agreement (including, without limitation, (a) the applicable fees and charges of, and expenses incurred by, the Depositary and (b) taxes), or (ii) if additional ADSs are not so distributed, take all actions necessary so that each ADS issued and outstanding after the ADS Record Date shall, to the extent permissible by law, thenceforth also represent rights and interests in the additional integral number of Shares distributed upon the Deposited Securities represented thereby (net of (a) the applicable fees and charges of, and expenses incurred by, the Depositary and (b) taxes).  In lieu of delivering fractional ADSs, the Depositary shall sell the number of Shares or ADSs, as the case may be, represented by the aggregate of such fractions and distribute the net proceeds upon the terms described in Section 4.1.  In the event that the Depositary determines that any distribution in property (including Shares) is subject to any tax or other governmental charges which the Depositary is obligated to withhold, or, if the Company in the fulfillment of its obligation under Section 5.7, has furnished an opinion of U.S. counsel determining that Shares must be registered under the Securities Act or other laws in order to be distributed to Holders (and no such registration statement has been declared effective), the Depositary may dispose of all or a portion of such property (including Shares and rights to subscribe therefor) in such amounts and in such manner, including by public or private sale, as the Depositary deems necessary and practicable, and the Depositary shall distribute the net proceeds of any such sale (after deduction of (a) taxes and (b) fees and charges of, and expenses incurred by, the Depositary) to Holders entitled thereto upon the terms described in Section 4.1.  The Depositary shall hold and/or distribute any unsold balance of such property in accordance with the provisions of the Deposit Agreement.  Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for in this Section 4.2, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in this Section 4.2, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.2 where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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Section 4.3        Elective Distributions in Cash or Shares.  Whenever the Company intends to make a distribution payable at the election of the holders of Deposited Securities in cash or in additional Shares, the Company shall give timely notice thereof to the Depositary at least sixty (60) days prior to the proposed distributionspecifying, inter alia, the record date applicable to holders of Deposited Securities entitled to receive such elective distribution and whether or not it wishes such elective distribution to be made available to Holders of ADSs.  Upon the timely receipt of a notice indicating that the Company wishes such elective distribution to be made available to Holders of ADSs, the Depositary shall consult with the Company to determine, and the Company shall assist the Depositary in its determination, whether it is lawful and reasonably practicable to make such elective distribution available to the Holders of ADSs.  The Depositary shall make such elective distribution available to Holders only if (i) the Company shall have timely requested that the elective distribution be made available to Holders, (ii) the Depositary shall have determined, upon consultation with the Company, that such distribution is reasonably practicable and (iii) the Depositary shall have received satisfactory documentation within the terms of Section 5.7.  If the above conditions are not satisfied or if the Company requests such elective distribution not to be made available to Holders of ADSs, the Depositary shall establish an ADS Record Date on the terms described in Section 4.9 and, to the extent permitted by law, distribute to the Holders, on the basis of the same determination as is made in England and Wales in respect of the Shares for which no election is made, either (X) cash upon the terms described in Section 4.1 or (Y) additional ADSs representing such additional Shares upon the terms described in Section 4.2.  If the above conditions are satisfied, the Depositary shall establish an ADS Record Date on the terms described in Section 4.9 and establish procedures to enable Holders to elect the receipt of the proposed distribution in cash or in additional ADSs.  The Company shall assist the Depositary in establishing such procedures to the extent necessary.  If a Holder elects to receive the proposed distribution (X) in cash, the distribution shall be made upon the terms described in Section 4.1, or (Y) in ADSs, the distribution shall be made upon the terms described in Section 4.2.  Nothing herein shall obligate the Depositary to make available to Holders a method to receive the elective distribution in Shares (rather than ADSs).  There can be no assurance that Holders generally, or any Holder in particular, will be given the opportunity to receive elective distributions on the same terms and conditions as the holders of Shares.  Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for in this Section 4.3, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in this Section 4.3, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.3 where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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Section 4.4        Distribution of Rights to Purchase Additional ADSs.

(a)       Distribution to ADS Holders.  Whenever the Company intends to distribute to the holders of the Deposited Securities rights to subscribe for additional Shares, the Company shall give timely notice thereof to the Depositary at least sixty (60) days prior to the proposed distributionspecifying, inter alia, the record date applicable to holders of Deposited Securities entitled to receive such distribution and whether or not it wishes such rights to be made available to Holders of ADSs.  Upon the timely receipt of a notice indicating that the Company wishes such rights to be made available to Holders of ADSs, the Depositary shall consult with the Company to determine, and the Company shall assist the Depositary in its determination, whether it is lawful and reasonably practicable to make such rights available to the Holders.  The Depositary shall make such rights available to Holders only if (i) the Company shall have timely requested that such rights be made available to Holders, (ii) the Depositary shall have received satisfactory documentation within the terms of Section 5.7, and (iii) the Depositary shall have determined that such distribution of rights is reasonably practicable.  In the event any of the conditions set forth above are not satisfied or if the Company requests that the rights not be made available to Holders of ADSs, the Depositary shall proceed with the sale of the rights as contemplated in Section 4.4(b) below.  In the event all conditions set forth above are satisfied, the Depositary shall establish an ADS Record Date (upon the terms described in Section 4.9) and establish procedures to (x) distribute rights to purchase additional ADSs (by means of warrants or otherwise), (y) enable the Holders to exercise such rights (upon payment of the subscription price and of the applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes), and (z) deliver ADSs upon the valid exercise of such rights.  The Company shall assist the Depositary to the extent necessary in establishing such procedures.  Nothing herein shall obligate the Depositary to make available to the Holders a method to exercise rights to subscribe for Shares (rather than ADSs).

(b)       Sale of Rights.  If (i) the Company does not timely request the Depositary to make the rights available to Holders or requests that the rights not be made available to Holders, (ii) the Depositary fails to receive satisfactory documentation within the terms of Section 5.7, or determines, upon consultation with the Company, it is not reasonably practicable to make the rights available to Holders, or (iii) any rights made available are not exercised and appear to be about to lapse, the Depositary shall, upon consultation with the Company, determine whether it is lawful and reasonably practicable to sell such rights, in a riskless principal capacity, at such place and upon such terms (including public or private sale) as it may deem practicable.  The Company shall assist the Depositary to the extent necessary to determine such legality and practicability.  The Depositary shall, upon such sale, convert and distribute proceeds of such sale (net of applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) upon the terms set forth in Section 4.1.

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(c)        Lapse of Rights.  If the Depositary is unable to make any rights available to Holders upon the terms described in Section 4.4(a) or to arrange for the sale of the rights upon the terms described in Section 4.4(b), the Depositary shall allow such rights to lapse.

The Depositary shall not be liable for (i) any failure to accurately determine whether it may be lawful or practicable to make such rights available to Holders in general or any Holders in particular, (ii) any foreign exchange exposure or loss incurred in connection with such sale, or exercise, or (iii) the content of any materials forwarded to the Holders on behalf of the Company in connection with the rights distribution.

Notwithstanding anything to the contrary in this Section 4.4, if registration (under the Securities Act or any other applicable law) of the rights or the securities to which any rights relate may be required in order for the Company to offer such rights or such securities to Holders and to sell the securities represented by such rights, the Depositary will not distribute such rights to the Holders (i) unless and until a registration statement under the Securities Act (or other applicable law) covering such offering is in effect or (ii) unless the Company furnishes the Depositary opinion(s) of counsel for the Company in the United States and counsel to the Company in any other applicable country in which rights would be distributed, in each case reasonably satisfactory to the Depositary, to the effect that the offering and sale of such securities to Holders and Beneficial Owners are exempt from, or do not require registration under, the provisions of the Securities Act or any other applicable laws.

In the event that the Company, the Depositary or the Custodian shall be required to withhold and does withhold from any distribution of Deposited Property (including rights) an amount on account of taxes or other governmental charges, the amount distributed to the Holders of ADSs representing such Deposited Property shall be reduced accordingly.  In the event that the Depositary determines that any distribution of Deposited Property (including Shares and rights to subscribe therefor) is subject to any tax or other governmental charges which the Depositary is obligated to withhold, the Depositary may dispose of all or a portion of such Deposited Property (including Shares and rights to subscribe therefor) in such amounts and in such manner, including by public or private sale, as the Depositary deems necessary and practicable to pay any such taxes or charges.

There can be no assurance that Holders generally, or any Holder in particular, will be given the opportunity to receive or exercise rights on the same terms and conditions as the holders of Shares or be able to exercise such rights.  Nothing herein shall obligate the Company to file any registration statement in respect of any rights or Shares or other securities to be acquired upon the exercise of such rights.

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Section 4.5        Distributions Other Than Cash, Shares or Rights to Purchase Shares.

(a)       Whenever the Company intends to distribute to the holders of Deposited Securities property other than cash, Shares or rights to purchase additional Shares, the Company shall give timely notice thereof to the Depositary and shall indicate whether or not it wishes such distribution to be made to Holders of ADSs.  Upon receipt of a notice indicating that the Company wishes such distribution to be made to Holders of ADSs, the Depositary shall consult with the Company, and the Company shall assist the Depositary, to determine whether such distribution to Holders is lawful and reasonably practicable.  The Depositary shall not make such distribution unless (i) the Company shall have requested the Depositary to make such distribution to Holders, (ii) the Depositary shall have received satisfactory documentation within the terms of Section 5.7, and (iii) the Depositary shall have determined, after consultation with the Company, that such distribution is reasonably practicable.

(b)       Upon receipt of reasonably satisfactory documentation and the request of the Company to distribute property to Holders of ADSs and after making the requisite determinations set forth in (a) above, the Depositary shall distribute the property so received to the Holders of record, as of the ADS Record Date, in proportion to the number of ADSs held by them respectively and in such manner as the Depositary may deem practicable for accomplishing such distribution (i) upon receipt of payment or net of the applicable fees and charges of, and expenses incurred by, the Depositary, and (ii) net of any taxes withheld.  The Depositary may dispose of all or a portion of the property so distributed and deposited, in such amounts and in such manner (including public or private sale) as the Depositary may deem practicable or necessary to satisfy any taxes (including applicable interest and penalties) or other governmental charges applicable to the distribution.

(c)       If (i) the Company does not request the Depositary to make such distribution to Holders or requests the Depositary not to make such distribution to Holders, (ii) the Depositary does not receive reasonably satisfactory documentation within the terms of Section 5.7, or (iii) the Depositary determines that all or a portion of such distribution is not reasonably practicable, the Depositary shall sell or cause such property to be sold in a public or private sale, at such place or places and upon such terms as it may deem practicable and shall (i) cause the proceeds of such sale, if any, to be converted into Dollars and (ii) distribute the proceeds of such conversion received by the Depositary (net of applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) to the Holders as of the ADS Record Date upon the terms of Section 4.1.  If the Depositary is unable to sell such property, the Depositary may dispose of such property for the account of the Holders in any way it deems reasonably practicable under the circumstances.

(d)       Neither the Depositary nor the Company shall be liable for (i) any failure to accurately determine whether it is lawful or practicable to make the property described in this Section 4.5 available to Holders in general or any Holders in particular, nor (ii) any loss incurred in connection with the sale or disposal of such property.

Section 4.6        Distributions with Respect to Deposited Securities in Bearer Form.Subject to the terms of this Article IV, distributions in respect of Deposited Securities that are held by the Depositary or the Custodian in bearer form shall be made to the Depositary for the account of the respective Holders of ADS(s) with respect to which any such distribution is made upon due presentation by the Depositary or the Custodian to the Company of any relevant coupons, talons, or certificates.  The Company shall promptly notify the Depositary of such distributions.  The Depositary or the Custodian shall promptly present such coupons, talons or certificates, as the case may be, in connection with any such distribution.

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Section 4.7        Redemption.  If the Company intends to exercise any right of redemption in respect of any of the Deposited Securities, the Company shall give timely notice thereof to the Depositary at least sixty (60) days prior to the intended date of redemptionwhich notice shall set forth the particulars of the proposed redemption.  Upon timely receipt of (i) such notice and (ii) satisfactory documentation given by the Company to the Depositary within the terms of Section 5.7, and only if, after consultation with the Company, the Depositary shall have determined that such proposed redemption is practicable, the Depositary shall provide to each Holder a notice setting forth the intended exercise by the Company of the redemption rights and any other particulars set forth in the Company’s notice to the Depositary.  The Depositary shall instruct the Custodian to present to the Company the Deposited Securities in respect of which redemption rights are being exercised against payment of the applicable redemption price.  Upon receipt of confirmation from the Custodian that the redemption has taken place and that funds representing the redemption price have been received, the Depositary shall convert, transfer, and distribute the proceeds (net of applicable (a) fees and charges of, and the expenses incurred by, the Depositary, and (b) taxes), retire ADSs and cancel ADRs, if applicable, upon delivery of such ADSs by Holders thereof and the terms set forth in Sections 4.1 and 6.2.  If less than all outstanding Deposited Securities are redeemed, the ADSs to be retired will be selected by lot or on a pro rata basis, as may be determined by the Depositary, after consultation with the Company.  The redemption price per ADS shall be the dollar equivalent of the per share amount received by the Depositary (adjusted to reflect the ratio of ADS(s) to Share(s)) upon the redemption of the Deposited Securities represented by ADSs (subject to the terms of Section 4.8 and the applicable fees and charges of, and expenses incurred by, the Depositary, and taxes) multiplied by the number of Deposited Securities represented by each ADS redeemed.

Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed redemption provided for in this Section 4.7, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in this Section 4.7, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.7 where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

Section 4.8        Conversion of Foreign Currency.  Whenever the Depositary or the Custodian shall receive Foreign Currency, by way of dividends or other distributions or the net proceeds from the sale of Deposited Property, which in the judgment of the Depositary can at such time be converted on a practicable basis, by sale or in any other manner that it may determine in accordance with applicable law, into Dollars transferable to the United States and distributable to the Holders entitled thereto, the Depositary shall convert or cause to be converted, by sale or in any other manner that it may reasonably determine, such Foreign Currency into Dollars, and shall distribute such Dollars (net of the fees and charges set forth in the Fee Schedule attached hereto as Exhibit B, and applicable taxes withheld) in accordance with the terms of the applicable sections of the Deposit Agreement.  The Depositary and/or its agent (which may be a division, branch or Affiliate of the Depositary) may act as principal for any conversion of Foreign Currency.  If the Depositary shall have distributed warrants or other instruments that entitle the holders thereof to such Dollars, the Depositary shall distribute such Dollars to the holders of such warrants and/or instruments upon surrender thereof for cancellation, in either case without liability for interest thereon.  Such distribution may be made upon an averaged or other practicable basis without regard to any distinctions among Holders on account of any application of exchange restrictions or otherwise.

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If such conversion or distribution generally or with regard to a particular Holder can be effected only with the approval or license of any government or agency thereof, the Depositary shall have authority to file such application for approval or license, if any, as it may deem desirable.  In no event, however, shall the Depositary be obligated to make such a filing.

If at any time the Depositary shall determine that in its judgment the conversion of any Foreign Currency and the transfer and distribution of proceeds of such conversion received by the Depositary is not practicable or lawful, or if any approval or license of any governmental authority or agency thereof that is required for such conversion, transfer and distribution is denied or, in the opinion of the Depositary, not obtainable at a reasonable cost or within a reasonable period, the Depositary may, in its discretion, (i) make such conversion and distribution in Dollars to the Holders for whom such conversion, transfer and distribution is lawful and practicable, (ii) distribute the Foreign Currency (or an appropriate document evidencing the right to receive such Foreign Currency) to Holders for whom this is lawful and practicable, or (iii) hold (or cause the Custodian to hold) such Foreign Currency (without liability for interest thereon) for the respective accounts of the Holders entitled to receive the same.

Section 4.9        Fixing of ADS Record Date.  Whenever (a) the Depositary shall receive notice of the fixing of a record date by the Company for the determination of holders of Deposited Securities entitled to receive any distribution (whether in cash, Shares, rights, or other distribution), (b) for any reason the Depositary causes a change in the number of Shares that are represented by each ADS, (c) the Depositary shall receive notice of any meeting of, or solicitation of consents or proxies of, holders of Shares or other Deposited Securities, or (d) the Depositary shall find it necessary or convenient in connection with the giving of any notice, solicitation of any consent or any other matter, the Depositary shall fix the record date (the “ADS Record Date”) for the determination of the Holders of ADS(s) who shall be entitled to receive such distribution, to give instructions for the exercise of voting rights at any such meeting, to give or withhold such consent, to receive such notice or solicitation or to otherwise take action, or to exercise the rights of Holders with respect to such changed number of Shares represented by each ADS.  The Depositary shall make reasonable efforts to establish the ADS Record Date as closely as possible to the applicable record date for the Deposited Securities (if any) set by the Company in England and Walesand shall not announce the establishment of any ADS Record Date prior to the relevant corporate action having been made public by the Company (if such corporate action affects the Deposited Securities).  Subject to applicable law and the provisions of Section 4.1 through 4.8 and to the other terms and conditions of the Deposit Agreement, only the Holders of ADSs at the close of business in New York on such ADS Record Date shall be entitled to receive such distribution, to give such voting instructions, to receive such notice or solicitation, or otherwise take action.

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Section 4.10      Voting of Deposited Securities.  As soon as practicable after receipt of notice of any meeting at which the holders of Deposited Securities are entitled to vote, or of solicitation of consents or proxies from holders of Deposited Securities, the Depositary shall fix the ADS Record Date in respect of such meeting or solicitation of consent or proxy in accordance with Section 4.9.  The Depositary shall, if requested by the Company in writing in a timely manner (except as otherwise contemplated in this Section 4.10, the Depositary having no obligation to take any further action if the request shall not have been received by the Depositary at least thirty (30) days (or such lesser number of days as mutually agreed to in writing by the Company and the Depositary) prior to the date of such vote or meeting), at the Company’s expense and provided no U.S. legal prohibitions exist, distribute as soon as practicable after receipt thereof to Holders as of the ADS Record Date: (a) such notice of meeting or solicitation of consent or proxy, (b) a statement that the Holders at the close of business on the ADS Record Date will be entitled, subject to any applicable law, the provisions of the Deposit Agreement, the Articles of Association and the provisions of or governing the Deposited Securities (which provisions, if any, shall be summarized in pertinent part by the Company), to instruct the Depositary as to the exercise of the voting rights, if any, pertaining to the Deposited Securities represented by such Holder’s ADSs, and (c) a brief statement as to the manner in which such voting instructions may be given or deemed to have been given in accordance with this Section 4.10, it being acknowledged that if no instructions are received prior to the deadline set for such purposes to the Depositary to give a discretionary proxy to a person designated by the Company.

The Company shall use its reasonable endeavors to notify the Depositary in writing of its intention to convene a meeting where the holders of Deposited Securities are entitled to vote, at least thirty (30) days (or such lesser number of days as mutually agreed to in writing by the Company and the Depositary) prior to the date of the proposed meeting.

Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to timely request that the Depositary distribute the information as provided for in this Section 4.10, if the Depositary so agrees after consultation with the Company, the Depositary will use commercially reasonable efforts to perform the actions contemplated in this Section 4.10, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.10, where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

Notwithstanding anything contained in the Deposit Agreement or any ADR, the Depositary may, to the extent not prohibited by law or regulations, or by the requirements of the stock exchange on which the ADSs are listed, in lieu of distribution of the materials provided to the Depositary in connection with any meeting of, or solicitation of consents or proxies from, holders of Deposited Securities, distribute to the Holders a notice that provides Holders with, or otherwise publicizes to Holders, instructions on how to retrieve such materials or receive such materials upon request (e.g., by reference to a website containing the materials for retrieval or a contact for requesting copies of the materials).

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The Depositary has been advised by the Company that under the Articles of Association as in effect on the date of the Deposit Agreement, voting at any meeting of shareholders of the Company is by show of hands unless (before or on the declaration of the result of the show of hands) a poll is demanded.  The Depositary will not join in demanding a poll, whether or not requested to do so by Holders of ADSs.  Under the Articles of Association as in effect on the date of the Deposit Agreement, at any meeting a resolution put to the vote of the meeting shall be decided on a show of hands, unless before or upon the declaration of the result of the show of hands a poll is demanded by: (a) the chairman of the meeting; (b) not less than five (5) members of the Company having the right to vote at the meeting; (c) a member or members of the Company representing not less than one tenth of the total voting rights of all the members of the Company having the right to vote at the meeting; or (d) a member or members of the Company holding shares in the Company conferring a right to vote at the meeting, being shares on which an aggregate sum has been paid up equal to not less than one tenth of the total sum paid up on all the shares conferring that right..

Voting instructions may be given only in respect of a number of ADSs representing an integral number of Deposited Securities.  Upon the timely receipt from a Holder of ADSs as of the ADS Record Date of voting instructions in the manner specified by the Depositary, the Depositary shall endeavor, insofar as practicable and permitted under applicable law, the provisions of the Deposit Agreement, Articles of Association and the provisions of the Deposited Securities, to vote, or cause the Custodian to vote, the Deposited Securities (in person or by proxy) represented by such Holder’s ADSs as follows: (a) in the event voting takes place at a shareholders’ meeting by a show of hands, the Depositary will instruct the Custodian to vote all Deposited Securities in accordance with the voting instructions received timely from a majority of Holders of ADSs who provided voting instructions, and (b) in the event voting takes place at a shareholders’ meeting by poll, the Depositary will instruct the Custodian to vote the Deposited Securities in accordance with the voting instructions timely received from the Holders of ADSs.  If voting is by poll and the Depositary does not receive voting instructions from a Holder as of the ADS Record Date on or before the date established by the Depositary for such purpose, such Holder shall be deemed, and the Depositary shall (unless otherwise specified in the notice distributed to Holders) deem such Holder, to have instructed the Depositary to give a discretionary proxy to a person designated by the Company to vote the Deposited Securities; provided, however, that no such discretionary proxy shall be given by the Depositary with respect to any matter to be voted upon as to which the Company informs the Depositary that (a) the Company does not wish such proxy to be given, (b) substantial opposition exists, or (c) the rights of holders of Deposited Securities may be materially adversely affected.

Deposited Securities represented by ADSs for which no timely voting instructions are received by the Depositary from the Holder shall not be voted (except (a) in the case voting is by show of hands, in which case the Depositary will instruct the Custodian to vote all Deposited Securities in accordance with the voting instructions received from a majority of Holders of ADSs who provided timely voting instructions, and (b) as contemplated in this Section 4.10).  Neither the Depositary nor the Custodian shall under any circumstances exercise any discretion as to voting and neither the Depositary nor the Custodian shall vote, attempt to exercise the right to vote, or in any way make use of, for purposes of establishing a quorum or otherwise, the Deposited Securities represented by ADSs, except pursuant to and in accordance with the voting instructions timely received from Holders or as otherwise contemplated herein.  If the Depositary timely receives voting instructions from a Holder which fail to specify the manner in which the Depositary is to vote the Deposited Securities represented by such Holder’s ADSs, the Depositary will deem such Holder (unless otherwise specified in the notice distributed to Holders) to have instructed the Depositary to vote in favor of the items set forth in such voting instructions.

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Notwithstanding anything to the contrary contained herein, the Depositary shall, if so requested in writing by the Company, represent all Deposited Securities (whether or not voting instructions have been received in respect of such Deposited Securities from Holders as of the ADS Record Date) for the sole purpose of establishing quorum at a meeting of shareholders.

Notwithstanding anything to the contrary contained in the Deposit Agreement or any ADR, the Depositary shall not have any obligation to take any action with respect to any meeting, or solicitation of consents or proxies, of holders of Deposited Securities if the taking of such action would violate U.S. or English laws.  The Company agrees to take any and all actions reasonably necessary and as permitted by any applicable law to enable Holders and Beneficial Owners to exercise the voting rights accruing to the Deposited Securities and to deliver to the Depositary an opinion of U.S. counsel addressing any actions requested to be taken if so requested by the Depositary.

There can be no assurance that Holders generally or any Holder in particular will receive the notice described above with sufficient time to enable the Holder to return voting instructions to the Depositary in a timely manner.

Section 4.11      Changes Affecting Deposited Securities.  Upon any change in nominal or par value, split‑up, cancellation, consolidation or any other reclassification of Deposited Securities, or upon any recapitalization, reorganization, merger, consolidation or sale of assets affecting the Company or to which it is a party, any property which shall be received by the Depositary or the Custodian in exchange for, or in conversion of, or replacement of, or otherwise in respect of, such Deposited Securities shall, to the extent permitted by law, be treated as new Deposited Property under the Deposit Agreement, and the ADSs shall, subject to the provisions of the Deposit Agreement, any ADR(s) evidencing such ADSs and applicable law, represent the right to receive such additional or replacement Deposited Property.  In giving effect to such change, split-up, cancellation, consolidation or other reclassification of Deposited Securities, recapitalization, reorganization, merger, consolidation or sale of assets, the Depositary may, with the Company’s approval, and shall, if the Company shall so request, subject to the terms of the Deposit Agreement (including, without limitation, (a) the applicable fees and charges of, and expenses incurred by, the Depositary, and (b) taxes) and receipt of an opinion of counsel to the Company reasonably satisfactory to the Depositary that such actions are not in violation of any applicable laws or regulations, (i) issue and deliver additional ADSs as in the case of a stock dividend on the Shares, (ii) amend the Deposit Agreement and the applicable ADRs, (iii) amend the applicable Registration Statement(s) on Form F-6 as filed with the Commission in respect of the ADSs, (iv) call for the surrender of outstanding ADRs to be exchanged for new ADRs, and (v) take such other actions as the Depositary, in consultation with the Company, considers appropriate to reflect the transaction with respect to the ADSs.  The Company agrees to, jointly with the Depositary, amend the Registration Statement on Form F-6 as filed with the Commission to permit the issuance of such new form of ADRs.  Notwithstanding the foregoing, in the event that any Deposited Property so received may not be, upon consultation with the Company, lawfully distributed to some or all Holders, the Depositary may, with the Company’s approval, and shall, if the Company requests, subject to receipt of an opinion of Company’s counsel reasonably satisfactory to the Depositary that such action is not in violation of any applicable laws or regulations, sell such Deposited Property at public or private sale, at such place or places and upon such terms as it may deem proper and may allocate the net proceeds of such sales (net of (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) for the account of the Holders otherwise entitled to such Deposited Property upon an averaged or other practicable basis without regard to any distinctions among such Holders and distribute the net proceeds so allocated to the extent practicable as in the case of a distribution received in cash pursuant to Section 4.1.  The Depositary shall not be responsible for (i) any failure to determine whether it may be lawful or practicable to make such Deposited Property available to Holders in general or to any Holder in particular, (ii) any foreign exchange exposure or loss incurred in connection with such sale or (iii) any liability to the purchaser of such Deposited Property.

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Section 4.12      Available Information.  The Company is subject to the periodic reporting requirements of the Exchange Act and, accordingly, is required to file or furnish certain reports with the Commission.  These reports can be retrieved from the Commission's website (www.sec.gov) and can be inspected and copied at the public reference facilities maintained by the Commission located (as of the date of the Deposit Agreement) at 100 F Street, N.E., Washington D.C.  20549.

Section 4.13      Reports.  The Depositary shall make available for inspection by Holders at its Principal Office, this Deposit Agreement, the provisions of or governing Deposited Securities and any reports and communications, including any proxy soliciting materials, received from the Company which are both (a) received by the Depositary, the Custodian, or the nominee of either of them as the holder of the Deposited Property and (b) made generally available to the holders of such Deposited Property by the Company.  The Depositary shall also provide or make available to Holders copies of such reports when furnished by the Company pursuant to Section 5.6.

Section 4.14      List of Holders.  Promptly upon written request by the Company, the Depositary shall furnish to the Company a list, as of a recent date, of the names, addresses and holdings of ADSs of all Holders.  Upon the written request of the Company, the Depositary shall, to the extent practicable, obtain and deliver to the Company a list of non-objecting Beneficial Owners.

Section 4.15      Taxation.  The Depositary will, and will instruct the Custodian to, forward to the Company or its agents such information from its records as the Company may reasonably request to enable the Company or its agents to file the necessary tax reports with governmental authorities or agencies.  The Depositary, the Custodian or the Company and its agents may file such reports as are necessary to reduce or eliminate applicable taxes on dividends and on other distributions in respect of Deposited Property under applicable tax treaties or laws for the Holders and Beneficial Owners.  In accordance with instructions from the Company and to the extent practicable, the Depositary or the Custodian will take reasonable administrative actions to obtain tax refunds, reduced withholding of tax at source on dividends and other benefits under applicable tax treaties or laws with respect to dividends and other distributions on the Deposited Property.  As a condition to receiving such benefits, Holders and Beneficial Owners of ADSs may be required from time to time, and in a timely manner, to file such proof of taxpayer status, residence and beneficial ownership (as applicable), to execute such certificates and to make such representations and warranties, or to provide any other information or documents, as the Company, the Depositary or the Custodian may deem necessary or proper to fulfill the Depositary’s or the Custodian’s obligations under applicable law.  The Depositary, the Custodian and the Company shall not have any obligation or liability to any person if any Holder or Beneficial Owner fails to provide such information or if such information does not reach the relevant tax authorities in time for any Holder or Beneficial Owner to obtain the benefits of any tax treatment.  The Holders and Beneficial Owners shall indemnify the Depositary, the Company, the Custodian and any of their respective directors, employees, agents and Affiliates against, and hold each of them harmless from, any claims by any governmental authority with respect to taxes, additions to tax, penalties or interest arising out of any refund of taxes, reduced rate of withholding at source or other tax benefit obtained.

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If the Company (or any of its agents) withholds from any distribution any amount on account of taxes or governmental charges, or pays any other tax in respect of such distribution (e.g., stamp duty tax, capital gains or other similar tax), the Company shall (and shall cause such agent to) remit promptly to the Depositary information about such taxes or governmental charges withheld or paid, and, if so requested, the tax receipt (or other proof of payment to the applicable governmental authority) therefor, in each case, in a form satisfactory to the Depositary.  The Depositary shall, to the extent required by U.S. law, report to Holders any taxes withheld by it or the Custodian, and, if such information is provided to it by the Company, any taxes withheld by the Company.  The Depositary and the Custodian shall not be required to provide the Holders with any evidence of the remittance by the Company (or its agents) of any taxes withheld, or of the payment of taxes by the Company, except to the extent the evidence is provided by the Company to the Depositary or the Custodian, as applicable.  Neither the Depositary or the Custodian shall be liable for the failure by any Holder or Beneficial Owner to obtain the benefits of credits on the basis of non‑U.S. tax paid against such Holder’s or Beneficial Owner’s income tax liability.  The Depositary is under no obligation to provide the Holders and Beneficial Owners with any information about the tax status of the Company.  The Depositary shall not incur any liability for any tax consequences that may be incurred by Holders and Beneficial Owners on account of their ownership of the ADSs, including without limitation, tax consequences resulting from the Company (or any of its subsidiaries) being treated as a “Passive Foreign Investment Company” (in each case as defined in the U.S. Internal Revenue Code of 1986, as amended, and the regulations issued thereunder) or otherwise.

ARTICLE V

THE DEPOSITARY, THE CUSTODIAN AND THE COMPANY

Section 5.1        Maintenance of Office and Transfer Books by the Registrar.  Until termination of the Deposit Agreement in accordance with its terms, the Registrar shall maintain in the Borough of Manhattan, the City of New York, an office and facilities for the issuance and delivery of ADSs, the acceptance for surrender of ADS(s) for the purpose of withdrawal of Deposited Securities, the registration of issuances, cancellations, transfers, combinations and split‑ups of ADS(s) and, if applicable, to countersign ADRs evidencing the ADSs so issued, transferred, combined or split-up, in each case in accordance with the provisions of the Deposit Agreement.

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The Registrar shall keep books for the registration of ADSs which at all reasonable times shall be open for inspection by the Company and by the Holders of such ADSs, provided that such inspection shall not be, to the Registrar’s knowledge, for the purpose of communicating with Holders of such ADSs in the interest of a business or object other than the business of the Company or other than a matter related to the Deposit Agreement or the ADSs.  The Company shall have the right to examine and copy the transfer and registration records of the Depositary or its agent, take copies thereof and require the Depositary or its agent, the Registrar and any co-transfer agents or co-registrars to supply copies of such portions of such records, to the extent practicable, as the Company may request from time to time.

The Registrar may close the transfer books with respect to the ADSs, at any time or from time to time, when deemed necessary or advisable by it in good faith in connection with the performance of its duties hereunder, or at the reasonable written request of the Company subject, in all cases, to Section 7.8.

If any ADSs are listed on one or more stock exchanges or automated quotation systems in the United States, the Depositary shall act as Registrar or appoint a Registrar or one or more co‑registrars for registration of issuances, cancellations, transfers, combinations and split-ups of ADSs and, if applicable, to countersign ADRs evidencing the ADSs so issued, transferred, combined or split-up,in accordance with any requirements of such exchanges or systems.  Such Registrar or co-registrars may, with notice to the Company as promptly as practicable, be removed and a substitute or substitutes appointed by the Depositary.

Section 5.2        Exoneration.  Notwithstanding any other provision of the Deposit Agreement or any ADR(s) to the contrary, neither the Depositary nor the Company shall be obligated to do or perform any act which is inconsistent with the provisions of the Deposit Agreement or incur any liability (to the extent not limited by Section 7.8(b)) (i) if the Depositary, the Custodian, the Company or their respective agents shall be prevented or forbidden from, or delayed in, doing or performing any act or thing required or contemplated by the terms of the Deposit Agreement, by reason of any provision of any present or future law or regulation of the United States, England and Wales, or any other country, or of any other governmental authority or regulatory authority or stock exchange, or on account of the potential criminal or civil penalties or restraint, or by reason of any provision, present or future, of the Articles of Association or any provision of or governing any Deposited Securities, or by reason of any act of God or war or other circumstances beyond its control (including, without limitation, nationalization, expropriation, currency restrictions, work stoppage, strikes, civil unrest, acts of terrorism, revolutions, rebellions, explosions and computer failure), (ii) by reason of any exercise of, or failure to exercise, any discretion provided for in the Deposit Agreement or in the Articles of Association or provisions of or governing Deposited Securities, (iii) for any action or inaction in reliance upon the advice of or information from legal counsel, accountants, any person presenting Shares for deposit, any Holder, any Beneficial Owner or authorized representative thereof, or any other person believed by it in good faith to be competent to give such advice or information, (iv) for the inability by a Holder or Beneficial Owner to benefit from any distribution, offering, right or other benefit which is made available to holders of Deposited Securities but is not, under the terms of the Deposit Agreement, made available to Holders of ADSs, (v) for any action or inaction of any clearing or settlement system (and any participant thereof) for the Deposited Property or the ADSs, or (vi) for any consequential or punitive damages (including lost profits) for any breach of the terms of the Deposit Agreement.

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The Depositary, its controlling persons, its agents, any Custodian and the Company, its Affiliates, its controlling persons and its agents may rely and shall be protected in acting upon any written notice, request or other document believed by it to be genuine and to have been signed or presented by the proper party or parties.

Section 5.3        Standard of Care.  The Company and the Depositary and their respective Affiliates, directors, officers, employees and agents assume no obligation and shall not be subject to any liability under the Deposit Agreement or any ADRs to any Holder(s) or Beneficial Owner(s), except that the Company and the Depositary agree to perform their respective obligations specifically set forth in the Deposit Agreement or the applicable ADRs without negligence or bad faith.

Without limitation of the foregoing, neither the Depositary, nor the Company, nor any of their respective Affiliates, directors, officers, controlling persons, employees or agents, shall be under any obligation to appear in, prosecute or defend any action, suit or other proceeding in respect of any Deposited Property or in respect of the ADSs, which in its opinion may involve it in expense or liability, unless indemnity satisfactory to it against all expense (including fees and disbursements of counsel) and liability be furnished as often as may be required (and no Custodian shall be under any obligation whatsoever with respect to such proceedings, the responsibility of the Custodian being solely to the Depositary).

The Depositary and its agents shall not be liable for any failure to carry out any instructions to vote any of the Deposited Securities, or for the manner in which any vote is cast or the effect of any vote, provided that any such action or omission is in good faith and without negligence and in accordance with the terms of the Deposit Agreement.  The Depositary shall not incur any liability for any failure to accurately determine whether any distribution or action may be lawful or reasonably practicable, for any investment risk associated with acquiring an interest in the Deposited Property, for the validity or worth of the Deposited Property, for the value of any Deposited Property or any distribution thereon, for any interest on Deposited Property, for any tax consequences that may result from the ownership of ADSs, Shares or other Deposited Property, for the credit‑worthiness of any third party. The Depositary shall not incur any liability for the content of any information submitted to it by the Company for distribution to the Holders or for any inaccuracy of any translation thereof, for allowing any rights to lapse upon the terms of the Deposit Agreement, for the failure or timeliness of any notice from the Company, or for any action of or failure to act by, or any information provided or not provided by, DTC or any DTC Participant.

The Depositary shall not be liable for any acts or omissions made by a successor depositary whether in connection with a previous act or omission of the Depositary or in connection with any matter arising wholly after the removal or resignation of the Depositary, provided that in connection with the issue out of which such potential liability arises the Depositary performed its obligations without negligence or bad faith while it acted as Depositary.

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Section 5.4        Resignation and Removal of the Depositary; Appointment of Successor Depositary.  The Depositary may at any time resign as Depositary hereunder by written notice of resignation delivered to the Company, such resignation to be effective on the earlier of (i) the 90th day after delivery thereof to the Company (whereupon the Depositary shall be entitled to take the actions contemplated in Section 6.2), or (ii) the appointment by the Company of a successor depositary and its acceptance of such appointment as hereinafter provided.

The Depositary, in such capacity, may at any time be removed by the Company by written notice of such removal, which removal shall be effective on the later of (i) the 90th day after delivery thereof to the Depositary (whereupon the Depositary shall be entitled to take the actions contemplated in Section 6.2), or (ii) the appointment by the Company of a successor depositary and its acceptance of such appointment as hereinafter provided.

In case at any time the Depositary acting hereunder shall resign or be removed, the Company shall use its best efforts to appoint a successor depositary, which shall be a bank or trust company having an office in the Borough of Manhattan, the City of New York.  Every successor depositary shall be required by the Company to execute and deliver to its predecessor and to the Company an instrument in writing accepting its appointment hereunder, and thereupon such successor depositary, without any further act or deed (except as required by applicable law), shall become fully vested with all the rights, powers, duties and obligations of its predecessor (other than as contemplated in Sections 5.8 and 5.9).  The predecessor depositary, upon payment of all sums due it and on the written request of the Company, shall, (i) execute and deliver an instrument transferring to such successor all rights and powers of such predecessor hereunder (other than as contemplated in Sections 5.8 and 5.9), (ii) duly assign, transfer and deliver all of the Depositary’s right, title and interest to the Deposited Property to such successor, and (iii) deliver to such successor a list of the Holders of all outstanding ADSs and such other information relating to ADSs and Holders thereof as the successor may reasonably request.  Any such successor depositary shall promptly provide notice of its appointment to such Holders.

Any entity into or with which the Depositary may be merged or consolidated shall be the successor of the Depositary without the execution or filing of any document or any further act.

Section 5.5        The Custodian.  The Depositary has initially appointed Citibank, N.A. (London) as Custodian for the purpose of the Deposit Agreement.  The Custodian or its successors in acting hereunder shall be authorized to act as custodian in England and Wales and shall be subject at all times and in all respects to the direction of the Depositary for the Deposited Property for which the Custodian acts as custodian and shall be responsible solely to it.  If any Custodian resigns or is discharged from its duties hereunder with respect to any Deposited Property and no other Custodian has previously been appointed hereunder, the Depositary shall, with notice to the Company as soon as practicable, promptly appoint a substitute custodian.  The Depositary shall require such resigning or discharged Custodian to Deliver, or cause the Delivery of, the Deposited Property held by it, together with all such records maintained by it as Custodian with respect to such Deposited Property as the Depositary may request, to the Custodian designated by the Depositary.  Whenever the Depositary determines, in its discretion, that it is appropriate to do so, it may appoint an additional custodian with respect to any Deposited Property, or discharge the Custodian with respect to any Deposited Property and appoint a substitute custodian, which shall thereafter be Custodian hereunder with respect to the Deposited Property.  Immediately upon any such change, the Depositary shall give notice thereof in writing to all Holders of ADSs, each other Custodian and the Company.

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Citibank may at any time act as Custodian of the Deposited Property pursuant to the Deposit Agreement, in which case any reference to Custodian shall mean Citibank solely in its capacity as Custodian pursuant to the Deposit Agreement.  Notwithstanding anything contained in the Deposit Agreement or any ADR to the contrary, the Depositary shall not be obligated to give notice to the Company, any Holders of ADSs or any other Custodian of its acting as Custodian pursuant to the Deposit Agreement.

Upon the appointment of any successor depositary, any Custodian then acting hereunder shall, unless otherwise instructed by the Depositary, continue to be the Custodian of the Deposited Property without any further act or writing, and shall be subject to the direction of the successor depositary.  The successor depositary so appointed shall, nevertheless, on the written request of any Custodian, execute and deliver to such Custodian all such instruments as may be proper to give to such Custodian full and complete power and authority to act on the direction of such successor depositary.

Section 5.6        Notices and Reports.  On or before the first date on which the Company gives notice, by publication or otherwise, of any meeting of holders of Shares or other Deposited Securities, or of any adjourned meeting of such holders, or of the taking of any action by such holders other than at a meeting, or of the taking of any action in respect of any cash or other distributions or the offering of any rights in respect of Deposited Securities, the Company shall transmit to the Depositary and the Custodian a copy of the notice thereof in the English language but otherwise in the form given or to be given to holders of Shares or other Deposited Securities. The Company shall also furnish to the Custodian and the Depositary a summary, in English, of any applicable provisions or proposed provisions of the Articles of Association that may be relevant or pertain to such notice of meeting or be the subject of a vote thereat.

The Company will also transmit to the Depositary (a) an English language version of the other notices, reports and communications which are made generally available by the Company to holders of its Shares or other Deposited Securities and (b) the English‑language versions of the Company’s annual and semi‑annual reports prepared in accordance with the applicable requirements of the Commission.  The Depositary shall arrange, at the request of the Company and at the Company’s expense, to provide copies thereof to all Holders or make such notices, reports and other communications available to all Holders on a basis similar to that for holders of Shares or other Deposited Securities or on such other basis as the Company may advise the Depositary or as may be required by any applicable law, regulation or stock exchange requirement.  The Company has delivered to the Depositary and the Custodian a copy of the Company’s Articles of Association along with the provisions of or governing the Shares and any other Deposited Securities issued by the Company in connection with such Shares, and promptly upon any amendment thereto or change therein, the Company shall deliver to the Depositary and the Custodian a copy of such amendment thereto or change therein.  The Depositary may rely upon such copy for all purposes of the Deposit Agreement.

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The Depositary will, at the expense of the Company, make available a copy of any such notices, reports or communications issued by the Company and delivered to the Depositary for inspection by the Holders of the ADSs at the Depositary’s Principal Office, at the office of the Custodian and at any other designated transfer office.

Section 5.7        Issuance of Additional Shares, ADSs etc.  The Company agrees that in the event it or any of its Affiliates proposes (i) an issuance, sale or distribution of additional Shares, (ii) an offering of rights to subscribe for Shares or other Deposited Securities, (iii) an issuance or assumption of securities convertible into or exchangeable for Shares, (iv) an issuance of rights to subscribe for securities convertible into or exchangeable for Shares, (v) an elective dividend of cash or Shares, (vi) a redemption of Deposited Securities, (vii) a meeting of holders of Deposited Securities, or solicitation of consents or proxies, relating to any reclassification of securities, merger or consolidation or transfer of assets, (viii) any assumption, reclassification, recapitalization, reorganization, merger, consolidation or sale of assets which affects the Deposited Securities, or (ix) a distribution of securities other than Shares, it will obtain U.S. legal advice and take all steps necessary to ensure that the application of the proposed transaction to Holders and Beneficial Owners does not violate the registration provisions of the Securities Act, or any other applicable laws (including, without limitation, the Investment Company Act of 1940, as amended, the Exchange Act and the securities laws of the states of the U.S.).  In support of the foregoing and in connection with any such transactions, the Company will furnish to the Depositary, if applicable, (a) a written opinion of U.S. counsel (reasonably satisfactory to the Depositary) stating whether such transaction (1) requires a registration statement under the Securities Act to be in effect or (2) is exempt from the registration requirements of the Securities Act and (b) an opinion of English counsel stating that (1) making the transaction available to Holders and Beneficial Owners does not violate the laws or regulations of England and Wales and (2) all requisite regulatory consents and approvals, if any, have been obtained in England and Wales.  If the filing of a registration statement is required, the Depositary shall not have any obligation to proceed with the transaction unless it shall have received evidence reasonably satisfactory to it that such registration statement has been declared, or has otherwise become, effective.  If, being advised by counsel, the Company determines that a transaction is required to be registered under the Securities Act, the Company will either (i) register such transaction to the extent necessary, (ii) alter the terms of the transaction to avoid the registration requirements of the Securities Act or (iii) direct the Depositary to take specific measures, in each case as contemplated in the Deposit Agreement, to prevent such transaction from violating the registration requirements of the Securities Act.  The Company agrees with the Depositary that neither the Company nor any of its Affiliates will at any time (i) deposit any Shares or other Deposited Securities, either upon original issuance or upon a sale of Shares or other Deposited Securities previously issued and reacquired by the Company or by any such Affiliate, or (ii) issue additional Shares, rights to subscribe for such Shares, securities convertible into or exchangeable for Shares or rights to subscribe for such securities or distribute securities other than Shares, unless such transaction and the securities issuable in such transaction do not violate the registration provisions of the Securities Act, or any other applicable laws (including, without limitation, the Investment Company Act of 1940, as amended, the Exchange Act and the securities laws of the states of the U.S.).

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Notwithstanding anything to the contrary contained in the Deposit Agreement, nothing in the Deposit Agreement shall be deemed to obligate the Company to file any registration statement in respect of any proposed transaction.

Section 5.8        Indemnification.  The Depositary agrees to indemnify the Company and its directors, officers, employees, agents and Affiliates against, and hold each of them harmless from, any direct loss, liability, tax, charge or expense of any kind whatsoever (including, but not limited to, the reasonable fees and expenses of counsel) which may arise out of acts performed or omitted by the Depositary or the Custodian (for so long as the Custodian is a branch of Citibank N.A.) under the terms hereof due to the negligence or bad faith of the Depositary or the Custodian (for so long as the Custodian is a branch of Citibank N.A.), as applicable.

The Company agrees to indemnify the Depositary, the Custodian and any of their respective directors, officers, employees, agents and Affiliates against, and hold each of them harmless from, any direct loss, liability, tax, charge or expense of any kind whatsoever (including, but not limited to, the reasonable fees and expenses of counsel) that may arise (a) out of, or in connection with, any offer, issuance, sale, resale, transfer, deposit or withdrawal of ADRs, ADSs, the Shares, or other Deposited Securities, as the case may be, (b) out of, or as a result of, any offering documents in respect thereof or (c) out of acts performed or omitted, including, but not limited to, any delivery by the Depositary on behalf of the Company of information regarding the Company, in connection with the Deposit Agreement, any ancillary or supplemental agreement entered into between the Company and the Depositary, the ADRs, the ADSs, the Shares, or any Deposited Property, in any such case (i) by the Depositary, the Custodian or any of their respective directors, officers, employees, agents and Affiliates, except to the extent such loss, liability, tax, charge or expense is due to the negligence or bad faith of any of them, or (ii) by the Company or any of its directors, officers, employees, agents and Affiliates.  The Company shall not indemnify the Depositary, the Custodian and any of their respective directors, officers, employees, agents and Affiliates against any liability or expense arising out of information relating to the Depositary or such Custodian, as the case may be, furnished to the Company by the Depositary or such Custodian for use in any registration statement, prospectus or preliminary prospectus relating to any Deposited Securities represented by the ADSs.

The obligations set forth in this Section shall survive the termination of the Deposit Agreement and the succession or substitution of any party hereto.

Any person seeking indemnification hereunder (an “indemnified person”) shall notify the person from whom it is seeking indemnification (the “indemnifying person”) of the commencement of any indemnifiable action or claim promptly after such indemnified person becomes aware of such commencement (provided that the failure to make such notification shall not affect such indemnified person’s rights to seek indemnification except to the extent the indemnifying person is materially prejudiced by such failure) and shall consult in good faith with the indemnifying person as to the conduct of the defense of such action or claim that may give rise to an indemnity hereunder, which defense shall be reasonable in the circumstances.  No indemnified person shall compromise or settle any action or claim that may give rise to an indemnity hereunder without the consent of the indemnifying person, which consent shall not be unreasonably withheld.

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Section 5.9        ADS Fees and Charges.  The Company, the Holders, the Beneficial Owners, persons depositing Shares or withdrawing Deposited Securities in connection with the issuance and cancellation of ADSs, and persons receiving ADSs upon issuance or whose ADSs are being cancelled shall be required to pay to the Depositary the Depositary’s fees and related charges identified as payable by them respectively in the Fee Schedule attached hereto as Exhibit B.  All ADS fees and charges so payable may be deducted from distributions or must be remitted to the Depositary, or its designee,and may, at any time and from time to time, be changed by agreement between the Depositary and the Company, but, in the case of ADS fees and charges payable by Holders and Beneficial Owners, only in the manner contemplated in Section 6.1.  The Depositary shall provide, without charge, a copy of its latest ADS fee schedule to anyone upon request.

ADS fees and charges for (i) the issuance of ADSs and (ii) the cancellation of ADSs will be payable by the person for whom the ADSs are so issued by the Depositary (in the case of ADS issuances) and by the person for whom ADSs are being cancelled (in the case of ADS cancellations).  In the case of ADSs issued by the Depositary into DTC or presented to the Depositary via DTC, the ADS issuance and cancellation fees and charges will be payable by the DTC Participant(s) receiving the ADSs from the Depositary or the DTC Participant(s) holding the ADSs being cancelled, as the case may be, on behalf of the Beneficial Owner(s) and will be charged by the DTC Participant(s) to the account(s) of the applicable Beneficial Owner(s) in accordance with the procedures and practices of the DTC participant(s) as in effect at the time.  ADS fees and charges in respect of distributions and the ADS service fee are payable by Holders as of the applicable ADS Record Date established by the Depositary.  In the case of distributions of cash, the amount of the applicable ADS fees and charges is deducted from the funds being distributed.  In the case of (i) distributions other than cash and (ii) the ADS service fee, the applicable Holders as of the ADS Record Date established by the Depositary will be invoiced for the amount of the ADS fees and charges and such ADS fees may be deducted from distributions made to Holders.  For ADSs held through DTC, the ADS fees and charges for distributions other than cash and the ADS service fee may be deducted from distributions made through DTC, and may be charged to the DTC Participants in accordance with the procedures and practices prescribed by DTC from time to time and the DTC Participants in turn charge the amount of such ADS fees and charges to the Beneficial Owners for whom they hold ADSs.  In the case of (i) registration of ADS transfers, the ADS transfer fee will be payable by the ADS Holder whose ADSs are being transferred or by the person to whom the ADSs are transferred, and (ii) conversion of ADSs of one series for ADSs of another series, the ADS conversion fee will be payable by the Holder whose ADSs are converted or by the person to whom the converted ADSs are delivered.

The Depositary may reimburse the Company for certain expenses incurred by the Company in respect of the ADR program established pursuant to the Deposit Agreement, by making available a portion of the ADS fees charged in respect of the ADR program or otherwise, upon such terms and conditions as the Company and the Depositary agree from time to time.  The Company shall pay to the Depositary such fees and charges, and reimburse the Depositary for such out-of-pocket expenses, as the Depositary and the Company may agree from time to time.  Responsibility for payment of such fees, charges and reimbursements may from time to time be changed by agreement between the Company and the Depositary.  Unless otherwise agreed, the Depositary shall present its statement for such fees, charges and reimbursements to the Company once every three months.  The charges and expenses of the Custodian are for the sole account of the Depositary.

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The obligation of Holders and Beneficial Owners to pay ADS fees and charges shall survive the termination of the Deposit Agreement.  As to any Depositary, upon the resignation or removal of such Depositary as described in Section 5.4, the right to collect ADS fees and charges shall extend for those ADS fees and charges incurred prior to the effectiveness of such resignation or removal.

Section 5.10      Restricted Securities Owners.  The Company agrees to advise in writing each of the persons or entities who, to the knowledge of the Company, holds Restricted Securities that such Restricted Securities are ineligible for deposit hereunder (except under the circumstances contemplated in Section 2.14) and, to the extent practicable, shall require each of such persons to represent in writing that such person will not deposit Restricted Securities hereunder (except under the circumstances contemplated in Section 2.14).

ARTICLE VI

AMENDMENT AND TERMINATION

Section 6.1        Amendment/Supplement.  Subject to the terms and conditions of this Section 6.1 and applicable law, the ADRs outstanding at any time, the provisions of the Deposit Agreement and the form of ADR attached hereto and to be issued under the terms hereof may at any time and from time to time be amended or supplemented by written agreement between the Company and the Depositary in any respect which they may deem necessary or desirable without the prior written consent of the Holders or Beneficial Owners.  Any amendment or supplement which shall impose or increase any fees or charges (other than charges in connection with foreign exchange control regulations, and taxes and other governmental charges, delivery and other such expenses), or which shall otherwise materially prejudice any substantial existing right of Holders or Beneficial Owners, shall not, however, become effective as to outstanding ADSs until the expiration of thirty (30) days after notice of such amendment or supplement shall have been given to the Holders of outstanding ADSs.  Notice of any amendment to the Deposit Agreement or any ADR shall not need to describe in detail the specific amendments effectuated thereby, and failure to describe the specific amendments in any such notice shall not render such notice invalid, provided, however, that, in each such case, the notice given to the Holders identifies a means for Holders and Beneficial Owners to retrieve or receive the text of such amendment (e.g., upon retrieval from the Commission’s, the Depositary’s or the Company’s website or upon request from the Depositary).  The parties hereto agree that any amendments or supplements which (i) are reasonably necessary (as agreed by the Company and the Depositary) in order for (a) the ADSs to be registered on Form F-6 under the Securities Act or (b) the ADSs to be settled solely in electronic book‑entry form and (ii) do not in either such case impose or increase any fees or charges to be borne by Holders, shall be deemed not to materially prejudice any substantial existing rights of Holders or Beneficial Owners.  Every Holder and Beneficial Owner at the time any amendment or supplement so becomes effective shall be deemed, by continuing to hold such ADSs, to consent and agree to such amendment or supplement and to be bound by the Deposit Agreement and the ADR, if applicable, as amended or supplemented thereby.  In no event shall any amendment or supplement impair the right of the Holder to surrender such ADS and receive therefor the Deposited Securities represented thereby, except in order to comply with mandatory provisions of applicable law.  Notwithstanding the foregoing, if any governmental body should adopt new laws, rules or regulations which would require an amendment of, or supplement to, the Deposit Agreement to ensure compliance therewith, the Company and the Depositary may amend or supplement the Deposit Agreement and any ADRs at any time in accordance with such changed laws, rules or regulations.  Such amendment or supplement to the Deposit Agreement and any ADRs in such circumstances may become effective before a notice of such amendment or supplement is given to Holders or within any other period of time as required for compliance with such laws, rules or regulations.

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Section 6.2        Termination.  The Depositary shall, at any time at the written direction of the Company, terminate the Deposit Agreement by distributing notice of such termination to the Holders of all ADSs then outstanding at least thirty (30) days prior to the date fixed in such notice for such termination.  If (i) ninety (90) days shall have expired after the Depositary shall have delivered to the Company a written notice of its election to resign, or (ii) ninety (90) days shall have expired after the Company shall have delivered to the Depositary a written notice of the removal of the Depositary, and, in either case, a successor depositary shall not have been appointed and accepted its appointment as provided in Section 5.4 of the Deposit Agreement, the Depositary may terminate the Deposit Agreement by distributing notice of such termination to the Holders of all ADSs then outstanding at least thirty (30) days prior to the date fixed in such notice for such termination.  The date so fixed for termination of the Deposit Agreement in any termination notice so distributed by the Depositary to the Holders of ADSs is referred to as the “Termination Date”.  Until the Termination Date, the Depositary shall continue to perform all of its obligations under the Deposit Agreement, and the Holders and Beneficial Owners will be entitled to all of their rights under the Deposit Agreement.

If any ADSs shall remain outstanding after the Termination Date, the Registrar and the Depositary shall not, after the Termination Date, have any obligation to perform any further acts under the Deposit Agreement, except that the Depositary shall, subject, in each case, to the terms and conditions of the Deposit Agreement, continue to (i) collect dividends and other distributions pertaining to Deposited Securities, (ii) sell Deposited Property received in respect of Deposited Securities, (iii) deliver Deposited Securities, together with any dividends or other distributions received with respect thereto and the net proceeds of the sale of any other Deposited Property, in exchange for ADSs surrendered to the Depositary (after deducting, or charging, as the case may be, in each case, the fees and charges of, and expenses incurred by, the Depositary, and all applicable taxes or governmental charges for the account of the Holders and Beneficial Owners, in each case upon the terms set forth in Section 5.9 of the Deposit Agreement), and (iv) take such actions as may be required under applicable law in connection with its role as Depositary under the Deposit Agreement.

At any time after the Termination Date, the Depositary may sell the Deposited Property then held under the Deposit Agreement and shall after such sale hold un-invested the net proceeds of such sale, together with any other cash then held by it under the Deposit Agreement, in an un-segregated account and without liability for interest, for the pro rata benefit of the Holders whose ADSs have not theretofore been surrendered.  After making such sale, the Depositary shall be discharged from all obligations under the Deposit Agreement except (i) to account for such net proceeds and other cash (after deducting, or charging, as the case may be, in each case, the fees and charges of, and expenses incurred by, the Depositary, and all applicable taxes or governmental charges for the account of the Holders and Beneficial Owners, in each case upon the terms set forth in Section 5.9 of the Deposit Agreement), and (ii) as may be required at law in connection with the termination of the Deposit Agreement.  After the Termination Date, the Company shall be discharged from all obligations under the Deposit Agreement, except for its obligations to the Depositary under Sections 5.8, 5.9 and 7.6 of the Deposit Agreement.  The obligations under the terms of the Deposit Agreement of Holders and Beneficial Owners of ADSs outstanding as of the Termination Date shall survive the Termination Date and shall be discharged only when the applicable ADSs are presented by their Holders to the Depositary for cancellation under the terms of the Deposit Agreement (except as specifically provided in the Deposit Agreement).

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ARTICLE VII

MISCELLANEOUS

Section 7.1        Counterparts.  The Deposit Agreement may be executed in any number of counterparts, each of which shall be deemed an original and all of such counterparts together shall constitute one and the same agreement.  Copies of the Deposit Agreement shall be maintained with the Depositary and shall be open to inspection by any Holder during business hours.

Section 7.2        No Third‑Party Beneficiaries/Acknowledgments.  The Deposit Agreement is for the exclusive benefit of the parties hereto (and their successors) and shall not be deemed to give any legal or equitable right, remedy or claim whatsoever to any other person, except to the extent specifically set forth in the Deposit Agreement.  Nothing in the Deposit Agreement shall be deemed to give rise to a partnership or joint venture among the parties nor establish a fiduciary or similar relationship among the parties.  The parties hereto acknowledge and agree that (i) Citibank and its Affiliates may at any time have multiple banking relationships with the Company,  the Holders, the Beneficial Owners, and their respective Affiliates,  (ii) Citibank and its Affiliates may own and deal in any class of securities of the Company and its Affiliates and in ADSs, and may be engaged at any time in transactions in which parties adverse to the Company, the Holders, the Beneficial Owners or their respective Affiliates may have interests, (iii) the Depositary and its Affiliates may from time to time have in their possession non-public information about the Company, the Holders, the Beneficial Owners, and their respective Affiliates, (iv) nothing contained in the Deposit Agreement shall (a) preclude Citibank or any of its Affiliates from engaging in such transactions or establishing or maintaining such relationships, or (b) obligate Citibank or any of its Affiliates to disclose such information, transactions or relationships, or to account for any profit made or payment received in such transactions or relationships,  (v) the Depositary shall not be deemed to have knowledge of any information any other division of Citibank or any of its Affiliates may have about the Company, the Holders, the Beneficial Owners, or any of their respective Affiliates, and (vi) the Company, the Depositary, the Custodian and their respective agents and controlling persons may be subject to the laws and regulations of jurisdictions other than the U.S. and England and Wales, and the authority of courts and regulatory authorities of such other jurisdictions, and, consequently, the requirements and the limitations of such other laws and regulations, and the decisions and orders of such other courts and regulatory authorities, may affect the rights and obligations of the parties to the Deposit Agreement.

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Section 7.3        Severability.  In case any one or more of the provisions contained in the Deposit Agreement or in the ADRs should be or become invalid, illegal or unenforceable in any respect, the validity, legality and enforceability of the remaining provisions contained herein or therein shall in no way be affected, prejudiced or disturbed thereby.

Section 7.4        Holders and Beneficial Owners as Parties; Binding Effect.  The Holders and Beneficial Owners from time to time of ADSs issued hereunder shall be parties to the Deposit Agreement and shall be bound by all of the terms and conditions hereof and of any ADR evidencing their ADSs by acceptance thereof or any beneficial interest therein.

Section 7.5        Notices.  Any and all notices to be given to the Company shall be in writing and shall be deemed to have been duly given if personally delivered or sent by mail, air courier or email as follows:

Address:            90 Harcourt Street, Dublin 2, Ireland

Attention:          Chief Financial Officer

Email:                rory.nealon@amrytpharma.com,

or to any other address which the Company may specify in writing to the Depositary.

Any and all notices to be given to the Depositary shall be in writing and deemed to have been duly given if personally delivered or sent by mail, air courier or cable, telex or facsimile transmission, confirmed by letter personally delivered or sent by mail or air courier, addressed to Citibank, N.A., 388 Greenwich Street, New York, New York 10013, U.S.A., Attention:  Depositary Receipts Department, or to any other address which the Depositary may specify in writing to the Company.

Any and all notices to be given to any Holder shall be deemed to have been duly given

(a)  if personally delivered or sent by mail or cable, telex or facsimile transmission, confirmed by letter, addressed to such Holder at the address of such Holder as it appears on the books of the Depositary or, if such Holder shall have filed with the Depositary a request that notices intended for such Holder be mailed to some other address, at the address specified in such request, or

(b)  if a Holder shall have designated such means of notification as an acceptable means of notification under the terms of the Deposit Agreement, by means of electronic messaging addressed for delivery to the e-mail address designated by the Holder for such purpose.  Notice to Holders shall be deemed to be notice to Beneficial Owners for all purposes of the Deposit Agreement.  Failure to notify a Holder or any defect in the notification to a Holder shall not affect the sufficiency of notification to other Holders or to the Beneficial Owners of ADSs held by such other Holders.  Any notices given to DTC under the terms of the Deposit Agreement shall (unless otherwise specified by the Depositary) constitute notice to the DTC Participants who hold the ADSs in their DTC accounts and to the Beneficial Owners of such ADSs.

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Delivery of a notice sent by mail, air courier or cable, telex or facsimile transmission shall be deemed to be effective at the time when a duly addressed letter containing the same (or a confirmation thereof in the case of a cable, telex or facsimile transmission) is deposited, postage prepaid, in a post-office letter box or delivered to an air courier service, without regard for the actual receipt or time of actual receipt thereof by a Holder.  The Depositary or the Company may, however, act upon any cable, telex or facsimile transmission received by it from any Holder, the Custodian, the Depositary, or the Company, notwithstanding that such cable, telex or facsimile transmission shall not be subsequently confirmed by letter.

Delivery of a notice by means of email or electronic messaging shall be deemed to be effective at the time of the initiation of the transmission by the sender (as shown on the sender’s records), notwithstanding that the intended recipient retrieves the message at a later date, fails to retrieve such message, or fails to receive such notice on account of its failure to maintain the designated e-mail address, its failure to designate a substitute e-mail address or for any other reason.

Section 7.6        Governing Law and Jurisdiction.  The Deposit Agreement, the ADRs and the ADSs shall be interpreted in accordance with, and all rights hereunder and thereunder and provisions hereof and thereof shall be governed by, the laws of the State of New York without reference to the principles of choice of law thereof.  Notwithstanding any other provision of the Deposit Agreement to the contrary, any ADR or any present or future provisions of the laws of the State of New York, the rights of holders of Shares and of any other Deposited Securities and the obligations and duties of the Company in respect of the holders of Shares and other Deposited Securities, as such, shall be governed by the laws of England and Wales (or, if applicable, such other laws as may govern the Deposited Securities).

Except as set forth in the following paragraph of this Section 7.6, the Company and the Depositary agree that the federal or state courts in the City of New York shall have jurisdiction to hear and determine any suit, action or proceeding and to settle any dispute between them that may arise out of or in connection with the Deposit Agreement and, for such purposes, each irrevocably submits to the non-exclusive jurisdiction of such courts.  The Company hereby irrevocably designates, appoints and empowers Aegerion Pharmaceuticals, Inc. (the “Agent”) now at 53 State Street, Suite 500, Boston, MA 02109 as its authorized agent to receive and accept for and on its behalf, and on behalf of its properties, assets and revenues, service by mail of any and all legal process, summons, notices and documents that may be served in any suit, action or proceeding brought against the Company in any federal or state court as described in the preceding sentence or in the next paragraph of this Section 7.6.  If for any reason the Agent shall cease to be available to act as such, the Company agrees to designate a new agent in New York on the terms and for the purposes of this Section 7.6 reasonably satisfactory to the Depositary.  The Company further hereby irrevocably consents and agrees to the service of any and all legal process, summons, notices and documents in any suit, action or proceeding against the Company, by service by mail of a copy thereof upon the Agent (whether or not the appointment of such Agent shall for any reason prove to be ineffective or such Agent shall fail to accept or acknowledge such service), with a copy mailed to the Company by registered or certified air mail, postage prepaid, to its address provided in Section 7.5.  The Company agrees that the failure of the Agent to give any notice of such service to it shall not impair or affect in any way the validity of such service or any judgment rendered in any action or proceeding based thereon.

44

Notwithstanding the foregoing, the Depositary and the Company unconditionally agree that in the event of any suit, action or proceeding against (a) the Company, (b) the Depositary in its capacity as Depositary under the Deposit Agreement, or (c) against both the Company and the Depositary, in any such case, in any state or federal court of the United States, and the Depositary or the Company have any claim, for indemnification or otherwise, against each other arising out of the subject matter of such suit, action or proceeding, then the Company and the Depositary may pursue such claim against each other in the state or federal court in the United States in which such suit, action, or proceeding is pending and, for such purposes, the Company and the Depositary irrevocably submit to the non-exclusive jurisdiction of such courts.  The Company agrees that service of process upon the Agent in the manner set forth in the preceding paragraph shall be effective service upon it for any suit, action or proceeding brought against it as described in this paragraph.

The Company irrevocably and unconditionally waives, to the fullest extent permitted by law, any objection that it may now or hereafter have to the laying of venue of any actions, suits or proceedings brought in any court as provided in this Section 7.6, and hereby further irrevocably and unconditionally waives and agrees not to plead or claim in any such court that any such action, suit or proceeding brought in any such court has been brought in an inconvenient forum.

The Company irrevocably and unconditionally waives, to the fullest extent permitted by law, and agrees not to plead or claim, any right of immunity from legal action, suit or proceeding, from setoff or counterclaim, from the jurisdiction of any court, from service of process, from attachment upon or prior to judgment, from attachment in aid of execution or judgment, from execution of judgment, or from any other legal process or proceeding for the giving of any relief or for the enforcement of any judgment, and consents to such relief and enforcement against it, its assets and its revenues in any jurisdiction, in each case with respect to any matter arising out of, or in connection with, the Deposit Agreement, any ADR or the Deposited Property.

EACH OF THE PARTIES TO THE DEPOSIT AGREEMENT (INCLUDING, WITHOUT LIMITATION, EACH HOLDER AND BENEFICIAL OWNER) IRREVOCABLY WAIVES, TO THE FULLEST EXTENT PERMITTED BY APPLICABLE LAW, ANY AND ALL RIGHT TO TRIAL BY JURY IN ANY LEGAL PROCEEDING AGAINST THE COMPANY AND/OR THE DEPOSITARY ARISING OUT OF, OR RELATING TO, THE DEPOSIT AGREEMENT, ANY ADR AND ANY TRANSACTIONS CONTEMPLATED THEREIN (WHETHER BASED ON CONTRACT, TORT, COMMON LAW OR OTHERWISE).

The provisions of this Section 7.6 shall survive any termination of the Deposit Agreement, in whole or in part.

45

Section 7.7        Assignment.  Subject to the provisions of Section 5.4, the Deposit Agreement may not be assigned by either the Company or the Depositary.

Section 7.8        Compliance with, and No Disclaimer under, U.S. Securities Laws.

(a)       Notwithstanding anything in the Deposit Agreement to the contrary, the withdrawal or delivery of Deposited Securities will not be suspended by the Company or the Depositary except as would be permitted by Instruction I.A.(1) of the General Instructions to Form F-6 Registration Statement, as amended from time to time, under the Securities Act.

(b)       Each of the parties to the Deposit Agreement (including, without limitation, each Holder and Beneficial Owner) acknowledges and agrees that no provision of the Deposit Agreement or any ADR shall, or shall be deemed to, disclaim any liability under the Securities Act or the Exchange Act, in each case to the extent established under applicable U.S. laws.

Section 7.9        English Law References.  Any summary of English laws and regulations and of the terms of the Company’s Articles of Association set forth in the Deposit Agreement have been provided by the Company solely for the convenience of Holders, Beneficial Owners and the Depositary.  While such summaries are believed by the Company to be accurate as of the date of the Deposit Agreement, (i) they are summaries and as such may not include all aspects of the materials summarized applicable to a Holder or Beneficial Owner, and (ii) these laws and regulations and the Company’s Articles of Association may change after the date of the Deposit Agreement.  Neither the Depositary nor the Company has any obligation under the terms of the Deposit Agreement to update any such summaries.

Section 7.10      Titles and References.

(a)       Deposit Agreement.  All references in the Deposit Agreement to exhibits, articles, sections, subsections, and other subdivisions refer to the exhibits, articles, sections, subsections and other subdivisions of the Deposit Agreement unless expressly provided otherwise.  The words “the Deposit Agreement”, “herein”, “hereof”, “hereby”, “hereunder”, and words of similar import refer to the Deposit Agreement as a whole as in effect at the relevant time between the Company, the Depositary and the Holders and Beneficial Owners of ADSs and not to any particular subdivision unless expressly so limited.  Pronouns in masculine, feminine and neuter gender shall be construed to include any other gender, and words in the singular form shall be construed to include the plural and vice versa unless the context otherwise requires.  Titles to sections of the Deposit Agreement are included for convenience only and shall be disregarded in construing the language contained in the Deposit Agreement.  References to “applicable laws and regulations” shall refer to laws and regulations applicable to ADRs, ADSs or Deposited Property as in effect at the relevant time of determination, unless otherwise required by law or regulation.

46

(b)       ADRs.  All references in any ADR(s) to paragraphs, exhibits, articles, sections, subsections, and other subdivisions refer to the paragraphs, exhibits, articles, sections, subsections and other subdivisions of the ADR(s) in question unless expressly provided otherwise.  The words “the Receipt”, “the ADR”, “herein”, “hereof”, “hereby”, “hereunder”, and words of similar import used in any ADR refer to the ADR as a whole and as in effect at the relevant time, and not to any particular subdivision unless expressly so limited.  Pronouns in masculine, feminine and neuter gender in any ADR shall be construed to include any other gender, and words in the singular form shall be construed to include the plural and vice versa unless the context otherwise requires.  Titles to paragraphs of any ADR are included for convenience only and shall be disregarded in construing the language contained in the ADR.  References to “applicable laws and regulations” shall refer to laws and regulations applicable to the Company, the Depositary, the Custodian, their agents and controlling persons, the ADRs, the ADSs and the Deposited Property as in effect at the relevant time of determination, unless otherwise required by law or regulation.

Section 7.11      Amendment and Restatement.  The Depositary shall arrange to have new ADRs printed that reflect the form of ADR attached to the Deposit Agreement.  All ADRs issued hereunder after the date hereof, whether upon the deposit of Shares or other Deposited Securities or upon the transfer, combination or split-up of existing ADRs, shall be substantially in the form of the specimen ADR attached as Exhibit A hereto.  However, American depositary receipts issued prior to the date hereof under the terms of the Original Deposit Agreement and outstanding as of the date hereof, which do not reflect the form of ADR attached hereto as Exhibit A, do not need to be called in for exchange and may remain outstanding until such time as the Holders thereof choose to surrender them for any reason under the Deposit Agreement.  The Depositary is authorized and directed to take any and all actions deemed necessary to effect the foregoing.

The Company hereby instructs the Depositary to (i) promptly send notice of the execution of the Deposit Agreement  to all holders of American depositary shares outstanding under the Original Deposit Agreement as of the date hereof and (ii) inform holders of American depositary shares issued as “certificated American depositary shares” and outstanding under the Original Deposit Agreement as of the date hereof that they have the opportunity, but are not required, to exchange their American depositary receipts for one or more ADR(s) issued pursuant to the Deposit Agreement.

Holders and Beneficial Owners of American depositary shares issued pursuant to the Original Deposit Agreement and outstanding as of the date hereof, shall, from and after the date hereof, be deemed Holders and Beneficial Owners of ADSs issued pursuant and be subject to all of the terms and conditions of the Deposit Agreement in all respects, provided, however, that any term of the Deposit Agreement that prejudices any substantial existing right of holders or beneficial owners of American depositary shares issued under the Original Deposit Agreement shall not become effective as to Holders and Beneficial Owners until thirty (30) days after notice of the amendments effectuated by the Deposit Agreement shall have been given to holders of ADSs outstanding as of the date hereof.

47

IN WITNESS WHEREOF, Amryt Pharma plc and CITIBANK, N.A. have duly executed the Deposit Agreement as of the day and year first above set forth and all Holders and Beneficial Owners shall become parties hereto upon acceptance by them of ADSs issued in accordance with the terms hereof, or upon acquisition of any beneficial interest therein.

AMRYT PHARMA PLC
By:
	Name:
	Title:

CITIBANK, N.A.
By:
	Name:
	Title:

48

EXHIBIT A

[FORM OF ADR]

 Number  CUSIP NUMBER: 

American Depositary Shares (each

American Depositary Share

representing the right to receive five

(5) fully paid ordinaryshares)

AMERICAN DEPOSITARY RECEIPT

for

AMERICAN DEPOSITARY SHARES

representing

DEPOSITED ORDINARY SHARES

of

AMRYT PHARMA PLC

(Incorporated under the laws of England and Wales)

CITIBANK, N.A., a national banking association organized and existing under the laws of the United States of America, as depositary (the “Depositary”), hereby certifies that _____________is the owner of ______________ American Depositary Shares (hereinafter “ADS”) representing deposited ordinary shares, including evidence of rights to receive such ordinary shares (the “Shares”), of Amryt Pharma plc, a public limited company incorporated under the laws of England and Wales, and its successors (the “Company”).  As of the date of issuance of this ADR, each ADS represents the right to receive five (5) Shares deposited under the Deposit Agreement (as hereinafter defined) with the Custodian, which at the date of issuance of this ADR is Citibank, N.A. (London) (the “Custodian”).  The ADS(s)-to-Share(s) ratio is subject to amendment as provided in Articles IV and VI of the Deposit Agreement.  The Depositary’s Principal Office is located at 388 Greenwich Street, New York, New York 10013, U.S.A.

A-1

(1)       The Deposit Agreement.  This American Depositary Receipt is one of an issue of American Depositary Receipts (“ADRs”), all issued and to be issued upon the terms and conditions set forth in the Amended and Restated Deposit Agreement, dated as of [●] [●], 2020 (as amended and supplemented from time to time, the “Deposit Agreement”), by and among the Company, the Depositary, and all Holders and Beneficial Owners from time to time of ADSs issued thereunder.  The Deposit Agreement sets forth the rights and obligations of Holders and Beneficial Owners of ADSs and the rights and duties of the Depositary in respect of the Shares deposited thereunder and any and all other Deposited Property (as defined in the Deposit Agreement) from time to time received and held on deposit in respect of the ADSs.  Copies of the Deposit Agreement are on file at the Principal Office of the Depositary and with the Custodian.  Each Holder and each Beneficial Owner, upon acceptance of any ADSs (or any interest therein) issued in accordance with the terms and conditions of the Deposit Agreement, or by continuing to hold, from and after the date hereof any American depositary shares issued and outstanding under the Original Deposit Agreement,shall be deemed for all purposes to (a) be a party to and bound by the terms of the Deposit Agreement and the applicable ADR(s), and (b) appoint the Depositary its attorney‑in‑fact, with full power to delegate, to act on its behalf and to take any and all actions contemplated in the Deposit Agreement and the applicable ADR(s), to adopt any and all procedures necessary to comply with applicable law and to take such action as the Depositary in its sole discretion may deem necessary or appropriate to carry out the purposes of the Deposit Agreement and the applicable ADR(s), the taking of such actions to be the conclusive determinant of the necessity and appropriateness thereof.  The manner in which a Beneficial Owner holds ADSs (e.g., in a brokerage account vs. as registered holder) may affect the rights and obligations of, the manner in which, and the extent to which, services are made available to, Beneficial Owners pursuant to the terms of the Deposit Agreement.

The statements made on the face and reverse of this ADR are summaries of certain provisions of the Deposit Agreement and the Articles of Association (as in effect on the date of the signing of the Deposit Agreement) and are qualified by and subject to the detailed provisions of the Deposit Agreement and the Articles of Association, to which reference is hereby made.

All capitalized terms not defined herein shall have the meanings ascribed thereto in the Deposit Agreement.

The Depositary makes no representation or warranty as to the validity or worth of the Deposited Property.  The Depositary has made arrangements for the acceptance of the ADSs into DTC.  Each Beneficial Owner of ADSs held through DTC must rely on the procedures of DTC and the DTC Participants to exercise and be entitled to any rights attributable to such ADSs.  The Depositary may issue Uncertificated ADSs subject, however, to the terms and conditions of Section 2.13 of the Deposit Agreement.

(2)       Surrender of ADSs and Withdrawal of Deposited Securities.  The Holder of this ADR (and of the ADSs evidenced hereby) shall be entitled to Delivery (at the Custodian’s designated office) of the Deposited Securities at the time represented by the ADSs evidenced hereby upon satisfaction of each of the following conditions: (i) the Holder (or a duly-authorized attorney of the Holder) has duly Delivered ADSs to the Depositary at its Principal Office the ADSs evidenced hereby (and, if applicable, this ADR evidencing such ADSs) for the purpose of withdrawal of the Deposited Securities represented thereby, (ii) if applicable and so required by the Depositary, this ADR Delivered to the Depositary for such purpose has been properly endorsed in blank or is accompanied by proper instruments of transfer in blank (including signature guarantees in accordance with standard securities industry practice), (iii) if so required by the Depositary, the Holder of the ADSs has executed and delivered to the Depositary a written order directing the Depositary to cause the Deposited Securities being withdrawn to be Delivered to or upon the written order of the person(s) designated in such order, and (iv) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 of, and Exhibit B to, the Deposit Agreement) have been paid,subject, however, in each case, to the terms and conditions of this ADR evidencing the surrendered ADSs, of the Deposit Agreement, of the Company’s Articles of Associationand of any applicable laws and the rules of CREST or any other applicable book-entry settlement system, if available, and to any provisions of or governing the Deposited Securities, in each case as in effect at the time thereof.

A-2

Upon satisfaction of each of the conditions specified above, the Depositary (i) shall cancel the ADSs Delivered to it (and, if applicable, this ADR(s) evidencing the ADSs so Delivered), (ii) shall direct the Registrar to record the cancellation of the ADSs so Delivered on the books maintained for such purpose, and (iii) shall direct the Custodian to Deliver, or cause the Delivery of, in each case,  without unreasonable delay, the Deposited Securities represented by the ADSs so canceled together with any certificate or other document of title for the Deposited Securities, or evidence of the electronic transfer thereof (if available), as the case may be, to or upon the written order of the person(s) designated in the order delivered to the Depositary for such purpose, subject however, in each case, to the terms and conditions of the Deposit Agreement, of this ADR evidencing the ADS so canceled, of the Articles of Association, of any applicable laws and of the rules of CREST or any other applicable book-entry settlement system, if available, and to the terms and conditions of  or governing the Deposited Securities, in each case as in effect at the time thereof.

The Depositary shall not accept for surrender ADSs representing less than one (1) Share.  In the case of Delivery to it of ADSs representing a number other than a whole number of Shares, the Depositary shall cause ownership of the appropriate whole number of Shares to be Delivered in accordance with the terms hereof, and shall, at the discretion of the Depositary, either (i) return to the person surrendering such ADSs the number of ADSs representing any remaining fractional Share, or (ii) sell or cause to be sold the fractional Share represented by the ADSs so surrendered and remit the proceeds of such sale (net of (a) applicable fees and charges of, and expenses incurred by, the Depositary and (b) taxes withheld) to the person surrendering the ADSs.

Notwithstanding anything to the contrary contained in this ADR or the Deposit Agreement, the Depositary may make delivery at the Principal Office of the Depositary of Deposited Property consisting of (i) any cash dividends or cash distributions, or (ii) any proceeds from the sale of any non-cash distributions, which are at the time held by the Depositary in respect of the Deposited Securities represented by the ADSs surrendered for cancellation and withdrawal.  At the request, risk and expense of any Holder so surrendering ADSs represented by this ADR, and for the account of such Holder, the Depositary shall direct the Custodian to forward (to the extent permitted by law) any Deposited Property (other than Deposited Securities) held by the Custodian in respect of such ADSs to the Depositary for delivery at the Principal Office of the Depositary.  Such direction shall be given by letter or, at the request, risk and expense of such Holder, by cable, telex or facsimile transmission.

A-3

(3)       Transfer, Combination and Split-up of ADRs.  The Registrar shall register the transfer of this ADR (and of the ADSs represented hereby) on the books maintained for such purpose and the Depositary shall (x) cancel this ADR and execute new ADRs evidencing the same aggregate number of ADSs as those evidenced by this ADR canceled by the Depositary, (y) cause the Registrar to countersign such new ADRs, and (z) Deliver such new ADRs to or upon the order of the person entitled thereto, if each of the following conditions has been satisfied:  (i) this ADR has been duly Delivered by the Holder (or by a duly authorized attorney of the Holder) to the Depositary at its Principal Office for the purpose of effecting a transfer thereof, (ii) this surrendered ADR has been properly endorsed or is accompanied by proper instruments of transfer (including signature guarantees in accordance with standard securities industry practice), (iii) this surrendered ADR has been duly stamped (if required by the laws of the State of New York or of the United States), and (iv) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 of, and Exhibit B to, the Deposit Agreement) have been paid, subject, however, in each case, to the terms and conditions of this ADR, of the Deposit Agreement and of applicable law, in each case as in effect at the time thereof.

The Registrar shall register the split-up or combination of this ADR (and of the ADSs represented hereby) on the books maintained for such purpose and the Depositary shall (x) cancel this ADR and execute new ADRs for the number of ADSs requested, but in the aggregate not exceeding the number of ADSs evidenced by this ADR canceled by the Depositary, (y) cause the Registrar to countersign such new ADRs, and (z) Deliver such new ADRs to or upon the order of the Holder thereof, if each of the following conditions has been satisfied:  (i) this ADR has been duly Delivered by the Holder (or by a duly authorized attorney of the Holder) to the Depositary at its Principal Office for the purpose of effecting a split‑up or combination hereof, and (ii) all applicable fees and charges of, and expenses incurred by, the Depositary and all applicable taxes and governmental charges (as are set forth in Section 5.9 of, and Exhibit B to, the Deposit Agreement) have been paid, subject, however, in each case, to the terms and conditions of this ADR, of the Deposit Agreement and of applicable law, in each case as in effect at the time thereof.

(4)       Pre-Conditions to Registration, Transfer, Etc.  As a condition precedent to the execution and Delivery, the registration of issuance, transfer, split‑up, combination or surrender, of any ADS, the delivery of any distribution thereon, or the withdrawal of any Deposited Property, the Depositary or the Custodian may require (i) payment from the depositor of Shares or presenter of ADSs or of this ADR of a sum sufficient to reimburse it for any tax or other governmental charge and any stock transfer or registration fee with respect thereto (including any such tax or charge and fee with respect to Shares being deposited or withdrawn) and payment of any applicable fees and charges of the Depositary as provided in Section 5.9 and Exhibit B to the Deposit Agreement and in this ADR, (ii) the production of proof satisfactory to it as to the identity and genuineness of any signature or any other matter contemplated by Section 3.1 of the Deposit Agreement, and (iii) compliance with (A) any laws or governmental regulations relating to the execution and Delivery of this ADR or ADSs or to the withdrawal of Deposited Securities and (B) such reasonable regulations as the Depositary and the Company may establish consistent with the provisions of this ADR, if applicable, the Deposit Agreement and applicable law.

A-4

The issuance of ADSs against deposits of Shares generally or against deposits of particular Shares may be suspended, or the deposit of particular Shares may be refused, or the registration of transfer of ADSs in particular instances may be refused, or the registration of transfer of ADSs generally may be suspended, during any period when the transfer books of the Company, the Depositary, a Registrar or the Share Registrar are closed or if any such action is deemed necessary or advisable by the Depositary (whereupon the Depositary shall notify the Company) or the Company, in good faith, at any time or from time to time because of any requirement of law or regulation, any government or governmental body or commission or any securities exchange on which the ADSs or Shares are listed, or under any provision of the Deposit Agreement or this ADR, if applicable, or under any provision of, or governing, the Deposited Securities, or because of a meeting of shareholders of the Company or for any other reason, subject, in all cases to Section 7.8 of the Deposit Agreement and paragraph (25) of this ADR.  Notwithstanding any provision of the Deposit Agreement or this ADR to the contrary, Holders are entitled to surrender outstanding ADSs to withdraw the Deposited Securities associated therewith at any time subject only to (i) temporary delays caused by closing the transfer books of the Depositary or the Company or the deposit of Shares in connection with voting at a shareholders’ meeting or the payment of dividends, (ii) the payment of fees, taxes and similar charges, (iii) compliance with any U.S. or foreign laws or governmental regulations relating to the ADSs or to the withdrawal of the Deposited Securities, and (iv) other circumstances specifically contemplated by Instruction I.A.(l) of the General Instructions to Form F-6 (as such General Instructions may be amended from time to time) under the Securities Act.

(5)       Compliance with Information Requests.  Notwithstanding any other provision of the Deposit Agreement or this ADR, each Holder and Beneficial Owner of the ADSs represented hereby agrees to comply with requests from the Company pursuant to applicable law, the rules and requirements of any stock exchange on which the Shares or ADSs are, or will be, registered, traded or listed, or the Articles of Association, which are made to provide information, inter alia, as to the capacity in which such Holder or Beneficial Owner owns ADSs (and the Shares represented by such ADSs, as the case may be) and regarding the identity of any other person(s) interested in such ADSs (and the Shares represented by such ADSs, as the case may be) and the nature of such interest and various other matters, whether or not they are Holders and/or Beneficial Owners at the time of such request.

(6)       Ownership Restrictions.  Notwithstanding any other provision contained in this ADR or of the Deposit Agreement to the contrary, the Company may restrict transfers of the Shares where such transfer might result in ownership of Shares exceeding limits imposed by applicable law or the Articles of Association.  The Company may also restrict, in such manner as it deems appropriate, transfers of the ADSs where such transfer may result in the total number of Shares represented by the ADSs owned by a single Holder or Beneficial Owner to exceed any such limits.  The Company may, in its sole discretion but subject to applicable law, instruct the Depositary to take action with respect to the ownership interest of any Holder or Beneficial Owner in excess of the limits set forth in the preceding sentence, including but not limited to, the imposition of restrictions on the transfer of ADSs, the removal or limitation of voting rights or the mandatory sale or disposition on behalf of a Holder or Beneficial Owner of the Shares represented by the ADSs held by such Holder or Beneficial Owner in excess of such limitations, if and to the extent such disposition is permitted by applicable law and the Articles of Association.  Nothing herein or in the Deposit Agreement shall be interpreted as obligating the Depositary or the Company to ensure compliance with the ownership restrictions described herein or in Section 3.5 of the Deposit Agreement.

A-5

(7)       Reporting Obligations and Regulatory Approvals.  Applicable laws and regulations may require holders and beneficial owners of Shares, including the Holders and Beneficial Owners of ADSs, to satisfy reporting requirements and obtain regulatory approvals in certain circumstances.  Holders and Beneficial Owners of ADSs are solely responsible for determining and complying with such reporting requirements and obtaining such approvals.  Each Holder and each Beneficial Owner hereby agrees to make such determination, file such reports, and obtain such approvals to the extent and in the form required by applicable laws and regulations as in effect from time to time.  Neither the Depositary, the Custodian, the Company or any of their respective agents or affiliates shall be required to take any actions whatsoever on behalf of Holders or Beneficial Owners to determine or satisfy such reporting requirements or obtain such regulatory approvals under applicable laws and regulations.

(8)       Liability for Taxes and Other Charges.  Any tax or other governmental charge payable by the Custodian or by the Depositary with respect to any Deposited Property, ADSs or this ADR shall be payable by the Holders and Beneficial Owners to the Depositary.  The Company, the Custodian and/or the Depositary may withhold or deduct from any distributions made in respect of Deposited Property held on behalf of such Holder and/or Beneficial Owner, and may sell for the account of a Holder and/or Beneficial Owner any or all of such Deposited Property and apply such distributions and sale proceeds in payment of, any taxes (including applicable interest and penalties) or charges that are or may be payable by Holders or Beneficial Owners in respect of the ADSs, Deposited Property and this ADR, the Holder and the Beneficial Owner hereof remaining liable for any deficiency.  The Custodian may refuse the deposit of Shares and the Depositary may refuse to issue ADSs, to deliver ADRs, register the transfer of ADSs, register the split-up or combination of ADRs and (subject to paragraph (25) of this ADR and Section 7.8 of the Deposit Agreement) the withdrawal of Deposited Property until payment in full of such tax, charge, penalty or interest is received.  Every Holder and Beneficial Owner agrees to indemnify the Depositary, the Company, the Custodian, and any of their respective agents, officers, employees and Affiliates for, and to hold each of them harmless from, any claims (including, without limitation, by any governmental authority or other person or entity) with respect to taxes (including applicable interest and penalties thereon) arising from any tax benefit obtained for such Holder and/or Beneficial Owner.  Notwithstanding anything to the contrary contained in the Deposit Agreement or this ADR, the obligations of Holders and Beneficial Owners under Section 3.2 of the Deposit Agreement shall survive any transfer of ADSs, any cancellation of ADSs and withdrawal of Deposited Securities, and the termination of the Deposit Agreement.

(9)       Representations and Warranties on Deposit of Shares.  Each person depositing Shares under the Deposit Agreement shall be deemed thereby to represent and warrant that (i) such Shares and the certificates therefor are duly authorized, validly allotted and issued, fully paid, not subject to any call for the payment of further capital and legally obtained by such person, (ii) all preemptive (and similar) rights, if any, with respect to such Shares have been validly waived, disapplied or exercised, (iii) the person making such deposit is duly authorized so to do, (iv) the Shares presented for deposit are free and clear of any lien, encumbrance, security interest, charge, mortgage or adverse claim, (v) the Shares presented for deposit are not, and the ADSs issuable upon such deposit will not be, Restricted Securities (except as contemplated in Section 2.14 of the Deposit Agreement), (vi) the Shares presented for deposit have not been stripped of any rights or entitlements and (vii) the deposit of the Shares does not violate any applicable provisions of English law.  Such representations and warranties shall survive the deposit and withdrawal of Shares, the issuance and cancellation of ADSs in respect thereof and the transfer of such ADSs.  If any such representations or warranties are false in any way, the Company and the Depositary shall be authorized, at the cost and expense of the person depositing Shares, to take any and all actions necessary to correct the consequences thereof.

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(10)Proofs, Certificates and Other Information.  Any person presenting Shares for deposit, any Holder and any Beneficial Owner may be required, and every Holder and Beneficial Owner agrees, from time to time to provide to the Company, the Depositary and the Custodian such proof of citizenship or residence, taxpayer status, payment of all applicable taxes or other governmental charges, exchange control approval, legal or beneficial ownership of ADSs and Deposited Property, compliance with applicable laws, the terms of the Deposit Agreement or this ADR evidencing the ADSs and the provisions of, or governing, the Deposited Property, to execute such certifications and to make such representations and warranties, and to provide such other information and documentation (or, in the case of Shares in registered form presented for deposit, such information relating to the registration on the books of the Company or of the Share Registrar) as the Depositary or the Custodian may deem necessary or proper or as the Company may reasonably require by written request to the Depositary consistent with its obligations under the Deposit Agreement and this ADR.  The Depositary and the Registrar, as applicable, and at the reasonable request of the Company, shall, to the extent practicable, may withhold the execution or delivery or registration of transfer of any ADR or ADS or the distribution or sale of any dividend or distribution of rights or of the proceeds thereof or, to the extent not limited by paragraph (25) and Section 7.8 of the Deposit Agreement, the delivery of any Deposited Property until such proof or other information is filed or such certifications are executed, or such representations and warranties are made or such other documentation or information is provided, in each case to the Depositary’s, the Registrar’s and the Company’s satisfaction.

(11)     ADS Fees and Charges.  The following ADS fees are payable under the terms of the Deposit Agreement:

(i) ADS Issuance Fee:  by any person for whom ADSs are issued (e.g., an issuance upon a deposit of Shares, upon a change in the ADS(s)-to-Share(s) ratio, or for any other reason), excluding issuances as a result of distributions described in paragraph (iv) below, a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) issued under the terms of the Deposit Agreement;

(ii) ADS Cancellation Fee:  by any person for whom ADSs are being cancelled (e.g., a cancellation of ADSs for Delivery of deposited Shares, upon a change in the ADS(s)-to-Share(s) ratio, or for any other reason), a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) cancelled;

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(iii) Cash Distribution Fee:  by any Holder of ADSs, a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) held for the distribution of cash dividends or other cash distributions (e.g., upon a sale of rights and other entitlements);

(iv) Stock Distribution /Rights Exercise Fee:  by any Holder of ADS(s), a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) held for the distribution of ADSs pursuant to (a) stock dividends or other free stock distributions, or (b) an exercise of rights to purchase additional ADSs;

(v) Other Distribution Fee:  by any Holder of ADS(s), a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) held for the distribution of securities other than ADSs or rights to purchase additional ADSs (e.g., spin-off shares);

(vi) Depositary Services Fee:  by any Holder of ADS(s), a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) held on the applicable record date(s) established by the Depositary;

(vii) Registration of ADS Transfer Fee:  by any Holder of ADS(s) being transferred or by any person to whom ADSs are transferred, a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) transferred (e.g., upon a registration of the transfer of registered ownership of ADSs, upon a transfer of ADSs into DTC and vice versa, or for any other reason); and

(viii) ADS Conversion Fee:  by any Holder of ADS(s) being converted or by any person to whom the converted ADSs are delivered, a fee not in excess of U.S. $5.00 per 100 ADSs (or fraction thereof) converted from one ADS series to another ADS series (e.g., upon conversion of Partial Entitlement ADSs for Full Entitlement ADSs, or upon conversion of Restricted ADSs into freely transferrable ADSs, and vice versa).

The Company, Holders, Beneficial Owners, persons depositing Shares or withdrawing Deposited Securities in connection with ADS issuances and cancellations, and persons for whom ADSs are issued or cancelled shall be responsible for the following ADS charges under the terms of the Deposit Agreement:

(a) taxes (including applicable interest and penalties) and other governmental charges;

(b) such registration fees as may from time to time be in effect for the registration of Shares or other Deposited Securities on the share register and applicable to transfers of Shares or other Deposited Securities to or from the name of the Custodian, the Depositary or any nominees upon the making of deposits and withdrawals, respectively;

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(c) such cable, telex and facsimile transmission and delivery expenses as are expressly provided in the Deposit Agreement to be at the expense of the person depositing Shares or withdrawing Deposited Securities or of the Holders and Beneficial Owners of ADSs;

(d) in connection with the conversion of Foreign Currency, the fees, expenses, spreads, taxes and other charges of the Depositary and/or conversion service providers (which may be a division, branch or Affiliate of the Depositary).  Such fees, expenses, spreads, taxes and other charges shall be deducted from the Foreign Currency;

(e) any reasonable and customary out-of-pocket expenses incurred in such conversion and/or on behalf of the Holders and Beneficial Owners in complying with currency exchange control or other governmental requirements; and

(f) the fees, charges, costs and expenses incurred by the Depositary, the Custodian, or any nominee in connection with the ADR program.

All ADS fees and charges so payable may be deducted from distributions or must be remitted to the Depositary, or its designee, and may, at any time and from time to time, be changed by agreement between the Depositary and Company but, in the case of ADS fees and charges payable by Holders and Beneficial Owners, only in the manner contemplated by paragraph (23) of this ADR and as contemplated in Section 6.1 of the Deposit Agreement.  The Depositary shall provide, without charge, a copy of its latest ADS fee schedule to anyone upon request.

ADS fees and charges for (i) the issuance of ADSs and (ii) the cancellation of ADSs will be payable by the person for whom the ADSs are so issued by the Depositary (in the case of ADS issuances) and by the person for whom ADSs are being cancelled (in the case of ADS cancellations).  In the case of ADSs issued by the Depositary into DTC or presented to the Depositary via DTC, the ADS issuance and cancellation fees and charges will be payable by the DTC Participant(s) receiving the ADSs from the Depositary or the DTC Participant(s) holding the ADSs being cancelled, as the case may be, on behalf of the Beneficial Owner(s) and will be charged by the DTC Participant(s) to the account(s) of the applicable Beneficial Owner(s) in accordance with the procedures and practices of the DTC Participant(s) as in effect at the time.  ADS fees and charges in respect of distributions and the ADS service fee are payable by Holders as of the applicable ADS Record Date established by the Depositary.  In the case of distributions of cash, the amount of the applicable ADS fees and charges is deducted from the funds being distributed.  In the case of (i) distributions other than cash and (ii) the ADS service fee, the applicable Holders as of the ADS Record Date established by the Depositary will be invoiced for the amount of the ADS fees and charges and such ADS fees may be deducted from distributions made to Holders.  For ADSs held through DTC, the ADS fees and charges for distributions other than cash and the ADS service fee may be deducted from distributions made through DTC, and may be charged to the DTC Participants in accordance with the procedures and practices prescribed by DTC from time to time and the DTC Participants in turn charge the amount of such ADS fees and charges to the Beneficial Owners for whom they hold ADSs.  In the case of (i) registration of ADS transfers, the ADS transfer fee will be payable by the ADS Holder whose ADSs are being transferred or by the person to whom the ADSs are transferred, and (ii) conversion of ADSs of one series for ADSs of another series, the ADS conversion fee will be payable by the Holder whose ADSs are converted or by the person to whom the converted ADSs are delivered.

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The Depositary may reimburse the Company for certain expenses incurred by the Company in respect of the ADR program established pursuant to the Deposit Agreement, by making available a portion of the ADS fees charged in respect of the ADR program or otherwise, upon such terms and conditions as the Company and the Depositary agree from time to time.  The Company shall pay to the Depositary such fees and charges, and reimburse the Depositary for such out-of-pocket expenses, as the Depositary and the Company may agree from time to time.  Responsibility for payment of such fees, charges and reimbursements may from time to time be changed by agreement between the Company and the Depositary.  Unless otherwise agreed, the Depositary shall present its statement for such fees, charges and reimbursements to the Company once every three months.  The charges and expenses of the Custodian are for the sole account of the Depositary.

The obligation of Holders and Beneficial Owners to pay ADS fees and charges shall survive the termination of the Deposit Agreement.  As to any Depositary, upon the resignation or removal of such Depositary as described in Section 5.4 of the Deposit Agreement, the right to collect ADS fees and charges shall extend for those ADS fees and charges incurred prior to the effectiveness of such resignation or removal.

(12)     Title to ADRs.  Subject to the limitations contained in the Deposit Agreement and in this ADR, it is a condition of this ADR, and every successive Holder of this ADR by accepting or holding the same consents and agrees, that title to this ADR (and to each Certificated ADS evidenced hereby) shall be transferable upon the same terms as a certificated security under the laws of the State of New York, provided that, in the case of Certificated ADSs, this ADR has been properly endorsed or is accompanied by proper instruments of transfer.  Notwithstanding any notice to the contrary, the Depositary and the Company may deem and treat the Holder of this ADR (that is, the person in whose name this ADR is registered on the books of the Depositary) as the absolute owner thereof for all purposes.  Neither the Depositary nor the Company shall have any obligation nor be subject to any liability under the Deposit Agreement or this ADR to any holder of this ADR or any Beneficial Owner unless, in the case of a holder of ADSs, such holder is the Holder of this ADR registered on the books of the Depositary or, in the case of a Beneficial Owner, such Beneficial Owner, or the Beneficial Owner’s representative, is the Holder registered on the books of the Depositary.

(13)     Validity of ADR.  The Holder(s) of this ADR (and the ADSs represented hereby) shall not be entitled to any benefits under the Deposit Agreement or be valid or enforceable for any purpose against the Depositary or the Company unless this ADR has been (i) dated, (ii) signed by the manual or facsimile signature of a duly-authorized signatory of the Depositary, (iii) countersigned by the manual or facsimile signature of a duly-authorized signatory of the Registrar, and (iv) registered in the books maintained by the Registrar for the registration of issuances and transfers of ADRs.  An ADR bearing the facsimile signature of a duly-authorized signatory of the Depositary or the Registrar, who at the time of signature was a duly authorized signatory of the Depositary or the Registrar, as the case may be, shall bind the Depositary, notwithstanding the fact that such signatory has ceased to be so authorized prior to the delivery of such ADR by the Depositary.

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(14)     Available Information; Reports; Inspection of Transfer Books.

The Company is subject to the periodic reporting requirements of the Exchange Act and, accordingly, is required to file or furnish certain reports with the Commission.  These reports can be retrieved from the Commission's website (www.sec.gov) and can be inspected and copied at the public reference facilities maintained by the Commission located (as of the date of the Deposit Agreement) at 100 F Street, N.E., Washington D.C.  20549.  The Depositary shall make available for inspection by Holders at its Principal Office, the Deposit Agreement, the provisions of or governing Deposited Securities and any reports and communications, including any proxy soliciting materials, received from the Company which are both (a) received by the Depositary, the Custodian, or the nominee of either of them as the holder of the Deposited Property and (b) made generally available to the holders of such Deposited Property by the Company.

The Registrar shall keep books for the registration of ADSs which at all reasonable times shall be open for inspection by the Company and by the Holders of such ADSs, provided that such inspection shall not be, to the Registrar’s knowledge, for the purpose of communicating with Holders of such ADSs in the interest of a business or object other than the business of the Company or other than a matter related to the Deposit Agreement or the ADSs.

The Registrar may close the transfer books with respect to the ADSs, at any time or from time to time, when deemed necessary or advisable by it in good faith in connection with the performance of its duties hereunder, or at the reasonable written request of the Company subject, in all cases, to paragraph (25) and Section 7.8 of the Deposit Agreement.

Dated:

CITIBANK, N.A. Transfer Agent and Registrar		CITIBANK, N.A.  as Depositary
By:		By:
	Authorized Signatory		Authorized Signatory

The address of the Principal Office of the Depositary is 388 Greenwich Street, New York, New York 10013, U.S.A.

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[FORM OF REVERSE OF ADR]

SUMMARY OF CERTAIN ADDITIONAL PROVISIONS

OF THE DEPOSIT AGREEMENT

(15)     Dividends and Distributions in Cash, Shares, etc.  (a) Cash Distributions: Upon the timely receipt by the Depositary of a notice from the Company that it intends to make a distribution of a cash dividend or other cash distribution, the Depositary shall establish the ADS Record Date upon the terms described in Section 4.9 of the Deposit Agreement.  Upon receipt of confirmation from the Custodian of receipt of (x) any cash dividend or other cash distribution on any Deposited Securities, or (y) proceeds from the sale of any Deposited Property held in respect of the ADSs under the terms of the Deposit Agreement, the Depositary will (i) if any amounts are received in a Foreign Currency, promptly convert or cause to be converted such cash dividend, distribution or proceeds into Dollars (subject to the terms and on the conditions of Section 4.8 of the Deposit Agreement), (ii) if applicable and unless previously established, establish the ADS Record Date upon the terms described in Section 4.9 of the Deposit Agreement, and (iii) distribute promptly the amount thus received from such conversion (net of (a) the applicable fees and charges described in the Fee Schedule attached as Exhibit B to the Deposit Agreement and (b) applicable taxes withheld) to the Holders entitled thereto as of the ADS Record Date in proportion to the number of ADSs held as of the ADS Record Date.  The Depositary shall distribute only such amount, however, as can be distributed without attributing to any Holder a fraction of one cent, and any balance not so distributed shall be held by the Depositary (without liability for interest thereon) and shall be added to and become part of the next sum received by the Depositary for distribution to Holders of ADSs outstanding at the time of the next distribution.  If the Company, the Custodian or the Depositary is required to withhold and does withhold from any cash dividend or other cash distribution in respect of any Deposited Securities, or from any cash proceeds from the sales of Deposited Property, an amount on account of taxes, duties or other governmental charges, the amount distributed to Holders on the ADSs shall be reduced accordingly.  Such withheld amounts shall be forwarded by the Company, the Custodian or the Depositary to the relevant governmental authority.  Evidence of payment thereof by the Company shall be forwarded by the Company to the Depositary upon request.  The Depositary will hold any cash amounts it is unable to distribute in a non-interest bearing account for the benefit of the applicable Holders and Beneficial Owners of ADSs until the distribution can be effected or the funds that the Depositary holds must be escheated as unclaimed property in accordance with the laws of the relevant states of the United States. Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for above, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in Section 4.1 of the Deposit Agreement, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in Section 4.1 of the Deposit Agreement where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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(b)  Share Distributions: Upon the timely receipt by the Depositary of a notice from the Company that it intends to make a distribution that consists of a dividend in, or free distribution of Shares, the Depositary shall establish the ADS Record Date upon the terms described in Section 4.9 of the Deposit Agreement.  Upon receipt of confirmation from the Custodian of the receipt of the Shares so distributed by the Company, the Depositary shall either (i) subject to Section 5.9 of the Deposit Agreement, distribute to the Holders as of the ADS Record Date in proportion to the number of ADSs held as of the ADS Record Date, additional ADSs, which represent in the aggregate the number of Shares received as such dividend, or free distribution, subject to the other terms of the Deposit Agreement (including, without limitation, (a) the applicable fees and charges of, and expenses incurred by, the Depositary and (b) taxes), or (ii) if additional ADSs are not so distributed, take all actions necessary so that each ADS issued and outstanding after the ADS Record Date shall, to the extent permissible by law, thenceforth also represent rights and interests in the additional integral number of Shares distributed upon the Deposited Securities represented thereby (net of (a) the applicable fees and charges of, and expenses incurred by, the Depositary, and (b) taxes).  In lieu of delivering fractional ADSs, the Depositary shall sell the number of Shares or ADSs, as the case may be, represented by the aggregate of such fractions and distribute the net proceeds upon the terms described in Section 4.1 of the Deposit Agreement.

In the event that the Depositary determines that any distribution in property (including Shares) is subject to any tax or other governmental charges which the Depositary is obligated to withhold, or, if the Company in the fulfillment of its obligations under Section 5.7 of the Deposit Agreement, has furnished an opinion of U.S. counsel determining that Shares must be registered under the Securities Act or other laws in order to be distributed to Holders (and no such registration statement has been declared effective), the Depositary may dispose of all or a portion of such property (including Shares and rights to subscribe therefor) in such amounts and in such manner, including by public or private sale, as the Depositary deems necessary and practicable, and the Depositary shall distribute the net proceeds of any such sale (after deduction of (a) taxes and (b) fees and charges of, and the expenses incurred by, the Depositary) to Holders entitled thereto upon the terms of Section 4.1 of the Deposit Agreement.  The Depositary shall hold and/or distribute any unsold balance of such property in accordance with the provisions of the Deposit Agreement. Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for above, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in Section 4.2 of the Deposit Agreement, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in Section 4.2 of the Deposit Agreement where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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(c)  Elective Distributions in Cash or Shares: Upon the timely receipt of a notice indicating that the Company wishes an elective distribution in cash or Shares to be made available to Holders of ADSs upon the terms described in the Deposit Agreement, the Company and the Depositary shall determine in accordance with the Deposit Agreement whether such distribution is lawful and reasonably practicable.  The Depositary shall make such elective distribution available to Holders only if (i) the Company shall have timely requested that the elective distribution be made available to Holders, (ii) the Depositary shall have determined, upon consultation with the Company, that such distribution is reasonably practicable and (iii) the Depositary shall have received satisfactory documentation within the terms of Section 5.7 of the Deposit Agreement. If the above conditions are satisfied, the Depositary shall, subject to the terms and conditions of the Deposit Agreement, establish an ADS Record Date according to paragraph (17) and Section 4.9 of the Deposit Agreement and establish procedures to enable the Holder hereof to elect to receive the proposed distribution in cash or in additional ADSs.  If a Holder elects to receive the distribution in cash, the distribution shall be made as in the case of a distribution in cash.  If the Holder hereof elects to receive the distribution in additional ADSs, the distribution shall be made as in the case of a distribution in Shares upon the terms described in the Deposit Agreement.  If such elective distribution is not reasonably practicable or if the Depositary did not receive satisfactory documentation set forth in the Deposit Agreement, the Depositary shall establish an ADS Record Date upon the terms of Section 4.9 of the Deposit Agreement and, to the extent permitted by law, distribute to Holders, on the basis of the same determination as is made in England and Wales in respect of the Shares for which no election is made, either (x) cash, upon the terms described in Section 4.1 of the Deposit Agreement or (y) additional ADSs representing such additional Shares, in each case, upon the terms described in Section 4.2 of the Deposit Agreement.  Nothing herein or in the Deposit Agreement shall obligate the Depositary to make available to the Holder hereof a method to receive the elective distribution in Shares (rather than ADSs).  There can be no assurance that the Holder hereof will be given the opportunity to receive elective distributions on the same terms and conditions as the holders of Shares. Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed distribution provided for above, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in Section 4.3 of the Deposit Agreement, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in Section 4.3 of the Deposit Agreement where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

(d)  Distribution of Rights to Purchase Additional ADSs: Upon the timely receipt by the Depositary of a notice indicating that the Company wishes rights to subscribe for additional Shares to be made available to Holders of ADSs, the Depositary upon consultation with the Company, shall determine, whether it is lawful and reasonably practicable to make such rights available to the Holders.  The Depositary shall make such rights available to any Holders only if (i) the Company shall have timely requested that such rights be made available to Holders, (ii) the Depositary shall have received satisfactory documentation within the terms of Section 5.7 of the Deposit Agreement, and (iii) the Depositary shall have determined that such distribution of rights is reasonably practicable.  If such conditions are not satisfied or if the Company requests that the rights not be made available to Holders of ADSs, the Depositary shall sell the rights as described below.  In the event all conditions set forth above are satisfied, the Depositary shall establish an ADS Record Date (upon the terms described in Section 4.9 of the Deposit Agreement) and establish procedures to (x) distribute rights to purchase additional ADSs (by means of warrants or otherwise), (y) enable the Holders to exercise such rights (upon payment of the subscription price and of the applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes), and (z) deliver ADSs upon the valid exercise of such rights.  Nothing herein or in the Deposit Agreement shall obligate the Depositary to make available to the Holders a method to exercise rights to subscribe for Shares (rather than ADSs).  If (i) the Company does not timely request the Depositary to make the rights available to Holders or requests that the rights not be made available to Holders, (ii) the Depositary fails to receive satisfactory documentation within the terms of Section 5,7 of the Deposit Agreement or determines, upon consultation with the Company, it is not reasonably practicable to make the rights available to Holders, or (iii) any rights made available are not exercised and appear to be about to lapse, the Depositary shall, upon consultation with the Company, determine whether it is lawful and reasonably practicable to sell such rights, in a riskless principal capacity, at such place and upon such terms (including public and private sale) as it may deem practicable.  The Depositary shall, upon such sale, convert and distribute proceeds of such sale (net of applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) upon the terms hereof and of Section 4.1 of the Deposit Agreement.  If the Depositary is unable to make any rights available to Holders upon the terms described in Section 4.4(a) of the Deposit Agreement or to arrange for the sale of the rights upon the terms described in Section 4.4(b) of the Deposit Agreement, the Depositary shall allow such rights to lapse.  The Depositary shall not be liable for (i) any failure to accurately determine whether it may be lawful or practicable to make such rights available to Holders in general or any Holders in particular, (ii) any foreign exchange exposure or loss incurred in connection with such sale or exercise, or (iii) the content of any materials forwarded to the Holders on behalf of the Company in connection with the rights distribution.

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Notwithstanding anything herein or in Section 4.4 of the Deposit Agreement to the contrary, if registration (under the Securities Act or any other applicable law) of the rights or the securities to which any rights relate may be required in order for the Company to offer such rights or such securities to Holders and to sell the securities represented by such rights, the Depositary will not distribute such rights to the Holders (i) unless and until a registration statement under the Securities Act (or other applicable law) covering such offering is in effect or (ii) unless the Company furnishes the Depositary opinion(s) of counsel for the Company in the United States and counsel to the Company in any other applicable country in which rights would be distributed, in each case reasonably satisfactory to the Depositary, to the effect that the offering and sale of such securities to Holders and Beneficial Owners are exempt from, or do not require registration under, the provisions of the Securities Act or any other applicable laws.  In the event that the Company, the Depositary or the Custodian shall be required to withhold and does withhold from any distribution of Deposited Property (including rights) an amount on account of taxes or other governmental charges, the amount distributed to the Holders of ADSs representing such Deposited Property shall be reduced accordingly.  In the event that the Depositary determines that any distribution of Deposited Property (including Shares and rights to subscribe therefor) is subject to any tax or other governmental charges which the Depositary is obligated to withhold, the Depositary may dispose of all or a portion of such Deposited Property (including Shares and rights to subscribe therefor) in such amounts and in such manner, including by public or private sale, as the Depositary deems necessary and practicable to pay any such taxes or charges.

There can be no assurance that Holders generally, or any Holder in particular, will be given the opportunity to receive or exercise rights on the same terms and conditions as the holders of Shares or be able to exercise such rights.  Nothing herein or in the Deposit Agreement shall obligate the Company to file any registration statement in respect of any rights or Shares or other securities to be acquired upon the exercise of such rights.

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(e)  Distributions other than Cash, Shares or Rights to Purchase Shares:  Upon receipt of a notice indicating that the Company wishes property other than cash, Shares or rights to purchase additional Shares to be made to Holders of ADSs, the Depositary shall determine whether such distribution to Holders is lawful and reasonably practicable.  The Depositary shall not make such distribution unless (i) the Company shall have requested the Depositary to make such distribution to Holders, (ii) the Depositary shall have received satisfactory documentation contemplated in Section 5.7 of the Deposit Agreement, and (iii) the Depositary shall have determined, after consultation with the Company, that such distribution is reasonably practicable.  Upon satisfaction of such conditions, the Depositary shall distribute the property so received to the Holders of record, as of the ADS Record Date, in proportion to the number of ADSs held by them respectively and in such manner as the Depositary may deem practicable for accomplishing such distribution (i) upon receipt of payment or net of the applicable fees and charges of, and expenses incurred by, the Depositary, and (ii) net of any taxes withheld.  The Depositary may dispose of all or a portion of the property so distributed and deposited, in such amounts and in such manner (including public or private sale) as the Depositary may deem practicable or necessary to satisfy any taxes (including applicable interest and penalties) or other governmental charges applicable to the distribution.

If the conditions above are not satisfied, the Depositary shall sell or cause such property to be sold in a public or private sale, at such place or places and upon such terms as it may deem practicable and shall (i) cause the proceeds of such sale, if any, to be converted into Dollars and (ii) distribute the proceeds of such conversion received by the Depositary (net of applicable (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) to the Holders as of the ADS Record Date upon the terms hereof and of Section 4.1 of the Deposit Agreement.  If the Depositary is unable to sell such property, the Depositary may dispose of such property for the account of the Holders in any way it deems reasonably practicable under the circumstances.

Neither the Depositary nor the Company shall be responsible for (i) any failure to determine whether it is lawful or practicable to make the property described in Section 4.5 of the Deposit Agreement available to Holders in general or any Holders in particular, nor (ii) any loss incurred in connection with the sale or disposal of such property.

(16)     Redemption.  Upon timely receipt of notice from the Company that it intends to exercise its right of redemption in respect of any of the Deposited Securities, and satisfactory documentation, and only if, after consultation with the Company, the Depositary shall have determined that such proposed redemption is practicable, the Depositary shall (to the extent practicable) provide to each Holder a notice setting forth the Company’s intention to exercise the redemption rights and any other particulars set forth in the Company’s notice to the Depositary.  The Depositary shall instruct the Custodian to present to the Company the Deposited Securities in respect of which redemption rights are being exercised against payment of the applicable redemption price. Upon receipt of confirmation from the Custodian that the redemption has taken place and that funds representing the redemption price have been received, the Depositary shall convert, transfer, and distribute the proceeds (net of applicable (a) fees and charges of, and the expenses incurred by, the Depositary, and (b) taxes), retire ADSs and cancel ADRs, if applicable, upon delivery of such ADSs by Holders thereof and the terms set forth in Sections 4.1 and 6.2 of the Deposit Agreement.  If less than all outstanding Deposited Securities are redeemed, the ADSs to be retired will be selected by lot or on a pro rata basis, as may be determined by the Depositary after consultation with the Company.  The redemption price per ADS shall be the dollar equivalent of the per share amount received by the Depositary (adjusted to reflect the ratio of ADS(s) to Share(s)) upon the redemption of the Deposited Securities represented by ADSs (subject to the terms of Section 4.8 of the Deposit Agreement and the applicable fees and charges of, and expenses incurred by, the Depositary, and taxes) multiplied by the number of Deposited Securities represented by each ADS redeemed. Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to give the Depositary timely notice of the proposed redemption provided for above, the Depositary agrees to use commercially reasonable efforts to perform the actions contemplated in Section 4.7 of the Deposit Agreement, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in Section 4.7 of the Deposit Agreement where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

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(17)     Fixing of ADS Record Date.  Whenever the Depositary shall receive notice of the fixing of a record date by the Company for the determination of holders of Deposited Securities entitled to receive any distribution (whether in cash, Shares, rights or other distribution), or whenever for any reason the Depositary causes a change in the number of Shares that are represented by each ADS, or whenever the Depositary shall receive notice of any meeting of, or solicitation of consents or proxies of, holders of Shares or other Deposited Securities, or whenever the Depositary shall find it necessary or convenient in connection with the giving of any notice, solicitation of any consent or any other matter, the Depositary shall fix the record date (the “ADS Record Date”) for the determination of the Holders of ADS(s) who shall be entitled to receive such distribution, to give instructions for the exercise of voting rights at any such meeting, to give or withhold such consent, to receive such notice or solicitation or to otherwise take action, or to exercise the rights of Holders with respect to such changed number of Shares represented by each ADS.  Subject to applicable law, the terms and conditions of this ADR and Sections 4.1 through 4.8 of the Deposit Agreement, only the Holders of ADSs at the close of business in New York on such ADS Record Date shall be entitled to receive such distribution, to give such voting instructions, to receive such notice or solicitation, or otherwise take action.

(18)     Voting of Deposited Securities.  As soon as practicable after receipt of notice of any meeting at which the holders of Deposited Securities are entitled to vote, or of solicitation of consents or proxies from holders of Deposited Securities, the Depositary shall fix the ADS Record Date in respect of such meeting or solicitation of consent or proxy in accordance with Section 4.9 of the Deposit Agreement.  The Depositary shall, if requested by the Company in writing in a timely manner (except as otherwise contemplated in Section 4.10 of the Deposit Agreement, the Depositary having no obligation to take any further action if the request shall not have been received by the Depositary at least thirty (30) days (or such lesser number of days as mutually agreed to in writing by the Company and the Depositary) prior to the date of such vote or meeting), at the Company’s expense and provided no U.S. legal prohibitions exist, distribute as soon as practicable after receipt thereof to Holders as of the ADS Record Date: (a) such notice of meeting or solicitation of consent or proxy, (b) a statement that the Holders at the close of business on the ADS Record Date will be entitled, subject to any applicable law, the provisions of the Deposit Agreement, the Articles of Association and the provisions of or governing the Deposited Securities (which provisions, if any, shall be summarized in pertinent part by the Company), to instruct the Depositary as to the exercise of the voting rights, if any, pertaining to the Deposited Securities represented by such Holder’s ADSs, and (c) a brief statement as to the manner in which such voting instructions may be given or deemed to have been given in accordance with Section 4.10 of the Deposit Agreement, it being acknowledged that if no instructions are received prior to the deadline set for such purposes to the Depositary to give a discretionary proxy to a person designated by the Company.

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The Company shall use its reasonable endeavors to notify the Depositary in writing of its intention to convene a meeting where the holders of Deposited Securities are entitled to vote, at least thirty (30) days (or such lesser number of days as mutually agreed to in writing by the Company and the Depositary) prior to the date of the proposed meeting.

Notwithstanding anything contained in the Deposit Agreement to the contrary, in the event the Company fails to timely request that the Depositary distribute the information as provided for in this Section 4.10 of the Deposit Agreement, if the Depositary so agrees after consultation with the Company, the Depositary will use commercially reasonable efforts to perform the actions contemplated in this Section 4.10 of the Deposit Agreement, and the Company, the Holders and the Beneficial Owners acknowledge that the Depositary shall have no liability for the Depositary’s failure to perform the actions contemplated in this Section 4.10 of the Deposit Agreement, where such notice has not been so timely given, other than its failure to use commercially reasonable efforts, as provided herein.

Notwithstanding anything contained in the Deposit Agreement or any ADR, the Depositary may, to the extent not prohibited by law or regulations, or by the requirements of the stock exchange on which the ADSs are listed, in lieu of distribution of the materials provided to the Depositary in connection with any meeting of, or solicitation of consents or proxies from, holders of Deposited Securities, distribute to the Holders a notice that provides Holders with, or otherwise publicizes to Holders, instructions on how to retrieve such materials or receive such materials upon request (e.g., by reference to a website containing the materials for retrieval or a contact for requesting copies of the materials).

The Depositary has been advised by the Company that under the Articles of Association as in effect on the date of the Deposit Agreement, voting at any meeting of shareholders of the Company is by show of hands unless (before or on the declaration of the result of the show of hands) a poll is demanded.  The Depositary will not join in demanding a poll, whether or not requested to do so by Holders of ADSs.  Under the Articles of Association as in effect on the date of the Deposit Agreement, at any meeting a resolution put to the vote of the meeting shall be decided on a show of hands, unless before or upon the declaration of the result of the show of hands a poll is demanded by: (a) the chairman of the meeting; (b) not less than five (5) members of the Company having the right to vote at the meeting; (c) a member or members of the Company representing not less than one tenth of the total voting rights of all the members of the Company having the right to vote at the meeting; or (d) a member or members of the Company holding shares in the Company conferring a right to vote at the meeting, being shares on which an aggregate sum has been paid up equal to not less than one tenth of the total sum paid up on all the shares conferring that right.

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Voting instructions may be given only in respect of a number of ADSs representing an integral number of Deposited Securities.  Upon the timely receipt from a Holder of ADSs as of the ADS Record Date of voting instructions in the manner specified by the Depositary, the Depositary shall endeavor, insofar as practicable and permitted under applicable law, the provisions of the Deposit Agreement, Articles of Association and the provisions of the Deposited Securities, to vote, or cause the Custodian to vote, the Deposited Securities (in person or by proxy) represented by such Holder’s ADSs as follows: (a) in the event voting takes place at a shareholders’ meeting by a show of hands, the Depositary will instruct the Custodian to vote all Deposited Securities in accordance with the voting instructions received timely from a majority of Holders of ADSs who provided voting instructions, and (b) in the event voting takes place at a shareholders’ meeting by poll, the Depositary will instruct the Custodian to vote the Deposited Securities in accordance with the voting instructions timely received from the Holders of ADSs.  If voting is by poll and the Depositary does not receive voting instructions from a Holder as of the ADS Record Date on or before the date established by the Depositary for such purpose, such Holder shall be deemed, and the Depositary shall (unless otherwise specified in the notice distributed to Holders) deem such Holder, to have instructed the Depositary to give a discretionary proxy to a person designated by the Company to vote the Deposited Securities; provided, however, that no such discretionary proxy shall be given by the Depositary with respect to any matter to be voted upon as to which the Company informs the Depositary that (a) the Company does not wish such proxy to be given, (b) substantial opposition exists, or (c) the rights of holders of Deposited Securities may be adversely affected.

Deposited Securities represented by ADSs for which no timely voting instructions are received by the Depositary from the Holder shall not be voted (except (a) in the case voting is by show of hands, in which case the Depositary will instruct the Custodian to vote all Deposited Securities in accordance with the voting instructions received from a majority of Holders of ADSs who provided timely voting instructions, and (b) as contemplated in this Section 4.10).  Neither the Depositary nor the Custodian shall under any circumstances exercise any discretion as to voting and neither the Depositary nor the Custodian shall vote, attempt to exercise the right to vote, or in any way make use of, for purposes of establishing a quorum or otherwise, the Deposited Securities represented by ADSs, except pursuant to and in accordance with the voting instructions timely received from Holders or as otherwise contemplated herein.  If the Depositary timely receives voting instructions from a Holder which fail to specify the manner in which the Depositary is to vote the Deposited Securities represented by such Holder’s ADSs, the Depositary will deem such Holder (unless otherwise specified in the notice distributed to Holders) to have instructed the Depositary to vote in favor of the items set forth in such voting instructions.

Notwithstanding anything to the contrary contained herein, the Depositary shall, if so requested in writing by the Company, represent all Deposited Securities (whether or not voting instructions have been received in respect of such Deposited Securities from Holders as of the ADS Record Date) for the sole purpose of establishing quorum at a meeting of shareholders.

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Notwithstanding anything to the contrary contained in the Deposit Agreement or any ADR, the Depositary shall not have any obligation to take any action with respect to any meeting, or solicitation of consents or proxies, of holders of Deposited Securities if the taking of such action would violate U.S. or English laws.  The Company agrees to take any and all actions reasonably necessary and as permitted by any applicable law to enable Holders and Beneficial Owners to exercise the voting rights accruing to the Deposited Securities and to deliver to the Depositary an opinion of U.S. counsel addressing any actions requested to be taken if so requested by the Depositary.

There can be no assurance that Holders generally or any Holder in particular will receive the notice described above with sufficient time to enable the Holder to return voting instructions to the Depositary in a timely manner.

(19)     Changes Affecting Deposited Securities.  Upon any change in nominal or par value, split‑up, cancellation, consolidation or any other reclassification of Deposited Securities, or upon any recapitalization, reorganization, merger, consolidation or sale of assets affecting the Company or to which it is a party, any property which shall be received by the Depositary or the Custodian in exchange for, or in conversion of, or replacement of, or otherwise in respect of, such Deposited Securities shall, to the extent permitted by law, be treated as new Deposited Property under the Deposit Agreement, and this ADR shall, subject to the provisions of the Deposit Agreement, this ADR evidencing such ADSs and applicable law, represent the right to receive such additional or replacement Deposited Property.  In giving effect to such change, split-up, cancellation, consolidation or other reclassification of Deposited Securities, recapitalization, reorganization, merger, consolidation or sale of assets, the Depositary may, with the Company’s approval, and shall, if the Company shall so request, subject to the terms of the Deposit Agreement (including, without limitation, (a) the applicable fees and charges of, and expenses incurred by, the Depositary, and (b) taxes) and receipt of an opinion of counsel to the Company reasonably satisfactory to the Depositary that such actions are not in violation of any applicable laws or regulations, (i) issue and deliver additional ADSs as in the case of a stock dividend on the Shares, (ii) amend the Deposit Agreement and the applicable ADRs, (iii) amend the applicable Registration Statement(s) on Form F-6 as filed with the Commission in respect of the ADSs, (iv) call for the surrender of outstanding ADRs to be exchanged for new ADRs, and (v) take such other actions as the Depositary, in consultation with the Company, considers appropriate to reflect the transaction with respect to the ADSs.  Notwithstanding the foregoing, in the event that any Deposited Property so received may not be, upon consultation with the Company, lawfully distributed to some or all Holders, the Depositary may, with the Company’s approval, and shall, if the Company requests, subject to receipt of an opinion of Company’s counsel reasonably satisfactory to the Depositary that such action is not in violation of any applicable laws or regulations, sell such Deposited Property at public or private sale, at such place or places and upon such terms as it may deem proper and may allocate the net proceeds of such sales (net of (a) fees and charges of, and expenses incurred by, the Depositary and (b) taxes) for the account of the Holders otherwise entitled to such Deposited Property upon an averaged or other practicable basis without regard to any distinctions among such Holders and distribute the net proceeds so allocated to the extent practicable as in the case of a distribution received in cash pursuant to Section 4.1 of the Deposit Agreement.  The Depositary shall not be responsible for (i) any failure to determine whether it may be lawful or practicable to make such Deposited Property available to Holders in general or to any Holder in particular, (ii) any foreign exchange exposure or loss incurred in connection with such sale or (iii) any liability to the purchaser of such Deposited Property.

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(20)     Exoneration.  Notwithstanding anything contained in the Deposit Agreement or this ADR to the contrary, neither the Depositary nor the Company shall be obligated to do or perform any act which is inconsistent with the provisions of the Deposit Agreement or incur any liability (to the extent not limited by paragraph (25) hereof and Section 7.9 (b) of the Deposit Agreement) (i) if the Depositary, the Custodian, the Company or their respective agents shall be prevented or forbidden from, or delayed in, doing or performing any act or thing required or contemplated by the terms of the Deposit Agreement and this ADR, by reason of any provision of any present or future law or regulation of the United States, England and Wales or any other country, or of any other governmental authority or regulatory authority or stock exchange, or on account of the potential criminal or civil penalties or restraint, or by reason of any provision, present or future, of the Articles of Association or any provision of or governing any Deposited Securities, or by reason of any act of God or war or other circumstances beyond its control (including, without limitation, nationalization, expropriation, currency restrictions, work stoppage, strikes, civil unrest, acts of terrorism, revolutions, rebellions, explosions and computer failure), (ii) by reason of any exercise of, or failure to exercise, any discretion provided for in the Deposit Agreement or in the Articles of Association or provisions of or governing Deposited Securities, (iii) for any action or inaction in reliance upon the advice of or information from legal counsel, accountants, any person presenting Shares for deposit, any Holder, any Beneficial Owner or authorized representative thereof, or any other person believed by it in good faith to be competent to give such advice or information, (iv) for the inability by a Holder or Beneficial Owner to benefit from any distribution, offering, right or other benefit which is made available to holders of Deposited Securities but is not, under the terms of the Deposit Agreement, made available to Holders of ADSs, (v) for any action or inaction of any clearing or settlement system (any participant thereof) for the Deposited Property or the ADSs, or (vi) for any consequential or punitive damages (including lost profits) for any breach of the terms of the Deposit Agreement.  The Depositary, its controlling persons, its agents, any Custodian and the Company, its Affiliates, its controlling persons and its agents may rely and shall be protected in acting upon any written notice, request or other document believed by it to be genuine and to have been signed or presented by the proper party or parties.

(21)     Standard of Care.  The Company and the Depositary and their respective Affiliates, directors, officers, employees and agents assume no obligation and shall not be subject to any liability under the Deposit Agreement or this ADR to any Holder(s) or Beneficial Owner(s), except that the Company and the Depositary agree to perform their respective obligations specifically set forth in the Deposit Agreement or this ADR without negligence or bad faith.  Without limitation of the foregoing, neither the Depositary, nor the Company, nor any of their respective Affiliates, directors, officers, controlling persons, employees or agents, shall be under any obligation to appear in, prosecute or defend any action, suit or other proceeding in respect of any Deposited Property or in respect of the ADSs, which in its opinion may involve it in expense or liability, unless indemnity satisfactory to it against all expense (including fees and disbursements of counsel) and liability be furnished as often as may be required (and no Custodian shall be under any obligation whatsoever with respect to such proceedings, the responsibility of the Custodian being solely to the Depositary).

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The Depositary and its agents shall not be liable for any failure to carry out any instructions to vote any of the Deposited Securities, or for the manner in which any vote is cast or the effect of any vote, provided that any such action or omission is in good faith and without negligence and in accordance with the terms of the Deposit Agreement.  The Depositary shall not incur any liability for any failure to accurately determine whether any distribution or action may be lawful or reasonably practicable, for any investment risk associated with acquiring an interest in the Deposited Property, for the validity or worth of the Deposited Property, for the value of any Deposited Property or any distribution thereon, for any interest on Deposited Property, for any tax consequences that may result from the ownership of ADSs, Shares or other Deposited Property, for the credit‑worthiness of any third party. The Depositary shall not incur any liability for the content of any information submitted to it by the Company for distribution to the Holders or for any inaccuracy of any translation thereof, for allowing any rights to lapse upon the terms of the Deposit Agreement, for the failure or timeliness of any notice from the Company, or for any action of or failure to act by, or any information provided or not provided by, DTC or any DTC Participant.

The Depositary shall not be liable for any acts or omissions made by a successor depositary whether in connection with a previous act or omission of the Depositary or in connection with any matter arising wholly after the removal or resignation of the Depositary, provided that in connection with the issue out of which such potential liability arises the Depositary performed its obligations without negligence or bad faith while it acted as Depositary.

(22)     Resignation and Removal of the Depositary; Appointment of Successor Depositary.  The Depositary may at any time resign as Depositary under the Deposit Agreement by written notice of resignation delivered to the Company, such resignation to be effective on the earlier of (i) the 90th day after delivery thereof to the Company (whereupon the Depositary shall be entitled to take the actions contemplated in Section 6.2 of the Deposit Agreement), or (ii) the appointment by the Company of a successor depositary and its acceptance of such appointment as provided in the Deposit Agreement.  The Depositary, in such capacity, may at any time be removed by the Company by written notice of such removal, which removal shall be effective on the later of (i) the 90th day after delivery thereof to the Depositary (whereupon the Depositary shall be entitled to take the actions contemplated in Section 6.2 of the Deposit Agreement), or (ii) the appointment by the Company of a successor depositary and its acceptance of such appointment as provided in the Deposit Agreement.  In case at any time the Depositary acting hereunder shall resign or be removed, the Company shall use its best efforts to appoint a successor depositary, which shall be a bank or trust company having an office in the Borough of Manhattan, the City of New York.  Every successor depositary shall be required by the Company to execute and deliver to its predecessor and to the Company an instrument in writing accepting its appointment hereunder, and thereupon such successor depositary, without any further act or deed (except as required by applicable law), shall become fully vested with all the rights, powers, duties and obligations of its predecessor (other than as contemplated in Sections 5.8 and 5.9 of the Deposit Agreement).  The predecessor depositary, upon payment of all sums due it and on the written request of the Company shall (i) execute and deliver an instrument transferring to such successor all rights and powers of such predecessor hereunder (other than as contemplated in Sections 5.8 and 5.9 of the Deposit Agreement), (ii) duly assign, transfer and deliver all of the Depositary’s right, title and interest to the Deposited Property to such successor, and (iii) deliver to such successor a list of the Holders of all outstanding ADSs and such other information relating to ADSs and Holders thereof as the successor may reasonably request. Any such successor depositary shall promptly provide notice of its appointment to such Holders.  Any entity into or with which the Depositary may be merged or consolidated shall be the successor of the Depositary without the execution or filing of any document or any further act.

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(23)     Amendment/Supplement.  Subject to the terms and conditions of this paragraph 23, and Section 6.1 of the Deposit Agreement and applicable law, this ADR and any provisions of the Deposit Agreement may at any time and from time to time be amended or supplemented by written agreement between the Company and the Depositary in any respect which they may deem necessary or desirable without the prior written consent of the Holders or Beneficial Owners.  Any amendment or supplement which shall impose or increase any fees or charges (other than charges in connection with foreign exchange control regulations, and taxes and other governmental charges, delivery and other such expenses), or which shall otherwise materially prejudice any substantial existing right of Holders or Beneficial Owners, shall not, however, become effective as to outstanding ADSs until the expiration of thirty (30) days after notice of such amendment or supplement shall have been given to the Holders of outstanding ADSs.  Notice of any amendment to the Deposit Agreement or any ADR shall not need to describe in detail the specific amendments effectuated thereby, and failure to describe the specific amendments in any such notice shall not render such notice invalid, provided, however, that, in each such case, the notice given to the Holders identifies a means for Holders and Beneficial Owners to retrieve or receive the text of such amendment (e.g., upon retrieval from the Commission’s, the Depositary’s or the Company’s website or upon request from the Depositary).  The parties hereto agree that any amendments or supplements which (i) are reasonably necessary (as agreed by the Company and the Depositary) in order for (a) the ADSs to be registered on Form F-6 under the Securities Act or (b) the ADSs to be settled solely in electronic book-entry form and (ii) do not in either such case impose or increase any fees or charges to be borne by Holders, shall be deemed not to materially prejudice any substantial existing rights of Holders or Beneficial Owners.  Every Holder and Beneficial Owner at the time any amendment or supplement so becomes effective shall be deemed, by continuing to hold such ADSs, to consent and agree to such amendment or supplement and to be bound by the Deposit Agreement and this ADR, if applicable, as amended or supplemented thereby.  In no event shall any amendment or supplement impair the right of the Holder to surrender such ADS and receive therefor the Deposited Securities represented thereby, except in order to comply with mandatory provisions of applicable law.  Notwithstanding the foregoing, if any governmental body should adopt new laws, rules or regulations which would require an amendment of, or supplement to, the Deposit Agreement to ensure compliance therewith, the Company and the Depositary may amend or supplement the Deposit Agreement and this ADR at any time in accordance with such changed laws, rules or regulations.  Such amendment or supplement to the Deposit Agreement and this ADR in such circumstances may become effective before a notice of such amendment or supplement is given to Holders or within any other period of time as required for compliance with such laws, rules or regulations.

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(24)     Termination.  The Depositary shall, at any time at the written direction of the Company, terminate the Deposit Agreement by distributing notice of such termination to the Holders of all ADSs then outstanding at least thirty (30) days prior to the date fixed in such notice for such termination.  If (i) ninety (90) days shall have expired after the Depositary shall have delivered to the Company a written notice of its election to resign, or (ii) ninety (90) days shall have expired after the Company shall have delivered to the Depositary a written notice of the removal of the Depositary, and, in either case, a successor depositary shall not have been appointed and accepted its appointment as provided in Section 5.4 of the Deposit Agreement, the Depositary may terminate the Deposit Agreement by distributing notice of such termination to the Holders of all ADSs then outstanding at least thirty (30) days prior to the date fixed in such notice for such termination.  The date so fixed for termination of the Deposit Agreement in any termination notice so distributed by the Depositary to the Holders of ADSs is referred to as the “Termination Date”.  Until the Termination Date, the Depositary shall continue to perform all of its obligations under the Deposit Agreement, and the Holders and Beneficial Owners will be entitled to all of their rights under the Deposit Agreement.  If any ADSs shall remain outstanding after the Termination Date, the Registrar and the Depositary shall not, after the Termination Date, have any obligation to perform any further acts under the Deposit Agreement, except that the Depositary shall, subject, in each case, to the terms and conditions of the Deposit Agreement, continue to (i) collect dividends and other distributions pertaining to Deposited Securities, (ii) sell Deposited Property received in respect of Deposited Securities, (iii) deliver Deposited Securities, together with any dividends or other distributions received with respect thereto and the net proceeds of the sale of any other Deposited Property, in exchange for ADSs surrendered to the Depositary (after deducting, or charging, as the case may be, in each case, the fees and charges of, and expenses incurred by, the Depositary, and all applicable taxes or governmental charges for the account of the Holders and Beneficial Owners, in each case upon the terms set forth in Section 5.9 of the Deposit Agreement), and (iv) take such actions as may be required under applicable law in connection with its role as Depositary under the Deposit Agreement.  At any time after the Termination Date, the Depositary may sell the Deposited Property then held under the Deposit Agreement and shall after such sale hold un-invested the net proceeds of such sale, together with any other cash then held by it under the Deposit Agreement, in an un-segregated account and without liability for interest, for the   pro rata benefit of the Holders whose ADSs have not theretofore been surrendered.  After making such sale, the Depositary shall be discharged from all obligations under the Deposit Agreement except (i) to account for such net proceeds and other cash (after deducting, or charging, as the case may be, in each case, the fees and charges of, and expenses incurred by, the Depositary, and all applicable taxes or governmental charges for the account of the Holders and Beneficial Owners, in each case upon the terms set forth in Section 5.9 of the Deposit Agreement), and (ii) as may be required at law in connection with the termination of the Deposit Agreement.  After the Termination Date, the Company shall be discharged from all obligations under the Deposit Agreement, except for its obligations to the Depositary under Sections 5.8, 5.9 and 7.6 of the Deposit Agreement.  The obligations under the terms of the Deposit Agreement of Holders and Beneficial Owners of ADSs outstanding as of the Termination Date shall survive the Termination Date and shall be discharged only when the applicable ADSs are presented by their Holders to the Depositary for cancellation under the terms of the Deposit Agreement (except as specifically provided in the Deposit Agreement).

(25)     Compliance with, and No Disclaimer under, U.S. Securities Laws.  (a) Notwithstanding any provisions in this ADR or the Deposit Agreement to the contrary, the withdrawal or delivery of Deposited Securities will not be suspended by the Company or the Depositary except as would be permitted by Instruction I.A.(1) of the General Instructions to the Form F-6 Registration Statement, as amended from time to time, under the Securities Act.

(b)       Each of the parties to the Deposit Agreement (including, without limitation, each Holder and Beneficial Owner) acknowledges and agrees that no provision of the Deposit Agreement or any ADR shall, or shall be deemed to, disclaim any liability under the Securities Act or the Exchange Act, in each case to the extent established under applicable U.S. laws.

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(26)     No Third Party Beneficiaries/Acknowledgements.   The Deposit Agreement is for the exclusive benefit of the parties hereto (and their successors) and shall not be deemed to give any legal or equitable right, remedy or claim whatsoever to any other person, except to the extent specifically set forth in the Deposit Agreement.  Nothing in the Deposit Agreement shall be deemed to give rise to a partnership or joint venture among the parties nor establish a fiduciary or similar relationship among the parties.  The parties hereto acknowledge and agree that (i) Citibank and its Affiliates may at any time have multiple banking relationships with the Company,  the Holders, the Beneficial Owners, and their respective Affiliates,  (ii) Citibank and its Affiliates may own and deal in any class of securities of the Company and its Affiliates and in ADSs, and may be engaged at any time in transactions in which parties adverse to the Company, the Holders, the Beneficial Owners or their respective Affiliates may have interests, (iii) the Depositary and its Affiliates may from time to time have in their possession non-public information about the Company, the Holders, the Beneficial Owners, and their respective Affiliates, (iv) nothing contained in the Deposit Agreement shall (a) preclude Citibank or any of its Affiliates from engaging in such transactions or establishing or maintaining such relationships, or (b) obligate Citibank or any of its Affiliates to disclose such information, transactions or relationships, or to account for any profit made or payment received in such transactions or relationships, (v) the Depositary shall not be deemed to have knowledge of any information any other division of Citibank or any of its Affiliates may have about the Company, the Holders, the Beneficial Owners, or any of their respective Affiliates, and (vi) the Company, the Depositary, the Custodian and their respective agents and controlling persons may be subject to the laws and regulations of jurisdictions other than the U.S. and  England and Wales, and the authority of courts and regulatory authorities of such other jurisdictions, and, consequently, the requirements and the limitations of such other laws and regulations, and the decisions and orders of such other courts and regulatory authorities, may affect the rights and obligations of the parties to the Deposit Agreement.

(27)     Governing Law / Waiver of Jury Trial.  The Deposit Agreement, the ADRs and the ADSs shall be interpreted in accordance with, and all rights hereunder and thereunder and provisions hereof and thereof shall be governed by, the laws of the State of New York without reference to the principles of choice of law thereof.  Notwithstanding anything contained in the Deposit Agreement to the contrary, any ADR or any present or future provisions of the laws of the State of New York, the rights of holders of Shares and of any other Deposited Securities and the obligations and duties of the Company in respect of the holders of Shares and other Deposited Securities, as such, shall be governed by the laws of England and Wales (or, if applicable, such other laws as may govern the Deposited Securities).

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EACH OF THE PARTIES TO THE DEPOSIT AGREEMENT (INCLUDING, WITHOUT LIMITATION, EACH HOLDER AND BENEFICIAL OWNER) IRREVOCABLY WAIVES, TO THE FULLEST EXTENT PERMITTED BY APPLICABLE LAW, ANY AND ALL RIGHT TO TRIAL BY JURY IN ANY LEGAL PROCEEDING AGAINST THE COMPANY AND/OR THE DEPOSITARY ARISING OUT OF, OR RELATING TO, THE DEPOSIT AGREEMENT, ANY ADR AND ANY TRANSACTIONS CONTEMPLATED THEREIN (WHETHER BASED ON CONTRACT, TORT, COMMON LAW OR OTHERWISE).

A-26

(ASSIGNMENT AND TRANSFER SIGNATURE LINES)

FOR VALUE RECEIVED, the undersigned Holder hereby sell(s), assign(s) and transfer(s) unto ______________________________ whose taxpayer identification number is _______________________ and whose address including postal zip code is ________________, the within ADR and all rights thereunder, hereby irrevocably constituting and appointing ________________________ attorney‑in‑fact to transfer said ADR on the books of the Depositary with full power of substitution in the premises.

Dated:	Name:
		By:
		Title:

NOTICE: The signature of the Holder to this assignment must correspond with the name as written upon the face of the within instrument in every particular, without alteration or enlargement or any change whatsoever.

If the endorsement be executed by an attorney, executor, administrator, trustee or guardian, the person executing the endorsement must give his/her full title in such capacity and proper evidence of authority to act in such capacity, if not on file with the Depositary, must be forwarded with this ADR.

SIGNATURE GUARANTEED

All endorsements or assignments of ADRs must be guaranteed by a member of a Medallion Signature Program approved by the Securities Transfer Association, Inc.

Legends

[The ADRs issued in respect of Partial Entitlement American Depositary Shares shall bear the following legend on the face of the ADR: “This ADR evidences ADSs representing 'partial entitlement' Shares of the Company and as such do not entitle the holders thereof to the same per-share entitlement as other Shares (which are ‘full entitlement’ Shares) issued and outstanding at such time.  The ADSs represented by this ADR shall entitle holders to distributions and entitlements identical to other ADSs when the Shares represented by such ADSs become ‘full entitlement’ Shares.”]

A-27

EXHIBIT B

FEE SCHEDULE

ADS FEES AND RELATED CHARGES

All capitalized terms used but not otherwise defined herein shall have the meaning given to such terms in the Deposit Agreement.  Except as otherwise specified herein, any reference to ADSs herein includes Partial Entitlement ADSs, Full Entitlement ADSs, Certificated ADSs, Uncertificated ADSs, and Restricted ADSs.

I.           ADS Fees

The following ADS fees are payable under the terms of the Deposit Agreement:

Service	Rate	By Whom Paid
(1)   Issuance of ADSs (e.g., an issuance upon a deposit of Shares, upon a change in the ADS(s)-to-Share(s) ratio, or for any other reason), excluding issuances as a result of distributions described in paragraph (4) below.	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) issued.	Person for whom ADSs are issued.
(2)   Cancellation of ADSs (e.g.,a cancellation of ADSs for Delivery of deposited Shares, upon a change in the ADS(s)-to-Share(s) ratio, or for any other reason).	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) cancelled.	Person for whom ADSs are being cancelled.
(3)   Distribution of cash dividends or other cash distributions (e.g., upon a sale of rights and other entitlements).	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) held.	Person to whom the distribution is made.
(4)   Distribution of ADSs pursuant to (i) stock dividends or other free stock distributions, or (ii) an exercise of rights to purchase additional ADSs.	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) held.	Person to whom the distribution is made.

B-1

(5)   Distribution of securities other than ADSs or rights to purchase additional ADSs (e.g., spin-off shares).	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) held.	Person to whom the distribution is made.
(6)   ADS Services.	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) held on the applicable record date(s) established by the Depositary.	Person holding ADSs on the applicable record date(s) established by the Depositary.
(7)   Registration of ADS Transfers (e.g., upon a registration of the transfer of registered ownership of ADSs, upon a transfer of ADSs into DTC and vice versa, or for any other reason).	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) transferred.	Person for whom or to whom ADSs are transferred.
(8)   Conversion of ADSs of one series for ADSs of another series (e.g., upon conversion of Partial Entitlement ADSs for Full Entitlement ADSs, or upon conversion of Restricted ADSs into freely transferable ADSs, and vice versa).	Up to U.S. $5.00 per 100 ADSs (or fraction thereof) converted.	Person for whom ADSs are converted or to whom the converted ADSs are delivered.

II.          Charges

The Company, Holders, Beneficial Owners, persons depositing Shares or withdrawing Deposited Securities in connection with ADS issuances and cancellations, and persons for whom ADSs are issued or cancelled shall be responsible for the following ADS charges under the terms of the Deposit Agreement:

(i) taxes (including applicable interest and penalties) and other governmental charges;

(ii) such registration fees as may from time to time be in effect for the registration of Shares or other Deposited Securities on the share register and applicable to transfers of Shares or other Deposited Securities to or from the name of the Custodian, the Depositary or any nominees upon the making of deposits and withdrawals, respectively;

(iii) such cable, telex and facsimile transmission and delivery expenses as are expressly provided in the Deposit Agreement to be at the expense of the person depositing Shares or withdrawing Deposited Property or of the Holders and Beneficial Owners of ADSs;

B-2

(iv) in connection with the conversion of Foreign Currency, the fees, expenses, spreads, taxes and other charges of the Depositary and/or conversion service providers (which may be a division, branch or Affiliate of the Depositary).  Such fees, expenses, spreads, taxes, and other charges shall be deducted from the Foreign Currency;

(v) any reasonable and customary out-of-pocket expenses incurred in such conversion and/or on behalf of the Holders and Beneficial Owners in complying with currency exchange control or other governmental requirements; and

(vi) the fees, charges, costs and expenses incurred by the Depositary, the Custodian, or any nominee in connection with the ADR program.

The above fees and charges may at any time and from time to time be changed by agreement between the Company and the Depositary.

B-3

EX-4.3

Exhibit 4.3

EXECUTION VERSION

DATED 24 September 2019

AMRYT PHARMA PLC

INSTRUMENT CONSTITUTING ZERO COST WARRANTS TO
SUBSCRIBE FOR ORDINARY SHARES OF £0.06 EACH

GIBSON, DUNN & CRUTCHER UK LLP

Telephone House

2-4 Temple Avenue, London EC4Y 0HB
020 7071 4000 Tel       020 7071 4244 Fax
Ref: 103347081 / File no. 06287.000001

CONTENTS

Clause Subject Matter		Page
1.	INTERPRETATION	1
2.	SUBSCRIPTION RIGHTS	2
3.	CONSTITUTION AND FORM OF WARRANTS	3
4.	GOVERNING LAW	3
SCHEDULE 1 CERTIFICATE OF WARRANT AND EXERCISE NOTICE		4
SCHEDULE 2 TERMS AND CONDITIONS OF THE WARRANTS		7

THIS INSTRUMENT is executed on 24 September 2019

BY:

AMRYT PHARMA PLC, registered in England and Wales with company number 12107859 whose registered office is at Dept 920a, 196 High Road, Wood Green, London N22 8HH (the “Company”).

BACKGROUND:

(A) The Company has by a resolution of its Directors passed on 23 September 2019 resolved to create Warrants to subscribe for Ordinary Shares to be constituted in accordance with this Instrument.

(B) This Instrument and the Schedules constitute the Warrants.

(C) The Warrants constituted by this Instrument are being granted by the Company to one or more persons subscribing for Ordinary Shares pursuant to a subscription for Ordinary Shares being undertaken by the Company on or around the date of this Instrument.

NOW THIS INSTRUMENT WITNESSES and the Company hereby declares as follows:

1.             INTERPRETATION

1.1 In this Instrument, the following expressions shall have the following meanings:

“Act” means the Companies Act 2006;

“Admission” means admission of the Subscription Shares to trading on AIM in accordance with the AIM Rules and to trading on Euronext Growth in accordance with the Euronext Growth Rules;

“ADRs” means the American Depositary Receipts issued or to be issued by the Company and “ADR” shall be construed accordingly;

“AIM” means the market of that name operated by London Stock Exchange;

“AIM Rules” means the AIM Rules for Companies and guidance notes relating to AIM published by the London Stock Exchange as in force at the date of this Instrument, or where the context requires, as amended modified or adapted from time to time;

“Business Day” a day (excluding a Saturday, Sunday or a public holiday) on which the clearing banks are open for normal business in the City of London and Dublin;

“Certificate” means a certificate in the form set out in Schedule 1 to this Instrument;

“Conditions” means the terms and conditions of the Warrants as set out in Schedule 2;

“CREST” the computerised settlement system operated by Euroclear UK & Ireland Limited to facilitate the transfer of title to shares in uncertificated form;

“Deed” means this Instrument;

“Directors” means the directors of the Company (from time to time);

“Euronext Growth” means the Euronext Growth Market, a market operated by Euronext Dublin;

“Euronext Growth Rules” means the Euronext Growth Rules for Companies published by Euronext Dublin governing admission to, and the operation of, Euronext Growth as in force as at the date of this Instrument or, where the context so requires, as amended or modified after the date of this Instrument;

“Exercise Notice” as defined in Condition 1.1;

“London Stock Exchange” London Stock Exchange plc;

“Ordinary Shares” means ordinary shares of £0.06 (6 pence) each in the capital of the Company;

“Registered Office” means the registered office for the time being of the Company;

“Register of Warrantholders” means the register maintained by the Company of the holders of Warrants;

“Special Resolution” has the meaning given to it in Condition 5.2;

“Subscription Period” means any time on and from Admission;

“Subscription Rights” means the right to subscribe for Ordinary Shares conferred by the Warrants as set out in Schedule 2;

“Subscription Shares” means the Ordinary Shares being subscribed for as referred to in recital (C);

“Warrantholder” means a registered holder for the time being of Warrants; and

“Warrants” means the zero cost warrants constituted by this Instrument.

1.2 Terms defined in the articles of association of the Company as in force on the date of this Deed (the “Articles”) shall, unless otherwise defined in this Deed, have the same meaning when used in this Deed and the schedules hereto.

1.3 Words denoting the singular shall include the plural and vice versa.

1.4 Words denoting the masculine gender shall include the feminine gender.

1.5 Words denoting persons only shall include corporations.

2. SUBSCRIPTION RIGHTS

2.1 The Company undertakes that upon the exercise of the Subscription Rights by a Warrantholder in accordance with the Conditions it shall allot and issue to that Warrantholder the relevant number of Ordinary Shares to be allotted and issued pursuant to the Subscription Rights in accordance with the Conditions and, if at the time of exercise of the Subscription Rights, the Company has an American Depositary Share programme, it shall procure that the Ordinary Shares allotted and issued are deposited with the relevant depositary or custodian and that an ADR certificate is issued to the Warrantholder.

2 

3. CONSTITUTION AND FORM OF WARRANTS

3.1 The Company has created, pursuant to a resolution of its Directors dated 23 September 2019 and subject to the provisions of this Deed, the Warrants. Each Warrant shall be in registered form and shall entitle the holder to subscribe at any time during the Subscription Period for one Ordinary Share.

3.2 The Warrants shall be granted to the proposed Warrantholder by deed poll. The Company acknowledges that it has received due consideration for the Warrants and no further or other consideration shall be required from the proposed Warrantholder.

3.3 Each Warrantholder shall be entitled to a Certificate. A Warrant shall bear the date on which the holder(s) thereof are entered on the Register of Warrantholders.

3.4 Joint holders of Warrants will be entitled to only one Certificate in respect of their joint holding and such Certificate will be delivered to the joint holder who is first-named on the Register of Warrantholders in respect of the joint holding or to such other person as the joint holders may in writing direct to the Company.

3.5 The Company shall comply with the Conditions, which shall be deemed to be incorporated in this Deed and shall be binding on the Company and the holders of the Warrants (who shall be deemed to have notice of their terms) and all persons claiming through or under them respectively.

4. GOVERNING LAW

This Deed is governed by, and shall be construed in accordance with, English law, and the courts of England shall have exclusive jurisdiction to settle any dispute or non-contractual claim which may arise out of or in connection with this Deed.

3 

SCHEDULE 1

CERTIFICATE OF WARRANT AND EXERCISE NOTICE

Certificate Number:

Amryt Pharma Plc (the “Company”)

Registered in England and Wales No: [●]

ZERO COST WARRANT REPRESENTING SUBSCRIPTION RIGHTS FOR ORDINARY SHARES OF £0.06 EACH (“ORDINARY SHARES”)

This is to certify that [●] is the holder of [●] Warrants to subscribe at any time for Ordinary Shares fully paid in the Company subject to the articles of association of the Company and to the terms of the Warrant Instrument dated [●] 2019. The Warrantholder shall be entitled to exercise Subscription Rights to subscribe for Ordinary Shares upon exercise of the Warrants and may exercise such Subscription Rights in whole or in part and from time to time during the Subscription Period.

An Exercise Notice is set out overleaf.

Dated	20[●]
Executed and delivered as a deed by		)
AMRYT PHARMA PLC		)	Signature
acting by		)
in the presence of:		)
		)	Name
	Signature of witness
	Name (in block capitals)
	Address of witness
	Occupation of witness

No transfer of any or all of the Subscription Rights represented by this Warrant will be registered without the production of this Warrant Certificate or an indemnity satisfactory to the Company.

4 

EXERCISE NOTICE

To: The Directors
Amryt Pharma Plc (the “Company”)

From: [Warrantholder]
[Address]

Date: [●]

Part A

I/We, the registered holder(s) of this Warrant hereby give notice of my/our wish to exercise my/our Subscription Rights on [●]. This exercise notice is provided in respect of [●] Ordinary Shares of £0.06 each in the capital of the Company (“Ordinary Shares”) in accordance with the particulars below.

Part B

I/We agree to accept the Ordinary Shares to be allotted pursuant hereto subject to the articles of association of the Company. I/We desire all of such Ordinary Shares to be registered in my/our name(s) and authorise the entry of [my/our name(s)]/[the name of the ADR custodian] in the [register of members]/[ADR register] in respect thereof.

Part C

EITHER

I/We hereby authorise the delivery of the Ordinary Shares to be allotted to me/us to the CREST Stock Account having the CREST Participant ID and CREST Member Account ID set out below and the despatch of a Warrant in my/our name(s) for any balance of my/our Subscription Rights remaining exercisable by post at my/our risk to the address shown above or if no address is given to the registered address of the first named Warrantholder.

CREST Participant ID
CREST Member Account ID
Signature(s) of registered Warrantholder(s)	Date(s)

OR

I/We hereby authorise the despatch of the Certificate in respect of the Ordinary Shares in the Company to be allotted to me/us and a Warrant in my/our name(s) for any balance of my/our Subscription Rights remaining exercisable by post at my/our risk to the address shown above or if no address is given to the registered address of the first named Warrantholder.

OR

I/We hereby authorise the delivery of the Ordinary Shares to be allotted to me/us to the [ADR custodian] having the account ID set out below and the despatch of a Warrant in my/our name(s) for any balance of my/our Subscription Rights remaining exercisable by post at my/our risk to the address shown above or if no address is given to the registered address of the first named Warrantholder.

5 

Account ID
Signature(s) of registered Warrantholder(s)	Date(s)

NOTES:

1. In the case of joint holdings, all Warrantholders must sign. In the case of a corporation, this notice must be executed under its common seal or under the hand of an officer or attorney of the corporation duly authorised in that behalf.

2. Please insert in Part A the number of Ordinary Shares in respect of which the Subscription Rights are to be exercised. If no number of Ordinary Shares is inserted but the notice is otherwise duly complete, the notice will be deemed to relate to the total number of Warrants held by the relevant Warrantholder(s) on the date of this notice.

3. In order to exercise the Subscription Rights, the registered Warrantholder(s) must complete this notice of subscription and lodge it at the registered office of the Company in accordance with the Warrant Instrument. The rights are subject to adjustment as set out in the Warrant Instrument and completion and lodgement of this notice will in that event be deemed to be an exercise of the rights as so adjusted.

6 

SCHEDULE 2

Terms and Conditions of the Warrants

1. SUBSCRIPTION RIGHTS

1.1 A Warrantholder shall have the right in respect of each Warrant held by it to subscribe for one Ordinary Share at any time within the Subscription Period. In no event shall any Warrantholder have the right to subscribe for, nor shall the Company issue to such Warrantholder (and to the extent issued such issuance shall be deemed null and void), Ordinary Shares to the extent that such subscription would result in the Warrantholder and its affiliates, including groups that include the Warrantholder and its affiliates, together beneficially owning more than 4.99 per cent. or more than 9.99 per cent. (as the case may be) of the issued and outstanding Ordinary Shares.

1.2 In order to exercise Subscription Rights, in whole or in part, a Warrantholder must lodge its Certificate at the Registered Office on any Business Day within the Subscription Period, having completed the notice of exercise set out on the Warrantholder’s Certificate (“Exercise Notice”). Once lodged, an Exercise Notice shall be irrevocable save with the consent of the Directors. Compliance must also be made with any statutory or regulatory requirements for the time being applicable (e.g. anti-money laundering procedures). An Exercise Notice which is not completed and lodged in accordance with this Condition shall be of no effect.

1.3 The Subscription Rights shall be exercised subject in each case to any applicable fiscal or other laws or regulations applicable in the jurisdiction in which the Warrantholder is located.

1.4 Any Ordinary Shares allotted upon exercise of a Warrant will be fully paid and will in all respects rank pari passu with the fully paid Ordinary Shares in issue on the relevant exercise date (except in any such case for any right excluded by mandatory provisions of applicable law), except that the Ordinary Shares so allotted will not rank for any rights, distributions or payments where the record date or other due date for the establishment of entitlement to the same falls prior to the relevant date of allotment.

1.5 A Warrantholder exercising a Warrant must pay to the relevant authority any applicable taxes and capital, stamp, issue and registration duties arising on exercise of the relevant Warrant (other than any issue, capital, stamp or registration duties (or any like or similar taxes or duties) payable or arising in the United Kingdom in respect of the allotment, issue and/or delivery of any ADR certificate or Ordinary Shares (as applicable) resulting from such exercise, which shall be payable by the Company) and such holder shall be responsible for all, if any, taxes arising by reference to any disposal or deemed disposal of a Warrant or interest therein in connection with such exercise.

1.6 Any Ordinary Shares to be issued on exercise of the Warrants will be allotted and issued in uncertificated form by the Company through CREST or through the relevant settlement system operated by the ADR custodian, unless the Warrantholder elects to hold the Ordinary Shares in certificated registered form or, at the time of issue, the Ordinary Shares are not a participating security in CREST.

7 

1.7 Where Ordinary Shares are to be issued through CREST or through the relevant settlement system operated by the ADR custodian, they will be delivered to the account specified by the Warrantholder in the Exercise Notice on the date of admission of the Ordinary Shares to trading on AIM. If a Warrantholder elects to hold the Ordinary Shares in certificated registered form, the Ordinary Shares will be delivered to the account specified by the Warrantholder in the Exercise Notice and certificates for the Ordinary Shares issued on exercise of the Warrants will be despatched to the address specified in the Exercise Notice by mail free of charge (but uninsured and at the risk of the person entitled thereto) no later than 14 days after due completion and lodging at the Registered Office of the Exercise Notice. In the event of a partial exercise by a Warrantholder of the Subscription Rights comprised in its Certificate, the Company shall at the time of issue of the Ordinary Shares despatch by mail free of charge (but uninsured and at the risk of the person entitled thereto) a fresh Certificate in the name of the Warrantholder for any balance of his Subscription Rights remaining exercisable.

2. ADJUSTMENT OF SUBSCRIPTION RIGHTS

Upon any sub-division or consolidation of the Ordinary Shares on or by reference to such a date, the number and/or nominal value of Ordinary Shares to be subscribed for on any subsequent exercise of the Subscription Rights will be increased or reduced, as the case may be, as the auditors for the time being of the Company acting as experts and not as arbitrators (“Auditors”) shall certify as being necessary in order that, after such adjustment, a Warrantholder will be in the same economic position as it had been prior to the adjustment and notice of any such adjustment will be sent to each Warrantholder within 21 days thereafter. A Certificate reflecting the adjusted entitlement of each Warrantholder will be issued by the Company to each Warrantholder within 14 days of the surrender by it of its existing Certificate.

3. ADMISSION TO LISTING AND TRADING

3.1 If at the time of the exercise of Warrants, the Ordinary Shares (or any of them or, as the case may be, ADRs) are admitted to trading on AIM, Euronext Growth or NASDAQ (or listed on the Official List of the Financial Conduct Authority and admitted to trading on the London Stock Exchange’s main market for listed securities, or admitted to listing and/or trading on any other market or exchange), the Company will as soon as reasonably practicable apply for admission to trading (and listing if applicable) of such Ordinary Shares or, as the case may be, the ADRs on the relevant exchange and shall use all reasonable endeavours to secure such admission.

3.2 No application has or will be made to any stock exchange for the Warrants to be listed or otherwise traded or dealt in.

4. WINDING UP

If, at any time during the Subscription Period, an order is made or an effective resolution is passed for the voluntary winding up of the Company (except for the purpose of reconstruction or amalgamation, in which case the Company will procure that each Warrantholder is granted by the reconstructed or amalgamated company a substituted warrant of a value equivalent to the value of his Warrants immediately prior to such reconstruction or amalgamation in substitution, as the Warrantholder(s) acknowledge(s), for and to the exclusion of the Warrant) each Warrantholder will be entitled for the purpose of ascertaining its rights in the winding up to be treated as if it had immediately before the date of the passing of the resolution fully exercised its rights to acquire Ordinary Shares pursuant to its Warrants and in that event it shall be entitled to receive out of the assets available in the liquidation pari passu with the holders of the Ordinary Shares such a sum as it would have received had it been the holder of all such Ordinary Shares to which it would have become entitled by virtue of such exercise. The rights of the Warrantholders under this Condition 4 shall be calculated by the Auditors whose determination shall (save in the case of manifest error) bind the Company and the Warrantholders. Subject to this Condition the Warrants shall lapse on liquidation of the Company.

8 

5. VARIATION OF RIGHTS

5.1 Subject to Condition 5.2, all or any of the rights for the time being attached to the Warrants may from time to time (whether or not the Company is being wound up) be altered or abrogated with the consent in writing of the Company and with either the consent in writing of any Warrantholders entitled to subscribe for not less than 75 per cent. of the Ordinary Shares which are subject to outstanding Warrants or with the sanction of a Special Resolution of the Warrantholders. All the provisions of the articles of association of the Company as to general meetings of the Company shall mutatis mutandis apply to any separate meeting of the Warrantholders as though the Warrants were a class of shares and as if such provisions were expressly set out in this Instrument but so that:

(a) the necessary quorum shall be the Warrantholders (present in person or by proxy) entitled to subscribe for one-third in nominal amount of the Ordinary Shares subject to outstanding Warrants;

(b) every Warrantholder present in person at such meeting shall be entitled on a show of hands to one vote and every such Warrantholder present in person or by proxy at any such meeting shall be entitled on a poll to one vote for every Ordinary Share for which it is entitled to subscribe pursuant to the Warrants;

(c) any Warrantholder(s) entitled to ten per cent. or more of the aggregate outstanding Warrants present in person or by proxy may demand or join in demanding a poll; or

(d) if at any adjourned meeting a quorum (as above defined) is not present those holders of outstanding Warrants who are then present in person or by proxy shall be a quorum.

5.2 “Special Resolution” for the purposes of this Condition 5 means a resolution proposed at a meeting of the Warrantholders duly convened and held and passed by a majority consisting of not less than 75 per cent. of the votes cast, whether on a show of hands or on a poll.

5.3 In addition, the Directors may amend the provisions of the Warrants without the consent of the Warrantholders if such amendment would not be prejudicial to the interests of the Warrantholders. Any such amendment shall be notified to the Warrantholders in writing as soon as reasonably practicable.

9 

6. TAKEOVER PROVISIONS

6.1 Subject to Condition 6.2, if at any time an offer is made to all holders of Ordinary Shares (or all such holders other than the offeror and/or any company controlled by the offeror and/or any person acting in concert with the offeror (the “Offeror”)) to acquire the whole or any part of such Ordinary Shares as a result of which the right to cast a majority of the votes which may ordinarily be cast on a poll at a general meeting may become vested in the Offeror (a “General Offer”), the Company shall give notice to the Warrantholders of such offer within five days of it being made; the publication of a scheme of arrangement under Part 26 of the Companies Act 2006 (“Scheme”) providing for the acquisition by any person of the whole or any part of such Ordinary Shares shall be deemed to be the making of an offer for the purposes of this Condition 6.1.

6.2 If, following or in conjunction with, a General Offer the Offeror makes available an offer (whether in cash or in the form of warrants to subscribe for ordinary shares of the Offeror or otherwise) for the Warrants which the financial advisers to the Company consider in their opinion (acting as experts not as arbitrators) is fair and reasonable having regard to the terms of the General Offer and any other circumstances which may appear to the financial advisers to be relevant (“Warrant Offer”), then if the Warrantholder does not accept such Warrant Offer or exercise his Subscription Rights within 28 days of the date of receipt of the notice of the Warrant Offer, any Director shall be authorised as attorney and agent for the Warrantholder to and shall:

(a) execute a transfer thereof in favour of the Offeror in consideration of the payment of the consideration provided under the terms of the Warrant Offer; and

(b) do such acts and things as may be necessary or appropriate in connection therewith,

subject in both (a) and (b) aforesaid and in all circumstances to the General Offer becoming or being declared wholly unconditional or in the case of a Scheme to it being sanctioned by the court and the order filed with the registrar of companies in accordance with section 899 of the Companies Act 2006.

6.3 Subject to Condition 6.2 the Subscription Rights will lapse to the extent that they have not been exercised within one month of the General Offer becoming or being declared wholly unconditional or in the case of a Scheme to it being sanctioned by the court and the order filed with the registrar of companies in accordance with section 899 of the Companies Act 2006.

7. LOST OR DESTROYED CERTIFICATES

7.1 If any Certificate is worn out or defaced then upon production of such Certificate to the Directors they may cancel it and issue a new replacement Certificate. If any Certificate be lost or destroyed then upon proof thereof to the reasonable satisfaction of the Directors (or in default of proof, on such indemnity as the Directors may deem adequate being given) a new replacement Certificate may be given to the persons entitled to such lost or destroyed Certificate free of charge (save as regards any payment pursuant to any such indemnity).

10 

7.2 An entry as to the issue of the new Certificate and indemnity (if any) shall be made in the Register of Warrantholders.

8. NOTICES

8.1 Any notice or other document (including a Certificate) may be given or sent to any Warrantholder by sending the same by post in a pre-paid envelope addressed to such Warrantholder to its registered address or to the address (if any) supplied by it to the Company for the giving of notice to it.

8.2 In the case of joint holders a notice given to the Warrantholder whose name stands first in the Register of Warrantholders shall be sufficient notice to all joint holders.

8.3 Any notice required to be given to the Company may be given either personally or by sending it by post to the Registered Office marked for the attention of the Company Secretary or to such other address as the Company may from time to time notify Warrantholders.

8.4 Any notice given or document sent by post shall be deemed to be served on the day after it is posted or, if such a day is not a Business Day, then on the next following Business Day. In proving such service or receipt it shall be sufficient to prove that the envelope containing the notice or document was properly addressed, stamped and posted.

8.5 Any Warrantholder who shall from time to time give to the Company an address at which any notice may be served upon him shall be entitled to have notice served on him at such address.

8.6 Any person who by operation of law, transmission or other means whatsoever shall become entitled to any Warrant shall be bound by every notice in respect of such Warrant which prior to his name and address being entered on the Register of Warrantholders shall be duly given to the person from whom he derives his title to such Warrant.

9. OTHER PROVISIONS

9.1 The Company shall ensure it has the relevant shareholder authorities to issue sufficient share capital to satisfy in full all Subscription Rights remaining exercisable.

9.2 The Company shall send to the Warrantholders a copy of every document sent by it to the holders of its Ordinary Shares at the same time as it is sent to such holders.

9.3 A Certificate shall be issued to each Warrantholder in respect of its registered holding of Warrants. The persons in whose names Warrants are registered will (except as required by law or where ordered by a court of competent jurisdiction) be deemed to be, and be treated as, the holders and absolute owners of the relevant Warrants for all purposes (regardless of any notice of ownership, trust or any interest in them or their theft or loss) and no person shall be liable for treating the holders as such.

9.4 The Warrants are direct and unsecured obligations of the Company, ranking pari passu and without any preference among themselves and (save for any obligations to be preferred by law) at least equally with the Company’s other present and future unsecured and unsubordinated obligations.

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9.5 A copy of the Warrant Instrument shall be kept at the Registered Office. A Warrantholder and any person authorised by a Warrantholder may at all reasonable times during office hours inspect such copy.

10. TRANSFER

10.1 The Warrants are not transferable except with the prior written consent of the Company. Where Warrants are transferred in accordance with this Condition, promptly upon receipt of the written transfer instrument and Certificate by the Company (and subject to compliance with any relevant statute relating to stamp duties), the Company shall register the transfer and issue a new Certificate to the transferee for the number of Warrants comprised in the transfer and, where applicable, a Certificate for the balance of any Warrants to the transferor.

10.2 No beneficial interest in any Warrant shall be disposed of without the presentation for registration of a transfer and Certificate in respect of such Warrant in accordance with this Condition.

11. PURCHASE

11.1 The Company and its subsidiaries shall have the right to offer to purchase Warrants by tender (available to all Warrantholders alike) or by private treaty, in either case at any price. All Warrants so purchased by the Company shall be cancelled and shall not be available for reissue or resale.

11.2 Any Warrant purchased by a subsidiary of the Company shall be sold to the Company and cancelled.

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IN WITNESS whereof this deed has been executed and delivered on the date first above written.

Executed and delivered as a deed by AMRYT PHARMA PLC acting by		) )	Signature
one director		)
in the presence of:		)
		)	Name	Joe Wiley
	Signature of witness
	Name (in block capitals)
	Address of witness		90 Harcourt Street
			Dublin 2, Ireland
	Occupation of witness		Solicitor

<signature page to Zero Cost Warrant Instrument>

13 

EX-10.1

Exhibit 10.1

UNITED STATES BANKRUPTCY COURT
SOUTHERN DISTRICT OF NEW YORK
---------------------------------------------------------	x
	:
In re:	:	Chapter 11
	:
Aegerion Pharmaceuticals, Inc., et al.,1	:	Case No. 19-11632 (MG)
	:
Debtors.	:	(Jointly Administered)
	:
---------------------------------------------------------	x

DEBTORS’ MODIFIED FIRST AMENDED JOINT CHAPTER 11 PLAN

Dated:	New York, New York
	August 29, 2019
		WILLKIE FARR & GALLAGHER LLP
		Counsel for the Debtors and Debtors in Possession
		787 Seventh Avenue
		New York, New York 10019
		(212) 728-8000

¹ The Debtors in these chapter 11 cases and the last four digits of each Debtor’s federal taxpayer identification number are Aegerion Pharmaceuticals, Inc. (0116), and Aegerion Pharmaceuticals Holdings, Inc. (1331). The Debtors’ executive headquarters are located at 245 First Street, Riverview II, 18th Floor, Cambridge, MA 02142.

TABLE OF CONTENTS

INTRODUCTION				1
ARTICLE I.			DEFINITIONS AND INTERPRETATION	1
ARTICLE II.			CERTAIN INTER-CREDITOR AND INTER-DEBTOR ISSUES	20
	2.1.	Settlement of Certain Inter-Creditor Issues		20
	2.2.	Formation of Debtor Group for Convenience Purposes		20
	2.3.	Intercompany Claims and Intercompany Interests		20
ARTICLE III.			DIP CLAIMS, ADMINISTRATIVE EXPENSE CLAIMS, FEE CLAIMS, U.S. TRUSTEE FEES AND PRIORITY TAX CLAIMS	21
	3.1.	DIP Claims		21
	3.2.	Administrative Expense Claims		22
	3.3.	Fee Claims		24
	3.4.	U.S. Trustee Fees		24
	3.5.	Priority Tax Claims		24
ARTICLE IV.			CLASSIFICATION OF CLAIMS AND INTERESTS	25
	4.1.	Classification of Claims and Interests		25
	4.2.	Unimpaired Classes of Claims		26
	4.3.	Impaired Classes of Claims		26
	4.4.	Separate Classification of Other Secured Claims		26
ARTICLE V.			TREATMENT OF CLAIMS AND INTERESTS	27
	5.1.	Priority Non-Tax Claims (Class 1)		27
	5.2.	Other Secured Claims (Class 2)		27
	5.3.	Bridge Loan Claims (Class 3)		28
	5.4.	Novelion Intercompany Loan Claims (Class 4)		28
	5.5.	Government Settlement Claims (Class 5)		29
	5.6.	Ongoing Trade Claims (Class 6A)		30
	5.7.	Other General Unsecured Claims (Class 6B)		31
	5.8.	Existing Securities Law Claims (Class 7)		31
	5.9.	Existing Interests (Class 8)		31
	5.10.	Special Provision Governing Unimpaired Claims		32
ARTICLE VI.			ACCEPTANCE OR REJECTION OF THE PLAN; EFFECT OF REJECTION BY ONE OR MORE CLASSES OF CLAIMS OR INTERESTS	32
	6.1.	Class Acceptance Requirement		32
	6.2.	Tabulation of Votes on a Non-Consolidated Basis		32

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	6.3.	Confirmation Pursuant to Section 1129(b) of the Bankruptcy Code or “Cramdown.”		32
	6.4.	Elimination of Vacant Classes		32
	6.5.	Voting Classes; Deemed Acceptance by Non-Voting Classes		33
	6.6.	Confirmation of All Cases		33
ARTICLE VII.			MEANS FOR IMPLEMENTATION	33
	7.1.	Non-Substantive Consolidation		33
	7.2.	Plan Funding Transaction		33
	7.3.	Rights Offering		34
	7.4.	Plan Funding		35
	7.5.	New Term Loan Facility; New Convertibles Notes		35
	7.6.	Authorization, Issuance and Delivery of Plan Securities by the Plan Investor		35
	7.7.	Continued Corporate Existence and Vesting of Assets		37
	7.8.	Cancellation of Existing Securities and Agreements		38
	7.9.	Boards		38
	7.10.	Management		39
	7.11.	Corporate Action		39
	7.12.	[Reserved]		40
	7.13.	Payment of Convertible Notes Trustee Fees		40
	7.14.	Comprehensive Settlement of Claims and Controversies		40
	7.15.	Additional Transactions Authorized Under This Plan		40
	7.16.	Shared Services Agreements		40
	7.17.	Acceptable		41
ARTICLE VIII.			DISTRIBUTIONS	41
	8.1.	Distributions		41
	8.2.	No Postpetition Interest on Claims		41
	8.3.	Date of Distributions		41
	8.4.	Distribution Record Date		42
	8.5.	Disbursing Agent		42
	8.6.	Delivery of Distributions in General		43
	8.7.	Delivery of Distributions on Convertible Notes Claims		43
	8.8.	Unclaimed Property		44
	8.9.	Satisfaction of Claims		44
	8.10.	Manner of Payment Under Plan		44
	8.11.	Fractional Shares; De Minimis Cash Distributions		44
	8.12.	Distributions on Account of Allowed Claims Only		45
	8.13.	No Distribution in Excess of Amount of Allowed Claim		45
	8.14.	Exemption from Securities Laws		45
	8.15.	Setoffs and Recoupments		46
	8.16.	Withholding and Reporting Requirements		46
	8.17.	Hart-Scott Rodino Antitrust Improvements Act		46

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ARTICLE IX.			PROCEDURES FOR RESOLVING CLAIMS	47
	9.1.	Claims Process		47
	9.2.	Amendment to Claims		47
	9.3.	Disputed Claims		47
	9.4.	Estimation of Claims		47
ARTICLE X.			EXECUTORY CONTRACTS AND UNEXPIRED LEASES	48
	10.1.	General Treatment		48
	10.2.	Claims Based on Rejection of Executory Contracts or Unexpired Leases		48
	10.3.	Cure of Defaults for Assumed Executory Contracts and Unexpired Leases		49
	10.4.	Effect of Confirmation Order on Assumption, Assumption and Assignment, and Rejection		50
	10.5.	Modifications, Amendments, Supplements, Restatements, or Other Agreements		51
	10.6.	Compensation and Benefit Programs		51
ARTICLE XI.			CONDITIONS PRECEDENT TO CONSUMMATION OF THE PLAN	52
	11.1.	Conditions Precedent to the Effective Date		52
	11.2.	Satisfaction and Waiver of Conditions Precedent		53
	11.3.	Effect of Failure of Conditions		53
ARTICLE XII.			EFFECT OF CONFIRMATION	54
	12.1.	Binding Effect		54
	12.2.	Discharge of Claims Against and Interests in the Debtors		54
	12.3.	Term of Pre-Confirmation Injunctions or Stays		54
	12.4.	Injunction Against Interference with the Plan		54
	12.5.	Injunction		55
	12.6.	Releases		56
	12.7.	Exculpation and Limitation of Liability		60
	12.8.	Injunction Related to Releases and Exculpation		60
	12.9.	Retention of Causes of Action/Reservation of Rights		61
	12.10.	Indemnification Obligations		61
ARTICLE XIII.			RETENTION OF JURISDICTION	62
ARTICLE XIV.			MISCELLANEOUS PROVISIONS	63
	14.1.	Exemption from Certain Transfer Taxes		63
	14.2.	Retiree Benefits		64
	14.3.	Dissolution of Creditors’ Committee		64
	14.4.	Termination of Professionals		64

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14.5.	Amendments	64
14.6.	Revocation or Withdrawal of this Plan	65
14.7.	Allocation of Plan Distributions Between Principal and Interest	65
14.8.	Severability	65
14.9.	Governing Law	66
14.10.	Section 1125(e) of the Bankruptcy Code	66
14.11.	Inconsistency	66
14.12.	Time	66
14.13.	Exhibits	66
14.14.	Notices	67
14.15.	Filing of Additional Documents	67
14.16.	Reservation of Rights	67

iv

INTRODUCTION²

Aegerion Pharmaceuticals, Inc. and Aegerion Pharmaceuticals Holdings, Inc., the debtors and debtors in possession in the above-captioned cases, propose the following joint chapter 11 plan of reorganization for the resolution of the Claims against and Interests in the Debtors.

Reference is made to the Disclosure Statement accompanying this Plan, including the exhibits and supplements thereto, for a discussion of the Debtors’ history, business, properties and operations, projections for those operations, risk factors, a summary and analysis of this Plan, and certain related matters including certain tax matters, and the securities and other consideration to be issued and/or distributed under this Plan. Subject to certain restrictions and requirements set forth in 11 U.S.C. § 1127, Fed. R. Bankr. P. 3019 and Sections 14.5 and 14.6 of this Plan, the Debtors, subject to the parties’ rights under the RSA and the Plan Funding Agreement, reserve the right to alter, amend, modify, revoke or withdraw this Plan prior to its substantial consummation.

The only Persons that are entitled to vote on this Plan are the holders of Bridge Loan Claims, Novelion Intercompany Loan Claims, and Other General Unsecured Claims. Such Persons are encouraged to read the Plan and the Disclosure Statement and their respective exhibits and schedules in their entirety before voting to accept or reject the Plan. No materials other than the Disclosure Statement, the respective schedules, notices and exhibits attached thereto and referenced therein have been authorized by the Bankruptcy Court for use in soliciting acceptances or rejections of the Plan.

ARTICLE I.

DEFINITIONS AND INTERPRETATION

A.        Definitions.

The following terms shall have the meanings set forth below (such meanings to be equally applicable to both the singular and plural):

1.1.        503(b)(9) Claims means Claims that have been timely and properly filed prior to the Bar Date and that are granted administrative expense priority treatment pursuant to section 503(b)(9) of the Bankruptcy Code.

1.2.        Acceptable shall have the meaning given it in Section 7.17 hereof.

1.3.        Ad Hoc Group means the ad hoc group of certain Bridge Loan Lenders and/or Convertible Noteholders that are signatories to the RSA and represented by Latham & Watkins, LLP and King & Spalding, LLP.

²       Capitalized terms not defined herein have the meanings given them in Article I herein.

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1.4.        Ad Hoc Group Fee Claim means any Claim, to the extent not previously paid, for the reasonable and documented out-of-pocket fees, expenses, costs and other charges incurred by the Ad Hoc Group (including those of Latham & Watkins, LLP, King & Spalding LLP and Ducera Partners LLC), the Debtors’ payment of which is provided for in the DIP Order, the RSA or this Plan, which Claim shall be Allowed on the Effective Date.

1.5.        Administrative Bar Date has the meaning set forth in Section 3.2(a) of this Plan.

1.6.        Administrative Expense Claim means any right to payment constituting a cost or expense of administration of the Chapter 11 Cases of the kind specified in section 503(b) of the Bankruptcy Code and entitled to priority pursuant to sections 328, 330, 363, 364(c)(1), 365, 503(b), 507(a)(2), or 507(b) of the Bankruptcy Code (other than a DIP Claim, Fee Claim or U.S. Trustee Fees) incurred during the period from the Petition Date to the Effective Date, including: (a) any actual and necessary costs and expenses of preserving the Estates, any actual and necessary costs and expenses of operating the Debtors’ business, and any indebtedness or obligations incurred or assumed by any of the Debtors during the Chapter 11 Cases; (b) 503(b)(9) Claims; and (c) any payment to be made under this Plan to cure a default under an assumed executory contract or unexpired lease.

1.7.        Aegerion means Aegerion Pharmaceuticals, Inc., a Delaware corporation.

1.8.        Aegerion Holdings means Aegerion Pharmaceuticals Holdings, Inc., a Delaware corporation.

1.9.        Allowed means, with respect to a Claim under this Plan, a Claim that is an Allowed Claim or an Allowed __________ Claim.

1.10.      Allowed Claim or Allowed __________ Claim (with respect to a specific type of Claim, if specified) means: (a) any Claim (or a portion thereof) as to which no action to dispute, disallow, deny, equitably subordinate or otherwise limit recovery with respect thereto, or alter the priority thereof (including a claim objection), has been timely commenced within the applicable period of limitation fixed by this Plan or applicable law, or, if an action to dispute, disallow, deny, equitably subordinate or otherwise limit recovery with respect thereto, or alter priority thereof, has been timely commenced, to the extent such Claim has been allowed (whether in whole or in part) by a Final Order of a court of competent jurisdiction with respect to the subject matter; or (b) any Claim or portion thereof that is allowed (i) in any contract, instrument, or other agreement entered into in connection with this Plan, (ii) pursuant to the terms of this Plan, (iii) by Final Order of the Bankruptcy Court, or (iv) with respect to an Administrative Expense Claim only (x) that was incurred by a Debtor in the ordinary course of business during the Chapter 11 Cases to the extent due and owing without defense, offset, recoupment or counterclaim of any kind, and (y) that is not otherwise disputed.

1.11.      Amended Certificates of Formation means the amended and restated certificates of formation or similar constitutive document for the Reorganized Debtors (as may be amended, modified or supplemented from time to time), on terms and conditions reasonably satisfactory to the Debtors, the Required Parties, and the Committee. A form of the Amended Certificate of Formation will be filed as part of the Plan Supplement.

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1.12.       Amended Memorandum of Association means the amended and restated memorandum of association for the Plan Investor (as may be amended, modified or supplemented from time to time), on terms and conditions Acceptable to the Debtors, the Required Parties, and the Committee. A form of the Amended Memorandum of Association shall be included in the Plan Supplement.

1.13.       Applicable Interest Rate means interest accruing at the Federal Judgment Rate, or such other rate of interest required to render such Claim Unimpaired as may be determined by the Bankruptcy Court.

1.14.      Athyrium means Athyrium Capital Management, LP and its affiliates and the investment funds managed or advised by any of the foregoing.

1.15.      Backstop Commitment means the commitment of the Backstop Parties to purchase Unsubscribed Shares as set forth in the Backstop Commitment Agreement.

1.16.      Backstop Commitment Agreement means that certain Backstop Subscription Agreement, by and among the Plan Investor and the Backstop Parties as required pursuant to the terms of the RSA (as amended, modified and/or supplemented from time to time in accordance with the terms therein).

1.17.      Backstop Commitment Fee means a commitment fee, pursuant to and as consideration for the obligations of the Backstop Parties under the Backstop Commitment Agreement, equal to 5% of the Rights Offering Amount and the Plan Investor Equity Raise Amount, in the aggregate, earned immediately upon the Subscription Commencement Date and payable by the Plan Investor on the Effective Date as set forth in, and subject to the terms and conditions of the Backstop Commitment Agreement.

1.18.      Backstop Parties means the entities party to the Backstop Commitment Agreement.

1.19.      Ballot means the form distributed by the Debtors or the Claims Agent to holders of impaired Claims entitled to vote on this Plan on which the acceptance or rejection of this Plan is to be indicated.

1.20.      Bankruptcy Code means title 11 of the United States Code, as amended from time to time, as applicable to the Chapter 11 Cases.

1.21.      Bankruptcy Court means the United States Bankruptcy Court for the Southern District of New York, or any other court exercising competent jurisdiction over the Chapter 11 Cases or any proceeding therein.

1.22.      Bankruptcy Rules means the Federal Rules of Bankruptcy Procedure, as promulgated by the Supreme Court of the United States under section 2075 of title 28 of the United States Code, as amended from time to time, as applicable to the Chapter 11 Cases, and any local rules of the Bankruptcy Court.

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1.23.      Bar Date means any deadline for filing proofs of Claim, including Claims arising prior to the Petition Date (including 503(b)(9) Claims) and Administrative Expense Claims, as established by an order of the Bankruptcy Court or under the Plan.

1.24.      Bridge Loan means the New Money Bridge Loan and the Roll Up Loan.

1.25.      Bridge Loan Administrative Agent means Cantor Fitzgerald Securities, or its successors and assigns, in its capacity as collateral agent and administrative agent for the Bridge Loan Lenders under the Bridge Loan Credit Agreement.

1.26.      Bridge Loan Claim means the New Money Bridge Loan Claim and the Roll Up Claim.

1.27.      Bridge Loan Credit Agreement means that certain Bridge Credit Agreement, dated as of November 8, 2018 (as amended, modified or supplemented from time to time), among Aegerion, as borrower, Aegerion Holdings, as guarantor, the Bridge Loan Administrative Agent, as administrative agent and collateral agent, and the Bridge Loan Lenders, including all agreements, documents, notes, instruments and any other agreements delivered pursuant thereto or in connection therewith (in each case, as amended, modified or supplemented from time to time).

1.28.      Bridge Loan Lender means any lender, in its capacity as such, in connection with the Bridge Loan under the Bridge Loan Credit Agreement, and its successors and assigns.

1.29.      Business Day means any day other than a Saturday, Sunday, a “legal holiday,” as defined in Bankruptcy Rule 9006(a), or a day on which banks are not open for general business in New York, New York.

1.30.      Cash means the legal currency of the United States and equivalents thereof.

1.31.      Causes of Action means any and all actions, causes of action (including causes of action under sections 510, 541, 544, 545, 546, 547, 548, 549, 550 and 553 of the Bankruptcy Code), suits, accounts, controversies, obligations, judgments, damages, demands, debts, rights, agreements, promises, rights to legal remedies, rights to equitable remedies, rights to payment, and claims (as defined in section 101(5) of the Bankruptcy Code), whether known or unknown, reduced to judgment, not reduced to judgment, liquidated, unliquidated, fixed, contingent, matured, unmatured, disputed, undisputed, secured, unsecured and whether asserted or assertable directly or derivatively, whether arising before, on, or after the Petition Date, in contract or tort, arising in law, equity or otherwise.

1.32.      Chapter 11 Cases means the jointly-administered cases under chapter 11 of the Bankruptcy Code commenced by the Debtors on the Petition Date in the Bankruptcy Court and captioned In re Aegerion Pharmaceuticals, Inc., et al., Case No. 19-11632 (MG).

1.33.      Claim means any “claim” as defined in section 101(5) of the Bankruptcy Code against any Debtor or property of any Debtor, including any Claim arising after the Petition Date.

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1.34.      Claims Agent means Prime Clerk LLC or any other entity approved by the Bankruptcy Court to act as the Debtors’ claims and noticing agent pursuant to 28 U.S.C. §156(c).

1.35.      Class means each category of Claims or Interests established under Article IV of this Plan pursuant to sections 1122 and 1123(a)(1) of the Bankruptcy Code.

1.36.      Class 4 New Common Stock Distribution means the shares of New Common Stock available for distribution under the Plan to Class 4, which shall equal 16.5% of the New Common Stock Distribution (including any New Common Stock issuable upon exercise of the New Warrants), subject to the Prepetition Shared Services Adjustment and the Prepetition Transaction Proceeds Adjustment which shall, in each case, result in a reduction of the New Common Stock Distribution to be distributed under the Plan to Class 4 in an amount equal to the ratable reduction of the Allowed Novelion Intercompany Loan Claim.

1.37.      Class 6B New Common Stock Distribution means the shares of New Common Stock available for distribution under the Plan to Class 6B, which shall equal 83.5% of the New Common Stock Distribution (including any New Common Stock issuable upon exercise of the New Warrants), subject to the Prepetition Shared Services Adjustment and the Prepetition Transaction Proceeds Adjustment which shall, in each case, result in an increase of the New Common Stock Distribution to be distributed under the Plan to Class 6B in an amount equal to the reduction of the New Common Stock Distribution to be distributed under the Plan to Class 4.

1.38.      Collateral means any property, wherever located, or interest in property of the Estates subject to a Lien to secure the payment or performance of a Claim.

1.39.      Competition Laws means the Hart-Scott-Rodino Antitrust Improvements Act of 1976, as amended, and any other competition or merger control law.

1.40.      Confirmation Date means the date on which the Clerk of the Bankruptcy Court enters the Confirmation Order on the docket of the Bankruptcy Court.

1.41.      Confirmation Hearing means a hearing to be held by the Bankruptcy Court regarding confirmation of this Plan, as such hearing may be adjourned or continued from time to time.

1.42.      Confirmation Order means the order of the Bankruptcy Court confirming this Plan pursuant to section 1129 of the Bankruptcy Code, the form and substance of which shall be Acceptable to the Debtors, the Required Parties, and the Committee as may be amended, modified, or supplemented from time to time with the consent of the Debtors, each of the Required Parties, and the Committee.

1.43.      Consenting Lenders means, as of the relevant time, Novelion, the Bridge Loan Lenders and the Convertible Noteholders that are party to the RSA.

1.44.      Convertible Noteholder means any holder, in its capacity as such, of the Convertible Notes pursuant to the Convertible Notes Indenture.

5

1.45.      Convertible Notes means the 2.00% convertible senior unsecured notes due 2019 issued pursuant to the Convertible Notes Indenture in the aggregate outstanding principal amount of $302,500,000.

1.46.      Convertible Notes Claim means all Claims against any Debtor, related to, arising under, on in connection with, the Convertible Notes Indenture and the Convertible Notes.

1.47.      Convertible Notes Indenture means that certain Indenture, dated as of August 15, 2014, governing the issuance of the Convertible Notes, by and between Aegerion, as issuer, and the Convertible Notes Trustee, as trustee, including all agreements, documents, notes, instruments and any other agreements delivered pursuant thereto or in connection therewith (in each case, as amended, modified or supplemented from time to time).

1.48.      Convertible Notes Trustee means The Bank of New York Mellon Trust Company, N.A., or its successors and assigns, in its capacity as trustee for the Convertible Noteholders under the Convertible Notes Indenture.

1.49.      Convertible Notes Trustee Fees means all outstanding reasonable and documented fees, expenses and compensation of the Convertible Notes Trustee (including the fees and expenses of its outside counsel and other professionals), whether prior to or after the Effective Date, to the extent provided under the Convertible Notes Indenture.

1.50.      Committee means the official committee of unsecured creditors appointed in the Chapter 11 Cases pursuant to the Notice of Appointment of Committee of Unsecured Creditors [Docket No. 56], as may be reconstituted from time to time.

1.51.      Cure Amount has the meaning set forth in Section 10.3(a) of this Plan.

1.52.      Cure Dispute has the meaning set forth in Section 10.3(c) of this Plan.

1.53.      Cure Schedule has the meaning set forth in Section 10.3(b) of this Plan.

1.54.      Debtor(s) means, individually or collectively, as the context requires, (a) Aegerion, and (b) Aegerion Holdings, which commenced the Chapter 11 Cases on the Petition Date.

1.55.      DIP Administrative Agent means Cantor Fitzgerald Securities, solely in its capacity as administrative agent and collateral agent under the DIP Financing Agreement, or any other administrative agent appointed pursuant to the terms therein.

1.56.      DIP Claims means all Claims of the DIP Administrative Agent and/or the DIP Lenders related to, arising under, or in connection with a DIP Order and the DIP Financing Documents, including Claims for all principal amounts outstanding, interest, fees, reasonable and documented expenses (including the reasonable and documented expenses of counsel as set forth in the DIP Financing Agreement), costs and other charges of the DIP Administrative Agent and the DIP Lenders in respect of the obligations of the Debtors arising under the DIP Financing Agreement.

6

1.57.      DIP Financing Agreement means the Senior Secured Super-Priority Debtor in Possession Financing Agreement, dated as of May 20, 2019, by and among the Debtors, the DIP Administrative Agent, and the DIP Lenders, as the same may be modified, amended or supplemented from time to time, in accordance with the terms thereof.

1.58.      DIP Financing Documents means the DIP Financing Agreement and all other agreements, documents and instruments entered into in connection with the DIP Financing Agreement.

1.59.      DIP Lenders means, collectively, and as of the relevant time, those lenders that are party to the DIP Financing Agreement.

1.60.      DIP Order means the order or orders of the Bankruptcy Court authorizing and approving the Debtors’ entry into the DIP Financing Agreement or the Debtors’ use of cash collateral.

1.61.      Disallowed means a finding or conclusion of law of the Bankruptcy Court in a Final Order, or provision in this Plan or the Confirmation Order, disallowing a Claim or Interest.

1.62.      Disbursing Agent means the applicable Reorganized Debtor, or the entity designated by such Reorganized Debtor, to distribute the Plan Consideration.

1.63.      Disclosure Statement means the disclosure statement that relates to this Plan, including all exhibits and schedules annexed thereto or referred to therein (in each case, as it or they may be amended, modified, or supplemented from time to time), which shall be in form and substance Acceptable to the Debtors and each of the Required Parties.

1.64.      Disclosure Statement Hearing means a hearing held by the Bankruptcy Court to consider approval of the Disclosure Statement as containing adequate information as required by section 1125 of the Bankruptcy Code, as the same may be adjourned or continued from time to time.

1.65.      Disclosure Statement Order means an order of the Bankruptcy Court approving the Disclosure Statement as having adequate information in accordance with section 1125 of the Bankruptcy Code.

1.66.      Disputed Claim means, with respect to a Claim or Interest, that portion (including, when appropriate, the whole) of such Claim or Interest that: (a) (i) has not been scheduled by the Debtors in their Schedules, or has been scheduled in a lesser amount or priority than the amount or priority asserted by the holder of such Claim or Interest, or (ii) has been scheduled as contingent, unliquidated or disputed and for which no proof of claim has been timely filed; (b) is the subject of an objection or request for estimation filed in the Bankruptcy Court which has not been withdrawn or overruled by a Final Order; and/or (c) is otherwise disputed by any of the Debtors or Reorganized Debtors in accordance with applicable law or contract, which dispute has not been withdrawn, resolved, or overruled by Final Order.

1.67.      Distribution Date means: (a) with respect to DIP Claims, Bridge Loan Claims, and Novelion Intercompany Loan Claims, the Effective Date (or as soon thereafter as reasonably

7

practicable), (b) with respect to Administrative Expense Claims, Priority Non-Tax Claims, U.S. Trustee Fees, Priority Tax Claims, Other Secured Claims, Other General Unsecured Claims and Ongoing Trade Claims, the date that is the latest of: (i) the Effective Date (or any date within fifteen (15) days thereafter); (ii) the date such Claim would be due and payable in the ordinary course of business; and (iii) the date that is fifteen (15) days after such Claim becomes an Allowed Claim or otherwise becomes payable under the Plan (or, if such date is not a Business Day, on the next Business Day thereafter), and (c) with respect to Fee Claims, the date (or as soon thereafter as reasonably practicable) that such Claims are allowed by Final Order.

1.68.      Distribution Record Date means with respect to all Classes, the Effective Date.

1.69.      DTC means The Depository Trust Company.

1.70.      Effective Date means the date specified by the Debtors (such date being Acceptable to the Required Parties), in consultation with the Committee, in a notice filed with the Bankruptcy Court as the date on which this Plan shall take effect, which date shall be the first Business Day on which all of the conditions set forth in Section 11.1 of this Plan have been satisfied or waived and no stay of the Confirmation Order is in effect.

1.71.      Eligible Holders means any holder of a Claim in Class 4 and Class 6B as of the record date set forth in the Rights Offering Procedures.

1.72.      Estate means each estate created in the Chapter 11 Cases pursuant to section 541 of the Bankruptcy Code.

1.73.      Estimation Order means an order or orders of the Bankruptcy Court estimating for voting and/or distribution purposes (under section 502(c) of the Bankruptcy Code) the allowed amount of any Claim. The defined term Estimation Order includes the Confirmation Order if the Confirmation Order grants the same relief that would have been granted in a separate Estimation Order.

1.74.      Existing Interests means all existing Interests (other than Intercompany Interests) in the Debtors that are outstanding immediately prior to the Effective Date.

1.75.      Existing Plan Investor Debt means the principal amount of indebtedness owing by the Plan Investor plus all accrued and unpaid fees and accrued interest, in the aggregate amount as of the Effective Date.

1.76.      Existing Securities Law Claim means any Claim, whether or not the subject of an existing lawsuit: (a) arising from rescission of a purchase or sale of any debt or equity securities of any Debtor or an affiliate of any Debtor; (b) for damages arising from the purchase or sale of any such security; (c) for violations of the securities laws, misrepresentations, or any similar Claims, including, to the extent related to the foregoing or otherwise subject to subordination under section 510(b) of the Bankruptcy Code, any attorneys’ fees, other charges, or costs incurred on account of the foregoing Claims; or (d) reimbursement, contribution, or indemnification on account of any such Claim.

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1.77.      Federal Judgment Rate means the interest rate applicable to a judgment entered on the Petition Date that is subject to 28 U.S.C. § 1961, as determined in accordance with that statute.

1.78.      Fee Claim means a Claim by a Professional Person for compensation, indemnification or reimbursement of expenses pursuant to sections 327, 328, 330, 331, 503(b) or 1103(a) of the Bankruptcy Code in connection with the Chapter 11 Cases, including in connection with final fee applications of such Professional Persons.

1.79.      Final Order means an order, ruling or judgment of the Bankruptcy Court (or other court of competent jurisdiction) entered by the Clerk of the Bankruptcy Court on the docket in the Chapter 11 Cases (or by the clerk of such other court of competent jurisdiction on the docket of such court), which has not been reversed, vacated, or stayed and as to which (a) the time to appeal, petition for certiorari, or move for a new trial, reargument, or rehearing has expired and as to which no appeal, petition for certiorari, or other proceeding for a new trial, reargument, or rehearing shall then be pending, or (b) if an appeal, writ of certiorari, new trial, reargument, or rehearing thereof has been sought, such order or judgment of the Bankruptcy Court shall have been affirmed by the highest court to which such order was appealed, or certiorari shall have been denied, or a new trial, reargument, or rehearing shall have been denied or resulted in no modification of such order, and the time to take any further appeal, petition for certiorari, or move for a new trial, reargument, or rehearing shall have expired; provided, that no order or judgment shall fail to be a Final Order solely because of the possibility that a motion under Rule 60 of the Federal Rules of Civil Procedure has been or may be filed with respect to such order or judgment; provided, further, that no order or judgment shall fail to be a Final Order solely because of the susceptibility of a Claim to a challenge under section 502(j) of the Bankruptcy Code.

1.80.      General Unsecured Claim means any Claim against a Debtor other than: (a) Bridge Loan Claim; (b) a Novelion Intercompany Loan Claim; (c) an Other Secured Claim; (d) a DIP Claim; (e) an Administrative Expense Claim; (f) a Fee Claim or an Ad Hoc Group Fee Claim; (g) a Priority Tax Claim; (h) a Priority Non-Tax Claim; (i) an Intercompany Claim; (j) an Existing Securities Law Claim; (k) U.S. Trustee Fees; (l) a Government Settlement Claim; and (m) an Other Novelion Claim.

1.81.      Government Settlement Agreements means the settlement agreements and judgments set forth on Schedule 1.81 hereto.

1.82.      Government Settlement Claims means all Claims against any Debtor held by a governmental unit (as defined in section 101(27) of the Bankruptcy Code) and relators arising from or relating to criminal and civil fines or other amounts required to be paid pursuant to the Government Settlement Agreements.

1.83.      Highbridge means, collectively, Highbridge MSF International Ltd., 1992 Tactical Credit Master Fund, L.P., Highbridge SCF Special Situations SPV, L.P., and Highbridge SCF Loan SPV, L.P.

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1.84.      Implementation Memorandum means the memorandum describing the sequencing of the actions, transfers and other corporate transactions making up, or otherwise to be effectuated pursuant to, the Plan and the Transaction Documents. A substantially final form of the Implementation Memorandum, in form and substance Acceptable to the Debtors, the Required Parties, and the Committee, will be contained in the Plan Supplement.

1.85.      Intercompany Claim means any Claim, Cause of Action, or remedy held by or asserted against a Debtor by (a) another Debtor, or (b) a non-Debtor subsidiary of a Debtor. For the avoidance of doubt, “Intercompany Claim” shall not include any Novelion Intercompany Loan Claim.

1.86.      Intercompany Interest means any Interest held by a Debtor in another Debtor.

1.87.      Interest means the interest (whether legal, equitable, contractual or otherwise) of any holders of any class of equity securities of any of the Debtors, represented by shares of common or preferred stock or other instruments evidencing an ownership interest in any of the Debtors, whether or not certificated, transferable, voting or denominated “stock” or a similar security, or any option, warrant or right, contractual or otherwise, to acquire any such interest.

1.88.      Lien has the meaning set forth in section 101(37) of the Bankruptcy Code.

1.89.      New Common Stock means, collectively, the shares of authorized common stock of the Plan Investor (or, at the option of the Plan Investor, American Depositary Shares representing common stock), the number of which shall be determined in accordance with the Plan Funding Agreement, to be issued by the Plan Investor (or a new holding company established to hold 100% of the equity of the Plan Investor and which will assume the Plan Investor’s obligations under, and in accordance with the terms of, the Plan Funding Agreement) on the Effective Date in connection with the implementation of this Plan and the Plan Funding Agreement or upon exercise of the New Warrants.

1.90.      New Common Stock Distribution means, collectively, the Class 4 New Common Stock Distribution and the Class 6B New Common Stock Distribution, which shall equal 61.4% of the New Common Stock.

1.91.      New Convertible Noteholder means any holder, in its capacity as such, of the New Convertible Notes pursuant to the New Convertible Notes Indenture.

1.92.      New Convertible Notes means the new 5.00% convertible senior unsecured notes issued by Reorganized Aegerion and guaranteed by the Plan Investor pursuant to the New Convertible Notes Indenture in the aggregate principal amount of $125,000,000.

1.93.      New Convertible Notes Indenture means that certain indenture, dated as of the Effective Date, by and between Reorganized Aegerion, as issuer, the Plan Investor, as guarantor, and certain other entities identified therein as “guarantors” and the New Convertible Notes Trustee, as trustee, including all agreements, documents, notes, instruments and any other agreements delivered pursuant thereto or in connection therewith (in each case, as amended, modified or supplemented from time to time), having the material terms set forth on Schedule 1.93 hereto, and a substantially final form of which will be contained in the Plan Supplement.

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1.94.      New Convertible Notes Trustee means the financial institution to be identified in the Plan Supplement, or its successors and assigns, in its capacity as trustee for the New Convertible Noteholders under the New Convertible Notes Indenture.

1.95.      New Equity Interests means the new common stock of each of the Reorganized Debtors.

1.96.      New Money Bridge Loan means the first lien term loans in the aggregate principal amount of $50,000,000 made pursuant to the Bridge Loan Credit Agreement.

1.97.      New Money Bridge Loan Claim means any Claim related to, arising under, or in connection with, the New Money Bridge Loan, which shall be Allowed on the Effective Date in the aggregate principal amount of $50,000,000 plus accrued and unpaid fees and interest through the Effective Date.

1.98.      New Registration Rights Agreement means the shareholders’ agreement, to be dated as of the Effective Date, among the Plan Investor, Athyrium and Highbridge, which shall be subject to the consent of the Plan Investor and in form and substance Acceptable to the Debtors, Athyrium, and Highbridge, and a substantially final form of which will be contained in the Plan Supplement.

1.99.      New Term Loan Agent means the financial agent to be identified in the Plan Supplement, solely in its capacity as the administrative agent and collateral agent under the New Term Loan Agreement, and any of its successors or assigns.

1.100.    New Term Loan Agreement means that certain first lien term loan agreement governing the New Term Loan Facility, by and among Reorganized Aegerion, as borrower, the

Plan Investor and certain other entities identified as “guarantors” in the New Term Loan Agreement, and the New Term Loan Agent, as administrative agent and collateral agent, including all agreements, documents, notes, instruments and any other agreements delivered pursuant thereto or in connection therewith (in each case, as amended, modified or supplemented from time to time), having the material terms set forth on Schedule 1.100 hereto, and a substantially final form of which will be contained in the Plan Supplement.

1.101.    New Term Loan Facility means the new first lien term loan facility, the terms of which shall be set forth in the New Term Loan Agreement, which shall be in the original principal amount equal to (a) the New Money Bridge Loan Claim plus (b) the Existing Plan Investor Debt.

1.102.    New Term Loan Facility Lenders means the lenders party to the New Term Loan Agreement.

1.103.    New Term Loan Facility Obligations means the obligations of Reorganized Aegerion and the other obligors party thereto under the New Term Loan Agreement.

1.104. New Warrants means a perpetual warrant issued by the Plan Investor, with a nominal exercise price, to purchase a number of shares of New Common Stock equal to the number of shares that a Person entitled to receive New Common Stock hereunder would

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otherwise have received had it not elected to receive New Warrants in lieu thereof, the terms of which will provide that it will not be exercisable for a period of sixty (60) days following notice of exercise (subject to customary exceptions) and unless such exercise otherwise complies with applicable law, the form of which warrant shall provide for customary anti-dilution protection in respect of stock splits, stock dividends, reverse stock splits and similar transactions and is reasonably acceptable to the Debtors, the Required Lenders, the Plan Investor, and the Committee.

1.105.    Novelion means Novelion Therapeutics Inc.

1.106.    Novelion Intercompany Loan means the term loan in the original principal amount of $40,000,000 made pursuant to the Novelion Intercompany Loan Credit Agreement.

1.107.    Novelion Intercompany Loan Claim means all Claims related to, arising under, or in connection with, the Novelion Intercompany Loan Credit Agreement, which shall be Allowed on the Effective Date in the aggregate amount of $36,340,173 less an amount equal to (a) the Prepetition Shared Services Adjustment plus (b) the Prepetition Transaction Proceeds Adjustment.

1.108.    Novelion Intercompany Loan Credit Agreement means that certain Amended and Restated Loan and Security Agreement, dated as of March 15, 2018 (as amended, modified or supplemented from time to time), among Aegerion, as borrower, and Novelion, as lender, including all agreements, documents, notes, instruments and any other agreements delivered pursuant thereto or in connection therewith (in each case, as amended, modified or supplemented from time to time).

1.109.    Ongoing Trade Claim means any General Unsecured Claim that is an obligation of the Debtors to third-party providers of goods and services to the Debtors that facilitate the Debtors’ operations in the ordinary course of business and will continue to do so after the Debtors’ emergence from the Chapter 11 Cases.

1.110.    Other General Unsecured Claim means any General Unsecured Claim against a Debtor other than an Ongoing Trade Claim, including (a) Claims held by a former officer, director or employee of the Debtors or the Debtors’ non-Debtor subsidiaries for indemnification, contribution, or advancement of expenses pursuant to any Debtor’s certificate of incorporation, by-laws, operating agreement, or similar organizational document, or any indemnification or contribution agreement, (b) the Convertible Notes Claims, and (c) any Claim based on damages arising from the rejection of an executory contract or unexpired lease.

1.111.    Other Novelion Claim means any Claim held by Novelion or a wholly-owned direct or indirect subsidiary thereof (excluding Aegerion and its Subsidiaries) against the Debtors and their non-Debtor affiliates other than (i) the Novelion Intercompany Loan Claim, (ii) Novelion’s Interests in Aegerion, and (iii) any Claim for payments under Section 1 of the Shared Services Amendment.

1.112.    Other Secured Claim means any Secured Claim against a Debtor other than a Bridge Loan Claim or a Novelion Intercompany Loan Claim.

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1.113.    Person means any individual, corporation, partnership, association, indenture trustee, limited liability company, cooperative, organization, joint stock company, joint venture, estate, fund, trust, unincorporated organization, governmental unit or any political subdivision thereof, or any other entity or organization of whatever nature.

1.114.    Petition Date means May 20, 2019, the date on which the Debtors commenced the Chapter 11 Cases.

1.115.    PFA Order shall have the meaning given it in the Plan Funding Agreement.

1.116.    Plan means this joint chapter 11 plan proposed by the Debtors, including the exhibits, supplements, appendices and schedules hereto, either in its present form or as the same may be altered, amended or modified from time to time in accordance with the provisions of the Bankruptcy Code and the terms hereof.

1.117.    Plan Cash means (a) the Debtors’ Cash on hand as of the Effective Date, (b) Cash generated from operations prior to the Effective Date, and (c) borrowings under the DIP Financing Agreement.

1.118.    Plan Consideration means, with respect to any Class of Claims entitled to distributions under this Plan, Cash, New Common Stock, New Warrants, New Convertible Notes, and New Term Loan Facility Obligations, as the context requires.

1.119.    Plan Distributions means the Plan Consideration distributed under this Plan.

1.120.    Plan Documents means the documents, other than this Plan, to be executed, delivered, assumed, and/or performed in connection with the consummation of this Plan, including the documents to be included in the Plan Supplement and any and all exhibits to this Plan and the Disclosure Statement, including the Plan Funding Agreement, the RSA, the Backstop Commitment Agreement, the Rights Offering Procedures, and any and all exhibits to the Plan and the Disclosure Statement, each of which shall be in form and substance Acceptable to the Debtors, each of the Required Parties and the Committee.

1.121.    Plan Funding Agreement means that certain Plan Funding Agreement, dated as of May 20, 2019, among Aegerion and the Plan Investor (as may be amended, modified and/or supplemented from time to time in accordance with its terms), pursuant to which the Plan Investor will acquire 100% of the New Equity Interests in Reorganized Aegerion in exchange for New Common Stock of the Plan Investor (including New Common Stock issuable upon the exercise of New Warrants) in the amounts set forth in this Plan and the Plan Funding Agreement.

1.122.    Plan Investor means (a) Amryt Pharma plc, on behalf of itself and/or one or more of its affiliates, and (b) in the case of the issuance of the New Common Stock and for purposes of Article XII of the Plan and for purposes of the New Registration Rights Agreement, Amryt Pharma plc or a new holding company established to hold 100% of the equity of the Plan Investor and will assume the Plan Investor’s obligations under and in accordance with the Plan Funding Agreement.

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1.123.    Plan Investor Equity Raise means the additional equity raise conducted by the Plan Investor, for shares of New Common Stock (including New Common Stock to be issuable upon exercise of the New Warrants) to be issued by the Plan Investor for an aggregate purchase price equal to the Plan Investor Equity Raise Amount to certain existing shareholders of the Plan Investor, for the benefit of the Plan Investor and the Reorganized Debtors and backstopped by the Backstop Parties.

1.124.    Plan Investor Equity Raise Amount means $18,000,000 plus any portion of the Rights Offering Amount that is not timely, duly and validly subscribed and paid for by the Eligible Holders that timely vote to accept the Plan in accordance with the Rights Offering Procedures.

1.125.    Plan Securities means, collectively, the New Convertible Notes, the New Common Stock, the New Warrants, the Subscription Rights, and the Rights Offering Stock.

1.126.    Plan Supplement means the supplemental appendix to this Plan (as may be amended, modified and/or supplemented from time to time), to be filed no later than five (5) calendar days prior to the deadline for filing objections to this Plan or such other earlier or later date(s) as expressly set forth in this Plan, which may contain, among other things, draft forms, signed copies, or summaries of material terms, as the case may be, of (a) the Amended Certificates of Formation, (b) the Amended Memorandum of Association, (c) the list of proposed officers and directors of each of the Plan Investor and the Reorganized Debtors, pursuant to the rights set forth in the New Registration Rights Agreement, (d) the New Term Loan Agreement, (e) the New Convertible Notes Indenture, (f) the Schedule of Rejected Contracts and Leases, (g) the New Registration Rights Agreement, (h) the Implementation Memorandum, (i) an agreement evidencing, or the form of, New Warrants, and (j) any additional documents filed with the Bankruptcy Court before the Effective Date as amendments to the Plan Supplement; provided, that unless consent rights are otherwise expressly set forth in this Plan, each of the documents in the Plan Supplement (whether or not set forth above), including any alternation, restatement, modification or replacement thereto, shall be in form and substance Acceptable to the Debtors, each of the Required Parties and the Committee.

1.127.    Prepetition Shared Services Adjustment means an amount equal to the additional funding needs of Novelion, if any, pursuant to the Shared Services Agreements, in the sole discretion of Aegerion and the Bridge Loan Lenders (and solely to the extent permitted by the DIP Financing Documents), to the extent Aegerion’s share of costs related to post-April 1, 2019 employee costs, audit costs and data room expenses exceed $1,970,000 in the aggregate, which additional funding shall be deemed to reduce the Novelion Intercompany Loan Claim by $1.50 for every $1.00 paid by Aegerion above the $1,970,000 cap.

1.128.    Prepetition Transaction Proceeds Adjustment means an amount equal to the aggregate amount withdrawn from the Novelion Segregated Licensing Account (as defined in the DIP Order) in accordance with the terms of the DIP Order, which aggregate amount withdrawn shall be deemed to reduce the Novelion Intercompany Loan Claim by $1.75 for every $1.00 withdrawn.

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1.129.    Priority Non-Tax Claim means any Claim, other than a DIP Claim, an Administrative Expense Claim, a Fee Claim, an Ad Hoc Group Fee Claim or a Priority Tax Claim, entitled to priority in payment as specified in section 507(a) of the Bankruptcy Code.

1.130.    Priority Tax Claim means any Claim of a governmental unit (as defined in section 101(27) of the Bankruptcy Code) of the kind entitled to priority in payment under sections 502(i) and 507(a)(8) of the Bankruptcy Code.

1.131.    Pro Rata Share means (a) with respect to any distribution on account of an Allowed Claim, a distribution equal in amount to the ratio (expressed as a percentage) that the amount of such Allowed Claim bears to the aggregate amount of all Allowed Claims entitled to share in the relevant Plan Distribution, and (b) with respect to an Eligible Holder’s participation in the Rights Offering, a distribution equal in amount to the ratio (expressed as a percentage) that the amount of such Eligible Holder’s Subscription Rights bears to the aggregate amount of all Rights Offering Stock distributed to Eligible Holders as determined pursuant to the Rights Offering.

1.132.    Professional Person(s) means all Persons retained by order of the Bankruptcy Court in connection with the Chapter 11 Cases, pursuant to sections 327, 328, 330, 363, or 1103 of the Bankruptcy Code, excluding any ordinary course professionals retained pursuant to an order of the Bankruptcy Court.

1.133.    Rebate Obligation means any cash expenditures in France made in connection with a “cohort ATU” that is authorized by the French National Agency for Medicines and Health Products Safety or any similar rebates in Spain or the United States.

1.134.    Released Parties means, collectively, and each solely in its capacity as such: (a) the Debtors, their respective non-Debtor subsidiaries, and the Reorganized Debtors; (b) Novelion; (c) the DIP Administrative Agent and the DIP Lenders; (d) the Bridge Loan Administrative Agent; (e) the Convertible Notes Trustee; (f) the Bridge Loan Lenders; (g) the Consenting Lenders; (h) the members of the Ad Hoc Group; (i) the Plan Investor; (j) the Committee and each of its current and former members solely in their capacity as members of the Committee; (k) each of such parties’ respective predecessors, successors, assigns, subsidiaries, owners, affiliates, managed accounts, funds or funds under common management; and (l) each of the foregoing parties’ (described in clauses (a)-(k)) respective current and former officers, directors, managers, managing members, employees, members, principals, shareholders, agents, advisory board members, management companies, fund advisors, partners, attorneys, financial advisors or other professionals or representatives, together with their successors and assigns, in each case solely in their capacity as such; provided, however, that (i) former directors, officers and employees of the Debtors who were not directors, officers or employees of any of the Debtors or any of the Debtors’ non-Debtor subsidiaries at any time after the Petition Date shall not be deemed Released Parties regardless of whether they would otherwise meet the definition of “Released Parties”, (ii) such attorneys and professional advisors described in subsection (l) shall only include those that provided services related to the Chapter 11 Cases and the transactions contemplated by this Plan (and do not include the attorneys and law firms retained by the Debtors in the ordinary course of business during these Chapter 11 Cases), (iii) no Person shall be a Released Party if it objects to the releases provided for in Article XII of this

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Plan, and (iv) the shareholders of Novelion, in their capacities as shareholders, shall not be deemed “Released Parties” as to Novelion.

1.135.    Releasing Parties means, collectively, and each solely in its capacity as such: (a) the Debtors, their respective non-Debtor subsidiaries, and the Reorganized Debtors; (b) Novelion; (c) the DIP Administrative Agent and the DIP Lenders; (d) the Bridge Loan Administrative Agent; (e) the Convertible Notes Trustee; (f) the Bridge Loan Lenders; (g) the Consenting Lenders; (h) the members of the Ad Hoc Group; (i) the Plan Investor; (j) the Committee and each of its members solely in their capacity as members of the Committee; (k) each of such parties’ respective predecessors, successors, assigns, subsidiaries, owners, affiliates, managed accounts, funds or funds under common management; (l) each of the foregoing parties’ (described in clauses (a)-(k)) respective current and former officers, directors, managers, managing members, employees, members, principals, shareholders, agents, advisory board members, management companies, fund advisors, partners, attorneys, financial advisors or other professionals or representatives, together with their successors and assigns, in each case solely in their capacity as such; (m) holders of Claims who vote to accept the Plan; (n) holders of Claims who vote to reject the Plan but who vote to “opt in” to the Third Party Release; and (o) all holders of Claims and Interests not described in clauses (a)-(n) who elect to opt-in to the Third Party Release; provided, however, that (i) notwithstanding anything to the contrary herein, the scope of the “Releasing Parties” shall be subject to the limitations set forth in Section 12.6(b) herein, (ii) former directors, officers and employees of the Debtors who were not directors, officers or employees of any of the Debtors or any of the Debtors’ non-Debtor subsidiaries at any time after the Petition Date shall not be deemed Releasing Parties regardless of whether they would otherwise meet the definition of “Releasing Parties”, and (iii)(a) shareholders of Novelion, solely in their capacities as shareholders, shall not be deemed “Releasing Parties”, and (b) Novelion shall not be deemed a “Releasing Party” as to Novelion’s shareholders (solely in their capacities as such).

1.136.    Reorganized Aegerion means Aegerion on and after the Effective Date.

1.137.    Reorganized Debtor(s) means, as the context requires, the applicable Debtor(s) on and after the Effective Date, after giving effect to the restructuring transactions occurring on the Effective Date in accordance with this Plan.

1.138.    Required Consenting Lenders means the Required Consenting Lenders as defined in the RSA.

1.139.    Required Parties means the Required Parties as defined in the RSA.

1.140.    Rights Offering means the offering of Subscription Rights to Eligible Holders to purchase shares of New Common Stock (including New Common Stock to be issuable upon the exercise of New Warrants) to be issued by the Plan Investor on the Effective Date pursuant to the Plan, for an aggregate purchase price of the Rights Offering Amount, to be conducted in reliance upon the exemption from registration under the Securities Act provided in section 1145 of the Bankruptcy Code.

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1.141.    Rights Offering Amount means $42,000,000 minus any portion of the Rights Offering Amount that is not timely, duly and validly subscribed and paid for by the Eligible Holders that timely vote to accept the Plan in accordance with the Rights Offering Procedures.

1.142.    Rights Offering Exercise Price means the purchase price for each share of Rights Offering Stock, as set forth in the Rights Offering Procedures and approved by the Bankruptcy Court. The Rights Offering Exercise Price for the Rights Offering Stock will be set at a per share price that is based upon the Rights Offering Stock equaling 13.61% of the New Common Stock of the Plan Investor (after giving effect to the Rights Offering and the Plan Investor Equity Raise, but prior to the any management incentive plan, conversion of the New Convertible Notes, or any contingent value rights issued to existing shareholders of the Plan Investor).

1.143.    Rights Offering Procedures means the procedures governing the Rights Offering, which procedures are attached as an exhibit to the Disclosure Statement, and shall be Acceptable to the Debtors and each of the Required Parties.

1.144.    Rights Offering Stock means shares of New Common Stock (including New Common Stock issuable upon the exercise of New Warrants) issued by the Plan Investor on the Effective Date pursuant to the Rights Offering.

1.145.    Roll Up Loan Claim means any Claim related to, arising under, or in connection with the Roll Up Loans, which shall be Allowed on the Effective Date in the aggregate principal amount of $22,500,000, plus accrued and unpaid fees and interest through the Effective Date.

1.146.    Roll Up Loans means first lien term loans in the aggregate principal amount of $22,500,000 that were funded by the Bridge Loan Lenders pursuant to the Bridge Loan Credit Agreement to repurchase and retire, at par, an equal amount of Convertible Notes held by the Bridge Loan Lenders.

1.147.    RSA means that certain Restructuring Support Agreement, dated as of May 20, 2019, inclusive of all exhibits thereto, by and among the Debtors, the Plan Investor and the Consenting Lenders.

1.148.    Schedule of Rejected Contracts and Leases means a schedule of the contracts and leases to be rejected pursuant to section 365 of the Bankruptcy Code and Section 10.1 hereof, which shall be contained in the Plan Supplement.

1.149.    Schedules means the schedules of assets and liabilities filed in the Chapter 11 Cases, as amended or supplemented from time to time.

1.150.    SEC has the meaning set forth in Section 12.6(d) of this Plan.

1.151.    Secured Claim means a Claim: (a) that is secured by a valid, perfected and enforceable Lien on Collateral, to the extent of the value of the Claim holder’s interest in such Collateral as of the Confirmation Date; or (b) to the extent that the holder thereof has a valid right of setoff pursuant to section 553 of the Bankruptcy Code.

1.152.    Securities Act means the Securities Act of 1933, as amended.

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1.153.    Shared Services Agreements means, collectively, that certain Master Service Agreement, dated as of December 1, 2016, between Novelion and Aegerion (as amended, modified or supplemented from time to time), and that certain Master Service Agreement, dated as of December 1, 2016, between Novelion Services USA, Inc. and Aegerion (as amended, modified or supplemented from time to time), in each case as amended pursuant to the Shared Services Amendment, which provided for an upfront payment of $3,123,000, plus up to $1,970,000 in payments for additional services through the Effective Date plus, if applicable, the Prepetition Shared Services Adjustment.

1.154.    Shared Services Amendment means that certain Amendment to Shared Services Agreements, dated as of May 20, 2019, by and between Aegerion, Novelion, and Novelion Services USA, Inc.

1.155.    State Government Settlement Agreements shall have the meaning given it in Section 5.5 hereof.

1.156.    Subscription Commencement Date means the date on which the Rights Offering commences, as specified in the Rights Offering Procedures.

1.157.    Subscription Rights means the non-transferable, non-certificated subscription rights of Eligible Holders to purchase shares of Rights Offering Stock on the terms and subject to the conditions set forth in the Plan, the Rights Offering Procedures, and the Backstop Commitment Agreement.

1.158.    Subsidiary means any corporation, association or other business entity of which at least the majority of the securities or other ownership interest is owned or controlled by a Debtor and/or one or more subsidiaries of the Debtor.

1.159.    Third Party Releases means the releases set forth in Section 12.6(b) of this Plan.

1.160.    Transaction Documents means this Plan, the Plan Funding Agreement, the RSA, and each other contract, exhibit, schedule, certificate and other document being delivered pursuant to, or in furtherance of the transactions contemplated by this Plan, the Plan Funding Agreement or the RSA.

1.161.    Unimpaired means, with respect to a Claim, Equity Interest, or Class of Claims or Equity Interests, not “impaired” within the meaning of sections 1123(a)(4) and 1124 of the Bankruptcy Code.

1.162.    Unsubscribed Shares means shares of New Common Stock that are not timely, duly and validly subscribed and paid for in connection with the Plan Investor Equity Raise, including any Rights Offering Stock that are not timely, duly and validly subscribed and paid for by the Eligible Holders that timely vote to accept the Plan in accordance with the Rights Offering Procedures.

1.163.    U.S. Trustee means the United States Trustee for Region 2.

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1.164.    U.S. Trustee Fees means fees arising under 28 U.S.C. § 1930(a)(6) and, to the extent applicable, accrued interest thereon arising under 31 U.S.C. § 3717.

B. Interpretation; Application of Definitions and Rules of Construction.

Unless otherwise specified, all section or exhibit references in this Plan are to the respective section in, or exhibit to, this Plan. The words “herein,” “hereof,” “hereto,” “hereunder,” and other words of similar import refer to this Plan as a whole and not to any particular section, subsection, or clause contained therein. Whenever from the context it is appropriate, each term, whether stated in the singular or the plural, will include both the singular and the plural. Any term that is not otherwise defined herein, but that is used in the Bankruptcy Code or the Bankruptcy Rules, shall have the meaning given to that term in the Bankruptcy Code or the Bankruptcy Rules, as applicable. The rules of construction contained in section 102 of the Bankruptcy Code, other than section 102(5), shall apply to the construction of this Plan. Any reference in this Plan to an existing document or exhibit filed or to be filed means such document or exhibit as it may have been or may be amended, modified, or supplemented. Subject to the provisions of any contracts, certificates or articles of incorporation, instruments, releases, or other agreements or documents entered into in connection with this Plan, the rights and obligations arising under this Plan shall be governed by, and construed and enforced in accordance with, federal law, including the Bankruptcy Code and Bankruptcy Rules. The captions and headings in this Plan are for convenience of reference only and shall not limit or otherwise affect the provisions hereof. Any reference to an entity as a holder of a Claim or Interest includes that entity’s successors and assigns. Any reference to directors or board of directors includes managers, managing members or any similar governing body, as the context requires.

C. Appendices and Plan Documents.

All Plan Documents and appendices to this Plan are incorporated into this Plan by reference and are a part of this Plan as if set forth in full herein. The documents contained in the exhibits and Plan Supplement shall be approved by the Bankruptcy Court pursuant to the Confirmation Order. Holders of Claims and Interests may inspect a copy of the Plan Documents, once filed, in the Office of the Clerk of the Bankruptcy Court during normal business hours, or via the Claims Agent’s website at http://cases.primeclerk.com/aegerion, or obtain a copy of any of the Plan Documents by a written request sent to the Claims Agent at the following address:

Aegerion Ballot Processing

c/o Prime Clerk LLC

One Grand Central Place

60 East 42nd Street, Suite 1440

New York, NY 10165

Phone: 844-627-5368 (U.S. toll free)

or 347-292-3524 (international)

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ARTICLE II.

CERTAIN INTER-CREDITOR AND INTER-DEBTOR ISSUES

2.1.        Settlement of Certain Inter-Creditor Issues.

The treatment of Claims and Interests under this Plan represents, among other things, the settlement and compromise of certain potential inter-creditor disputes.

2.2.        Formation of Debtor Group for Convenience Purposes.

The Plan groups the Debtors together solely for purposes of describing treatment under the Plan, confirmation of the Plan and making Plan Distributions in respect of Claims against and Interests in the Debtors under the Plan. Such grouping shall not affect any Debtor’s status as a separate legal entity, change the organizational structure of the Debtors’ business enterprise, constitute a change of control of any Debtor for any purpose, cause a merger or consolidation of any legal entities, nor cause the transfer of any assets or the assumption of any liabilities; and, except as otherwise provided by or permitted in the Plan, all Debtors shall continue to exist as separate legal entities.

2.3.        Intercompany Claims and Intercompany Interests.

(a) Intercompany Claims.

Notwithstanding anything to the contrary herein, on or after the Effective Date, any and all Intercompany Claims shall, at the option of the Debtors or the Reorganized Debtors, as applicable, and as Acceptable to the Required Parties, and the Committee, either be (i) extinguished, canceled and/or discharged on the Effective Date, or (ii) reinstated and otherwise survive the Debtors’ restructuring by virtue of such Intercompany Claims being left unimpaired. To the extent any such Intercompany Claim is reinstated, or otherwise adjusted (including by contribution, distribution in exchange for new debt or equity, or otherwise), paid or continued as of the Effective Date, any such transaction may be effected on or after the Effective Date without any further action by the Bankruptcy Court, act or action under applicable law, regulation, order or rule or the vote, consent, authorization or approval of any Person.

(b) Intercompany Interests.

No Intercompany Interests shall be cancelled pursuant to this Plan, and all Intercompany Interests shall be unaffected by the Plan and continue in place following the Effective Date, solely for the administrative convenience of maintaining the existing corporate structure of the Debtors and the Reorganized Debtors.

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ARTICLE III.

DIP CLAIMS, ADMINISTRATIVE EXPENSE CLAIMS, 

FEE CLAIMS, U.S. TRUSTEE FEES AND PRIORITY TAX CLAIMS

The Plan constitutes a joint plan of reorganization for all of the Debtors. All Claims and Interests, except DIP Claims, Administrative Expense Claims, Fee Claims, Ad Hoc Group Fee Claim, U.S. Trustee Fees and Priority Tax Claims, are placed in the Classes set forth in Article IV below. In accordance with section 1123(a)(1) of the Bankruptcy Code, DIP Claims, Administrative Expense Claims, Fee Claims, U.S. Trustee Fees and Priority Tax Claims have not been classified, and the holders thereof are not entitled to vote on this Plan. A Claim or Interest is placed in a particular Class only to the extent that such Claim or Interest falls within the description of that Class and is classified in other Classes to the extent that any portion of the Claim or Interest falls within the description of such other Classes.

A Claim or Interest is placed in a particular Class for all purposes, including voting, confirmation and distribution under this Plan and under sections 1122 and 1123(a)(1) of the Bankruptcy Code. However, a Claim or Interest is placed in a particular Class for the purpose of receiving Plan Distributions only to the extent that such Claim or Interest is an Allowed Claim or Allowed Interest and has not been paid, released or otherwise settled prior to the Effective Date.

3.1.        DIP Claims.

On the Effective Date, the DIP Claims shall be Allowed and shall not be subject to any avoidance, reductions, setoff, offset, recoupment, recharacterization, subordination (whether equitable, contractual, or otherwise), counterclaims, cross-claims, defenses, disallowance, impairment, objection, or any other challenges under any applicable law or regulation by any Person. In full satisfaction, settlement, release and discharge of the Allowed DIP Claims, on the Effective Date, Allowed DIP Claims shall (a) be paid in Cash to the greatest extent possible from available Cash of the Debtors (as reasonably agreed by the Debtors and the DIP Lenders), and (b) to the extent the Allowed DIP Claims are not paid in full in Cash on the Effective Date, receive New Convertible Notes in an amount equal to the amount of the Allowed DIP Claims not receiving Cash pursuant to the foregoing clause (a). Payment of any unpaid fees and expenses of the DIP Administrative Agent shall be paid to the DIP Administrative Agent in cash on the Effective Date. Distributions on account of Allowed DIP Claims other than Cash will not be distributed to the DIP Administrative Agent but instead shall be distributed directly to the DIP Lenders as reflected on the registry maintained by the DIP Administrative Agent as of the Confirmation Date. The Debtors will request such registry from the DIP Administrative Agent. Upon satisfaction of the Allowed DIP Lender Claims as set forth in this Section 3.1 of the Plan, all Liens and security interests granted to secure such obligations, whether in the Chapter 11 Cases or otherwise, shall be terminated and of no further force or effect.

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3.2.         Administrative Expense Claims.

(a) Time for Filing Administrative Expense Claims.

The holder of an Administrative Expense Claim, other than the holder of:

(i) a Fee Claim;

(ii) a DIP Claim;

(iii) a 503(b)(9) Claim;

(iv) an Ad Hoc Group Fee Claim;

(v) an Administrative Expense Claim that has been Allowed on or before the Effective Date;

(vi) an Administrative Expense Claim for an expense or liability incurred and payable in the ordinary course of business by a Debtor;

(vii) an Administrative Expense Claim on account of fees and expenses incurred on or after the Petition Date by ordinary course professionals retained by the Debtors pursuant to an order of the Bankruptcy Court;

(viii) an Administrative Expense Claim held by an officer, director or employee of the Debtors serving in such capacity immediately prior to the occurrence of the Effective Date solely in their capacity as such (whether or not also an officer, director or employee of Novelion), for indemnification, contribution, or advancement of expenses pursuant to (A) any Debtor’s certificate of incorporation, by-laws, operating agreement, or similar organizational document, (B) any employment, director or similar agreement, or (C) any indemnification or contribution agreement approved by the Bankruptcy Court;

(ix) an Administrative Expense Claim arising, in the ordinary course of business, out of the employment by one or more Debtors of an individual from and after the Petition Date, but only to the extent that such Administrative Expense Claim is solely for outstanding wages, commissions, accrued benefits, or reimbursement of business expenses;

(x) a Claim for adequate protection arising under the DIP Order;

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(xi) an Administrative Expense Claim of Novelion or Novelion Services USA, Inc. arising out of or related to the Shared Services Agreements;

(xii) an Intercompany Claim;

(xiii) an Administrative Expense Claim described in 11 U.S.C. § 503(b)(1)(B)-(C), if the holder is a Governmental Unit; or

(xiv) U.S. Trustee Fees,

must file with the Bankruptcy Court and serve on the Reorganized Debtors, the Claims Agent, and the U.S. Trustee, proof of such Administrative Expense Claim within thirty (30) days after the Effective Date (the “Administrative Bar Date”). Such proof of Administrative Expense Claim must include at a minimum: (1) the name of the applicable Debtor that is purported to be liable for the Administrative Expense Claim and if the Administrative Expense Claim is asserted against more than one Debtor, the exact amount asserted to be owed by each such Debtor; (2) the name of the holder of the Administrative Expense Claim; (3) the asserted amount of the Administrative Expense Claim; (4) the basis of the Administrative Expense Claim; and (5) supporting documentation for the Administrative Expense Claim. FAILURE TO FILE AND SERVE SUCH PROOF OF ADMINISTRATIVE EXPENSE CLAIM TIMELY AND PROPERLY SHALL RESULT IN SUCH CLAIM BEING FOREVER BARRED AND DISCHARGED.

(b) Treatment of Administrative Expense Claims.

Except to the extent that a holder of an Allowed Administrative Expense Claim agrees to a different treatment, on, or as soon thereafter as is reasonably practicable, the later of the Effective Date and the first Business Day after the date that is fifteen (15) calendar days after the date an Administrative Expense Claim becomes an Allowed Claim, the holder of such Allowed Administrative Expense Claim shall receive from the applicable Reorganized Debtor Cash in an amount equal to such Allowed Claim; provided, however, that Allowed Administrative Expense Claims representing liabilities incurred in the ordinary course of business by any of the Debtors, as debtors in possession, shall be paid by the applicable Reorganized Debtor in the ordinary course of business, consistent with past practice and in accordance with the terms and subject to the conditions of any orders or agreements governing, instruments evidencing, or other documents relating to, such liabilities.

Any Claim related to fees and expenses, contribution or indemnification obligations, payable or owing by the Debtors to the Ad Hoc Group, the Plan Investor, or the Backstop Parties under the RSA, the Backstop Commitment Agreement, the Plan Funding Agreement, or the PFA Order shall constitute an Allowed Administrative Expense Claim and shall be paid in Cash on the Effective Date or as soon thereafter as is reasonably practicable without the need to file a proof of such Claim with the Bankruptcy Court in accordance with Section 3.2(a) hereof and without further order of the Bankruptcy Court.

Any Claim then payable or owing by the Debtors to Novelion or Novelion Services, USA, Inc. arising out of or related to the Shared Services Agreements shall be paid in

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Cash on the Effective Date from Plan Cash, without the need to file a proof of such Claim with the Bankruptcy Court in accordance with Section 3.2(a) hereof and without further order of the Bankruptcy Court.

3.3.        Fee Claims.

(a) Time for Filing Fee Claims.

Any Professional Person seeking allowance of a Fee Claim shall file with the Bankruptcy Court its final application for allowance of compensation for services rendered and reimbursement of expenses incurred prior to the Effective Date and in connection with the preparation and prosecution of such final application no later than forty-five (45) calendar days after the Effective Date or such other date as established by the Bankruptcy Court. Objections to such Fee Claims, if any, must be filed and served no later than sixty-five (65) calendar days after the Effective Date or such other date as established by the Bankruptcy Court.

(b) Treatment of Fee Claims.

All Professional Persons seeking allowance by the Bankruptcy Court of a Fee Claim shall be paid in full in Cash in such amounts as are approved by the Bankruptcy Court: (i) upon the later of (x) the Effective Date, and (y) three (3) calendar days after the date upon which the order relating to the allowance of any such Fee Claim is entered, or (ii) upon such other terms as may be mutually agreed upon between the holder of such Fee Claim and the Reorganized Debtors. On the Effective Date, the Reorganized Debtors shall reserve and hold in a segregated account Cash in an amount equal to all accrued but unpaid Fee Claims as of the Effective Date, which Cash shall be disbursed solely to the holders of Allowed Fee Claims with the remainder to be reserved until all Fee Claims have been either Allowed and paid in full or Disallowed by Final Order, at which time any remaining Cash in the segregated account shall become the sole and exclusive property of the Reorganized Debtors, provided that the Debtors’ and the Reorganized Debtors’ obligations to pay Allowed Fee Claims shall not be limited or deemed limited to funds held in any escrow account. To the extent that funds held in any escrow account for Allowed Fee Claims are insufficient to satisfy the Allowed amount of Fee Claims owing to the Professional Person, the Reorganized Debtors shall pay such amounts within ten (10) Business Days of entry of the order approving such Fee Claims.

3.4.         U.S. Trustee Fees.

The Debtors or Reorganized Debtors, as applicable, shall pay all outstanding U.S. Trustee Fees of a Debtor on an ongoing basis on the date such U.S. Trustee Fees become due, until such time as a final decree is entered closing the applicable Chapter 11 Case, the applicable Chapter 11 Case is converted or dismissed, or the Bankruptcy Court orders otherwise.

3.5.        Priority Tax Claims.

Except to the extent that a holder of an Allowed Priority Tax Claim agrees to different treatment, each holder of an Allowed Priority Tax Claim shall receive, in the Debtors’ or Reorganized Debtors’ discretion, either: (a) on, or as soon thereafter as is reasonably practicable, the later of the Effective Date and the first Business Day after the date that is thirty

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(30) calendar days after the date a Priority Tax Claim becomes an Allowed Claim, Cash in an amount equal to such Claim; or (b) deferred Cash payments following the Effective Date, over a period ending not later than five (5) years after the Petition Date, in an aggregate amount equal to the Allowed amount of such Priority Tax Claim (with any interest to which the holder of such Priority Tax Claim may be entitled calculated in accordance with section 511 of the Bankruptcy Code); provided, however, that all Allowed Priority Tax Claims that are not due and payable on or before the Effective Date shall be paid in the ordinary course of business as they become due.

ARTICLE IV.

CLASSIFICATION OF CLAIMS AND INTERESTS

4.1.        Classification of Claims and Interests.

The following table designates the Classes of Claims against and Interests in the Debtors, and specifies which Classes are: (a) impaired or unimpaired by this Plan; (b) entitled to vote to accept or reject this Plan in accordance with section 1126 of the Bankruptcy Code; or (c) deemed to accept or reject this Plan.

Class	Designation	Impairment	Entitled to Vote
Class 1	Priority Non-Tax Claims	No	No (Presumed to accept)
Class 2	Other Secured Claims	No	No (Presumed to accept)
Class 3	Bridge Loan Claims	Yes	Yes
Class 4	Novelion Intercompany Loan Claims	Yes	Yes
Class 5	Government Settlement Claims	No	No (Presumed to accept)
Class 6A	Ongoing Trade Claims	No	No (Presumed to accept)
Class 6B	Other General Unsecured Claims	Yes	Yes
Class 7	Existing Securities Law Claims	Yes	No (Deemed to reject)
Class 8	Existing Interests	Yes	No (Deemed to reject)

If a controversy arises regarding whether any Claim or Interest is properly classified under the Plan, the Bankruptcy Court shall, upon proper motion and notice, determine such controversy at the Confirmation Hearing. If the Bankruptcy Court finds that the classification of any Claim or Interest is improper, then such Claim or Interest shall be reclassified and any Ballot previously cast by the holder of such Claim or Interest shall be counted in, and the Claim or Interest shall receive the treatment prescribed in, the Class in which the Bankruptcy Court determines such Claim or Interest should have been classified, without the necessity of resoliciting any votes on the Plan.

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4.2.         Unimpaired Classes of Claims.

The following Classes of Claims are unimpaired and, therefore, presumed to have accepted this Plan and are not entitled to vote on this Plan under section 1126(f) of the Bankruptcy Code:

(a)      Class 1: Class 1 consists of all Priority Non-Tax Claims.

(b)      Class 2: Class 2 consists of all Other Secured Claims.

(c)      Class 5: Class 5 consists of all Government Settlement Claims.

(d)      Class 6A: Class 6A consists of all Ongoing Trade Claims.

4.3.          Impaired Classes of Claims.

        (a)       The following Classes of Claims are impaired and entitled to vote on this Plan:

    (i)        Class 3: Class 3 consists of all Bridge Loan Claims.

    (ii)       Class 4: Class 4 consists of all Novelion Intercompany Loan Claims.

   (iii)       Class 6B: Class 6B consists of all Other General Unsecured Claims.

(b)       The following Classes of Claims and Interests are impaired and deemed to have rejected this Plan and, therefore, are not entitled to vote on this Plan under section 1126(g) of the Bankruptcy Code:

            (i)        Class 7: Class 7 consists of all Existing Securities Law Claims.

     (ii)      Class 8: Class 8 consists of all Existing Interests.

4.4.         Separate Classification of Other Secured Claims.

Although all Other Secured Claims have been placed in one Class for purposes of nomenclature, each Other Secured Claim, to the extent secured by a Lien on Collateral different than that securing any additional Other Secured Claims, shall be treated as being in a separate sub-Class for the purpose of receiving Plan Distributions.

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ARTICLE V.

TREATMENT OF CLAIMS AND INTERESTS

5.1.         Priority Non-Tax Claims (Class 1).

(a)       Treatment: The legal, equitable and contractual rights of the holders of Priority Non-Tax Claims are unaltered by this Plan. Except to the extent that a holder of an Allowed Priority Non-Tax Claim agrees to a different treatment, on the applicable Distribution Date, each holder of an Allowed Priority Non-Tax Claim shall receive Cash from the applicable Reorganized Debtor in an amount equal to such Allowed Claim.

(b)       Voting: The Priority Non-Tax Claims are Unimpaired Claims. In accordance with section 1126(f) of the Bankruptcy Code, the holders of Priority Non-Tax Claims are conclusively presumed to accept this Plan and are not entitled to vote to accept or reject the Plan, and the votes of such holders will not be solicited with respect to such Allowed Priority Non-Tax Claims.

5.2.         Other Secured Claims (Class 2).

      (a)       Treatment: The legal, equitable and contractual rights of the holders of Other Secured Claims are unaltered by this Plan. Except to the extent that a holder of an Allowed Other Secured Claim agrees to a different treatment, on the applicable Distribution Date each holder of an Allowed Other Secured Claim shall receive, at the election of the Reorganized Debtors: (i) Cash in an amount equal to such Allowed Claim; or (ii) such other treatment that will render such Other Secured Claim unimpaired pursuant to section 1124 of the Bankruptcy Code; provided, however, that Other Secured Claims incurred by a Debtor in the ordinary course of business may be paid in the ordinary course of business in accordance with the terms and conditions of any agreements relating thereto, in the discretion of the applicable Debtor or Reorganized Debtor without further notice to or order of the Bankruptcy Court. Each holder of an Allowed Other Secured Claim shall retain the Liens securing its Allowed Other Secured Claim as of the Effective Date until full and final satisfaction of such Allowed Other Secured Claim is made as provided herein. On the full payment or other satisfaction of each Allowed Other Secured Claim in accordance with the Plan, the Liens securing such Allowed Other Secured Claim shall be deemed released, terminated and extinguished, in each case without further notice to or order of the Bankruptcy Court, act or action under applicable law, regulation, order or rule or the vote, consent, authorization or approval of any Person.

        (b)       Deficiency Claims: To the extent that the value of the Collateral securing any Other Secured Claim is less than the Allowed amount of such Other Secured Claim, the undersecured portion of such Allowed Claim shall be treated for all purposes under this Plan as an Other General Unsecured Claim and shall be classified as a Class 6B Other General Unsecured Claim.

        (c)       Voting: The Allowed Other Secured Claims are Unimpaired Claims. In accordance with section 1126(f) of the Bankruptcy Code, the holders of Allowed Other Secured Claims are conclusively presumed to accept this Plan and are not entitled to vote to accept or

27

reject the Plan, and the votes of such holders will not be solicited with respect to such Allowed Other Secured Claims.

5.3.         Bridge Loan Claims (Class 3).

(a)       Treatment: The Bridge Loan Claims shall be Allowed under this Plan, and shall not be subject to any avoidance, reductions, setoff, offset, recoupment, recharacterization, subordination (whether equitable, contractual, or otherwise), counterclaims, cross-claims, defenses, disallowance, impairment, objection, or any other challenges under any applicable law or regulation by any Person. Except to the extent that a holder of a Bridge Loan Claim agrees to different treatment with respect to such holder’s Claim, on the applicable Distribution Date, or as soon as practicable thereafter, each holder of a Bridge Loan Claim shall receive, subject to the terms of this Plan, in full and final satisfaction, settlement, release and discharge of its Bridge Loan Claim:

(i) New Money Bridge Loan Claim: receipt of New Term Loan Facility Obligations on a dollar for dollar basis on account of its New Money Bridge Loan Claim.

(ii) Roll Up Loan Claim: receipt of New Convertible Notes on a dollar for dollar basis on account of its Roll Up Loan Claim.

(b)       Voting: The Bridge Loan Claims are impaired Claims. Holders of such Claims are entitled to vote to accept or reject the Plan. The Bridge Loan Lenders, as reflected on the registry maintained by the Bridge Loan Administrative Agent on the date the Disclosure Statement Order is entered on the Bankruptcy Court’s docket, rather than the Bridge Loan Administrative Agent, will vote on the Plan. The Debtors will request such registry from the Bridge Loan Administrative Agent and votes will be solicited directly by the Debtors with respect to such Bridge Loan Claims.

5.4.          Novelion Intercompany Loan Claims (Class 4).

(a)       Treatment: The Novelion Intercompany Loan Claim shall be Allowed under this Plan, and shall not be subject to any avoidance, reductions, setoff, offset, recoupment, recharacterization, subordination (whether equitable, contractual, or otherwise), counterclaims, cross-claims, defenses, disallowance, impairment, objection, or any other challenges under any applicable law or regulation by any Person. Except to the extent that the holder of the Novelion Intercompany Loan Claim agrees to different treatment, on the applicable Distribution Date, or as soon as practicable thereafter, the holder of the Novelion Intercompany Loan Claim shall receive, in full and final satisfaction, release and discharge of the Novelion Intercompany Loan Claim, the Class 4 New Common Stock Distribution. For the avoidance of doubt, in satisfaction of the Novelion Intercompany Loan Claim in accordance with this Section 5.4, Novelion shall waive and release any and all Other Novelion Claims, and Novelion shall not be entitled to any distribution or consideration on account thereof, except as provided in Section 1 of the Shared Services Agreements pursuant to Section 7.16 hereof.

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(b)       Voting: The Novelion Intercompany Loan Claim is an impaired Claim. The holder of such Claim is entitled to vote to accept or reject the Plan, and the vote of such holder will be solicited with respect to such Novelion Intercompany Loan Claim.

5.5.         Government Settlement Claims (Class 5).

(a)       Treatment: Except to the extent that a holder of a Government Settlement Claim agrees to a different treatment, Government Settlement Claims shall be unimpaired by this Plan and shall remain obligations of the Reorganized Debtors to the extent not satisfied and/or paid on or before the Effective Date. The Government Settlement Agreements shall be deemed assumed by the Debtors, and binding upon the Reorganized Debtors and the applicable parties thereto as of and following the Effective Date (however, the foregoing shall not constitute a determination whether such agreements are executory contracts subject to section 365 of the Bankruptcy Code).

Notwithstanding the foregoing, the applicable federal government parties to the federal Government Settlement Agreements have agreed not to exercise rights under the federal Government Settlement Agreements to accelerate or increase monetary obligations under those agreements based solely on the fact of the commencement of the Chapter 11 Cases or the fact of the consummation of the transactions contemplated by this Plan, the Plan Funding Agreement and/or the other Transaction Documents, including the occurrence of any Fundamental Transaction (as defined in the Government Settlement Agreements), by virtue of the consummation of any such transactions or the failure of the New Common Stock of the Plan Investor to be listed on the NASDAQ or other US stock exchange, provided that all of the Debtors’ and Reorganized Debtors’ payment and other obligations under the federal Government Settlement Agreements continue to be fulfilled in accordance with the terms of those agreements during the Chapter 11 Cases and after the Reorganized Debtors’ emergence from the Chapter 11 Cases. Except as expressly set forth in this paragraph, the applicable federal government parties to the federal Government Settlement Agreements reserve all rights under those agreements, including but not limited to any right to require accelerated or increased payments if the Debtors and/or Reorganized Debtors fail to comply with their payment or other obligations under those agreements.

With respect to the non-federal government parties to the Government Settlement Agreements listed at Schedule 1.81(b)-(cc), (jj) to the Plan (the “State Government Settlement Agreements”), the Debtors and Reorganized Debtors expressly agree that any provisions regarding default and acceleration in the State Government Settlement Agreements (including, but not limited to, the “Relators’ Letter Agreement”) as written and agreed to on or about February 14, 2018, as the result of a global settlement in United States et al., ex rel. Clarke et al., v. Aegerion Pharmaceuticals, Inc., et al., Civil Action No. 13-11785, as filed in the United States District Court for the District of Massachusetts, shall continue to apply as set forth in those agreements, notwithstanding any provisions of the Plan, Plan Documents, and Confirmation Order. Nothing in the Plan, Plan Documents, and Confirmation Order releases, nullifies, precludes, modifies, or enjoins the enforcement of the terms of the State Governmental Settlement Agreements by and between the Debtors and any parties thereto. For the sake of clarity, nothing in the Plan, Plan Documents, and Confirmation Order shall divest any court, commission, or tribunal of jurisdiction over any matters related to the State Government

29

Settlement Agreements or confer on the Bankruptcy Court jurisdiction over any matter related to the State Government Settlement Agreements. Notwithstanding the foregoing, the monetary obligations under the State Government Settlement Agreements shall not be accelerated or increased as a result of the commencement of the Chapter 11 Cases or the consummation of the Plan, the Plan Funding Agreement, the Rights Offering, the New Term Loan Facility, and the New Convertible Notes, including the occurrence of any Fundamental Transaction (as defined in the State Government Settlement Agreements), by virtue of the consummation of any such transactions or the failure of the New Common Stock of the Plan Investor to be listed on the NASDAQ or other US stock exchange; provided that such transactions are consummated by October 31, 2019, as such date may be extended with the consent of the Debtors, the Required Parties and the applicable government parties to the State Government Settlement Agreements; provided further that, following the effective date of the Plan, all terms of the State Government Settlement Agreements will be in full force and effect, including, but not limited to any right to require accelerated or increased payments upon the occurrence of any Fundamental Transaction occurring after the Effective Date.

Nothing in the foregoing paragraphs affects or limits the provisions of Section 12.6(d)-(e) of the Plan.

(b)       Voting: The Government Settlement Claims are Unimpaired Claims. In accordance with section 1126(f) of the Bankruptcy Code, the holders of the Government Settlement Claims are conclusively presumed to accept this Plan and are not entitled to vote to accept or reject the Plan, and the votes of such holders will not be solicited with respect to the Government Settlement Claims.3

5.6.         Ongoing Trade Claims (Class 6A).

(a)       Treatment: Except to the extent that a holder of an Allowed Ongoing Trade Claim agrees to a different treatment, on the applicable Distribution Date each holder of an Allowed Ongoing Trade Claim shall, at the election of the Reorganized Debtors (in consultation with the Committee), and to the extent that such Allowed Ongoing Trade Claim was not previously paid pursuant to an order of the Bankruptcy Court: (i) be paid in full in Cash on the applicable Distribution Date, plus postpetition interest at the Applicable Interest Rate, computed daily from the Petition Date through the applicable Distribution Date, from Plan Cash (unless and until fully exhausted), or (ii) as to any Ongoing Trade Claim incurred in the ordinary course of business where payment comes due, based on normal credit terms, following the Effective Date, receive (a) such treatment that leaves unaltered all legal, equitable, or contractual rights to which the holder of such Allowed Ongoing Trade Claim is entitled in accordance with section 1124 of the Bankruptcy Code, or (b) such other treatment as agreed by the Reorganized Debtors

³ It is the Debtors’ position that the Government Settlement Claims are Unimpaired Claims and that Class 5 is presumed to accept this Plan under section 1126(f) of the Bankruptcy Code. However, the holders of Government Settlement Claims reserve their rights to dispute the Debtors’ position and/or to object to this Plan by the deadline established by the Bankruptcy Court. Further, even if the holders of Government Settlement Claims are presumed to accept this Plan, that presumption in no way affects or limits the provisions of section 12.6(d)-(e) of this Plan.

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and the holder of such Allowed General Unsecured Claim, to their mutual satisfaction, that would render such Ongoing Trade Claim Unimpaired.

(b)       Voting: The Allowed Ongoing Trade Claims are Unimpaired Claims. In accordance with section 1126(f) of the Bankruptcy Code, the holders of Allowed Ongoing Trade Claims are conclusively presumed to accept this Plan and are not entitled to vote to accept or reject the Plan, and the votes of such holders will not be solicited with respect to such Allowed Ongoing Trade Claims.

5.7.         Other General Unsecured Claims (Class 6B).

(a)       Treatment: Except to the extent that a holder of an Allowed Other General Unsecured Claim agrees to less favorable treatment, each holder of an Allowed Other General Unsecured Claim shall receive, on the applicable Distribution Date and in full and final satisfaction, settlement and release of such Allowed Other General Unsecured Claim, its Pro Rata Share of: (i) New Convertible Notes in the principal amount of $125,000,000 less the portion of New Convertible Notes distributed to (x) holders of DIP Claims (to the extent the DIP Claims are not repaid in full in Cash and receive a distribution of New Convertible Notes pursuant to Section 3.1 hereof), and (y) the holders of Roll Up Loan Claims pursuant to Section 5.3(a)(ii) hereof; and (ii) the Class 6B New Common Stock Distribution (including any New Common Stock issuable upon exercise of the New Warrants).

(b)       Voting: The Other General Unsecured Claims are impaired Claims. Holders of such Claims are entitled to vote to accept or reject the Plan, and the votes of such holders will be solicited with respect to such Other General Unsecured Claims.

5.8.         Existing Securities Law Claims (Class 7).

(a)       Treatment: Holders of Existing Securities Law Claims shall not receive or retain any distribution under the Plan on account of such Existing Securities Law Claims.

(b)       Voting: The Existing Securities Law Claims are impaired Claims. In accordance with section 1126(g) of the Bankruptcy Code, the holders of Existing Securities Law Claims are conclusively deemed to reject this Plan and are not entitled to vote to accept or reject the Plan, and the votes of such holders will not be solicited with respect to such Existing Securities Law Claims.

5.9.         Existing Interests (Class 8).

(a)       Treatment: Existing Interests shall be discharged, cancelled, released and extinguished, and holders thereof shall not receive or retain any distribution under the Plan on account of such Existing Interests.

(b)       Voting: The Existing Interests are impaired Interests. In accordance with section 1126(g) of the Bankruptcy Code, the holders of Existing Interests are conclusively deemed to reject this Plan and are not entitled to vote to accept or reject the Plan, and the votes of such holders will not be solicited with respect to such Existing Interests.

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5.10.       Special Provision Governing Unimpaired Claims.

For the avoidance of doubt, notwithstanding anything to the contrary in this Plan, the Plan Supplement, or in the Confirmation Order, until a holder of an Allowed Unimpaired Claim has received payment on account of such holder’s Allowed Claim that renders such Claim Unimpaired in accordance with this Plan, (a) such Claim shall not be deemed settled, satisfied, resolved, released, discharged, barred or enjoined by any provision of the Plan or the Confirmation Order, and (b) the property of each Debtors’ Estates that vests in the applicable Reorganized Debtor pursuant to the Plan shall not be free and clear of such Claim.

ARTICLE VI.

ACCEPTANCE OR REJECTION OF 

THE PLAN; EFFECT OF REJECTION BY ONE 

OR MORE CLASSES OF CLAIMS OR INTERESTS

6.1.         Class Acceptance Requirement.

A Class of Claims shall have accepted the Plan if it is accepted by at least two- thirds (2/3) in dollar amount and more than one-half (1/2) in number of holders of the Allowed Claims in such Class that have voted on the Plan calculated in accordance with the Disclosure Statement Order.

6.2.         Tabulation of Votes on a Non-Consolidated Basis.

All votes on the Plan shall be tabulated on a non-consolidated basis by Class and by Debtor for the purpose of determining whether the Plan satisfies sections 1129(a)(8) and/or (10) of the Bankruptcy Code.

6.3.         Confirmation Pursuant to Section 1129(b) of the Bankruptcy Code or “Cramdown.”

Because certain Classes are deemed to have rejected this Plan, the Debtors will request confirmation of this Plan, as it may be modified and amended from time to time, under section 1129(b) of the Bankruptcy Code with respect to such Classes. Subject to Sections 14.5 and 14.6 of this Plan, the Debtors reserve the right (subject to the parties’ rights under the RSA and the Plan Funding Agreement) to alter, amend, modify, revoke or withdraw this Plan or any Plan Document in order to satisfy the requirements of section 1129(b) of the Bankruptcy Code, if necessary. Subject to Sections 14.5 and 14.6 of this Plan, the Debtors also reserve the right to request confirmation of the Plan, as it may be modified, supplemented or amended from time to time, with respect to any Class that affirmatively votes to reject the Plan.

6.4.         Elimination of Vacant Classes.

Any Class of Claims or Interests that does not have a holder of an Allowed Claim or Allowed Interest or a Claim or Interest temporarily Allowed as of the date of the Confirmation Hearing shall be deemed eliminated from the Plan for purposes of voting to accept or reject the

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Plan and for purposes of determining acceptance or rejection of the Plan pursuant to section 1129(a)(8) of the Bankruptcy Code.

6.5.         Voting Classes; Deemed Acceptance by Non-Voting Classes.

If a Class contains Claims or Interests eligible to vote and no holders of Claims or Interests eligible to vote in such Class vote to accept or reject the Plan, the Plan shall be deemed accepted by such Class.

6.6.         Confirmation of All Cases.

Except as otherwise specified herein, the Plan shall not be deemed to have been confirmed unless and until the Plan has been confirmed as to each of the Debtors; provided, however, that the Debtors, subject to the parties’ rights under the RSA and the Plan Funding Agreement, may at any time waive this Section 6.6.

ARTICLE VII.

MEANS FOR IMPLEMENTATION

7.1.         Non-Substantive Consolidation.

The Plan is a joint plan that does not provide for substantive consolidation of the Debtors’ Estates, and on the Effective Date, the Debtors’ Estates shall not be deemed to be substantively consolidated for purposes hereof. Except as specifically set forth herein, nothing in this Plan shall constitute or be deemed to constitute an admission that any one of the Debtors is subject to or liable for any claim against any other Debtor. Additionally, claimants holding Claims and Interests against multiple Debtors, to the extent Allowed in each Debtor’s Chapter 11 Case, will be treated as holding a separate Claim or separate Interest, as applicable, against each Debtor’s Estate, provided, however, that no holder of an Allowed Claim shall be entitled to receive more than payment in full of such Allowed Claim (plus postpetition interest, if and to the extent provided in this Plan), and such Claims will be administered and treated in the manner provided in this Plan.

7.2.         Plan Funding Transaction.

On the Effective Date, subject to the terms and conditions set forth in the Plan Funding Agreement and the Implementation Memorandum and in exchange for New Common Stock in the Plan Investor or a newly formed holding company of the Plan Investor (“New Amryt”) and the other obligations of the Plan Investor and/or New Amryt under the Plan Funding Agreement and this Plan, Aegerion shall sell to the Plan Investor or New Amryt one hundred percent (100%) of the New Equity Interests in Reorganized Aegerion. From and after the Effective Date, the Plan Investor or New Amryt shall directly and indirectly own the Reorganized Debtors. The existing shareholders of the Plan Investor shall own 38.6% of New Common Stock and the holders of Class 4 and Class 6B Claims shall collectively own 61.4% of the New Common Stock (prior to completion of the $42 million Rights Offering and $18 million Plan Investor Equity Raise). The transfer of the New Equity Interests of Reorganized Aegerion to the Plan Investor or New Amryt, and any and all action to be taken in connection therewith,

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shall be authorized without the need for any further board, corporate or shareholder action of the Debtors or Novelion. The issuance of the New Common Stock requires the approval of the U.K. Panel on Takeovers and Mergers. Further, the formation of New Amryt will be effected pursuant to a scheme of arrangement that requires the approval of both the (a) shareholders of the Plan Investor (with a voting threshold of 75% of those voting and a majority in number of those voting) and (b) courts of England and Wales. The scheme of arrangement will be undertaken pursuant to Part 26 of the Companies Act of 2006 and will involve an application by the Plan Investor to the High Court of Justice in England and Wales to sanction the scheme of arrangement to allow New Amryt to become the holding company of the Plan Investor group, following which the rights and obligations of the Plan Investor under the Plan Funding Agreement will be assumed by New Amryt. In consideration for the cancellation of each Plan Investor shareholder’s interest in the Plan Investor, each Plan Investor shareholder will receive shares in New Amryt and certain contingent value rights. The New Common Stock is anticipated to be listed for trading on the Alternative Investment Market operated by the London Stock Exchange plc. (“AIM”) and on the Euronext Growth Market operated by Euronext Dublin (“Euronext”) and will require the approval of AIM and Euronext for such admission and trading. The issuance of the New Common Stock is also subject to confirmation of the Plan.

7.3.             Rights Offering.

(a)       Purpose. The proceeds of the sale of the Rights Offering Stock and Plan Investor Equity Raise shall be used to provide a new equity raise of $60 million — $42 million of which is on account of the Rights Offering conducted under the Plan and $18 million of which is on account of the Plan Investor Equity Raise, which shall be available for ordinary course operations and general corporate purposes.

(b)       Rights Offering. In accordance with the New Registration Rights Agreement, the Rights Offering Procedures and the Backstop Commitment Agreement, and as provided in the Implementation Memorandum, each Eligible Holder that timely votes to accept the Plan shall receive Subscription Rights to acquire its respective Pro Rata Share of Rights Offering Stock pursuant to the terms set forth in this Plan and in the Rights Offering Procedures. With respect to each Eligible Holder that timely votes to accept the Plan, each Subscription Right shall represent the right to acquire one share of Rights Offering Stock for the Rights Offering Exercise Price.

(c)       Backstop Commitment. The Plan Investor Equity Raise will be correspondingly increased by the aggregate amount of the Rights Offering Amount that is not timely, duly and validly subscribed and paid for by the Eligible Holders that timely vote to accept the Plan in accordance with the Rights Offering Procedures, and in accordance with, and subject to the limitations of, the provisions of the Backstop Commitment Agreement, and as further described below, upon exercise of the put option of the Plan Investor, the Backstop Parties shall be severally, and not jointly, required to purchase their applicable portion of the Unsubscribed Shares (allocated pro rata among the Backstop Parties based upon their respective Backstop Commitments) in the event that the Plan Investor has been unable to effect a private placement of the entire Plan Investor Equity Raise Amount.

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(d)       Commitment Fee. On the Effective Date, the Backstop Parties shall receive from the Plan Investor their respective portion of the Backstop Commitment Fee pursuant to the terms of the Backstop Commitment Agreement. The Backstop Commitment Fee shall be fully earned immediately upon the Subscription Commencement Date and payable by the Plan Investor (and not the Debtors) on the Effective Date pursuant to the terms and conditions of the Backstop Commitment Agreement.

7.4.         Plan Funding.

The Debtors’ Cash obligations under the Plan will be funded from Plan Cash and proceeds from the Rights Offering and the Plan Investor Equity Raise; provided however (i) that only Plan Cash shall be used for payment of Government Settlement Claims that become due and payable prior to the Effective Date, DIP Claims, Fee Claims, Ad Hoc Group Fee Claim and the Convertible Notes Trustee Fees and (ii) only proceeds from the Rights Offering and Plan Investor Equity Raise will be used to pay the Rebate Obligations or to repay any portion of the DIP Obligations incurred to pay Rebate Obligations.

7.5.         New Term Loan Facility; New Convertibles Notes.

On the Effective Date, subject to the Implementation Memorandum, without any requirement of further action by stockholders or directors of the Debtors, each of the Reorganized Debtors shall be authorized to enter into the New Term Loan Facility, in the estimated amount of $81.9 million (which is an amount equal to (a) the New Money Bridge Loan Claims plus (b) the Existing Plan Investor Debt), and the New Convertible Notes Indenture, governing approximately $125 million of New Convertible Notes, as well as any notes, documents or agreements in connection therewith, including, without limitation, any documents required in connection with the creation or perfection of the Liens on any Collateral securing the New Term Loan Facility.

7.6.       Authorization, Issuance and Delivery of Plan Securities by the Plan Investor.

(a)       On the Effective Date, subject to the Implementation Memorandum, the Plan Investor is authorized to issue or cause to be issued those Plan Securities to be issued by it in accordance with the terms of this Plan and the Plan Funding Agreement and to take any and all action associated therewith, without the need for any further Bankruptcy Court, corporate, limited liability company, member or shareholder action.

(b)       On the Effective Date, subject to the Implementation Memorandum, the Plan Investor shall issue and cause to be delivered the New Common Stock and the New Warrants available in the New Common Stock Distribution to the Reorganized Debtors, who will then deliver such New Common Stock and New Warrants directly to the holders of the Novelion Intercompany Loan Claims and Other General Unsecured Claims in accordance with the terms this Plan.

(c)       On the Effective Date, subject to the Implementation Memorandum, the Plan Investor shall issue and cause to be delivered the Rights Offering Stock to the Reorganized Debtors, who will then deliver such Rights Offering Stock directly to Eligible Holders who vote

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in favor of the Plan and exercise their Subscription Rights in accordance with the terms of this Plan, the Rights Offering Procedures, and the Backstop Commitment Agreement.

(d)       As a condition to receiving any Plan Securities under this Plan or pursuant to the Rights Offering or the Plan Investor Equity Raise, the Bridge Lenders shall have executed and delivered to the Plan Investor a signature page to the New Registration Rights Agreement. The New Registration Rights Agreement shall be executed and in full force and effect on the Effective Date.

(e)       Notwithstanding anything to the contrary herein, (x) any Person that would be entitled to receive more than 9.99% (but no more than 16%) of the aggregate amount of the New Common Stock issued as of the Effective Date (excluding New Common Stock issued pursuant to any management incentive plan and any New Common Stock reserved for issuance to any person other than such Person pursuant to New Warrants or the New Convertible Notes or any other warrant, option or agreement) or (y) with the consent of the Debtors and Plan Investor, any other Person entitled to receive New Common Stock hereunder, may elect to receive New Warrants on a one-for-one basis in lieu of all or any portion of the shares of New Common Stock that would otherwise be issued to such Person under the Plan; provided that such Person notifies the Debtors in writing of such election (and the percentage of shares of New Common Stock to be issuable thereunder) no later than two (2) Business Days after the Confirmation Date, provided, further, that, with respect to clause (x), without the consent of the Debtors and the Plan Investor, such Person may only elect to receive New Warrants in lieu of such portion of New Common Stock that would otherwise be issued to such Person under the Plan in excess of 7.5% of the aggregate amount of New Common Stock issued as of the Effective Date (excluding New Common Stock issued pursuant to any management incentive plan and any New Common Stock reserved for issuance to any person other than such Person pursuant to New Warrants or the New Convertible Notes or any other warrant, option or agreement).

(f)       Notwithstanding anything to the contrary herein, (x) any Person that would be entitled to receive more than 4.99% (but no more than 6.0%) of the aggregate amount of the New Common Stock issued as of the Effective Date (excluding New Common Stock issued pursuant to any management incentive plan and any New Common Stock reserved for issuance to any person other than such Person pursuant to New Warrants or the New Convertible Notes or any other warrant, option or agreement) or (y) with the consent of the Debtors and Plan Investor, any other Person entitled to receive New Common Stock hereunder, may elect to receive New Warrants on a one-for-one basis in lieu of all or any portion of the shares of New Common Stock that would otherwise be issued to such Person under the Plan; provided that such Person notifies the Debtors in writing of such election (and the percentage of shares of New Common Stock to be issuable thereunder) no later than two (2) Business Days after the Confirmation Date, provided, further, that, with respect to clause (x), without the consent of the Debtors and the Plan Investor, such Person may only elect to receive New Warrants in lieu of such portion of New Common Stock that would otherwise be issued to such Person under the Plan in excess of 4.5% of the aggregate amount of New Common Stock issued as of the Effective Date (excluding New Common Stock issued pursuant to any management incentive plan and any New Common Stock reserved for

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issuance to any person other than such Person pursuant to New Warrants or the New Convertible Notes or any other warrant, option or agreement).

7.7.         Continued Corporate Existence and Vesting of Assets.

(a)       General.

(i) Except as otherwise provided in this Plan, the Debtors shall continue to exist after the Effective Date as Reorganized Debtors in accordance with the applicable laws of the respective jurisdictions in which they are incorporated or organized and pursuant to the Amended Certificates of Formation for the purposes of satisfying their obligations under the Plan and the continuation of their business. On or after the Effective Date, each Reorganized Debtor, in its discretion, may take any and all action as permitted by applicable law and such Reorganized Debtor’s organizational documents, as such Reorganized Debtor may determine is reasonable and appropriate, including, but not limited to, causing: (w) a Reorganized Debtor to be merged into another Reorganized Debtor, or its Subsidiary and/or affiliate; (x) a Reorganized Debtor to be dissolved; (y) the legal name of a Reorganized Debtor to be changed; or (z) the closure of a Reorganized Debtor’s case on the Effective Date or any time thereafter.

(ii) On the Effective Date or as soon as reasonably practicable thereafter, the Reorganized Debtors may take any and all action as may be necessary or appropriate to effect any transaction described in, approved by, contemplated by, or necessary to effectuate the Plan, including: (1) the execution and delivery of appropriate agreements or other documents of merger, consolidation, restructuring, conversion, disposition, transfer, dissolution or liquidation containing terms that are consistent with the terms of the Plan and that satisfy the applicable requirements of applicable law and any other terms to which the applicable entities may agree; (2) the execution and delivery of appropriate instruments of transfer, assignment, assumption or delegation of any asset, property, right, liability, debt or obligation on terms consistent with the terms of the Plan and having other terms for which the applicable parties agree; (3) the filing of appropriate certificates or articles of incorporation, reincorporation, merger, consolidation, conversion or dissolution pursuant to applicable state law; and (4) all other actions that the applicable entities determine to be necessary or appropriate, including making filings or recordings that may be required by applicable law.

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(b)       Revesting of Assets. Except as otherwise provided in this Plan, on and after the Effective Date, all property of the Estates, wherever located, including all claims, rights and Causes of Action and any property, wherever located, acquired by the Debtors under or in connection with this Plan, shall revest in the Reorganized Debtors, as applicable, free and clear of all Claims, Liens, charges, other encumbrances and Interests. On and after the Effective Date, except as otherwise provided in this Plan, each applicable Reorganized Debtor may operate its business and may use, acquire and dispose of property, wherever located, and each Reorganized Debtor may prosecute, compromise or settle any Claims (including any Administrative Expense Claims) and Causes of Action without supervision of or approval by the Bankruptcy Court and free and clear of any restrictions of the Bankruptcy Code or the Bankruptcy Rules other than restrictions expressly imposed by this Plan or the Confirmation Order. Without limiting the foregoing, the Reorganized Debtors may pay the charges incurred on or after the Effective Date for Professional Persons’ fees, disbursements, expenses or related support services without application to the Bankruptcy Court.

7.8.         Cancellation of Existing Securities and Agreements.

Except for the purpose of evidencing a right to distribution under this Plan, and except as otherwise set forth in this Plan (including Section 2.3 hereof), on the Effective Date, subject to the Implementation Memorandum, all agreements, including all intercreditor agreements, instruments, and other documents evidencing, related to or connected with any Claim or Interest, other than Intercompany Interests, and any rights of any holder in respect thereof, shall be deemed cancelled, discharged and of no force or effect. The holders of or parties to such cancelled instruments, securities and other documentation will have no rights arising from or relating to such instruments, securities and other documentation or the cancellation thereof, except the rights provided for pursuant to this Plan. Notwithstanding anything to the contrary herein, each of the Bridge Loan Credit Agreement, Novelion Intercompany Loan Credit Agreement and the Convertible Notes Indenture shall continue in effect solely to the extent necessary to: (a) permit holders of Bridge Loan Claims, Novelion Intercompany Loan Claims and Convertible Notes Claims (or the Convertible Notes Indenture Trustee) to receive Plan Distributions on account of such respective claims and make further distributions to holders of Claims, in accordance with the Plan, as applicable; (b) permit the Bridge Loan Administrative Agent and the Convertible Notes Trustee to seek compensation and/or reimbursement of fees and expenses in accordance with the terms of this Plan and/or the Convertible Notes Indenture, including through the exercise of the charging Lien provided under the Convertible Notes Indenture; and (c) preserving the right of the Convertible Notes Indenture Trustee to indemnification from the Debtors pursuant and subject to the terms of the Convertible Notes Indenture. Except as provided pursuant to this Plan, upon satisfaction of the Bridge Loan Claims and Convertible Notes Claims, each of the Bridge Loan Administrative Agent and the Convertible Notes Trustee, shall be discharged of all of their respective obligations associated with the Bridge Loan and the Convertible Notes, respectively.

7.9.         Boards.

(a)       As of the Effective Date, the initial board of directors of each of the Reorganized Debtors and the Plan Investor shall consist of those individuals set forth in the Plan Supplement to be filed with the Bankruptcy Court on or before the date of the Confirmation

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Hearing. The compensation arrangement for any insider of the Debtors that shall become an officer of a Reorganized Debtor or the Plan Investor shall be disclosed in the Plan Supplement and selected in accordance with the terms set forth in the New Registration Rights Agreement.

(b)       Unless reappointed pursuant to Section 7.9(a) of the Plan, the members of the board of directors of each Debtor prior to the Effective Date shall have no continuing obligations to the Reorganized Debtors in their capacities as such on and after the Effective Date, each such member shall be deemed to have resigned or shall otherwise cease to be a director of the applicable Debtor on the Effective Date. Commencing on the Effective Date, each of the directors of each of the Reorganized Debtors shall serve pursuant to the terms of the applicable organizational documents of such Reorganized Debtor and may be replaced or removed in accordance with such organizational documents.

7.10.       Management.

As of the Effective Date, the individuals who will serve in certain senior management positions of the Reorganized Debtors shall consist of those individuals set forth in the Plan Supplement and shall be Acceptable to the Debtors and each of the Required Parties in accordance with the applicable terms of the Transaction Documents. The compensation arrangement for any insider of the Debtors that shall become an officer of a Reorganized Debtor shall be in form and substance Acceptable to the Debtors and each of the Required Parties and disclosed in the Plan Supplement to be filed with the Bankruptcy Court on or before the date of the Confirmation Hearing.

7.11.       Corporate Action.

(a)       The Reorganized Debtors shall serve on the U.S. Trustee quarterly reports of the disbursements made by each Reorganized Debtor on an entity-by-entity basis, within 15 days after the conclusion of each such period, until such time as a final decree is entered closing the applicable Chapter 11 Case or the applicable Chapter 11 Case is converted or dismissed. Any such reports shall be prepared consistent with (both in terms of content and format) any applicable Bankruptcy Court and U.S. Trustee guidelines. Any deadline for filing Administrative Expense Claims shall not apply to U.S. Trustee Fees.

(b)       On the Effective Date, the Amended Memorandum of Association, the Amended Certificates of Formation and any other applicable amended and restated corporate organizational documents of each of the Reorganized Debtors shall be deemed authorized in all respects.

(c)       Any action under the Plan to be taken by or required of the Debtors or the Reorganized Debtors, including the adoption or amendment of certificates of formation, incorporation and by-laws, the issuance of securities and instruments, or the selection of officers or directors shall be authorized and approved in all respects, without any requirement of further action by any of the Debtors’ or the Reorganized Debtors’ equity holders, sole members, boards of directors or boards of managers, or similar body, as applicable.

(d)       The Debtors and the Reorganized Debtors shall be authorized to execute, deliver, file, and record such documents (including the Plan Documents), contracts, instruments,

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releases and other agreements and take such other action as may be necessary to effectuate and further evidence the terms and conditions of the Plan, without the necessity of any further Bankruptcy Court, corporate, limited liability company, board, member, or shareholder approval or action. In addition, the selection of the Persons who will serve as the initial directors, officers and managers of the Reorganized Debtors as of the Effective Date shall be deemed to have occurred and be effective on and after the Effective Date without any requirement of further action by the board of directors, board of managers, or equity holders of the applicable Reorganized Debtor.

7.12.       [Reserved].

7.13.       Payment of Convertible Notes Trustee Fees.

On the Effective Date, the Debtors shall pay in full in Cash all unpaid Convertible Notes Trustee Fees from Plan Cash, regardless of whether such fees and expenses were incurred before or after the Petition Date, without application by any party to the Bankruptcy Court and without notice and a hearing pursuant to section 1129(a)(4) of the Bankruptcy Code or otherwise. Notwithstanding anything to the contrary in the Plan, the Convertible Notes Trustee Fees shall not be subject to the Administrative Bar Date.

7.14.       Comprehensive Settlement of Claims and Controversies.

Pursuant to Bankruptcy Rule 9019 and in consideration for the distributions and other benefits provided under this Plan, the provisions of this Plan will constitute a good faith compromise and settlement of all Claims or controversies relating to the rights that a holder of a Claim or Interest may have with respect to any Allowed Claim or Allowed Interest or any distribution to be made pursuant to this Plan on account of any Allowed Claim or Allowed Interest. The entry of the Confirmation Order will constitute the Bankruptcy Court’s approval, as of the Effective Date (but subject to Section 5.10 hereof), of the compromise or settlement of all such claims or controversies and the Bankruptcy Court’s finding that all such compromises or settlements are: (i) in the best interest of the Debtors, the Reorganized Debtors, and their respective Estates and property, and of holders of Claims or Interests; and (ii) fair, equitable and reasonable.

7.15.       Additional Transactions Authorized Under This Plan.

On or prior to the Effective Date, as shall be Acceptable to the Required Parties and the Committee, the Debtors shall be authorized to take any such actions as may be necessary or appropriate to reinstate Claims or Interests or render Claims or Interests not impaired, as provided for under this Plan.

7.16.       Shared Services Agreements.

The Shared Services Agreements, as amended, shall be assumed by order of the Bankruptcy Court, and shall terminate on the Effective Date in accordance with the terms of the Shared Services Agreements.

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7.17.       Acceptable.

As used herein, the term “Acceptable” shall mean (x) when in reference to any document, or any amendment, modification or change to such document, in form and substance reasonably acceptable to the applicable parties, and (y) when in reference to any individual, reasonably acceptable to the applicable parties.

ARTICLE VIII.

DISTRIBUTIONS

8.1.         Distributions.

The Disbursing Agent shall make all Plan Distributions to the appropriate holders of Allowed Claims in accordance with the terms of this Plan. Distributions to holders of Allowed Other General Unsecured Claims (solely on account of Convertible Notes Claims) shall be made by the Convertible Notes Trustee and deemed completed when made to the Convertible Notes Trustee as Disbursing Agent. Plan Distributions on account of the Bridge Loan Claim (other than the payment of any unpaid fees and expenses of the Bridge Loan Administrative Agent which shall be paid to the Bridge Loan Administrative Agent in cash on the Effective Date) shall not be made to the Bridge Loan Administrative Agent but instead shall be distributed directly to the Bridge Loan Lenders as reflected on the registry maintained by the Bridge Loan Administrative Agent as of the Confirmation Date. The Debtors will request such registry from the Bridge Loan Administrative Agent.

8.2.         No Postpetition Interest on Claims.

Other than as specifically provided in the Plan or the Confirmation Order, or required by applicable bankruptcy or non-bankruptcy law, postpetition interest shall not accrue or be paid on any Claims, and no holder of a Claim shall be entitled to interest accruing on such Claim on or after the Petition Date.

8.3.         Date of Distributions.

Unless otherwise provided herein, any Plan Distributions and deliveries to be made hereunder shall be made on the applicable Distribution Date; provided, that the Reorganized Debtors may utilize periodic distribution dates to the extent that use of a periodic distribution date does not delay payment of the Allowed Claim more than sixty (60) days. For the avoidance of doubt, and notwithstanding anything herein to the contrary, all such Plan Distributions and deliveries that are to be made in Cash hereunder on the applicable Distribution Date shall be made from Plan Cash unless otherwise provided herein. In the event that any payment or act under this Plan is required to be made or performed on a date that is not a Business Day, then the making of such payment or the performance of such act may be completed on or as soon as reasonably practicable after the next succeeding Business Day, but shall be deemed to have been completed as of the required date.

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8.4.         Distribution Record Date.

As of the close of business on the Distribution Record Date, the various lists of holders of Claims in each of the Classes, as maintained by the Debtors, or their agents, shall be deemed closed and there shall be no further changes in the record holders of any of the Claims after the Distribution Record Date. Neither the Debtors nor the Disbursing Agent shall have any obligation to recognize any transfer of Claims occurring after the close of business on the Distribution Record Date. Additionally, with respect to payment of any Cure Amounts or any Cure Disputes in connection with the assumption and/or assignment of the Debtors’ executory contracts and unexpired leases, neither the Debtors, the Disbursing Agent nor the Plan Investor shall have any obligation to recognize or deal with any party other than the non-Debtor party to the applicable executory contract or unexpired lease, even if such non-Debtor party has sold, assigned or otherwise transferred its Claim for a Cure Amount.

8.5.         Disbursing Agent.

(a)       Powers of Disbursing Agent. The Disbursing Agent shall be empowered to: (i) effectuate all actions and execute all agreements, instruments, and other documents necessary to perform its duties under this Plan; (ii) make all applicable Plan Distributions or payments contemplated hereby; (iii) employ professionals to represent it with respect to its responsibilities; and (iv) exercise such other powers as may be vested in the Disbursing Agent by order of the Bankruptcy Court (including any order issued after the Effective Date), pursuant to this Plan, or as deemed by the Disbursing Agent to be necessary and proper to implement the provisions hereof.

(b)       Expenses Incurred by the Disbursing Agent on or After the Effective Date. Except as otherwise ordered by the Bankruptcy Court, and subject to the written agreement of the Reorganized Debtors, the amount of any reasonable and documented fees and expenses incurred by the Disbursing Agent on or after the Effective Date (including taxes) and any reasonable compensation and expense reimbursement Claims (including reasonable attorney and other professional fees and expenses) of the Disbursing Agent shall be paid in Cash by the Reorganized Debtors. The foregoing fees and expenses shall be paid in the ordinary course, upon presentation of invoices to the Reorganized Debtors and without the need for approval by the Bankruptcy Court, as set forth in Section 3.2(b) of this Plan. In the event that the Disbursing Agent and the Reorganized Debtors are unable to resolve a dispute with respect to the payment of the Disbursing Agent’s fees, costs and expenses, the Disbursing Agent may elect to submit any such dispute to the Bankruptcy Court for resolution.

(c)       Bond. The Disbursing Agent shall not be required to give any bond or surety or other security for the performance of its duties unless otherwise ordered by the Bankruptcy Court and, in the event that the Disbursing Agent is so otherwise ordered, all costs and expenses of procuring any such bond or surety shall be borne by the Reorganized Debtors. Furthermore, any such entity required to give a bond shall notify the Bankruptcy Court and the U.S. Trustee in writing before terminating any such bond that is obtained.

(d)       Cooperation with Disbursing Agent. The Reorganized Debtors shall use all commercially reasonable efforts to provide the Disbursing Agent with the amount of Claims

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and the identity and addresses of holders of Claims, in each case, that are entitled to receive Plan Distributions, as set forth in the Debtors’ or the applicable Reorganized Debtors’ books and records. The Reorganized Debtors will cooperate in good faith with the Disbursing Agent to comply with the withholding and reporting requirements outlined in Section 8.16 of this Plan.

8.6.         Delivery of Distributions in General.

Subject to the provisions contained in this Article VIII, the applicable Disbursing Agent will issue, or cause to be issued, and authenticate, as applicable, all Plan Consideration, and subject to Bankruptcy Rule 9010, make all Plan Distributions or payments to any holder of an Allowed Claim as and when required by this Plan at: (a) the address of such holder on the books and records of the Debtors or their agents; or (b) at the address in any written notice of address change delivered to the Debtors or the applicable Disbursing Agent, including any addresses included on any filed proofs of Claim or transfers of Claim filed with the Bankruptcy Court. In the event that any Plan Distribution to any holder is returned as undeliverable, no distribution or payment to such holder shall be made unless and until the applicable Disbursing Agent has been notified of the then current address of such holder, at which time or as soon as reasonably practicable thereafter such Plan Distribution shall be made to such holder without interest; provided, however, such Plan Distributions or payments shall be deemed unclaimed property under section 347(b) of the Bankruptcy Code at the expiration of the later of one year from (i) the Effective Date, and (ii) the first Distribution Date after such holder’s Claim is first Allowed.

8.7.         Delivery of Distributions on Convertible Notes Claims.

The Convertible Notes Indenture Trustee shall be deemed to be the holder of all Allowed Convertible Notes Claims in Class 6B for purposes of distributions to be made hereunder, and all distributions on account of such Allowed Claims shall be made to or at the direction of the Convertible Notes Indenture Trustee except as otherwise provided herein. As soon as practicable following the Effective Date, the Convertible Notes Indenture Trustee shall arrange to deliver or direct the delivery of such distributions to or on behalf of the holders of Allowed Convertible Notes Claims in Class 6B in accordance with the terms of the Convertible Notes Indenture and the Plan. Distributions of the New Convertible Notes to be held through DTC shall be made through the facilities of DTC in accordance with DTC’s customary practices. All New Convertible Notes to be distributed pursuant to the Plan shall be issued in the names of such holders, their nominees of record, or their permitted designees as of the Distribution Record Date in accordance with DTC’s book-entry procedures, to the extent applicable; provided that such New Convertible Notes are permitted to be held through DTC’s book-entry system; provided, further, that to the extent that the New Convertible Notes are not eligible for distribution in accordance with DTC’s customary practices, the Reorganized Debtors will take such reasonable actions as may be required to cause distributions of the New Convertible Notes under the Plan. No distributions will be made other than through DTC if the New Convertible Notes are permitted to be held through DTC’s book entry system. Any distribution that otherwise would be made to any holder eligible to receive a distribution of a security available solely through DTC who does not own or hold an account eligible to receive a distribution through DTC on a relevant distribution date shall be forfeited. The Reorganized Debtors will cause distributions of New Common Stock to be made to the CREST account of the holders of

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Allowed Convertible Notes Claims, or failing that, to the Convertible Notes Indenture Trustee to be held on behalf of the holders of Allowed Convertible Notes Claims and in accordance with the customary practices of the applicable depositary. All New Common Stock to be distributed pursuant to the Plan shall be issued in the names of such holders, their nominees of record, or their permitted designees as of the Distribution Record Date; provided, that to the extent that the New Common Stock is American Depositary Shares representing common stock or is not eligible for distribution as set forth herein, the Reorganized Debtors will take such reasonable actions as may be required to cause distributions of the New Common Stock under the Plan. Notwithstanding anything in the Plan to the contrary, and without limiting the exculpation and release provisions of the Plan, the Convertible Notes Indenture Trustee shall not have any liability to any entity with respect to distributions made or directed to be made by the Convertible Notes Indenture Trustee except for fraud or intentional misconduct.

8.8.         Unclaimed Property.

Except with respect to holders of Unimpaired Claims, one year from the later of (i) the Effective Date, and (ii) the first Distribution Date after such holder’s Claim is first Allowed, all unclaimed property, wherever located, or interests in property distributable hereunder on account of such Claim shall revert to the Reorganized Debtors or their respective successors or assigns of the Reorganized Debtors, and any claim or right of the holder of such Claim to such property, wherever located, or interest in property shall be discharged and forever barred. The Reorganized Debtors and the Disbursing Agent shall have no obligation to attempt to locate any holder of an Allowed Claim other than by reviewing the Debtors’ books and records, and the proofs of Claim filed against the Debtors, as reflected on the claims register maintained by the Claims Agent.

8.9.         Satisfaction of Claims.

Unless otherwise specifically provided herein, any Plan Distributions and deliveries to be made on account of Allowed Claims hereunder shall be in complete settlement, satisfaction and discharge of such Allowed Claims.

8.10.       Manner of Payment Under Plan.

Except as specifically provided herein, at the option of the Reorganized Debtors, any Cash payment to be made hereunder may be made by a check or wire transfer or as otherwise required or provided in applicable agreements or customary practices of the Debtors or the applicable Reorganized Debtor, as the case may be.

8.11.       Fractional Shares; De Minimis Cash Distributions.

Neither the Reorganized Debtors nor the Disbursing Agent shall have any obligation to make a Plan Distribution that is less than one (1) share of New Common Stock or $50.00 in Cash. No fractional shares of New Common Stock shall be distributed. When any Plan Distribution would otherwise result in the issuance of a number of shares of New Common Stock that is not a whole number, the shares of the New Common Stock subject to such Plan Distribution will be rounded to the next higher or lower whole number as follows: (i) fractions equal to or greater than ½ will be rounded to the next higher whole number; and (ii) fractions

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less than ½ will be rounded to the next lower whole number; provided, that the foregoing shall not apply to any rounding of the Rights Offering Stock, the distribution of which shall be governed by the Rights Offering Procedures and Section 7.3 of this Plan. The total number of shares of New Common Stock to be distributed on account of Allowed Claims will be adjusted as necessary to account for the rounding provided for in this Plan. No consideration will be provided in lieu of fractional shares that are rounded down. Fractional shares of New Common Stock that are not distributed in accordance with this Section 8.11 shall be cancelled.

8.12.       Distributions on Account of Allowed Claims Only.

Notwithstanding anything herein to the contrary, no Plan Distribution shall be made on account of a Claim until such Claim becomes an Allowed Claim plus any postpetition interest on such Claim, to the extent such interest is permitted under this Plan.

8.13.       No Distribution in Excess of Amount of Allowed Claim.

Notwithstanding anything herein to the contrary, no holder of an Allowed Claim shall, on account of such Allowed Claim, receive a Plan Distribution of a value in excess of the Allowed amount of such Claim, plus any postpetition interest on such Claim, to the extent such interest is permitted under this Plan.

8.14.       Exemption from Securities Laws.

The issuance of and the distribution under the Plan of the Plan Securities shall be exempt from registration under the Securities Act and any other applicable securities laws pursuant to section 1145 of the Bankruptcy Code, to the maximum extent permitted thereunder.

The New Common Stock (including the Rights Offering Stock and New Common Stock issuable upon the exercise of New Warrants) issued under the Plan will be issued without registration under the Securities Act or any similar federal, state, or local law in reliance upon section 1145 of the Bankruptcy Code. New Common Stock (including the Rights Offering Stock and New Common Stock issuable upon the exercise of New Warrants) issued under the Plan in reliance upon section 1145 of the Bankruptcy Code shall be exempt from, among other things, the registration requirements of Section 5 of the Securities Act and any other applicable U.S. state or local law requiring registration prior to the offering, issuance, distribution, or sale of securities except with respect to an entity that is an “underwriter” as defined in subsection (b) of section 1145 of the Bankruptcy Code. For the avoidance of doubt, Novelion shall not be deemed an “underwriter” as defined in subsection (b) of section 1145 of the Bankruptcy Code. The New Common Stock (including the Rights Offering Stock and New Common Stock issuable upon the exercise of New Warrants) issued pursuant to section 1145 of the Bankruptcy Code also does not constitute “restricted securities” as defined in Rule 144(a)(3) under the Securities Act, and, subject to the terms of the New Registration Rights Agreement and the Amended Memorandum of Association, is freely tradable and transferable by any holder thereof that: (a) is not an “affiliate” of the Reorganized Debtors as defined in Rule 144(a)(1) under the Securities Act; (b) has not been such an “affiliate” within 90 days of such transfer; and (c) has not acquired the New Common Stock from an “affiliate” within one year of such transfer. For the avoidance of doubt, while the Rights Offering shall be conducted in reliance upon the exemption from registration

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under the Securities Act provided in section 1145 of the Bankruptcy Code, the Debtors are not seeking such an exemption for the New Common Stock issued pursuant to the Plan Investor Equity Raise or with respect to any shares purchased by existing shareholders of the Plan Investor that are not Eligible Holders.

8.15.       Setoffs and Recoupments.

Except as expressly provided in this Plan, each Reorganized Debtor may, pursuant to section 553 of the Bankruptcy Code, set off and/or recoup against any Plan Distributions to be made on account of any Allowed Claim, any and all claims, rights and Causes of Action that such Reorganized Debtor may hold against the holder of such Allowed Claim to the extent such setoff or recoupment is either (a) agreed in amount among the relevant Reorganized Debtor(s) and holder of such Allowed Claim, or (b) otherwise adjudicated by the Bankruptcy Court or another court of competent jurisdiction; provided, however, that neither the failure to effectuate a setoff or recoupment nor the allowance of any Claim hereunder shall constitute a waiver or release by a Reorganized Debtor or its successor of any and all claims, rights and Causes of Action that such Reorganized Debtor or its successor may possess against the applicable holder.

8.16.       Withholding and Reporting Requirements.

In connection with this Plan and all Plan Distributions hereunder, the Reorganized Debtors shall comply with all withholding and reporting requirements imposed by any federal, state, provincial, local or foreign taxing authority, and all Plan Distributions hereunder shall be subject to any such withholding and reporting requirements. The Reorganized Debtors shall be authorized to take any and all action that may be necessary or appropriate to comply with such withholding and reporting requirements, including requiring a holder of a Claim to submit appropriate tax and withholding certifications. Notwithstanding any other provision of this Plan: (a) each holder of an Allowed Claim that is to receive a Plan Distribution under this Plan shall have sole and exclusive responsibility for the satisfaction and payment of any tax obligations imposed by any governmental unit, including income, withholding and other tax obligations on account of such distribution; and (b) no Plan Distributions shall be required to be made to or on behalf of such holder pursuant to this Plan unless and until such holder has made arrangements satisfactory to the Reorganized Debtors for the payment and satisfaction of such tax obligations or has, to the Reorganized Debtors’ satisfaction, established an exemption therefrom.

8.17.       Hart-Scott Rodino Antitrust Improvements Act.

Any New Common Stock to be distributed under the Plan to an entity required to file a Premerger Notification and Report Form under the Competition Laws shall not be distributed until the notification and waiting period applicable under such Competition Laws to such entity shall have expired or been terminated or any applicable authorizations, approvals, clearances or consents have been obtained.

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ARTICLE IX.

PROCEDURES FOR RESOLVING CLAIMS

9.1.         Claims Process.

Other than with respect to Fee Claims, only the Reorganized Debtors shall be entitled to object to Claims after the Effective Date. Any objections to those Claims (other than Administrative Expense Claims) shall be served and filed on or before the later of: (a) the date that is 180 days after the Effective Date; and (b) such other date as may be fixed by the Bankruptcy Court, whether fixed before or after the date specified in clause (a) hereof. Any Claims filed after the Bar Date or Administrative Bar Date, as applicable, shall be deemed disallowed and expunged in their entirety without further order of the Bankruptcy Court or any action being required on the part of the Debtors or the Reorganized Debtors, unless the Person wishing to file such untimely Claim has received the Bankruptcy Court’s authorization to do so. Notwithstanding any authority to the contrary, an objection to a Claim shall be deemed properly served on the claimant if the objecting party effects service in any of the following manners: (a) in accordance with Federal Rule of Civil Procedure 4, as modified and made applicable by Bankruptcy Rule 7004; (b) by first class mail, postage prepaid, on the signatory on the proof of claim as well as all other representatives identified in the proof of claim or any attachment thereto; or (c) if counsel has agreed to or is otherwise deemed to accept service, by first class mail, postage prepaid, on any counsel that has appeared on the claimant’s behalf in the Chapter 11 Cases (so long as such appearance has not been subsequently withdrawn). From and after the Effective Date, the Reorganized Debtors may settle or compromise any Disputed Claim without approval of the Bankruptcy Court.

9.2.       Amendment to Claims.

From and after the Effective Date, no proof of Claim may be amended to increase or assert additional claims not reflected in a previously timely filed Claim (or Claim scheduled on the applicable Debtor’s Schedules, unless superseded by a filed Claim), and any such Claim shall be deemed disallowed and expunged in its entirety without further order of the Bankruptcy Court or any action being required on the part of the Debtors or the Reorganized Debtors unless the claimant has obtained the Bankruptcy Court’s prior approval to file such amended or increased Claim. Notwithstanding anything to the contrary in this Section, proofs of Claim and amendments of any kind to proofs of Claim may be filed by Governmental Units in accordance with the deadlines set by the Order Establishing Deadline for Filing Proofs of Claim and Approving the Form and Manner of Notice Thereof [Docket No. 51].

9.3.         Disputed Claims.

Disputed Claims shall not be entitled to any Plan Distributions unless and until they become Allowed Claims.

9.4.         Estimation of Claims.

The Debtors and/or Reorganized Debtors may request that the Bankruptcy Court enter an Estimation Order with respect to any Claim, pursuant to section 502(c) of the

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Bankruptcy Code, for purposes of determining the Allowed amount of such Claim regardless of whether any Person has previously objected to such Claim or whether the Bankruptcy Court has ruled on any such objection, and the Bankruptcy Court shall retain jurisdiction to estimate any Claim at any time (including during the pendency of any appeal with respect to the allowance or disallowance of such Claims). In the event that the Bankruptcy Court estimates any contingent or unliquidated Claim for allowance or distribution purposes, that estimated amount will constitute either the Allowed amount of such Claim or a maximum limitation on such Claim, as determined by the Bankruptcy Court. If the estimated amount constitutes a maximum limitation on such Claim, the objecting party may elect to pursue any supplemental proceedings to object to any ultimate allowance of such Claim. All of the objection, estimation, settlement, and resolution procedures set forth in the Plan are cumulative and not exclusive of one another. Claims may be estimated and subsequently compromised, settled, resolved or withdrawn by any mechanism approved by the Bankruptcy Court.

ARTICLE X.

EXECUTORY CONTRACTS AND UNEXPIRED LEASES

10.1.       General Treatment.

As of and subject to the occurrence of the Effective Date and the payment of any applicable Cure Amount, all executory contracts and unexpired leases of the Debtors shall be deemed assumed, except that: (a) any executory contracts and unexpired leases that previously have been assumed or rejected pursuant to a Final Order of the Bankruptcy Court shall be treated as provided in such Final Order; (b) any executory contracts and unexpired leases listed on the Schedule of Rejected Contracts and Leases shall be deemed rejected as of the Effective Date; and (c) all executory contracts and unexpired leases that are the subject of a separate motion to assume or reject under section 365 of the Bankruptcy Code pending on the Effective Date shall be treated as provided for in the Final Order resolving such motion. Subject to the occurrence of the Effective Date, entry of the Confirmation Order by the Bankruptcy Court shall constitute approval of the assumptions and rejections described in this Section 10.1 pursuant to sections 365(a) and 1123 of the Bankruptcy Code. Each executory contract and unexpired lease assumed pursuant to this Section 10.1 shall revest in and be fully enforceable by the applicable Reorganized Debtor in accordance with its terms, except as modified by the provisions of the Plan, or any order of the Bankruptcy Court authorizing and providing for its assumption, or applicable federal law. Without determining whether any of the Government Settlement Agreements is an executory contract subject to section 365 of the Bankruptcy Code, the Government Settlement Agreements shall be deemed assumed by the Debtors, and binding upon the Reorganized Debtors and the applicable parties thereto as of and following the Effective Date (provided that the foregoing shall not constitute a determination whether such agreements are executory contracts subject to section 365 of the Bankruptcy Code). Nothing in the foregoing paragraph affects or limits the provisions of Section 12.6(d)-(e) of the Plan.

10.2.       Claims Based on Rejection of Executory Contracts or Unexpired Leases.

Except as otherwise explicitly set forth in the Plan, all Claims arising from the rejection of executory contracts or unexpired leases, if evidenced by a timely filed proof of

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claim, will be treated as Other General Unsecured Claims. Upon receipt of the Plan Distribution provided in Section 5.7 of the Plan, all such Claims shall be discharged, and shall not be enforceable against the Debtors, the Estates, the Reorganized Debtors or their respective properties or interests in property. In the event that the rejection of an executory contract or unexpired lease by any of the Debtors pursuant to the Plan results in damages to the other party or parties to such contract or lease, a Claim for such damages, if not evidenced by a timely filed proof of claim, shall be forever barred and shall not be enforceable against the Debtors or the Reorganized Debtors, or their respective properties or interests in property as agents, successors or assigns, unless a proof of claim is filed with the Bankruptcy Court and served upon counsel for the Debtors and the Reorganized Debtors on or before the date that is thirty (30) days after the effective date of such rejection (which may be the Effective Date, the date on which the Debtors reject the applicable contract or lease as provided in Section 10.3(c) below, or pursuant to an order of the Bankruptcy Court).

10.3.       Cure of Defaults for Assumed Executory Contracts and Unexpired Leases.

(a)       Except to the extent that less favorable treatment has been agreed to by the non-Debtor party or parties to each such executory contract or unexpired lease to be assumed pursuant to the Plan, any monetary defaults arising under such executory contract or unexpired lease shall be satisfied, pursuant to section 365(b)(1) of the Bankruptcy Code, by payment of the appropriate amount (the “Cure Amount”) in full in Cash on the later of thirty (30) days after: (i) the Effective Date; or (ii) the date on which any Cure Dispute relating to such Cure Amount has been resolved (either consensually or through judicial decision).

(b)       No later than ten (10) calendar days prior to the commencement of the Confirmation Hearing, the Debtors, in consultation with the Plan Investor, shall file a schedule (the “Cure Schedule”) setting forth the Cure Amount, if any, for each executory contract and unexpired lease to be assumed pursuant to Section 10.1 of the Plan, and serve such Cure Schedule on each applicable counterparty. Any party that fails to object to the applicable Cure Amount listed on the Cure Schedule within ten (10) calendar days of the filing thereof shall be forever barred, estopped and enjoined from disputing the Cure Amount set forth on the Cure Schedule (including a Cure Amount of $0.00) and/or from asserting any Claim against the applicable Debtor or Reorganized Debtor arising under section 365(b)(1) of the Bankruptcy Code except as set forth on the Cure Schedule.

(c)       In the event of a dispute (each, a “Cure Dispute”) regarding: (i) the Cure Amount; (ii) the ability of the applicable Reorganized Debtor to provide “adequate assurance of future performance” (within the meaning of section 365 of the Bankruptcy Code) under the contract or lease to be assumed; or (iii) any other matter pertaining to the proposed assumption, the cure payments required by section 365(b)(1) of the Bankruptcy Code shall be made following the entry of a Final Order resolving such Cure Dispute and approving the assumption. To the extent a Cure Dispute relates solely to the Cure Amount, the applicable Debtor may assume and/or assume and assign the applicable contract or lease prior to the resolution of the Cure Dispute provided that such Debtor reserves Cash in an amount sufficient to pay the full amount asserted as the required cure payment by the non-Debtor party to such contract or lease (or such smaller amount as may be fixed or estimated by the Bankruptcy Court). To the extent the Cure Dispute is resolved or determined against the applicable Debtor or Reorganized Debtor, as

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applicable, such Debtor or Reorganized Debtor, as applicable, may reject the applicable executory contract or unexpired lease after such determination, and the counterparty may thereafter file a proof of claim in the manner set forth in Section 10.2 hereof.

10.4.       Effect of Confirmation Order on Assumption, Assumption and Assignment, and Rejection.

Subject to the occurrence of the Effective Date, entry of the Confirmation Order by the Bankruptcy Court shall constitute entry of an order by the Bankruptcy Court pursuant to sections 365(a) and 1123(b) of the Bankruptcy Code approving the assumptions, assumptions and assignments and rejections described in this Article X and determining that: (a) with respect to such rejections, such rejected executory contracts and unexpired leases are burdensome and that the rejection therein is in the best interests of the Estates; (b) with respect to such assumptions, to the extent necessary, that the applicable Reorganized Debtor has (i) cured, or provided adequate assurance that the applicable Reorganized Debtor will promptly cure, any default in accordance with section 365(b)(1)(A) of the Bankruptcy Code, (ii) compensated or provided adequate assurance that it or its affiliate will promptly compensate the counterparty for any actual pecuniary loss to such party resulting from such default, and (iii) provided adequate assurance of future performance under such executory contract or unexpired lease; and (c) with respect to any assignment, to the extent necessary, that the applicable Reorganized Debtor or the proposed assignee has (i) cured, or provided adequate assurance that it or its affiliate will promptly cure, any default in accordance with section 365(b)(1)(A) of the Bankruptcy Code, (ii) compensated or provided adequate assurance that the applicable Reorganized Debtor or the proposed assignee will promptly compensate the counterparty for any actual pecuniary loss to such party resulting from such default, and (iii) that “adequate assurance of future performance” (within the meaning of section 365 of the Bankruptcy Code) by the assignee has been demonstrated and no further adequate assurance is required. Assumption of any executory contract or unexpired lease and satisfaction of the Cure Amounts shall result in the full discharge, release and satisfaction of any claims or defaults, whether monetary or nonmonetary, including defaults of provisions restricting the change in control or ownership interest composition or other bankruptcy-related defaults, arising under any assumed executory contract or unexpired lease at any time before the date such executory contract or unexpired lease is assumed. Each executory contract and unexpired lease assumed pursuant to this Article X shall revest in and be fully enforceable by the applicable Debtor in accordance with its terms, except as modified by the provisions of the Plan, or any order of the Bankruptcy Court authorizing and providing for its assumption, or applicable federal law. To the maximum extent permitted by law, to the extent any provision in any executory contract or unexpired lease assumed pursuant to the Plan restricts or prevents, or purports to restrict or prevent, or is breached or deemed breached by, the assumption of such executory contract or unexpired lease (including any “change of control” provision), then such provision shall be deemed modified such that the transactions contemplated by the Plan shall not entitle the non-Debtor party thereto to terminate such executory contract or unexpired lease or to exercise any other default-related rights with respect thereto. Any party that fails to timely file a Cure Dispute on the basis that consent to assume or assume and assign the applicable executory contract is a condition to such assumption or assumption and assignment, shall be deemed to have consented to the assumption or assumption and assignment, as applicable, of such contract.

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10.5.       Modifications, Amendments, Supplements, Restatements, or Other Agreements.

Unless otherwise provided in the Plan, each assumed or assumed and assigned executory contract and unexpired lease shall include all modifications, amendments, supplements, restatements or other agreements that in any manner affect such executory contract or unexpired lease, and all executory contracts and unexpired leases related thereto, if any, including all easements, licenses, permits, rights, privileges, immunities, options, rights of first refusal and any other interests, unless any of the foregoing agreements has been previously rejected or is rejected under the Plan or otherwise.

Modifications, amendments, supplements and restatements to prepetition executory contracts and unexpired leases that have been executed by the Debtors during the Chapter 11 Cases shall not be deemed to alter the prepetition nature of the executory contract or unexpired lease, or the validity, priority or amount of any Claims that may arise in connection therewith.

10.6.       Compensation and Benefit Programs.

Subject to the paragraph immediately following this paragraph, and except as otherwise expressly provided in this Plan, the Plan Funding Agreement, in a prior order of the Bankruptcy Court or to the extent subject to a motion pending before the Bankruptcy Court as of the Effective Date, all employment and severance policies, and all compensation and benefit plans, policies, and programs of the Debtors applicable to their respective employees, retirees and non-employee directors including all savings plans, unfunded retirement plans, healthcare plans, disability plans, severance benefit plans, incentive plans, and life, accidental death and dismemberment insurance plans, and paid time off policies, in each case, as existing on the Petition Date, are treated as executory contracts under the Plan and, on the Effective Date, will be assumed pursuant to the provisions of sections 365 and 1123 of the Bankruptcy Code except for Persons not employees of the Debtors as of the Petition Date.

Each of the Debtors may, prior to the Effective Date and subject to the parties’ rights under the RSA and the Plan Funding Agreement, enter into employment agreements with employees that become effective on or prior to the Effective Date and survive consummation of this Plan. Any such agreements (or a summary of the material terms thereof) shall be in form and substance Acceptable to the Plan Investor and be included in the Plan Supplement or otherwise filed with the Bankruptcy Court on or before the date of the Confirmation Hearing.

On the Effective Date, the Debtors or the Reorganized Debtors, as applicable, shall pay any amounts outstanding under the Debtors’ key executive incentive program and key employee retention plan authorized to be paid as of that date pursuant to an order of the Bankruptcy Court. For the avoidance of doubt, and notwithstanding anything herein to the contrary, any payments of amounts outstanding under the Debtors’ key executive incentive program and key employee retention plan authorized to be paid as of the Effective Date pursuant to an order of the Bankruptcy Court or otherwise, including, without limitation, any and all amounts that are outstanding or will become outstanding as a result of any “change of control” or similar transaction, shall be paid from Plan Cash.

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ARTICLE XI.

CONDITIONS PRECEDENT TO
CONSUMMATION OF THE PLAN

11.1.       Conditions Precedent to the Effective Date.

The occurrence of the Effective Date is subject to:

(a)       the RSA not having been terminated and remaining in full force and effect and the PFA Order having become a Final Order and remaining in full force and effect; provided that a termination as to a breaching Consenting Lender, where the termination occurs only as to such Consenting Lender and the RSA remains in full force and effect with respect to the other parties, shall not mean the RSA has been terminated or is not in full force and effect for purposes of this paragraph;

(b)      the Plan Funding Agreement not having been terminated and remaining in full force and effect and the transactions contemplated thereunder having been substantially consummated as of the Effective Date;

(c)       the Rights Offering having been consummated and the Backstop Commitment Agreement not having been terminated and remaining in full force and effect;

(d)       the Disclosure Statement Order, in form and substance Acceptable to the Debtors and each of the Required Parties, having been entered by the Bankruptcy Court and remaining in full force and effect;

(e)       the Confirmation Order, in form and substance Acceptable to the Debtors, each of the Required Parties and the Committee, having become a Final Order and remaining in full force and effect;

(f)       all fees and expenses then due and payable or owed by the Debtors under the Plan Funding Agreement, the PFA Order, the RSA and the Backstop Commitment Agreement having been paid;

(g)       the Convertible Notes Trustee Fees shall have been paid in full in Cash;

(h)      any non-technical and/or immaterial amendments, modifications or supplements to the Plan being Acceptable to the Debtors, the Committee, and each of the Required Parties, except as otherwise provided in Section 14.5 of this Plan; and

(k)       all actions and all agreements, instruments or other documents necessary to implement the terms and provisions of this Plan, including, without limitation, the Plan Funding Agreement and the other documents included in the Plan Supplement, in form and substance Acceptable to the Debtors, the Committee, and each of the Required Parties as set forth in the RSA, the Plan Funding Agreement, and herein, to be entered into by the applicable Debtors being executed and delivered, and any conditions (other than the occurrence of the

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Effective Date or certification by a Debtor that the Effective Date has occurred) contained therein having been satisfied or waived in accordance therewith.

11.2.       Satisfaction and Waiver of Conditions Precedent.

Except as otherwise provided herein, any actions taken on the Effective Date shall be deemed to have occurred simultaneously and no such action shall be deemed to have occurred prior to the taking of any other such action. Any of the conditions set forth in Sections 11.1 of this Plan may be waived in whole or part upon agreement by the Debtors, each of the Required Parties and the Committee and as the case may be, without notice and a hearing, and the Debtors’ benefits under any “mootness” doctrine, but only to the extent applicable, shall be unaffected by any provision hereof. The failure to assert the non-satisfaction of any such conditions shall not be deemed a waiver of any other rights hereunder, and each such right shall be deemed an ongoing right that may be asserted or waived (as set forth herein) at any time or from time to time.

11.3.       Effect of Failure of Conditions.

If all of the conditions to effectiveness have not been satisfied (as provided in Section 11.1 hereof) or duly waived (as provided in Section 11.2 hereof) and the Effective Date has not occurred on or before the Outside Date (as defined in the Plan Funding Agreement) or, subject to the parties’ rights under the RSA and the Plan Funding Agreement, by such later date as set forth by the Debtors in a notice filed with the Bankruptcy Court prior to the expiration of such period, then the Debtors, the Required Parties or the Committee may file a motion to vacate the Confirmation Order. Notwithstanding the filing of such a motion, the Confirmation Order shall not be vacated if all of the conditions to consummation set forth in Section 11.1 hereof are either satisfied or duly waived by the Debtors, the Required Parties and the Committee before the Bankruptcy Court enters an order granting the relief requested in such motion. If the Confirmation Order is vacated pursuant to this Section 11.3, this Plan shall be null and void in all respects, the Confirmation Order shall be of no further force or effect, no Plan Distributions shall be made, the Debtors, the Plan Investor, and all holders of Claims and Interests shall be restored to the status quo ante as of the day immediately preceding the Confirmation Date as though the Confirmation Date had never occurred (except that the Plan Investor, or any of its designees, shall retain its rights to the extent provided under the Transaction Documents), and upon such occurrence, nothing contained in this Plan shall: (a) constitute a waiver or release of any Claims against or Interests in the Debtors; (b) prejudice in any manner the rights of the Plan Investor or the holder of any Claim against or Interest in the Debtors; or (c) constitute an admission, acknowledgment, offer or undertaking by any Debtor or any other Person with respect to any matter set forth in the Plan.

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ARTICLE XII.

EFFECT OF CONFIRMATION

12.1.       Binding Effect.

Except as otherwise provided in section 1141(d)(3) of the Bankruptcy Code and subject to the occurrence of the Effective Date, on and after the Confirmation Date, the provisions of this Plan shall bind any holder of a Claim against, or Interest in, the Debtors and inure to the benefit of and be binding on such holder’s respective successors and assigns, whether or not the Claim or Interest of such holder is impaired under this Plan and whether or not such holder has accepted this Plan.

12.2.       Discharge of Claims Against and Interests in the Debtors.

Upon the Effective Date and in consideration of the Plan Distributions, if any, except as otherwise provided herein (including in Section 5.10) or in the Confirmation Order, each Person that is a holder (as well as any trustees and agents for or on behalf of such Person) of a Claim or Interest shall be deemed to have forever waived, released, and discharged the Debtors, to the fullest extent permitted by section 1141 of the Bankruptcy Code, of and from any and all Claims, Interests, rights and liabilities that arose prior to the Effective Date. Except as otherwise provided herein (including in Section 5.10), upon the Effective Date, all such holders of Claims and Interests shall be forever precluded and enjoined, pursuant to sections 105, 524 and 1141 of the Bankruptcy Code, from prosecuting or asserting any such discharged Claim against or terminated Interest in any Debtor, any Reorganized Debtor. For the avoidance of doubt, ancillary security enforcement, insolvency processes and/or other proceedings may be deployed in any relevant jurisdictions to implement the transactions set out in this Plan, including this Plan’s discharge provisions, in order to ensure that they are fully effective.

12.3.       Term of Pre-Confirmation Injunctions or Stays.

Unless otherwise provided herein, all injunctions or stays provided in the Chapter 11 Cases arising prior to the Confirmation Date in accordance with sections 105 or 362 of the Bankruptcy Code, or otherwise, and in existence on the Confirmation Date, shall remain in full force and effect until the Effective Date.

12.4.       Injunction Against Interference with the Plan.

Upon the entry of the Confirmation Order, all holders of Claims and Interests and other Persons, along with their respective present or former affiliates, employees, agents, officers, directors, or principals, shall be enjoined from taking any actions, whether in the United States or elsewhere, to interfere with the implementation or consummation of this Plan. Moreover, solely to the extent provided in this Plan or under applicable law, the property dealt with by this Plan is transferred to, or vests in (or both, as applicable) the Reorganized Debtors free and clear of all Claims and Interests pursuant to section 1141(c) of the Bankruptcy Code. As such, to the fullest extent permissible under applicable law, no Person holding a Claim or Interest may receive any payment from, or seek recourse against, any assets that are to be distributed under this Plan other than assets required to be distributed to that Person under this

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Plan. As of the Confirmation Date, subject to the occurrence of the Effective Date, to the fullest extent permissible under applicable law, all Persons are precluded and barred from asserting against any property to be distributed under this Plan any Claims, rights, Causes of Action, liabilities, Interests, or other action or remedy based on any act, omission, transaction, or other activity that occurred before the Confirmation Date except as expressly provided in this Plan or the Confirmation Order. Each of the Reorganized Debtors, as applicable, is expressly authorized hereby to seek to enforce such injunction.

12.5.       Injunction.

Except as otherwise provided in this Plan, including Sections 5.10 and 12.8, or the Confirmation Order, as of the Confirmation Date, but subject to the occurrence of the Effective Date, all Persons who have held, hold or may hold Claims against or Interests in the Debtors or the Estates are, with respect to any such Claims or Interests, permanently enjoined after the Confirmation Date from: (i) commencing, conducting or continuing in any manner, directly or indirectly, any suit, action or other proceeding of any kind (including any proceeding in a judicial, arbitral, administrative or other forum) against the Reorganized Debtors, the Estates or any of their property, wherever located, or any direct or indirect transferee of any property, wherever located, of, or direct or indirect successor in interest to, any of the foregoing Persons or any property, wherever located, of any such transferee or successor, on account of or in connection with or with respect to any released, settled, compromised, or exculpated Claims, Interests or Causes of Action arising against the Debtors and/or their Estates; (ii) enforcing, levying, attaching (including any pre-judgment attachment), collecting or otherwise recovering by any manner or means, whether directly or indirectly, any judgment, award, decree or order against the Reorganized Debtors, the Estates or any of their property, wherever located, or any direct or indirect transferee of any property, wherever located, of, or direct or indirect successor in interest to, any of the foregoing Persons, or any property, wherever located, of any such transferee or successor, on account of or in connection with or with respect to any released, settled, compromised, or exculpated Claims, Interests or Causes of Action arising against the Debtors and/or their Estates; (iii) creating, perfecting or otherwise enforcing in any manner, directly or indirectly, any encumbrance of any kind against the Reorganized Debtors, or the Estates or any of their property, wherever located, or any direct or indirect transferee of any property, wherever located, of, or successor in interest to, any of the foregoing Persons, on account of or in connection with or with respect to any released, settled, compromised, or exculpated Claims, Interests or Causes of Action arising against the Debtors and/or their Estates; (iv) acting or proceeding in any manner, in any place whatsoever, that does not conform to or comply with the provisions of this Plan to the full extent permitted by applicable law; and (v) commencing or continuing, in any manner or in any place, any action that does not comply with or is inconsistent with the provisions of this Plan; provided, however, that nothing contained herein shall preclude such Persons from exercising their rights, or obtaining benefits, pursuant to and consistent with the terms of this Plan. For the avoidance of doubt, ancillary security enforcement, insolvency processes and/or other proceedings may be deployed in any relevant jurisdictions to implement the transactions set out in this Plan, including the injunctions set forth in this Section 12.5, in order to ensure that they are fully effective. Each of the Reorganized Debtors, as applicable, is expressly authorized hereby to seek to enforce such injunction.

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12.6.       Releases.

(a)       Releases by the Debtors. Except as otherwise provided in the Plan or the Confirmation Order, as of the Effective Date, the Debtors, as, debtors in possession, and any person seeking to exercise the rights of the Debtors’ Estates, including without limitation, any successor to the Debtors or any representative of the Debtors’ Estates appointed or selected pursuant to sections 1103, 1104 or 1123(b)(3) of the Bankruptcy Code or under chapter 7 of the Bankruptcy Code, shall be deemed to forever release, waive and discharge all claims (as such term “claim” is defined in section 101(5) of the Bankruptcy Code), obligations, suits, judgments, damages, demands, debts, rights, causes of action (including, but not limited to, the Causes of Action) and liabilities (other than the rights of the Debtors to enforce the Plan and the contracts, instruments, releases and other agreements or documents delivered thereunder) against any Released Party, whether liquidated or unliquidated, fixed or contingent, matured or unmatured, known or unknown, foreseen or unforeseen, existing or hereafter arising, in law, equity or otherwise that are based in whole or in part on any act, omission, transaction, event or other occurrence taking place on or prior to the Effective Date in any way relating to the Debtors, the Reorganized Debtors, the purchase, sale or rescission of the purchase or sale of any security of the Debtors, the subject matter of, or the transactions or events giving rise to, any Claim or Interest that is treated in the Plan, the parties released pursuant to this Section 12.6, the Chapter 11 Cases, the RSA, the DIP Financing Agreement, the Plan Funding Agreement, or this Plan or the Disclosure Statement, and that could have been asserted by or on behalf of the Debtors or their Estates, whether directly, indirectly, derivatively or in any representative or any other capacity; provided, however, that in no event shall anything in this Section 12.6(a) be construed as a release of any Person’s gross negligence, fraud, or willful misconduct, each as determined by a Final Order, for matters with respect to the Debtors and/or their affiliates. Entry of the Confirmation Order shall constitute the Bankruptcy Court’s approval, pursuant to Bankruptcy Rule 9019, of the releases herein, which includes by reference each of the related provisions and definitions contained herein, and further, shall constitute the Bankruptcy Court’s finding that the releases herein are: (1) in exchange for the good and valuable consideration provided by the Released Parties; (2) a good faith settlement and compromise of the claims released by the releases herein; (3) in the best interests of the Debtors and all holders of Claims and Interests; (4) fair, equitable and reasonable; (5) given and made after reasonable investigation by the Debtors and after notice and opportunity for hearing; and (6) a bar to any of the Debtors asserting any claim released by the releases herein against any of the Released Parties.

(b)       Third Party Releases. Except as otherwise provided in the Plan, the Plan Funding Agreement or the Confirmation Order, on the Effective Date each Releasing Party, in consideration for the obligations of the Debtors under the Plan, the distributions under the Plan and other contracts, instruments, releases, agreements or documents executed and delivered in connection with the Plan, will be deemed to have consented to the Plan and the restructuring embodied herein for all purposes and deemed to forever release, waive and discharge all claims (as such term is defined in section 101(5) of the Bankruptcy Code), including but not limited to any claim sounding in law or equity or asserting a tort, breach of any duty or contract, violations of the common law, any federal or state statute, any federal or state securities laws or otherwise, demands, debts, rights, causes of action (including without limitation, the Causes of Action) or liabilities (other than the right to enforce the obligations of any party under the Plan and the contracts, instruments, releases, agreements and documents delivered under or in connection

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with the Plan), including, without limitation, any claims for any such loss such holder may suffer, have suffered or be alleged to suffer as a result of the Debtors commencing the Chapter 11 Cases or as a result of the Plan being consummated, against any Released Party, whether liquidated or unliquidated, fixed or contingent, matured or unmatured, known or unknown, foreseen or unforeseen, existing or hereafter arising, in law, equity or otherwise that are based in whole or in part on any act or omission, transaction, event or other occurrence taking place on or prior to the Effective Date in any way relating to the Debtors, the Reorganized Debtors, the Chapter 11 Cases, the Plan or the Disclosure Statement; provided, however, that in no event shall anything in this Section 12.6(b) be construed as a release of any Person’s gross negligence, fraud, or willful misconduct, each as determined by a Final Order, for matters with respect to the Debtors and/or their affiliates. Entry of the Confirmation Order shall constitute the Bankruptcy Court’s approval, pursuant to Bankruptcy Rule 9019, of the releases of holders of Claims and Interests, which includes by reference each of the related provisions and definitions contained herein, and further, shall constitute the Bankruptcy Court’s finding that the releases herein are: (1) in exchange for the good and valuable consideration provided by the Released Parties; (2) a good faith settlement and compromise of the claims herein; (3) in the best interests of the Debtors and all holders of Claims and Interests; (4) fair, equitable and reasonable; (5) given and made after notice and opportunity for hearing; and (6) a bar to any holder of a Claim or Interest asserting any Claim released by the releases herein against any of the Released Parties.

(c)       Notwithstanding anything to the contrary contained herein: (i) except to the extent permissible under applicable law, as such law may be extended or interpreted subsequent to the Effective Date, the releases provided for in this Section 12.6 of the Plan shall not release any non-Debtor entity from any liability arising under (a) the Internal Revenue Code or any state, city or municipal tax code, (b) any criminal laws of the United States or any state, city or municipality, or (c) any environmental laws of the United States or any state, city or municipal tax code; and (ii) the releases set forth in this Section 12.6 shall not release any (a) claims, right, or Causes of Action for money borrowed from or owed to the Debtors by any of their directors, officers or former employees, as set forth in the Debtors’ books and records, (b) any claims against any Person to the extent such Person asserts a crossclaim, counterclaim and/or claim for setoff which seeks affirmative relief against a Debtor or any of its officers, directors, or representatives, (c) claims against any Person arising from or relating to such Person’s gross negligence, fraud, or willful misconduct, each as determined by a Final Order of the Bankruptcy Court, and (d) any Unimpaired Claims unless and until holders of Unimpaired Claims have received payment on account of such Claims that render such claims Unimpaired in accordance with the Plan.

(d)       Notwithstanding any language to the contrary contained in this Plan, the Disclosure Statement, and/or the Confirmation Order, no provision of the Plan or the Confirmation Order shall (i) preclude the United States Securities and Exchange Commission (the “SEC”) from enforcing its police or regulatory powers; or (ii) enjoin, limit, impair, or delay the SEC from commencing or continuing any claims, causes of action, proceedings or investigations against any non-Debtor person or non-Debtor entity in any forum.

(e)       As to any Governmental Unit (as defined in section 101(27) of the Bankruptcy Code), nothing in the Plan, Plan Documents, or Confirmation Order shall limit or expand the scope of discharge, release or injunction to which the Debtors or Reorganized

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Debtors are entitled under the Bankruptcy Code, if any. The discharge, release, and injunction provisions contained in the Plan, Plan Documents, or Confirmation Order are not intended and shall not be construed to bar any Governmental Unit from, subsequent to the Confirmation Order, pursuing any police or regulatory action.

Accordingly, notwithstanding anything contained in the Plan, Plan Documents, or Confirmation Order to the contrary, nothing in the Plan, Plan Documents, or Confirmation Order shall discharge, release, impair or otherwise preclude: (1) any liability to any Governmental Unit that is not a “claim” within the meaning of section 101(5) of the Bankruptcy Code; (2) any Claim of any Governmental Unit arising on or after the Confirmation Date; (3) any valid right of setoff or recoupment of any Governmental Unit against any of the Debtors; or (4) any liability of the Debtors or Reorganized Debtors under police or regulatory statutes or regulations to any Governmental Unit as the owner, lessor, lessee or operator of property that such entity owns, operates or leases after the Confirmation Date. Nor shall anything in the Plan, Plan Documents, or Confirmation Order: (i) enjoin or otherwise bar any Governmental Unit from asserting or enforcing, outside the Bankruptcy Court, any liability described in the preceding sentence; or (ii) divest any court, commission, or tribunal of jurisdiction to determine whether any liabilities asserted by any Governmental Unit are discharged or otherwise barred by the Plan, Plan Documents, Confirmation Order, or the Bankruptcy Code.

Moreover, nothing in the Plan, Plan Documents, or Confirmation Order shall release or exculpate any non-debtor, including any Released Parties and/or exculpated parties, from any liability to any Governmental Unit, including but not limited to any liabilities arising under the Internal Revenue Code, the environmental laws, or the criminal laws against the Released Parties and/or exculpated parties, nor shall anything in the Plan, Plan Documents, or Confirmation Order enjoin any Governmental Unit from bringing any claim, suit, action or other proceeding against any non-Debtor for any liability whatsoever; provided, however, that the foregoing sentence shall not limit the scope of discharge granted to the Debtors under sections 524 and 1141 of the Bankruptcy Code.

Nothing contained in the Plan, Plan Documents, or Confirmation Order shall be deemed to determine the tax liability of any person or entity, including but not limited to the Debtors and the Reorganized Debtors, nor shall the Plan, Plan Documents, or Confirmation Order be deemed to have determined the federal and/or state tax treatment of any item, distribution, or entity, including the federal and/or state tax consequences of the Plan and/or Plan Documents, nor shall anything in the Plan, Plan Documents, or Confirmation Order be deemed to have conferred jurisdiction upon the Bankruptcy Court to make determinations as to federal and/or state tax liability and federal and/or state tax treatment except as provided under 11 U.S.C. § 505.

Article X of the Plan regarding Executory Contracts and Unexpired Leases, and Section 7.8 of the Plan regarding Cancellation of Existing Securities and Agreements, shall not apply to the Government Settlement Agreements. The Government Settlement Agreements shall be unimpaired by the Plan, Plan Documents, and Confirmation Order, and shall remain obligations of the Debtors and the Reorganized Debtors, and all rights, obligations, and duties under the Government Settlement Agreements shall be preserved as if the Debtors’ bankruptcy cases were never filed. All Governmental Units reserve all rights with respect to the Government

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Settlement Agreements, and nothing contained in the Plan, Plan Documents, or Confirmation Order shall discharge, release, impair, or otherwise preclude any liability to any Governmental Unit arising from or relating to the Government Settlement Agreements. Any amounts owed to Governmental Units under the Government Settlement Agreements shall be paid in full when due in the ordinary course and nothing in the Plan, Plan Documents, or Confirmation Order shall be interpreted to set cure amounts, authorize the assignment or rejection of any Government Settlement Agreement, or require any Governmental Unit to approve of and consent to the assignment of any Government Settlement Agreement. The Debtors and Reorganized Debtors expressly agree that any provisions regarding default in the Government Settlement Agreements shall continue to apply as set forth in those agreements, irrespective of any provisions of the Plan, Plan Documents, and Confirmation Order. For the avoidance of doubt, nothing contained in the Plan, Plan Documents, or Confirmation Order shall divest any court, commission, or tribunal of jurisdiction over any matters related to the Government Settlement Agreements, or confer on the Bankruptcy Court jurisdiction over any matter related to the Government Settlement Agreements.

Notwithstanding the foregoing, the applicable federal government parties to the federal Government Settlement Agreements have agreed not to exercise rights under the federal Government Settlement Agreements to accelerate or increase monetary obligations under those agreements based solely on the fact of the commencement of the Chapter 11 Cases or the fact of the consummation of the transactions contemplated by this Plan, the Plan Funding Agreement and/or the other Transaction Documents, including the occurrence of any Fundamental Transaction (as defined in the Government Settlement Agreements), by virtue of the consummation of any such transactions or the failure of the New Common Stock of the Plan Investor to be listed on the NASDAQ or other US stock exchange, provided that all of the Debtors’ and Reorganized Debtors’ payment and other obligations under the federal Government Settlement Agreements continue to be fulfilled in accordance with the terms of those agreements during the Chapter 11 Cases and after the Reorganized Debtors’ emergence from the Chapter 11 Cases.

With respect to the non-federal government parties to the State Government Settlement Agreements listed at Schedule 1.81(b)-(cc), (jj) to the Plan, the Debtors and Reorganized Debtors expressly agree that any provisions regarding default and acceleration in the State Government Settlement Agreements (including, but not limited to, the “Relators’ Letter Agreement”) as written and agreed to on or about February 14, 2018, as the result of a global settlement in United States et al., ex rel. Clarke et al., v. Aegerion Pharmaceuticals, Inc., et al., Civil Action No. 13-11785, as filed in the United States District Court for the District of Massachusetts, shall continue to apply as set forth in those agreements, notwithstanding any provisions of the Plan, Plan Documents, and Confirmation Order. Nothing in the Plan, Plan Documents, and Confirmation Order releases, nullifies, precludes, modifies, or enjoins the enforcement of the terms of the State Governmental Settlement Agreements by and between the Debtors and any parties thereto. For the sake of clarity, nothing in the Plan, Plan Documents, and Confirmation Order shall divest any court, commission, or tribunal of jurisdiction over any matters related to the State Government Settlement Agreements or confer on the Bankruptcy Court jurisdiction over any matter related to the State Government Settlement Agreements. Notwithstanding the foregoing, the monetary obligations under the State Government Settlement Agreements shall not be accelerated or increased as a result of the commencement of the Chapter

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11 Cases or the consummation of the Plan, the Plan Funding Agreement, the Rights Offering, the New Term Loan Facility, and the New Convertible Notes, including the occurrence of any Fundamental Transaction (as defined in the State Government Settlement Agreements), by virtue of the consummation of any such transactions or the failure of the New Common Stock of the Plan Investor to be listed on the NASDAQ or other US stock exchange; provided that such transactions are consummated by October 31, 2019, as such date may be extended with the consent of the Debtors, the Required Parties and the applicable government parties to the State Government Settlement Agreements; provided further that, following the effective date of the Plan, all terms of the State Government Settlement Agreements will be in full force and effect, including, but not limited to any right to require accelerated or increased payments upon the occurrence of any Fundamental Transaction occurring after the Effective Date.

Nothing in the foregoing paragraphs affects or limits the provisions of Section 12.6(d)-(e) of the Plan.

12.7.       Exculpation and Limitation of Liability.

To the extent permissible under section 1125(e) of the Bankruptcy Code, on the Effective Date, for good and valuable consideration, to the maximum extent permissible under applicable law, including the New York Rules of Professional Conduct, none of the Released Parties shall have or incur any liability to any holder of any Claim or Interest or any other Person for any act or omission in connection with, or arising out of the negotiation, implementation and execution of this Plan, the Chapter 11 Cases, the RSA, the Plan Funding Agreement, the Disclosure Statement, the DIP Financing Agreement, the solicitation of votes for and the pursuit of confirmation of this Plan, the consummation of this Plan, or the administration of this Plan or the property to be distributed under this Plan, including all documents ancillary thereto, all decisions, actions, inactions and alleged negligence or misconduct relating thereto and all activities leading to the promulgation and confirmation of this Plan except for gross negligence or willful misconduct, each as determined by a Final Order of the Bankruptcy Court. For purposes of the foregoing, it is expressly understood that any act or omission effected with the approval of the Bankruptcy Court conclusively will be deemed not to constitute gross negligence, or willful misconduct unless the approval of the Bankruptcy Court was obtained by fraud or misrepresentation, and in all respects, the applicable Persons shall be entitled to rely on the written advice of counsel with respect to their duties and responsibilities under, or in connection with, the Chapter 11 Cases, the Plan, and the administration thereof. Notwithstanding anything to the contrary herein, nothing in the Plan shall limit the liability of attorneys to their respective clients pursuant to Rule 1.8(h) of the New York Rules of Professional Conduct.

12.8.       Injunction Related to Releases and Exculpation.

The Confirmation Order shall permanently enjoin the commencement or prosecution by any Person, whether directly, derivatively or otherwise, of any claims, obligations, suits, judgments, damages, demands, debts, rights, Causes of Action or liabilities released pursuant to this Plan, including the claims, obligations, suits, judgments, damages, demands, debts, rights, Causes of Action or liabilities released in or encompassed by Sections

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12.6 and 12.7 of this Plan. Each of the Reorganized Debtors, as applicable, is expressly authorized hereby to seek to enforce such injunction.

12.9.       Retention of Causes of Action/Reservation of Rights.

Subject to Sections 12.6, 12.7, 12.8 and 12.9 of this Plan and except as expressly set forth herein, nothing contained in this Plan or the Confirmation Order shall be deemed to be a waiver or relinquishment of any rights, claims or Causes of Action, rights of setoff, or other legal or equitable defenses that the Debtors had immediately prior to the Effective Date on behalf of the Estates or of themselves in accordance with any provision of the Bankruptcy Code or any applicable non-bankruptcy law. Subject to Sections 12.6, 12.7, 12.8 and 12.9 of this Plan and except as expressly set forth herein, the Reorganized Debtors shall have, retain, reserve, and be entitled to assert all such claims, Causes of Action, rights of setoff, or other legal or equitable defenses as fully as if the Chapter 11 Cases had not been commenced, and all of the Debtors’ legal and/or equitable rights respecting any Claim left unimpaired, as set forth in Articles IV and V of this Plan, may be asserted after the Confirmation Date to the same extent as if the Chapter 11 Cases had not been commenced.

Notwithstanding any provision in the Plan or any order entered in these Chapter 11 Cases, the Debtors and Reorganized Debtors forever waive, relinquish, and release any and all Causes of Action the Debtors and their Estates had, have, or may have that arise under chapter 5 of the Bankruptcy Code (and analogous non-bankruptcy law) against any holder of an Allowed Claim in Class 6A under the Plan, provided, however, that for the avoidance of doubt, nothing in this Section or the Plan shall release any claims or Causes of Action against former directors, officers or employees who were not directors, officers or employees of any of the Debtors or any of the Debtors’ non-Debtor subsidiaries at any time after the Petition Date.

12.10.     Indemnification Obligations.

Notwithstanding anything to the contrary contained in this Plan, including Section 10.1 of this Plan, subject to the occurrence of the Effective Date, the existing obligations of the Debtors to indemnify, defend, reimburse, exculpate, advance fees and expenses to, or limit the liability of directors, officers or employees as of the Petition Date who were directors, officers or employees of any of the Debtors, or any of the Debtors’ non-Debtor subsidiaries, solely in their capacity as such, at any time after the Petition Date (whether or not also an officer, director or employee of Novelion), against any Causes of Action, remain unaffected thereby after the Effective Date and are not discharged. On and after the Effective Date, none of the Reorganized Debtors shall terminate or otherwise reduce the coverage under any directors’ and officers’ insurance policies in effect on the Petition Date, and all directors and officers of the Debtors, regardless of whether such person was a director or officer of the Debtors as of the Petition Date shall be entitled to the full benefits of any such policy (to the extent such director or officer is entitled to any benefits thereunder) for the full term of such policy, but solely to the extent, and as provided in, each such policy regardless of whether such directors and/or officers remain in such positions after the Effective Date. For the avoidance of doubt, all obligations of the Debtors to indemnify, defend, reimburse, exculpate, advance fees and expenses to, or limit the liability of former directors, officers or employees who were not directors, officers or employees of any of the Debtors or any of the Debtors’ non-Debtor subsidiaries at any time after the Petition Date,

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against any Causes of Action, are classified as Other General Unsecured Claims and shall be discharged on the Effective Date.

ARTICLE XIII.

RETENTION OF JURISDICTION

Pursuant to sections 105 and 1142 of the Bankruptcy Code and notwithstanding entry of the Confirmation Order and the occurrence of the Effective Date, on and after the Effective Date, the Bankruptcy Court shall retain exclusive jurisdiction, pursuant to 28 U.S.C. §§ 1334 and 157, over all matters arising in, arising under, or related to the Chapter 11 Cases for, among other things, the following purposes:

(a)       To hear and determine applications for the assumption or rejection of executory contracts or unexpired leases and the Cure Disputes resulting therefrom;

(b)       To determine any motion, adversary proceeding, application, contested matter, and other litigated matter pending on or commenced after the Confirmation Date;

(c)       To hear and resolve any disputes arising from or relating to (i) any orders of the Bankruptcy Court granting relief under Bankruptcy Rule 2004, or (ii) any protective orders entered by the Bankruptcy Court in connection with the foregoing;

(d)       To ensure that Plan Distributions to holders of Allowed Claims are accomplished as provided herein;

(e)       To consider Claims or the allowance, classification, priority, compromise, estimation, or payment of any Claim, including any Administrative Expense Claim;

(f)        To enter, implement, or enforce such orders as may be appropriate in the event the Confirmation Order is for any reason stayed, reversed, revoked, modified or vacated;

(g)       To issue and enforce injunctions, enter and implement other orders, and take such other actions as may be necessary or appropriate to restrain interference by any Person with the consummation, implementation, or enforcement of this Plan, the Confirmation Order, or any other order of the Bankruptcy Court;

(h)       To hear and determine any application to modify this Plan in accordance with section 1127 of the Bankruptcy Code, to remedy any defect or omission or reconcile any inconsistency in this Plan, the Disclosure Statement, or any order of the Bankruptcy Court, including the Confirmation Order, in such a manner as may be necessary to carry out the purposes and effects thereof;

(i)         To hear and determine all Fee Claims;

(j)         To hear and determine disputes arising in connection with the interpretation, implementation, or enforcement of this Plan, the Confirmation Order, any

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transactions or payments contemplated hereby, or any agreement, instrument, or other document governing or relating to any of the foregoing;

(k)       To take any action and issue such orders, including any such action or orders as may be necessary after occurrence of the Effective Date and/or consummation of the Plan, as may be necessary to construe, enforce, implement, execute, and consummate this Plan, including any release or injunction provisions set forth herein, or to maintain the integrity of this Plan following consummation;

(l)         To determine such other matters and for such other purposes as may be provided in the Confirmation Order;

(m)       To hear and determine all disputes involving the existence, nature or scope of the discharge, releases and injunction provisions contained in the Plan;

(n)       To hear and determine matters concerning state, local and federal taxes in accordance with sections 346, 505, and 1146 of the Bankruptcy Code;

(o)       To hear and determine any other matters related hereto and not inconsistent with the Bankruptcy Code and title 28 of the United States Code;

(p)       To resolve any disputes concerning whether a Person had sufficient notice of the Chapter 11 Cases, the Disclosure Statement Hearing, the Confirmation Hearing, any applicable Bar Date, or the deadline for responding or objecting to a Cure Amount, for the purpose of determining whether a Claim or Interest is discharged hereunder, or for any other purpose;

(q)       To recover all assets of the Debtors and property of the Estates, wherever located; and

(r)        To enter a final decree closing each of the Chapter 11 Cases.

If the Bankruptcy Court abstains from exercising, or declines to exercise, jurisdiction or is otherwise without jurisdiction over any matter arising in, arising under, or related to the Chapter 11 Cases, the provisions of this Article XIII shall have no effect on and shall not control, limit, or prohibit the exercise of jurisdiction by any other court having competent jurisdiction with respect to such matter.

ARTICLE XIV.

MISCELLANEOUS PROVISIONS

14.1.       Exemption from Certain Transfer Taxes.

To the fullest extent permitted by applicable law, all sale transactions consummated by the Debtors and approved by the Bankruptcy Court on and after the Confirmation Date through and including the Effective Date, including any transfers effectuated under this Plan, the sale by the Debtors of any owned property pursuant to section 363(b) of the Bankruptcy Code, and any assumption, assignment, and/or sale by the Debtors of their interests

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in unexpired leases of non-residential real property or executory contracts pursuant to section 365(a) of the Bankruptcy Code, shall constitute a “transfer under a plan” within the purview of section 1146 of the Bankruptcy Code, and shall not be subject to any stamp, real estate transfer, mortgage recording, or other similar tax.

14.2.       Retiree Benefits.

On and after the Effective Date, pursuant to section 1129(a)(13) of the Bankruptcy Code, the Reorganized Debtors shall continue to pay all retiree benefits (within the meaning of, and subject to the limitations of, section 1114 of the Bankruptcy Code), if any, at the level established in accordance with section 1114 of the Bankruptcy Code, at any time prior to the Confirmation Date, for the duration of the period for which any applicable Debtor had obligated itself to provide such benefits. Nothing herein shall: (a) restrict the Debtors’ or the applicable Reorganized Debtors’ right to modify the terms and conditions of the retiree benefits, if any, as otherwise permitted pursuant to the terms of the applicable plans, non-bankruptcy law, or section 1114(m) of the Bankruptcy Code; or (b) be construed as an admission that any such retiree benefits are owed by the Debtors.

14.3.       Dissolution of Creditors’ Committee.

The Committee shall be automatically dissolved on the Effective Date and all members, employees or agents thereof shall be released and discharged from all rights and duties arising from, or related to, the Chapter 11 Cases, provided that, notwithstanding the foregoing, the Committee and its professionals shall have the right to file, prosecute, review, and object to any applications for compensation and reimbursement of expenses filed in accordance with Section 3.3 hereof.

14.4.       Termination of Professionals.

On the Effective Date, the engagement of each Professional Person retained by the Debtors and the Creditors’ Committee shall be terminated without further order of the Bankruptcy Court or act of the parties; provided, however, such Professional Persons shall be entitled to prosecute their respective Fee Claims and represent their respective constituents with respect to applications for allowance and payment of such Fee Claims, and the Reorganized Debtors shall be responsible for the reasonable and documented fees, costs and expenses associated with the prosecution of such Fee Claims. Nothing herein shall preclude any Reorganized Debtor from engaging a former Professional Person on and after the Effective Date in the same capacity as such Professional Person was engaged prior to the Effective Date.

14.5.       Amendments.

This Plan may be amended, modified, or supplemented by the Debtors, subject to the parties’ rights under the RSA and the Plan Funding Agreement, in the manner provided for by section 1127 of the Bankruptcy Code or as otherwise permitted by law, without additional disclosure pursuant to section 1125 of the Bankruptcy Code, except as otherwise ordered by the Bankruptcy Court. In addition, after the Confirmation Date, so long as such action does not adversely affect the Plan Investor or the treatment of holders of Allowed Claims pursuant to this Plan, the Debtors may make appropriate technical adjustments, remedy any defect or omission or

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reconcile any inconsistencies in this Plan, the Plan Documents and/or the Confirmation Order, with respect to such matters as may be necessary to carry out the purposes and effects of this Plan, and any holder of a Claim that has accepted this Plan shall be deemed to have accepted this Plan as amended, modified, or supplemented. The Debtors may make such technical adjustments and modifications to this Plan without further order or approval of the Bankruptcy Court; provided, however, that, such technical adjustments and modifications are immaterial or do not adversely affect the Plan Investor or the treatment of holders of Claims or Interests under the Plan.

14.6.       Revocation or Withdrawal of this Plan.

Subject to the parties’ rights under the RSA and the Plan Funding Agreement, the Debtors reserve the right to revoke or withdraw this Plan prior to the Effective Date. If the Debtors revoke or withdraw this Plan, in accordance with the preceding sentence, prior to the Effective Date as to any or all of the Debtors, or if confirmation or consummation as to any or all of the Debtors does not occur, then, with respect to such Debtors: (a) this Plan shall be null and void in all respects; (b) any settlement or compromise embodied in this Plan (including the fixing or limiting to an amount of any Claim or Interest or Class of Claims or Interests), assumption or rejection of executory contracts or leases affected by this Plan, and any document or agreement executed pursuant to this Plan shall be deemed null and void, provided, however, that the Plan Investor, or any of its designees, shall retain its rights to the extent provided under the Transaction Documents; and (c) nothing contained in this Plan shall (i) constitute a waiver or release of any Claims by or against, or any Interests in, such Debtors or any other Person, (ii) prejudice in any manner the rights of such Debtors or any other Person or (iii) constitute an admission of any sort by the Debtors or any other Person.

14.7.       Allocation of Plan Distributions Between Principal and Interest.

To the extent that any Allowed Claim entitled to a distribution under the Plan consists of indebtedness and other amounts (such as accrued but unpaid interest thereon), such distribution shall be allocated first to the principal amount of the Claim (as determined for federal income tax purposes) and then, to the extent the consideration exceeds the principal amount of the Claim, to such other amounts.

14.8.       Severability.

If, prior to the entry of the Confirmation Order, any term or provision of this Plan is held by the Bankruptcy Court to be invalid, void, or unenforceable, the Bankruptcy Court, at the request of the Debtors, shall have the power to alter and interpret such term or provision to make it valid or enforceable to the maximum extent practicable, consistent with the original purpose of the term or provision held to be invalid, void, or unenforceable, and such term or provision shall then be applicable as altered or interpreted. Notwithstanding any such holding, alteration, or interpretation, the remainder of the terms and provisions of this Plan will remain in full force and effect and will in no way be affected, impaired, or invalidated by such holding, alteration, or interpretation. The Confirmation Order shall constitute a judicial determination and shall provide that each term and provision of this Plan, as it may have been altered or interpreted in accordance with the foregoing, is valid and enforceable pursuant to its terms.

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14.9.       Governing Law.

Except to the extent that the Bankruptcy Code or other U.S. federal law is applicable, or to the extent a Plan Document or exhibit or schedule to the Plan provides otherwise, the rights, duties, and obligations arising under this Plan and the Plan Documents shall be governed by, and construed and enforced in accordance with, the laws of the State of New York, without giving effect to the principles of conflict of laws thereof to the extent such principles would result in the application of the laws of any other jurisdiction.

14.10.     Section 1125(e) of the Bankruptcy Code.

The Debtors have, and upon confirmation of this Plan shall be deemed to have, solicited acceptances of this Plan in good faith and in compliance with the applicable provisions of the Bankruptcy Code, and the Debtors (and each of their respective affiliates, agents, directors, officers, employees, advisors, and attorneys) participated in good faith and in compliance with the applicable provisions of the Bankruptcy Code in the offer, issuance, sale, solicitation and/or purchase of the securities offered and sold under this Plan, and therefore are not, and on account of such offer, issuance, sale, solicitation, and/or purchase will not be, liable at any time for the violation of any applicable law, rule, or regulation governing the solicitation of acceptances or rejections of this Plan or offer, issuance, sale, or purchase of the securities offered and sold under this Plan.

14.11.     Inconsistency.

In the event of any inconsistency among the Plan, the Disclosure Statement, the Plan Documents (other than the Plan Funding Agreement), the RSA, any exhibit to the Plan or any other instrument or document created or executed pursuant to the Plan, the provisions of the Plan shall govern; provided, however, that the Plan Funding Agreement shall control and take precedence in the event of any inconsistency between the Plan Funding Agreement, any provision of this Plan, and any of the foregoing documents; provided, further, however, that the parties to the Plan Funding Agreement and the RSA shall use commercially reasonable efforts to eliminate any such inconsistency by agreement prior to the provisions of this section becoming applicable and enforceable.

14.12.     Time.

In computing any period of time prescribed or allowed by this Plan, unless otherwise set forth herein or determined by the Bankruptcy Court, the provisions of Bankruptcy Rule 9006 shall apply.

14.13.     Exhibits.

All exhibits to this Plan (including, without limitation, the Plan Documents, all documents filed with the Plan Supplement, and the Plan Funding Agreement and all exhibits and ancillary agreements thereto) are incorporated and are a part of this Plan as if set forth in full herein.

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14.14.     Notices.

All notices or requests in connection with the Plan shall be in writing (including by facsimile or electronic mail transmission) and, unless otherwise provided herein, shall be deemed to have been duly given or made only when actually delivered or, in the case of notice by facsimile or electronic mail transmission, when received and telephonically confirmed, addressed as follows:

Aegerion Pharmaceuticals, Inc.

245 First Street

Riverview II, 18th Floor

Cambridge, MA 02142

Attention: John R. Castellano

Email: JCastellano@alixpartners.com

with a copy to:

Willkie Farr & Gallagher LLP

787 Seventh Avenue

New York, NY 10019

Attention: Paul V. Shalhoub, Esq. and Andrew S. Mordkoff, Esq.

Email: pshalhoub@willkie.com; amordkoff@willkie.com

Facsimile: (212) 728-8111

Counsel to the Debtors

14.15.     Filing of Additional Documents.

On or before substantial consummation of the Plan, the Debtors shall file with the Bankruptcy Court such agreements and other documents as may be necessary or appropriate to effectuate and further evidence the terms and conditions of the Plan.

14.16.     Reservation of Rights.

Except as expressly set forth herein, the Plan shall have no force or effect unless and until the Bankruptcy Court enters the Confirmation Order. None of the filing of this Plan, any statement or provision contained herein, or the taking of any action by the Debtors with respect to this Plan shall be or shall be deemed to be, an admission or waiver of any rights of the Debtors with respect to any Claims or Interests prior to the Effective Date.

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 Dated:   August 29, 2019

New York, New York

Respectfully submitted,
AEGERION PHARMACEUTICALS, INC., on behalf of itself and its affiliated Debtors
By:	/s/ John R. Castellano
	Name:  John R. Castellano
	Title:  Chief Restructuring Officer

Counsel:

WILLKIE FARR & GALLAGHER LLP

Paul V. Shalhoub, Esq.

Andrew S. Mordkoff, Esq.

787 Seventh Avenue

New York, NY 10019

(212) 728-8000

Counsel for the Debtors and Debtors in Possession

Schedule 1.81

Government Settlement Agreements

The Government Settlement Agreements are comprised of the following settlement agreements and judgments:

(a) Civil Settlement Agreement, dated September 22, 2017,

(b) Indiana State Settlement Agreement, dated August 21, 2017,

(c) Mississippi State Settlement Agreement, dated August 21, 2017,

(d) South Carolina State Settlement Agreement, dated August 21, 2017,

(e) Arizona State Settlement Agreement, dated August 22, 2017,

(f) Michigan State Settlement Agreement, dated August 23, 2017,

(g) New Jersey State Settlement Agreement, dated August 23, 2017,

(h) Connecticut State Settlement Agreement, dated August 24, 2017,

(i) Georgia State Settlement Agreement, dated August 24, 2017,

(j) Ohio State Settlement Agreement, dated August 28, 2017,

(k) Alabama State Settlement Agreement, dated August 28, 2017,

(l) Illinois State Settlement Agreement, dated August 31, 2017,

(m) Florida State Settlement Agreement, dated September 1, 2017,

(n) Tennessee State Settlement Agreement, dated September 1, 2017,

(o) New York State Settlement Agreement, dated September 6, 2017,

(p) Pennsylvania State Settlement Agreement, dated September 6, 2017,

(q) Louisiana State Settlement Agreement, dated September 8, 2017,

(r) Iowa State Settlement Agreement, dated September 12, 2017,

(s) Virginia State Settlement Agreement, dated September 12, 2017,

(t) Nebraska State Settlement Agreement, dated September 13, 2017,

(u) West Virginia State Settlement Agreement, dated September 14, 2017,

(v) Colorado State Settlement Agreement, dated September 18, 2017,

(w) Nevada State Settlement Agreement, dated September 19, 2017,

(x) Kentucky State Settlement Agreement, dated September 20 2017,

(y) California State Settlement Agreement, dated September 21, 2017,

(z) Wisconsin State Settlement Agreement, dated September 21, 2017,

(aa) Texas State Settlement Agreement, dated September 26, 2017,

(bb) Missouri State Settlement Agreement, dated September 27, 2017,

(cc) Oklahoma State Settlement Agreement, dated September 28, 2017,

(dd) Deferred Prosecution Agreement, dated September 22, 2017,

(ee) Corporate Integrity Agreement, dated September 22, 2017,

(ff) Final Judgment, dated September 25, 2017,

(gg) Plea Agreement, dated January 12, 2018,

(hh) Criminal Judgment, dated January 30, 2018,

(ii) Consent Decree of Permanent Injunction, dated March 20, 2019, and

(jj) Side Letter Agreement, dated February 14, 2018.

SCHEDULE 1.93

MATERIAL TERMS OF THE NEW CONVERTIBLE NOTES INDENTURE

SCHEDULE 1.100

MATERIAL TERMS OF THE NEW TERM LOAN FACILITY

EX-10.2

Exhibit 10.2

Execution Version

RESTRUCTURING SUPPORT AGREEMENT

This Restructuring Support Agreement (as amended, restated, supplemented or otherwise modified from time to time in accordance with the terms set forth herein, this “Agreement”), dated as of May 20, 2019, is made by and among: (a) Aegerion Pharmaceuticals, Inc. (“Aegerion”) and each of its subsidiaries that are party hereto (collectively with Aegerion, the “Company”); (b) each of the undersigned holders (each, a “Consenting Lender” and, collectively, the “Consenting Lenders”, including any holders that execute a Lender Joinder (as defined below) after the date hereof) of claims (as defined in section 101(5) of title 11 of the United States Code (the “Bankruptcy Code”)) against the Company (the “Claims”) arising under or in connection with: (i) that certain Indenture, dated as of August 15, 2014 (as amended, supplemented or otherwise modified prior to the date hereof, the “Convertible Notes Indenture”and a holder of such Claims, the “Consenting Noteholders”), (ii) that certain Bridge Credit Agreement, dated as of November 8, 2018 (as amended, supplemented or otherwise modified prior to the date hereof, the “Bridge Credit Agreement” and a holder of such Claims, the “Consenting Bridge Lenders”), and/or (iii) that certain Amended and Restated Loan and Security Agreement, dated as of March 15, 2018 (as amended, restated, supplemented or otherwise modified from time to time prior to the date hereof, the “Intercompany Credit Agreement” and the holder of such Claims, presently Novelion Therapeutics Inc. (“Novelion Therapeutics”) or a wholly-owned direct or indirect subsidiary thereof (excluding Aegerion and its subsidiaries, and including Novelion Services USA, Inc. (“Novelion Services”), and collectively, “Novelion”)¹, in its capacity as such and as a holder of other Claims against and equity interests in the Company (including Claims in connection with the Amended Shared Services Agreements (as defined below)), and the Intercompany Credit Agreement, together with the Convertible Notes Indenture and the Bridge Credit Agreement, and their respective ancillary and related documents, the “Credit Documents”); and (c) Amryt Pharma plc (the “Plan Investor” and collectively with the Consenting Lenders, the “Plan Support Parties”). The Company and each of the Plan Support Parties are each referred to herein as a “Party”, and collectively, as the “Parties”. Each of the Consenting Noteholders, the Consenting Bridge Lenders and Novelion, as applicable, are referred to herein as a “Consenting Class.” Capitalized terms used but not otherwise defined herein have the meanings ascribed to such terms in the Plan (as defined below).

RECITALS

WHEREAS, the Company has determined that it would be in its best interests to implement a restructuring of its indebtedness and other obligations through the prosecution of “pre-negotiated” chapter 11 cases (the “Bankruptcy Cases”) in the United States Bankruptcy Court for the Southern District of New York (the “Bankruptcy Court”);

WHEREAS, the Parties have agreed that, in connection with the restructuring of the Company, the Plan Investor will acquire 100% of the equity of reorganized Aegerion in exchange for equity of the Plan Investor as set forth in, and the Parties shall otherwise consummate the transactions contemplated by, the Plan Funding Agreement (as defined below) and related documents, including the Plan (as defined below) and the other Definitive Documentation (as

¹ For purposes of this Agreement, Novelion shall not be deemed to be an Affiliate of Aegerion and Aegerion shall not be deemed to be an Affiliate of Novelion.

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defined below) (collectively, the “Transaction”), on the terms and subject to the conditions set forth in the Plan Funding Agreement;

WHEREAS, the Parties have agreed on the terms of the Transaction, which are memorialized in this Agreement and: (a) the proposed chapter 11 plan for the Company, substantially in the form attached hereto as Exhibit A (as may be amended, modified, or supplemented from time to time, including any schedules and exhibits attached thereto, in each case, in accordance with the terms hereof, the “Plan”); and (b) the Plan Funding Agreement between the Company and the Plan Investor, attached hereto as Exhibit B (as the same may be amended, modified, or supplemented from time to time, in accordance with the terms hereof, the “Plan Funding Agreement”), and executed concurrently herewith; and

WHEREAS, subject to the terms hereof and, as required, appropriate approvals of the Bankruptcy Court, the following sets forth the agreement between the Parties concerning their respective obligations in connection with the Transaction and the Bankruptcy Cases.

NOW, THEREFORE, in consideration of the foregoing, the Parties agree as follows:

AGREEMENT

Section 1.         Chapter 11 Plan and Definitive Documentation.

1.1 Support of Plan and Definitive Documentation.

(a) Subject to the terms of this Agreement, including the terms set forth in the immediately following sentence, so long as the Termination Date (as defined below) has not occurred, the Company agrees to: (i) use reasonable best efforts to take any actions, and do or cause to be done all things, necessary, appropriate or advisable in furtherance of the Transaction and the consummation thereof as promptly as practicable (and, in any event, within the time frames contemplated by this Agreement); (ii) commence the Bankruptcy Cases and file and seek approval on an interim and final (to the extent applicable) basis of “first day” motions (including (x) a motion seeking approval of a postpetition credit facility (the “DIP Facility” or the “DIP Credit Agreement”), substantially in the form attached hereto as Exhibit C) as may be amended, modified, or supplemented from time to time in accordance with the terms hereof, as well as the other Loan Documents (as defined in the DIP Credit Agreement, (y) a motion seeking approval of the Company’s assumption of (A) that certain Master Service Agreement dated as of December 1, 2016, but effective as of November 29, 2016, between Novelion Therapeutics and Aegerion and (B) that certain Master Service Agreement dated as of December 1, 2016, but effective as of November 29, 2016, between Novelion Services and Aegerion, each as amended by that certain Amendment to Shared Services Agreements dated as of May 20, 2019, between Novelion Therapeutics, Novelion Services and Aegerion (collectively, the “Amended Shared Services Agreements”) and (z) a motion (the “PFA Approval Motion”) seeking approval of the PFA Order (as defined below)) and with respect to all other “first day” motions, in the forms of the most recent drafts distributed in writing to the Plan Support Parties prior to the execution and delivery of this Agreement, as the same may be amended, modified or supplemented from time to time in accordance with the terms

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hereof (all such “first day” motions, collectively, the “First Day Motions”)); (iii) file the Plan and a related disclosure statement (as may be amended, modified or supplemented from time to time in accordance with the terms hereof, the “Disclosure Statement”), substantially in the form annexed hereto as Exhibit D, with the Bankruptcy Court and seek approval of the Disclosure Statement and confirmation of the Plan pursuant to the Confirmation Order (as defined below); (iv) act in good faith and use reasonable best efforts to support and complete successfully the solicitation of votes in favor of the Plan in accordance with the terms of this Agreement; (v) furnish any information reasonably requested by the Plan Investor (in the form and substance so requested) in connection with any application, notification or other document filed by or on behalf of the Plan Investor in connection with the Transaction, which information shall not contain any untrue statement of material fact or omit to state any material fact required to be stated therein or necessary in order to make the statements therein, in light of the circumstances under which they are made, not misleading and (vi) use reasonable best efforts to obtain any and all regulatory approvals and third-party approvals required, or otherwise reasonably requested by, any of the Plan Support Parties, to consummate or make effective the Transaction. Notwithstanding anything contained herein to the contrary, the Company is expressly permitted to take any and all actions contemplated by Sections 6.9 of the Plan Funding Agreement (such Sections of the Plan Funding Agreement and the actions contemplated thereby are sometimes referred to herein as the “Permitted Solicitation Activities”) and, so long as such actions are taken in accordance with the terms set forth therein, the Company shall not be deemed to be in breach of the terms set forth herein.

(b) Subject to the terms of this Agreement, so long as the Termination Date has not occurred, each Consenting Lender hereby agrees that it shall: (i) subject to the receipt by such Consenting Lender of the Disclosure Statement and Solicitation Materials (as defined below) approved by the Bankruptcy Court, and subject to the acknowledgements set forth in Section 8 of this Agreement, timely vote its Claims, now or hereafter beneficially owned by such Consenting Lender or for which the Consenting Lender now or hereafter serves as the nominee, investment manager or advisor for beneficial holders or over which it otherwise has voting power, to accept the Plan and otherwise in support and favor of the Transaction; provided that such vote shall be immediately revoked and deemed void ab initio upon termination of this Agreement as to such Consenting Lender prior to the confirmation of the Plan pursuant to the terms hereof; (ii) not change or withdraw (or cause to be changed or withdrawn) any such vote, subject to the proviso in the immediately preceding clause (i) of this Section 1.1(b); (iii) not, directly or indirectly, (x) object to, delay, impede or take any other action to interfere with acceptance, approval, confirmation or implementation of the Plan or the Transaction (or support any other person’s efforts to do any of the foregoing), (y) except as to Novelion at the request of the Company in connection with Permitted Solicitation Activities, (A) initiate, solicit, encourage or facilitate any inquiries, proposals or offers from any Person other than the Plan Investor and its Affiliates (as defined in the Plan Funding Agreement) and its and their respective advisors, consultants, legal counsel, investment bankers, agents and other representatives (with respect to any Person, the “Representatives” thereof) that are providing services in connection with the Transaction, relating to,

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or that could reasonably result in, alone or together with any other related transactions, any merger, acquisition, exchange, divestiture, sale of material assets or equity, business combination, recapitalization, joint venture, or other transaction directly or indirectly involving the equity, voting power or all or a material portion of the assets of Novelion or the Company or any of their respective subsidiaries, or any other similar transaction that would serve as an alternative to the Transaction or could reasonably be expected to impede, interfere with, prevent or delay the consummation of the Transaction or otherwise dilute in any material respect the benefits reasonably expected by the Plan Support Parties (any such transaction, an “Alternative Transaction”); (B) participate in discussions or negotiations with any Person regarding Novelion or the Company or any of their respective subsidiaries, the Plan, or the Transaction with respect to, or that would reasonably be expected to result in, an Alternative Transaction; or (C) propose, support, solicit, encourage, or participate in the formulation of any chapter 11 plan or any other restructuring or reorganization of the Company in the Bankruptcy Cases other than the Plan, or (z) otherwise take any action that would in any material respect interfere with, delay or postpone the consummation of the Transaction or otherwise dilute in any material respect the benefits reasonably expected by the Plan Support Parties; (iv) use its reasonable best efforts to take any and all necessary, appropriate or advisable actions in furtherance of the Transaction and the consummation thereof as promptly as practicable (and, in any event, within the time frames contemplated by this Agreement), including supporting the confirmation of the Plan and entry of the Confirmation Order; and supporting (and not objecting to) the First Day Motions; and (v) use reasonable best efforts to obtain any and all regulatory approvals and third-party approvals required, or otherwise reasonably requested, by the Company or any of the Plan Support Parties, to consummate or make effective the Transaction.

(c) Each Consenting Lender hereby agrees that, (i) so long as the Termination Date has not occurred and (ii) in the event the Termination Date occurs pursuant to (x) Section 2.2(a) of this Agreement, (y) Sections 2.2(c), 2.2(g) or 2.2(h) of this Agreement on or after the date the Company receives any solicited or unsolicited bona fide Company Alternative Proposal (as defined in the Plan Funding Agreement) that has not been withdrawn or terminated or (z) as elected by any Consenting Lender pursuant to Section 2.1 of this Agreement (other than Sections 2.1(k), 2.1(p) or 2.1(q) of this Agreement) on or after the date the Company receives any solicited or unsolicited bona fide Company Alternative Proposal that has not been withdrawn or terminated, each Consenting Lender shall vote against any Alternative Transaction, Company Alternative Transaction or Company Alternative Proposal, and any plan of reorganization that supports any of the foregoing, in the Bankruptcy Court and use reasonable best efforts to oppose the Bankruptcy Court’s approval of any such Alternative Transaction, Company Alternative Transaction or Company Alternative Proposal; provided, however, the foregoing obligation in the case of clause (ii) above shall lapse if, following inquiry in writing by the Consenting Lenders regarding whether the Plan Investor continues to be willing to consummate the Transaction in accordance with the Definitive Documentation, the Plan Investor does not agree within five (5) business days following the inquiry (subject to withdrawal at any time upon five (5) business

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days’ notice) that it would be willing to re-execute and deliver the Definitive Documents promptly after the other parties thereto re-execute and deliver same and consummate the Transaction in accordance with the Definitive Documentation if re-executed by the parties thereto. Notwithstanding anything to the contrary herein or in the Plan Funding Agreement, each Consenting Lender’s obligations under this Section 1.1(c) shall survive the Termination Date and shall remain in full force and effect until the earlier of the consummation of the Plan, any Alternative Transaction or Company Alternative Transaction.

(d) So long as the Termination Date has not occurred, the Plan Investor hereby agrees that it shall, and shall cause its Affiliates to, comply with the terms set forth in the Plan Funding Agreement until the closing of the Transaction contemplated thereby.

(e) The Backstop Parties hereby agree to backstop the Rights Offering and in connection therewith to execute the Backstop Commitment Agreement on or prior to the date of entry of the Disclosure Statement Order in substantially the form attached to Exhibit E hereto.

(f) Without limiting any other provision hereof, until the Termination Date, the Company and each of the Plan Support Parties hereby agrees to use reasonable best efforts to negotiate in good faith each of the definitive agreements and documents referenced in, or reasonably necessary to effectuate, the Transaction, this Agreement and the Plan, which shall consist of, among other things: (i) all amendments, exhibits and supplements to the Plan and to the Disclosure Statement; (ii) the PFA Order and the Rights Offering procedures and agreements; (iii) the solicitation materials in respect of the Plan (such materials, collectively, the “Solicitation Materials”), and the order to be entered by the Bankruptcy Court approving the Disclosure Statement and Solicitation Materials as containing, among other things, “adequate information” as required by section 1125 of the Bankruptcy Code (the “Disclosure Statement Order”); (iv) the order to be entered by the Bankruptcy Court confirming the Plan (the “Confirmation Order”) and pleadings in support of entry of the Confirmation Order; (v) the Interim CC Order (as defined below) and the Final DIP Order (as defined below) to the extent not attached as exhibits to the DIP Credit Agreement; (vi) the Amended Shared Services Agreements; and (vii) such other documents, pleadings, agreements or supplements as may be reasonably necessary to implement the Transaction, including, but not limited to, the new convertible notes indenture and the credit agreement for the new first lien secured credit facility in accordance with the term sheets attached hereto as Exhibits F and G, respectively (collectively, as may be amended, modified or supplemented from time to time, (and together with the Plan, the Disclosure Statement and any other definitive agreements and documents attached as exhibits hereto, the “Definitive Documentation”)), which Definitive Documentation shall be in form and substance consistent with the terms hereof and otherwise reasonably satisfactory to the Company and each of the Required Parties (as defined below). For the avoidance of doubt, any references herein to any document constituting Definitive Documentation (including, without limitation, the Plan, the Disclosure Statement, the Solicitation Materials, the Disclosure Statement Order and the Confirmation Order) shall mean such document in form and

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substance consistent with the terms hereof and otherwise reasonably satisfactory to the Company and each of the Required Parties.

(g) Subject to the terms of this Agreement, so long as the Termination Date has not occurred, the Company and each Consenting Lender hereby agrees not to: (i) file any motion, application, adversary proceeding or cause of action (A) challenging the validity, enforceability, perfection or priority of, or seeking avoidance or subordination of any Claims (in any capacity) of a Consenting Lender or the liens securing such Claims, or (B) otherwise seeking to impose liability upon or enjoin a Consenting Lender (in any capacity); or (ii) support any motion, application, adversary proceeding or cause of action referred to in the immediately preceding clause (i) filed by a third party, or consent to the standing of any such third party to bring such motion, application, adversary proceeding or cause of action.

For the avoidance of doubt, each of the Consenting Lenders, the Plan Investor and the Company also agrees, severally with respect to itself and not jointly, that, unless this Agreement is terminated in accordance with the terms hereof and subject to the Permitted Solicitation Activities and the right of each of the Plan Support Parties to take any action as may be set forth in this Agreement, the Plan Funding Agreement (including the actions contemplated by Section 6.9 of the Plan Funding Agreement in accordance with the terms set forth therein) or any other Definitive Documentation, it shall take such steps as are reasonably necessary to support, achieve approval of and consummate the Transaction on the terms set forth in this Agreement, the Plan Funding Agreement and the other Definitive Documentation and it will not take any action that would be expected to, in any material respect, interfere with, delay, or postpone the effectuation of the Transaction.

(h) As used herein, the following terms shall have the following meanings: “Required Consenting Lenders” shall mean, as of the applicable date of determination, (i) the Consenting Lenders that own at least 50.1% of principal indebtedness outstanding (“Obligations”) and held by all Consenting Lenders party hereto under the Convertible Notes Indenture, (ii) Consenting Lenders that own at least 66.7% of the Obligations held by all Consenting Lenders party hereto under the Bridge Credit Agreement, and (iii) Consenting Lenders that own at least 50.1% of the Obligations held by all Consenting Lenders party hereto under the Intercompany Credit Agreement. “Required Consenting Bridge Lenders/Noteholders” shall mean (i) the Consenting Lenders that own at least 50.1% of the Obligations held by all Consenting Lenders party hereto under the Convertible Notes Indenture, and (ii) Consenting Lenders that own at least 66.7% of the Obligations held by all Consenting Lenders party hereto under the Bridge Credit Agreement. “Required Consenting Intercompany Lenders” shall mean Consenting Lenders that own at least 50.1% of the Obligations held by all Consenting Lenders party hereto under the Intercompany Credit Agreement.

Section 2.         Termination Events.

2.1 Plan Support Party Termination Events.

Subject to the terms set forth in Section 2.5, the occurrence of any of the following shall be a “Plan Support Party Termination Event”:

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(a) 11:59 p.m. (prevailing Eastern Time) on the date that is one (1) business day after the date hereof unless prior thereto the Bankruptcy Cases have commenced in the Bankruptcy Court (the “Petition Date”);

(b) solely in the case of the Plan Investor, one (1) business day after the Petition Date, unless prior thereto the Company has filed the PFA Approval Motion;

(c) three (3) business days after the Petition Date, unless prior thereto the Bankruptcy Court has entered an order on an interim basis authorizing the Company to use cash collateral (the “Interim CC Order”);

(d) solely in the case of the Plan Investor, twenty-one (21) calendar days after the Petition Date (subject to a seven (7) day extension if the Bankruptcy Court so requires), unless prior thereto the Bankruptcy Court has entered an order approving the PFA Approval Motion (the “PFA Order”);

(e) solely in the case of Novelion, if the Company defaults in its payment obligations under the Amended Shared Services Agreements, and such default remains uncured after the running of any applicable cure period, or has filed a motion to reject the Amended Shared Services Agreements;

(f) thirty-five (35) calendar days after the Petition Date, unless prior thereto the Bankruptcy Court has entered an order on a final basis authorizing the Company to enter into the DIP Facility (the “Final DIP Order”);

(g) sixty (60) calendar days after the Petition Date, unless prior thereto the Bankruptcy Court has entered an order approving (i) the Disclosure Statement and authorizing the solicitation of votes on the Plan and (ii) the procedures with respect to the Rights Offering;

(h) one hundred twenty (120) calendar days after the Petition Date, unless prior thereto the Bankruptcy Court has entered the Confirmation Order;

(i) the Outside Date (as defined in the Plan Funding Agreement in the form attached as Exhibit B to this Agreement on the Petition Date), as extended pursuant to the definition thereof in the Plan Funding Agreement (in the form attached as Exhibit B to this Agreement on the Petition Date), unless prior thereto the effective date for the Plan has occurred;

(j) the occurrence of (A) any material breach by the Company of any of the undertakings or covenants of the Company set forth in this Agreement, or (B) any breach of any representation or warranty of the Company set forth in this Agreement unless the breach of such representation or warranty does not, and would not, reasonably be expected to, individually or together with any other uncured breaches, result in a Company Material Adverse Effect (as defined in the Plan Funding Agreement), unless, in each case, such breach is cured or waived by the Plan Support Parties within thirty (30) days after written notice of such breach is provided to the Company by any Party in accordance with the terms hereof;

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(k) solely in the case of the Consenting Lenders, the occurrence of any material breach by the Plan Investor of any of the undertakings or covenants, representations, or warranties of the Plan Investor set forth in this Agreement, unless, in each case, such breach is cured by the Plan Investor or waived by the Required Consenting Lenders within three (3) days after written notice of such breach is provided to the Plan Investor in accordance with the terms hereof;

(l) solely in the case of the Plan Investor, the occurrence of any material breach by any Consenting Lender of any of the undertakings or covenants, representations, or warranties of any Consenting Lender set forth in this Agreement, unless, in each case, such breach is cured by such Consenting Lender or waived by the Plan Investor within three (3) days after written notice of such breach is provided to such Consenting Lender in accordance with the terms hereof; provided, however, that, with respect to any termination as a result of a breach by a Consenting Lender as herein provided, the Plan Support Party Termination Event arising as a result of such breach shall apply only to the breaching Consenting Lender (at which point, for purposes of Section 2.1(v), such breaching Consenting Lender shall cease to be deemed a Consenting Lender hereunder) and this Agreement shall otherwise remain in full force and effect with respect to the Company and all other remaining Parties without limiting the terms set forth in Section 2.1(v);

(m) the filing of any pleading by the Company in the Bankruptcy Cases without the prior written consent of each of the Required Parties, that seeks to amend or modify this Agreement, the DIP Facility, the Backstop Commitment Agreement, the Rights Offering procedures, the Plan, the Disclosure Statement, the Plan Funding Agreement or any of the Definitive Documentation, which amendment, modification or filing is (i) materially inconsistent with this Agreement, the Plan, the Plan Funding Agreement and/or the Definitive Documentation, as applicable, and (ii) materially adverse to the applicable Plan Support Party(ies); and such motion or pleading has not been withdrawn prior to three (3) business days after the Company receives written notice from the Required Consenting Bridge Lenders/Noteholders or the Required Consenting Intercompany Lenders or the Plan Investor that such motion or pleading is (x) materially inconsistent with this Agreement, the Plan, the Plan Funding Agreement and/or the Definitive Documentation, and (y) materially adverse to such Plan Support Party; provided, that nothing contained in this subsection shall limit the Company’s ability to conduct the Permitted Solicitation Activities pursuant to the Plan Funding Agreement;

(n) the Company (i) withdraws the Plan, (ii) files, propounds or otherwise supports any plan of reorganization other than the Plan, or (iii) publicly announces its intention to do either of (i) or (ii); provided that nothing contained in this subsection shall limit the Company’s ability to conduct the Permitted Solicitation Activities pursuant to the Plan Funding Agreement;

(o) the Company files with the Bankruptcy Court any motion or application seeking authority to sell any material assets thereof without the prior written consent of the Required Parties;

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(p) any court of competent jurisdiction or other competent governmental or regulatory authority issues a final, non-appealable law or order, making illegal or otherwise preventing or prohibiting the consummation of the Transaction;

(q) any of the Bankruptcy Cases shall be dismissed or converted to a chapter 7 case, or a chapter 11 trustee with plenary powers, or a responsible officer or an examiner with enlarged powers relating to the operation of the businesses of the Company (powers beyond those set forth in section 1106(a)(3) and (4) of the Bankruptcy Code) shall be appointed in any of the Bankruptcy Cases or the Company shall file a motion or other request for such relief;

(r) the DIP Facility is terminated in accordance with the terms of the Final DIP Order or the Company’s right to use cash collateral is terminated in accordance with the terms of the Interim CC Order, the Final DIP Order or any separate cash collateral order that may have been entered in the Bankruptcy Cases;

(s) the Bankruptcy Court shall enter an order terminating, annulling, modifying or conditioning the automatic stay with respect to any material assets of the Company that would be reasonably likely to have a Company Material Adverse Effect (as defined in the Plan Funding Agreement), without the prior written consent of the Required Parties;

(t) the termination of the Plan Funding Agreement in accordance with the terms thereof;

(u) any of the orders of the Bankruptcy Court approving this Agreement, the DIP Facility (including the use of cash collateral), the Rights Offering procedures, the Plan Funding Agreement, the Plan or the Disclosure Statement, or the PFA Order, Confirmation Order or the Disclosure Statement Order or any other Definitive Documentation are reversed, vacated or otherwise materially modified in a manner inconsistent with this Agreement, the Plan Funding Agreement or the Plan and materially adverse to any of the Plan Support Parties without the written consent of the Plan Investor and written consent of the Required Consenting Bridge Lenders/Noteholders (to the extent Novelion is not materially adversely affected thereby), Novelion (to the extent Novelion but not any of the Consenting Bridge Lenders or Consenting Noteholders is materially adversely affected thereby) or the Required Consenting Lenders (if Novelion and other Consenting Lenders are materially adversely affected thereby), unless the Company promptly thereafter files a motion for reconsideration, reargument or rehearing and such reversal, vacation or other material modification is rescinded within thirty (30) days after the filing thereof;

(v) the Consenting Lenders at any time own less than 66.67% of the Obligations under each of the Convertible Notes Indenture, the Bridge Credit Agreement and the Intercompany Credit Agreement; provided that (i) no such Consenting Lender shall have the right to terminate this Agreement pursuant to this clause (v), and (ii) if any time the Consenting Lenders do not satisfy the foregoing threshold, then a Plan Support Party Termination Event shall not be deemed to have occurred under this clause (v) until the date that is fifteen (15) days following the date that such

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threshold shall have ceased to be satisfied, it being agreed that if the failure to satisfy such threshold shall have been cured (including by joining additional Consenting Lenders to this Agreement) on or prior to the expiration of such fifteen (15) days period, then a Plan Support Party Termination Event shall not be deemed to have occurred pursuant to this clause (v); and

(w) the Company loses the exclusive right to file and solicit acceptances of a chapter 11 plan; and

(x) (i) the Company or any Consenting Lender files any motion, application, adversary proceeding or cause of action (A) challenging the validity, enforceability, perfection or priority of, or seeking avoidance or subordination of any Claims (in any capacity) of a Consenting Lender or the liens securing such Claims, or (B) otherwise seeking to impose liability upon or enjoin a Consenting Lender (in any capacity); or (ii) the Company or any Consenting Lender supports any motion, application, adversary proceeding or cause of action referred to in the immediately preceding clause (i) filed by a third party, or consents to the standing of any such third party to bring such motion, application, adversary proceeding or cause of action.

2.2 Company Termination Events.

Subject to the terms set forth in Section 2.5, the occurrence of any of the following shall be a “Company Termination Event” and together with any Plan Support Party Termination Event, a “Termination Event”:

(a) the Company shall be entitled to terminate the Plan Funding Agreement pursuant to Section 8.1(b)(iii) thereof, subject to the terms and limitations thereof;

(b) the occurrence of (i) any material breach by the Plan Investor of any of the material undertakings or material covenants of the Plan Investor set forth in this Agreement, or (ii) any breach of any representation or warranty of the Plan Investor set forth in this Agreement unless the breach of such representation or warranty would not, individually or in the aggregate, reasonably be expected to have a Plan Investor Material Adverse Effect (as defined in the Plan Funding Agreement), unless, in each case, such breach is cured or waived within thirty (30) days after written notice of such breach is provided to the Plan Investor in accordance with the terms hereof.

(c) the occurrence of (i) any material breach by any Consenting Lender of any of the material undertakings or material covenants of such Consenting Lender set forth in this Agreement, or (ii) any breach of any representation or warranty of any Consenting Lender set forth in this Agreement unless the breach of such representation or warranty would not, individually or in the aggregate, reasonably be expected to have a material adverse effect on the ability of such Consenting Lender to consummate the Transaction as herein provided, unless, in each case, such breach is cured or waived within thirty (30) days after written notice of such breach is provided to such Consenting Lender in accordance with the terms hereof; provided, however, that, with respect to any termination as a result of a breach by a Consenting Lender as herein provided, the Company Termination Event arising

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as a result of such breach shall apply only to the breaching Consenting Lender and this Agreement shall otherwise remain in full force and effect with respect to the Company and all other remaining Parties, without limiting the terms set forth in the immediately following clause (d);

(d) the Consenting Lenders at any time own less than 66.67% of the Obligations under each of the Convertible Notes Indenture, the Bridge Credit Agreement and the Intercompany Credit Agreement, provided that if any time the Consenting Lenders do not satisfy the foregoing threshold, then a Company Termination Event shall not be deemed to have occurred under this clause (d) until the date that is thirty (30) days following the date that such threshold shall have ceased to be satisfied, it being agreed that if the failure to satisfy such threshold shall have been cured (including by joining additional Consenting Lenders to this Agreement) on or prior to the expiration of such thirty (30) days period, then a Company Termination Event shall not be deemed to have occurred pursuant to this clause (d);

(e) any court of competent jurisdiction or other competent governmental or regulatory authority issues a final, non-appealable law or order, making illegal or otherwise preventing or prohibiting the consummation of the Transaction;

(f) any of the Bankruptcy Cases shall be dismissed or converted to chapter 7;

(g) the termination of the Plan Funding Agreement in accordance with the provisions thereof; and

(h) the Outside Date (as defined in the Plan Funding Agreement in the form attached as Exhibit B to this Agreement on the Petition Date), as extended pursuant to the definition thereof in the Plan Funding Agreement (in the form attached as Exhibit B to this Agreement on the Petition Date), unless prior thereto the effective date for the Plan has occurred.

Notwithstanding the foregoing, any of the dates or deadlines set forth in Sections 2.1-2.2 of this Agreement may be extended by the written agreement of each of the Company and the Required Parties.

2.3 Company Termination Event Procedures.

Subject to the terms set forth in Section 2.5 and Section 2.7, upon the occurrence of any Company Termination Event, the Company may elect to terminate this Agreement by delivering written notice thereof to the other Parties; provided that if the Company exercises such right only in respect of one or more Consenting Lenders as contemplated by Section 2.2(c), then, subject to the terms set forth in Section 2.2(c), 2.2(d) and Section 2.7, this Agreement shall terminate only in respect of such Consenting Lender or Consenting Lenders (the date of the effectiveness of such termination, the “Company Termination Date”) and such Consenting Lender or Consenting Lenders shall cease to be deemed a Consenting Lender hereunder from and after such date.

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2.4 Plan Support Party Termination Event Procedures.

(a) Subject to the terms set forth in Section 2.5 and Section 2.7, the Plan Investor shall have the right to terminate this Agreement upon the occurrence of any Plan Support Party Termination Event (other than the Plan Support Termination Events set forth in Sections 2.1(e) and 2.1(1)) in accordance with this Section 2.4. Subject to the terms set forth in Section 2.5 and Section 2.7, the Required Consenting Bridge Lenders/Noteholders shall have the right to terminate this Agreement upon the occurrence of a Plan Support Party Termination Event (other than the Plan Support Termination Events set forth in Sections 2.1(b), 2.1(d), 2.1(e) and 2.1(1)) in accordance with this Section 2.4. Subject to the terms set forth in Section 2.5 and Section 2.7, the Required Consenting Intercompany Lenders shall have the right to terminate this Agreement upon the occurrence of a Plan Support Party Termination Event (other than the Plan Support Termination Events set forth in Sections 2.1(b), 2.1(d), 2.1(k) and 2.1(1)) in accordance with this Section 2.4. Subject to the terms set forth in the immediately preceding three sentences and Section 2.5 and Section 2.7, upon the occurrence of a Plan Support Party Termination Event, the Plan Investor, the Required Consenting Bridge Lenders/Noteholders or the Required Consenting Intercompany Lenders, as applicable, (in such capacity, the “Terminating Party”), may elect to terminate this Agreement with respect to such Terminating Party by delivering written notice thereof to the other Parties; provided that if the Plan Investor exercises such right only in respect of one or more Consenting Lenders as contemplated by Section 2.1(1), then, subject to the terms set forth in Section 2.1(1), 2.1(v) and Section 2.7, this Agreement shall terminate only in respect of such Consenting Lender or Consenting Lenders (the date of effectiveness of such termination, together with the Company Termination Date, being the “Termination Date”) and such Consenting Lender or Consenting Lenders shall cease to be deemed a Consenting Lender hereunder from and after such date. For the avoidance of doubt, the automatic stay arising pursuant to section 362 of the Bankruptcy Code shall be deemed waived or modified for purposes of providing notice or exercising rights hereunder, and the Company agrees it shall not take any action to enforce the automatic stay to prevent any valid termination of this Agreement and the PFA Order shall include a waiver of the automatic stay in connection therewith for purpose of providing notice or exercising rights hereunder.

(b) Notwithstanding anything herein to the contrary, but subject to Section 2.1(1), Section 2.1(v), Section 2.2(c) and Section 2.2(d) of this Agreement, if a Termination Date shall occur in respect of any Consenting Lender, such termination and Termination Date shall apply only to such Consenting Lender (and such Consenting Lender shall cease to be deemed a Consenting Lender hereunder from and after such Termination) and this Agreement shall otherwise remain in full force and effect with respect to the Company, the Plan Investor and all such remaining Consenting Lenders.

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2.5 Limitation on Termination.

Except with respect to a termination pursuant to Section 2.1(t), Section 2.2(a), Section 2.2(g) or Section 3 below, no Party shall have the right to terminate this Agreement if the Termination Event giving rise to such termination right is the result of the action or omission of such Party or any Affiliate thereof and the taking or failing to take such action by such Party or the applicable Affiliate thereof constitutes a breach of this Agreement, the Plan Funding Agreement or any other Definitive Document.

2.6 Consensual Termination.

In addition to any Termination Event otherwise set forth herein, this Agreement shall terminate immediately upon the written agreement of each of the Company, the Plan Investor, and the Required Consenting Lenders.

2.7 Effect of Termination.

Upon the valid termination of this Agreement, except as otherwise set forth herein (including if such termination only related to one or more Consenting Lenders but not this Agreement as an entirety): (a) this Agreement shall be of no further force and effect and each Party shall be released from its commitments, undertakings and agreements under this Agreement, and shall have the rights and remedies that it would have had it not entered into this Agreement, and shall be entitled to take all actions, whether with respect to the Transaction or otherwise, that it would have been entitled to take had it not entered into this Agreement; (b) any and all votes tendered by the Parties in respect of the Plan prior to such termination shall be deemed, for all purposes, to be null and void from the first instance and shall not be considered or otherwise used in any manner by the Parties in connection with this Agreement, the Transaction, the Plan or otherwise; and (c) if Bankruptcy Court permission shall be required for a Consenting Lender to change or withdraw (or cause to be changed or withdrawn) its vote in favor of the Plan, no Party to this Agreement shall oppose any attempt by such Party to change or withdraw (or cause to be changed or withdrawn) such vote. Notwithstanding the foregoing, nothing in this section or elsewhere in this Agreement, shall relieve any Party from (i) liability for such Party’s breach of such Party’s representations, warranties, covenants, undertakings or obligations hereunder or under any other Definitive Document (including the liability of any Consenting Lender with respect to the period before any Termination Date with respect to such Consenting Lender), or (ii) obligations under this Agreement or any other Definitive Document that expressly survive termination of this Agreement, including, without limitation, the Company’s obligation (if any) to pay professional fees and expenses pursuant to Section 9.12 hereof that accrued on or prior to the Termination Date or the Company’s obligations (if any) to make payments to the Plan Investor under the PFA Order. Except with respect to the obligations under this Agreement that expressly survive termination of this Agreement (including, without limitation, the Company’s obligation (if any) to pay professional fees and expenses pursuant to Section 9.12) and this Section 2.7 or the Company’s obligations (if any) to make payments to the Plan Investor under the PFA Order, this Agreement shall terminate automatically without any further required action or notice upon consummation of the Plan.

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Section 3.      Fiduciary Obligations.

3.1 The Company’s Fiduciary Obligations.

Notwithstanding anything to the contrary herein, but subject in all cases to compliance with the Plan Funding Agreement in all respects, the board of directors, board of managers, or such similar governing body of the Company, including any properly authorized committee thereof (each, a “Board”) shall be permitted to take (or permitted to refrain from taking) any action with respect to the Transaction as and to the extent permitted by Section 6.9 of the Plan Funding Agreement and may take such action without incurring any liability to the Consenting Lenders or the Plan Investor under this Agreement or the Plan as and to the extent permitted thereby; provided that nothing herein shall limit or otherwise affect the rights or remedies of (i) the Plan Investor under the Plan Funding Agreement or the PFA Order and (ii) the Consenting Lenders under Section 9.12 of this Agreement; provided, further, that nothing herein shall limit the rights of the Required Consenting Lender or the Plan Investor to terminate this Agreement to the extent the taking or refraining from taking any action pursuant to this Section 3.1 would otherwise constitute a Plan Support Party Termination Event (as determined without taking into account whether the taking or refraining from taking such action is permitted under this Section 3.1).

3.2 Consenting Lender Fiduciary Obligations.

Each Consenting Lender agrees not to request that the United States Trustee appoint an official committee of creditors or equity holders (either or both, an “Official Committee”) in the Bankruptcy Cases. Notwithstanding anything herein to the contrary, if any Consenting Lender is appointed to and serves on any Official Committee in the Bankruptcy Cases, the terms of this Agreement shall not be construed so as to limit such Consenting Lender’s exercise of its fiduciary duties to any person arising from its service on such Official Committee, and any such exercise of such fiduciary duties shall not be deemed to constitute a breach of the terms of this Agreement; provided that nothing in this Agreement shall be construed as requiring any Consenting Lender to serve on any Official Committee in any such chapter 11 case.

Section 4. Conditions Precedent to Agreement.

The obligations of the Parties and the effectiveness of this Agreement are subject to satisfaction of each of the following (the date upon which all such conditions are satisfied, the “Effective Date”): (x) execution and delivery of signature pages for the Plan Funding Agreement and the Amended Shared Services Agreements by each of the parties thereto; and (y) execution and delivery of signature pages for this Agreement by each of the Company, the Plan Investor and the Consenting Lenders (who, in any event, shall hold not less than 66.67% of the Obligations under each of the Convertible Notes Indenture, the Bridge Credit Agreement and the Intercompany Credit Agreement).

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Section 5. Effects of Exclusivity Agreement.

5.1 Retention of Advance Pending PFA Order.

Reference is made to the letter agreement, dated as of April 11, 2019 (the “Exclusivity Agreement”), by and among Aegerion, the Plan Investor, Novelion, Highbridge Capital Management LLC and Athyrium Capital Management, LP. Within two (2) business days following the date the Bankruptcy Court enters the PFA Order, the Plan Investor shall repay the entire Advance (as defined in the Exclusivity Agreement), including any previously applied portion of the Advance, to Aegerion by wire transfer of immediately available funds to an account identified by Aegerion. Until such time, the Plan Investor shall be entitled to retain the Advance, notwithstanding the occurrence of a No Reimbursement Event (as defined in the Exclusivity Agreement) by the execution and delivery of this Agreement or any other Definitive Documentation.

5.2 Use of Advance Upon Failure to Obtain PFA Order.

Upon any termination of this Agreement pursuant to Section 2.1(d) or upon any termination of this Agreement by any Party other than the Plan Investor at a time when the PFA Order has not been entered and the Plan Investor could have terminated this Agreement pursuant to Section 2.1(d), the Plan Investor shall be entitled to retain the Advance and use it to pay Expenses (as defined in the Exclusivity Agreement), notwithstanding the occurrence of a No Reimbursement Event by the execution and delivery of this Agreement or any other Definitive Documentation. The Advance and any right to payment of Expenses shall be treated as provided in Section 4(c) through 4(g) of the Exclusivity Agreement.

5.3 Effect on Exclusivity Agreement.

Except for the provisions of Section 4 of the Exclusivity Agreement that survive the execution and delivery of this Agreement as contemplated by this Section 5, the terms and conditions set forth in the Exclusivity Agreement shall expire and be of no further force and effect upon the execution and delivery of this Agreement.

Section 6. Representations, Warranties and Covenants.

6.1 Power and Authority.

Each Plan Support Party, severally with respect to itself and not jointly, represents, warrants, and covenants to the Company, and the Company, jointly and severally, represents, warrants, and covenants to each Plan Support Party, that (a) such Party has and shall maintain all requisite corporate, partnership, limited liability company or other applicable entity power and authority to enter into this Agreement and the other Definitive Documentation to which it is or will become a party and to carry out the transactions contemplated by, and perform its respective obligations under, this Agreement and such other Definitive Documentation, and (b) the execution and delivery of this Agreement and the other Definitive Documentation to which it is or will become a party and the performance of its obligations hereunder and thereunder have been duly authorized by all necessary action on its part.

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6.2 Enforceability.

Each Plan Support Party, severally with respect to itself and not jointly, represents and warrants to the Company, and the Company, jointly and severally, represents and warrants to each Plan Support Party, that this Agreement and each other Definitive Documentation to which it is or will become a party is (or will be) its legally valid and binding obligation, enforceable in accordance with its terms, except as enforcement may be limited by bankruptcy, insolvency, reorganization or other similar laws limiting creditors’ rights generally or by equitable principles relating to enforceability or ruling or approval of the Bankruptcy Court.

6.3 Governmental Consents.

Each Plan Support Party, severally with respect to itself and not jointly, represents and warrants to the Company, and the Company, jointly and severally, represents and warrants to each Plan Support Party that its execution, delivery, and performance of this Agreement and the other Definitive Documentation to which it is or will become a party does not and shall not require any registration or filing with, consent or approval of, or notice to, or other action to, with, or by, any federal, state, or other governmental authority or regulatory body, except: (a) as may be necessary and/or required by the Securities and Exchange Commission or federal securities laws, rules or regulations, national securities exchange, the Financial Conduct Authority or other applicable state or provincial securities or “blue sky” laws; (b) any of the foregoing as may be necessary and/or required in connection with the Bankruptcy Cases, including the approval of the Disclosure Statement and confirmation of the Plan by the Bankruptcy Court; (c) in the case of the Company or the Plan Investor, (i) filings of amended articles of incorporation or formation or other organizational or constating documents with applicable state or other local authorities that are required to implement the Transaction as contemplated by the Plan Funding Agreement, and (ii) other registrations, filings, consents, approvals, notices, or other actions that are reasonably necessary to maintain permits, licenses, qualifications, and governmental approvals to carry on the business of the Company or the Plan Investor; (d) authorizations, consents, orders or approvals of, or registrations or declarations with, any Governmental Entity (as defined in the Plan Funding Agreement), that have been or will be obtained or made prior to or on the closing date of the Transaction (the “Closing Date”), a true and complete list of which is set forth on Schedule 5.3 of the Plan Funding Agreement; and (e) any other registrations, filings, consents, approvals, notices, or other actions, the failure of which to make, obtain or take, as applicable, would not be reasonably likely to, individually or in the aggregate, (i) in the case, of the Company, have a Company Material Adverse Effect, (ii) in the case of the Plan Investor, have a Plan Investor Material Adverse Effect, or (iii) in the case of any Consenting Lender, materially delay or materially impair the ability of such Consenting Lender to consummate the Transaction.

6.4 Ownership.

Each Consenting Lender, severally and not jointly, represents, warrants, and covenants to the Company and the other Parties that, without limiting the ability of such Consenting Lender to sell, transfer or assign the Claims in accordance with and subject to the terms set forth in Section 9 of this Agreement, (a) such Party is either (i) the sole legal and beneficial owner of its share of the Claims and/or equity interests in the Company in the amounts indicated opposite its name on Schedule 6.4 of this Agreement, or (ii) such Consenting Lender has investment or voting discretion or control with respect to accounts for the holders or beneficial owners of the Claims and/or equity interests in the Company in the amounts indicated opposite its name on Schedule 6.4 of this

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Agreement; (b) it has full power and authority to vote on and consent to all matters concerning the Claims and/or equity interests in the Company in the amounts indicated opposite its name on Schedule 6.4 of this Agreement and to exchange, assign and transfer such Claims and/or equity interests as contemplated by the Transaction; and (c) other than pursuant to this Agreement and the other Definitive Documentation, such Claims and/or equity interests are and shall continue to be free and clear of any pledge, lien, security interest, charge, claim, equity, option, proxy, voting restriction, right of first refusal or other limitation on disposition, or encumbrances of any kind, that would adversely affect in any way such Consenting Lender’s performance of its obligations contained in this Agreement and the other Definitive Documentation at the time such obligations are required to be performed and the consummation of the Transaction.

6.5 No Conflict; Third Party Consents.

Each Plan Support Party, severally with respect to itself and not jointly, represents and warrants to the Company, and the Company, jointly and severally, represents and warrants, to each Plan Support Party that the execution, delivery and performance by such Party of this Agreement and the other Definitive Documentation to which it is or will become a party does not, and the consummation of the Transaction does not and will not (a) subject to receipt of the authorizations, consents, orders or approvals of, or registrations or declarations with, any federal, state, or other governmental authority or regulatory body that have been or will be obtained or made prior to or on the Closing Date with respect to the Transaction as set forth on Schedule 5.3 of the Plan Funding Agreement, violate any provision of law, rule or regulation applicable to it or its charter or bylaws (or other similar governing documents) in any material respect, (b) conflict with, result in a breach of or constitute (with due notice or lapse of time or both) a default under any contractual obligation to which it is a party in any material respect, except, in the case of the Company, for the filing of the Bankruptcy Cases, or (c) other than in respect of the Company as expressly contemplated by the Plan, require the consent or approval of, or notice to, or other action by, any creditor or shareholder of any Party or from any other Person in respect of any Party (including any contractual obligation of any Party), other than for any such consent, approval, notice or action, the failure of which to make or obtain, as would not reasonably be expected to be material to such Party or its ability to consummate the Transaction.

6.6 Publicity; Confidentiality.

(a) Publicity. Concurrently with or as promptly as practicable following the execution of this Agreement, the Parties (other than the Consenting Noteholders or Consenting Bridge Lenders) or some of the Parties shall issue the press release or press releases substantially in the form(s) attached to Schedule 6.6(a) (collectively, the “Initial Press Release”). Subject to the terms set forth in the immediately following sentence, none of the Parties will make, or permit any Affiliate thereof to make, any public statements, including any press releases, with respect to this Agreement, the other Definitive Documentation, or the Transaction unless such press release or public statement is consistent, in all material respects, with the Initial Press Release or receives the prior written consent of the Company, the Plan Investor and the Required Consenting Lenders. Notwithstanding anything to the contrary contained in the foregoing, any Party (or any Affiliate thereof) may (i) make disclosures required by any applicable law or applicable stock exchange requirements (it being acknowledged that Novelion intends to file a Current Report

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on Form 8-K and any equivalent filing as may be required by applicable Canadian securities laws in respect of the Transaction within the permitted statutory timeframe from the date of this Agreement (or in a periodic report in lieu of such Form 8-K, if timing so permits), and such filing and/or subsequent filings with the Securities and Exchange Commission may attach or otherwise file as exhibits this Agreement and/or other Definitive Documentation), in which case the Party required to make (or whose Affiliate is required to make) such disclosure will allow the other Parties reasonable time to comment on such disclosure in advance of the making or issuance thereof to the extent reasonably practicable, (ii) make disclosures that are expressly contemplated by this Agreement, the Plan Funding Agreement or the Plan, including (A) in the case of the Plan Investor, the filing and disclosure of the Admission Document and any other documentation in respect of the solicitation of the approval of its shareholders in respect of the Transaction, subject to compliance with the terms set forth in the Plan Funding Agreement, and (B) in the case of the Company, such disclosures as it is required to make in connection with the Bankruptcy Cases, including in connection with the solicitation of votes in support of the Plan, and (iii) make such disclosures as any Party or its Affiliates determines to be advisable or required in connection with any action or legal proceeding commenced by any Party against any other Party or any Affiliate thereof in respect of any dispute arising out of this Agreement, the other Definitive Documentation or the Transaction.

(b) Confidentiality. Any confidentiality agreement executed by any Party shall survive this Agreement and shall continue in full force and effect, subject to the terms thereof, irrespective of the terms hereof.

(c) Disclosure of Consenting Lender Information. Unless required by applicable law or regulation or requested by any regulatory authority, no Party shall disclose the amount of a Consenting Lender’s holdings of Claims without the prior written consent of such Consenting Lender; provided, however, that the Company may disclose the aggregate holdings and percentages of the Consenting Lenders, by Consenting Class, and, if required by the Bankruptcy Court, may disclose the amount of a Consenting Lender’s holdings of Claims without the prior written consent of such Consenting Lender. If any Party or any of its representatives receives a subpoena or other legal process as referred to in this Section 6.6 in connection with the Agreement, such Party shall provide the other Parties hereto with prompt written notice of any such request or requirement, to the fullest extent permissible and practicable under the circumstances (as advised by such Party’s internal or outside counsel), so that the other Parties may seek a protective order or other appropriate remedy or waiver of compliance with the provisions of this Agreement.

6.7          Acquired Interests. Each Consenting Lender severally, and not jointly, or jointly or severally, represents and warrants to the Plan Investor that it has not acquired an interest in shares (as such term is defined in the UK City Code of Takeovers and Mergers) in the Plan Investor during the course of the twelve months prior to the date of this Agreement (any such acquisition, a “Disqualifying Transaction”).

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6.8           UK Panel. Each Consenting Lender severally, and not jointly, or jointly or severally, hereby represents and warrants and undertakes to the Company that:

(a) neither it nor any of its Affiliates will enter into any Disqualifying Transaction in the period from the date of this Agreement until Closing of the Transaction except in the case of Highbridge MSF International Ltd., 1992 Tactical Credit Master Fund, L.P., Highbridge SCF Special Situations SPV, L.P., and Highbridge SCF Loan SPV, L.P. (the “Highbridge Funds”), the Highbridge Funds and its Affiliates may, with the consent of the UK Panel on Takeovers and Mergers (the “Panel”), purchase all or any portion of the shares issued by the Plan Investor pursuant to any Plan Investor Additional Equity Issuance (as defined in the Plan Funding Agreement); and

(b) neither it nor any of its Affiliates will knowingly take any action that it or such Affiliates knows at the time of such action constitutes “acting in concert” (as such term is defined in Rule 9.1 of the Takeover Code) with another Consenting Lender or any other third party with a view to obtain or seek to obtain control of the Company, and, if it or any of its Affiliates has actual knowledge that it or such Affiliate has been “acting in concert”, then it shall, or shall cause its Affiliate(s) to, advise the Company of such actions at least five (5) business days prior to the Company seeking shareholder approval of the “Rule 9 whitewash waiver” or, if such acting in concert has occurred during this five (5) business day period, no later than twenty-four (24) hours after the time such acting in concert has occurred.

6.9           Rule 9.1 Information. Athyrium Opportunities II Acquisition LP, Athyrium Opportunities III Acquisition LP, Highbridge MSF International Ltd., 1992 Tactical Credit Master Fund, L.P., Highbridge SCF Special Situations SPV, L.P., and Highbridge SCF Loan SPV, L.P., in each case severally, and not jointly, or jointly or severally, represents and warrants and undertakes to the Company that all of the information provided to the Panel in connection with the analysis undertaken for the purposes of Rule 9.1 is true and accurate in all material respects.

Section 7. Remedies.

It is understood and agreed by each of the Parties that any breach of this Agreement would give rise to irreparable harm for which money damages would not be an adequate remedy and accordingly the Parties agree that, in addition to any other remedies, each non-breaching Party shall be entitled to specific performance and injunctive or other equitable relief for any such breach without the posting of a bond or other security. The Parties agree to waive any defense in any action for specific performance that a remedy at law would be adequate. The Company and each of the Plan Support Parties agree that for so long as the Company and the Plan Support Parties have not taken any action to prejudice the enforceability of this Agreement (including without limitation, alleging in any pleading that this Agreement is unenforceable), and have taken such actions as are reasonably required or desirable for the enforcement hereof, then the Company and the Plan Support Parties shall have no liability for damages hereunder in the event a court determines that this Agreement is not enforceable. Each of the Parties to this Agreement acknowledges and agrees that, notwithstanding anything in this Agreement to the contrary, it shall have no, and agrees not to pursue any, recourse against (a) Novelion for breaches or threatened breaches hereunder by Aegerion or Aegerion’s Representatives to the extent such Representative

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was acting in its capacity as an Aegerion Representative (and not as a Novelion Representative) at the time of the alleged breach, or (b) Aegerion for breaches or threatened breaches hereunder by Novelion or Novelion’s Representatives to the extent such Representative was acting in its capacity as a Novelion Representative (and not as an Aegerion Representative) at the time of the alleged breach.

Section 8. Acknowledgement.

This Agreement and the Plan and transactions contemplated herein and therein are the product of negotiations among the Parties, together with their respective representatives. Notwithstanding anything herein to the contrary, this Agreement is not, and shall not be deemed to be, a solicitation of votes for the acceptance of the Plan or any chapter 11 plan for the purposes of sections 1125 and 1126 of the Bankruptcy Code or otherwise. Notwithstanding anything herein to the contrary, the Company will not solicit acceptances of the Plan from any Consenting Lender until such Consenting Lender has been provided with information required by section 1125 of the Bankruptcy Code.

Section 9. Miscellaneous Terms.

9.1           Assignment; Transfer Restrictions.

(a) Each Consenting Lender agrees, severally with respect to itself and not jointly, until the earlier of the date that this Agreement is validly terminated in accordance with its terms and the date that the closing of the Transaction occurs, not to, directly or indirectly, sell, assign, transfer, hypothecate or otherwise dispose of (including by participation) (a “Transfer”) any Claim against the Company or any interest therein or voting rights in respect thereof unless (i) (A) the transferee, assignee or equivalent is a Consenting Lender that is a party to and bound by this Agreement and, as applicable, the other Definitive Documentation (including the execution and delivery of a Lender Joinder in accordance with Subsection 9.1(c))), provided that upon the consummation of any Transfer by any Consenting Lender of any Claims, such Claims shall be, and shall automatically be deemed to be, subject to the terms of this Agreement and, as applicable, the other Definitive Documentation, or (B) as a condition precedent to the effectiveness of any such Transfer, the transferee thereof shall have executed and delivered a Lender Joinder in accordance with Subsection 9.1(c), and (ii) the consummation of such Transfer would not be reasonably expected to have or result in a material adverse impact on, or delay or impair the consummation of the Transaction in any material respect, within any of the time frames contemplated by this Agreement and the Plan Funding Agreement. Thereafter, such purchaser, transferee, assignee or other relevant Person shall be deemed to be a Consenting Lender for purposes of this Agreement and the other applicable Definitive Documentation and shall be bound by all of the terms hereof and thereof, and the transferor Consenting Lender shall be deemed to, automatically as of the consummation of such Transfer, relinquish its rights (and be released from its obligations) under this Agreement solely to the extent of such transferred Claims, it being understood and agreed that no such Transfer shall impact, effect or

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alter the rights and obligations of the Parties under the other Definitive Documentation except to the extent expressly set forth therein.

(b) Any Transfer of any Claim that does not comply with the procedures set forth in Subsection 9.1(a) of this Agreement shall be deemed void ab initio.

(c) Any person that seeks to receive or acquire a portion of the Claims pursuant to a Transfer of such Claims by a Consenting Lender shall be required, as a condition to the effectiveness of such Transfer, to be bound by all of the terms of this Agreement and, as applicable, the other Definitive Documentation (a “Joining Lender Party”) by duly executing and delivering to the Company and each other Party a joinder in the form of Exhibit H hereto (the “Lender Joinder”). The Joining Lender Party shall thereafter be deemed to be a “Consenting Lender” and a Party for all purposes under this Agreement and, as applicable, the other Definitive Documentation.

(d) With respect to the Claims held by the Joining Lender Party upon consummation of any Transfer, the Joining Lender Party shall be deemed to have made, with respect to itself, the representations and warranties of a Consenting Lender set forth in Section 6 of this Agreement to the Company.

(e) Subject to Subsection 9.1(a), this Agreement shall in no way be construed to preclude any Consenting Lender from acquiring additional Claims; provided that, any such Claims shall automatically be deemed to be subject to the terms of this Agreement and the other Definitive Documentation.

(f) Notwithstanding Section 9.1(a): (i) a Consenting Lender may Transfer any right, title, or interest in its Claims to an entity that is acting in its capacity as a Qualified Marketmaker without the requirement that the Qualified Marketmaker be or become a Consenting Lender only if such Qualified Marketmaker has purchased such Claims with a view to immediate resale of such Claims (by purchase, sale, assignment, transfer, participation or otherwise) as soon as reasonably practicable, and in no event later than the earlier of (A) three (3) business days prior to any voting deadline with respect to the Plan (solely if such Qualified Marketmaker acquires such Claims prior to such voting deadline) and (B) ten (10) business days of its acquisition to a transferee Consenting Lender that is or becomes a Consenting Lender (by executing and delivering the Lender Joinder in accordance with Subsection 9.1(c)); and (ii) to the extent that a Consenting Lender is acting solely in its capacity as a Qualified Marketmaker, it may Transfer any right, title, or interest in any Claims that such Consenting Lender, acting solely in its capacity as a Qualified Marketmaker, acquires from a holder of such Claims who is not a Consenting Lender without the requirement that the transferee be or become a Consenting Lender with respect to such Claims. Notwithstanding the foregoing, (w) if at the time of a proposed Transfer of any Claim to the Qualified Marketmaker in accordance with the foregoing, the date of such proposed Transfer is within three (3) business days of the voting deadline with respect to the Plan, the proposed transferor Consenting Lender shall first vote, and shall be deemed to have voted, such Claim in accordance with the requirements of Section 1.1(b) hereof prior to any Transfer or (x) if, after a Transfer in accordance with this Section 9.1(f), a

21

Qualified Marketmaker is holding a Claim on any date within three (3) business days of the voting deadline with respect to the Plan, such Qualified Marketmaker shall vote, and shall be deemed to have voted, such Claim in accordance with the requirements of Section 1.1(b) hereof as if it were a Consenting Lender and the definitive documentation in respect of any Transfer thereto shall require the foregoing, in form and substance reasonably acceptable to the Plan Investor, as a condition to any such Transfer. For these purposes, a “Qualified Marketmaker” means an entity that: (y) holds itself out to the market as standing ready in the ordinary course of its business to purchase from customers and sell to customers claims against the Company and its Affiliates (including debt securities or other debt) or enter into with customers long and short positions in claims against the Company and its Affiliates (including debt securities or other debt), in its capacity as a dealer or market maker in such claims against the Company and its Affiliates; and (z) is in fact regularly in the business of making a market in claims against issuers or borrowers (including debt securities or other debt). For avoidance of doubt, J.P. Morgan Chase & Co. together with its Affiliates, other than Highbridge Capital Management, LLC and its subsidiaries shall be deemed to be a Qualified Marketmaker.

9.2 Certain Additional Chapter 11 Related Matters.

The Company shall provide draft copies of all motions, applications and other documents that relate in any material respect to implementation of the Transaction (including all “first day” and “second day” motions and orders, the Plan, the Disclosure Statement, ballots and other Solicitation Materials in respect of the Plan, any proposed amended version of the Plan and/or the Disclosure Statement, the Confirmation Order and any other Definitive Documentation) it intends to file with the Bankruptcy Court to counsel for the Plan Investor and each Consenting Class, at least three (3) business days prior to the date when the Company intends to file any such pleading or other document with the Bankruptcy Court (provided that if delivery of such motions, orders or materials (other than the Plan, the Disclosure Statement or Confirmation Order) at least three (3) business days in advance is not reasonably practicable, such motion, application or other document shall be delivered as far in advance of such date of filing as is reasonably practicable) and, in each case shall, prior to the filing thereof, consult in good faith with such counsel regarding the form and substance of any such proposed filing.

9.3 No Third Party Beneficiaries.

This Agreement shall be solely for the benefit of the Company, the Plan Investor, and each Consenting Lender. No other person or entity shall be a third party beneficiary.

9.4 Entire Agreement.

This Agreement and the other Definitive Documentation, including exhibits and annexes hereto and thereto, constitutes the entire agreement of the Parties with respect to the subject matter hereof and thereof, including exhibits and annexes hereto and thereto, and supersedes all other prior negotiations, agreements and understandings, whether written or oral, among the Parties with respect to such subject; provided, however, that, subject to the terms and conditions of the Plan

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Funding Agreement, any confidentiality agreement executed by any Party shall survive this Agreement and shall continue in full force and effect, subject to the terms thereof.

9.5 Counterparts.

This Agreement may be executed in one or more counterparts, each of which shall be deemed an original and all of which shall constitute one and the same agreement. Delivery of an executed signature page of this Agreement by email or facsimile transmission shall be effective as delivery of a manually executed counterpart hereof.

9.6 Settlement Discussions.

This Agreement, the other Definitive Documentation and the Plan are part of a proposed settlement of disputes among certain of the Parties hereto. Nothing herein shall be deemed to be an admission of any kind. Pursuant to Federal Rule of Evidence 408 and any applicable state rules of evidence, this Agreement and the other Definitive Documentation and all negotiations relating hereto and thereto shall not be admissible into evidence in any proceeding other than a proceeding to enforce the terms of this Agreement, such other Definitive Documentation or in connection with the confirmation of the Plan.

9.7 Reservation of Rights.

In the event that, (x) the Transaction is not consummated in accordance with the terms and conditions hereof, the Plan Funding Agreement and the other Definitive Documentation, (y) a Termination Date occurs or (z) this Agreement is otherwise validly terminated for any reason, each Party fully reserves any and all of its respective rights, remedies and interests (if any) under the Credit Documents, the Plan Funding Agreement, the PFA Order, applicable law and in equity.

9.8 Governing Law; Waiver of Jury Trial.

(a) The Parties waive all rights to trial by jury in any jurisdiction in any action, suit, or proceeding brought to resolve any dispute between or among the Parties arising out of this Agreement, whether sounding in contract, tort or otherwise.

(b) This Agreement shall be governed by and construed in accordance with the Bankruptcy Code and the laws of the State of New York, without regard to any conflicts of law provision which would require the application of the law of any other jurisdiction. By its execution and delivery of this Agreement, each Party irrevocably and unconditionally agrees for itself that, subject to Subsection 9.8(c), any legal action, suit or proceeding brought by or against it with respect to any matter under or arising out of or in connection with this Agreement or for recognition or enforcement of any judgment rendered in any such action, suit or proceeding, shall be brought exclusively in any state or federal court of competent jurisdiction in New York County, State of New York, and by execution and delivery of this Agreement, each of the Parties hereby: (i) irrevocably accepts and submits itself to the exclusive jurisdiction of such court, generally and unconditionally, with respect to any such action, suit or proceeding; and (ii) waives any objection to laying venue in any such action, suit or proceeding.

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(c) Notwithstanding the foregoing, if the Bankruptcy Cases are commenced, nothing in Subsections 9.8(a)-9.8(b) shall limit the authority of the Bankruptcy Court, as applicable, to hear any matter related to or arising out of this Agreement, and each Party irrevocably and unconditionally consents to the jurisdiction and venue of the Bankruptcy Court, as applicable, to hear and determine such matters during the pendency of the Bankruptcy Cases.

9.9 Successors.

This Agreement is intended to bind the Parties and inure to the benefit of the Consenting Lenders, the Plan Investor and the Company and each of their respective successors and permitted assigns. Except in accordance with the express terms of this Agreement, no Party shall assign this Agreement or any rights or obligations hereunder without the prior written consent of the Company, the Plan Investor and the Required Consenting Lenders. For the avoidance of doubt, nothing contained in this Section 9.9 shall be deemed to permit any Transfer of any Claims other than in accordance with the terms of this Agreement.  

9.10 Acknowledgment of Counsel; Interpretation.

(a) Each of the Parties acknowledges that it is sophisticated and has been represented by counsel (or had the opportunity to and waived its right to do so) in connection with the negotiation and execution of this Agreement and the Transaction. Accordingly, the Parties do not intend that any rule of law or any legal decision or rules relating to the interpretation of contracts against the drafter of any particular clause or that would otherwise provide any Party with a defense to the enforcement of the terms of this Agreement against such Party based upon lack of legal counsel shall apply to this Agreement and each Party hereby expressly waives any such application or defense. Furthermore, prior drafts of this Agreement and any of the documents executed and delivered in connection herewith and the fact that any clauses have been added, deleted or otherwise modified from any prior drafts of this Agreement or any of the documents executed and delivered in connection herewith shall not be used as a rule of construction or otherwise constitute evidence of the intent of the Parties or the parties thereto, and no presumption or burden of proof shall arise favoring or disfavoring any such Party or parties by virtue of such prior drafts.

(b) When a reference is made in this Agreement to a Section, Schedule, Annex or Exhibit, such reference will be to a Section of, or a Schedule, Annex or Exhibit to, this Agreement unless otherwise indicated. Whenever the words “include,” “includes” or “including” are used in this Agreement, they will be deemed to be followed by the words “without limitation.” The words “hereof,” “herein” and “hereunder” and words of similar import when used in this Agreement will refer to this Agreement as a whole and not to any particular provision of this Agreement. The word “or” when used in this Agreement may have a disjunctive and not alternative meaning (i.e., where two items or qualities are separated by the word “or”, the existence of one item or quality shall not be deemed to be exclusive of the existence of the other and, as the context may require, the word “or” may be deemed to include the word “and”). All terms used herein with initial capital letters have the meanings ascribed to them herein. The definitions contained in this Agreement

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are applicable to the singular as well as the plural forms of such terms and to the masculine as well as to the feminine and neuter genders of such term. Unless otherwise expressly provided herein, any agreement, instrument or statute defined or referred to herein, or in any agreement or instrument that is referred to herein, means such agreement, instrument or statute as from time to time amended, modified or supplemented, including (in the case of agreements or instruments) by waiver or consent and (in the case of statutes) by succession of comparable successor statutes and references to all attachments thereto and instruments incorporated therein. All Exhibits, Annexes and Schedules annexed hereto or referred to herein are incorporated in and made a part of this Agreement as if set forth in full herein. If any time period for giving notice or taking action hereunder expires on a day that is not a business day, the applicable time period shall automatically be extended to the business day immediately following such day.

9.11 Amendments, Modifications, Waivers.

(a) Subject to the terms set forth in this Agreement, including in Section 9.1(b), this Agreement (including, without limitation, the Plan, the Plan Funding Agreement and the Disclosure Statement) may only be modified, amended or supplemented, and any of the terms thereof may only be waived with (i) in the case of any such modification, amendment or supplementation, the written consent by each of (a) the Company, (b) the Required Consenting Lenders, and (c) the Plan Investor (each of the Required Consenting Bridge Lenders/Noteholders, the Required Consenting Intercompany Lenders and the Plan Investor a “Required Party” and shall be referred to herein collectively as the “Required Parties”), and (ii) in the case of a waiver, by the Party or Parties waiving rights pursuant to the terms of such waiver, except that any waiver by the Required Consenting Bridge Lenders/Noteholders or the Required Consenting Intercompany Lenders shall be binding on all Consenting Noteholders and all Consenting Bridge Lenders and any waiver by the Required Consenting Intercompany Lenders shall be binding on all lenders under the Intercompany Credit Agreement; provided that, if the modification, amendment, supplement or waiver at issue adversely impacts the treatment or rights of any Consenting Lender (in its capacity as a Consenting Lender) in a materially different and materially disproportionate manner when compared to the effect thereof on other Consenting Lenders in its Consenting Class, the agreement in writing of such Consenting Lender whose treatment or rights are so adversely impacted shall also be required for such modification, amendment, supplement, or waiver to be effective with respect to such Consenting Lender; provided, further, that the waiver of a Termination Event arising from the breach by a Required Party of its obligations hereunder shall not require the consent of such breaching Required Party. If any ruling is made by the Panel that any provision of this Agreement is not permitted by the Takeover Code, such provision shall be given no effect. The Parties shall use reasonable efforts to replace such provision with a valid and enforceable provision which is acceptable to the Panel and carries out, as closely as possible, the intentions of the parties.

(b) Without prejudice to the other provisions of this Agreement, each of the Parties agrees to use its respective reasonable best efforts to take or cause to be taken, in

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good faith, all appropriate actions (including any amendments, modifications and supplements to this Agreement, the Plan and Disclosure Statement and the Plan Funding Agreement) as is reasonably necessary, appropriate and advisable to memorialize and effectuate the Transaction, including, without limitation, to obtain Bankruptcy Court confirmation of the Plan pursuant to a final order of the Bankruptcy Court; provided that no Party shall have any obligation to take any action or otherwise agree to any amendment, modification or supplement that (i) creates any additional material obligation on such Party or (ii) adversely affects in any material respect the treatment, obligations or rights of such Party (it being agreed that, for the avoidance of doubt, any change to the Plan that results in a diminution of the value of the property to be received by a Consenting Class under the Plan or alters the form in which such value is to be received by a Consenting Class under the Plan shall be deemed to adversely affect such Consenting Class or that results in a diminution of the value and/or increase in the liabilities of the Plan Investor shall be deemed to adversely affect the Plan Investor) whether such change is made directly to the treatment of a Consenting Class, the treatment of another Consenting Class, any term or provision relating to or impacting the Plan Investor or otherwise. Notwithstanding the foregoing, the Company may amend, modify or supplement the Plan and Disclosure Statement, from time to time, with the consent of any Required Parties (such consent not to be unreasonably withheld, conditioned or delayed), to cure any non-material ambiguity, defect (including any technical defect), inconsistency or clerical error; provided that any such amendment, modification or supplement does not adversely affect the rights, interests or treatment of any such Plan Support Parties under such Plan and Disclosure Statement.

9.12 Professional Fees.

The Company agrees to reimburse, in addition to its own advisors, all of the reasonable and documented out-of-pocket fees and expenses incurred by the Consenting Noteholders and the Consenting Bridge Lenders of Latham & Watkins, LLP and Ducera Partners LLC, under their respective engagement letters as in effect on the date hereof, in connection with the Transaction and implementation of the Plan (including, without limitation, fees and expenses incurred after the Petition Date); provided that only those fees and expenses in respect of Ducera Partners LLC that the Company shall be required to reimburse shall be those incurred as a result of the services expressly contemplated by the engagement letter by and between Ducera Partners LLC and Highbridge MSF International Ltd. (f/k/a 1992 MSF International Ltd.), 1992 Tactical Credit Master Fund, L.P., Athyrium Opportunities II Acquisition LP, and Athyrium Opportunities III Acquisition LP, dated as of December 7, 2018 (without giving effect to any subsequent amendment, restatement, supplement or modification thereof following such date), a true, complete and correct copy of which has been provided to the Company prior to the date hereof, in each case without the need to file any interim or final fee applications with the Bankruptcy Court, subject to the Company obtaining Bankruptcy Court approval of any postpetition payments pursuant to the Interim CC Order and the Final DIP Order; provided, however, that if this Agreement shall be terminated due to the breach by any Consenting Noteholder or Consenting Bridge Lender of its representations, warranties, covenants, undertakings or obligations hereunder or under any other Definitive Documentation, then the Company shall not be required to pay the expenses referred to in the preceding sentences of this Section 9.12 (except to the extent provided

26

in the Interim CC Order or the Final DIP Order, as applicable). For the avoidance of doubt, but subject to the foregoing, the Company’s obligation to pay professional fees and expenses pursuant to this Section 9.12 shall be unaffected by, and shall survive, termination of this Agreement; provided, however, that except as otherwise provided in the Interim CC Order or the Final DIP Order, as applicable, the Company shall only be obligated pursuant to this Agreement to pay such fees and expenses incurred through the Termination Date. For the avoidance of doubt, Novelion and the Plan Investor shall bear (and the Company shall have no liability in respect of other than as set forth in the Exclusivity Agreement) their own costs and expenses incurred in connection with the Transaction, including their respective professional fees incurred in connection with the Transaction, but without limitation of any rights of the Plan Investor to receive reimbursement of its costs and expenses (or a portion thereof) from the Company pursuant to the terms of the Plan Funding Agreement and, the Exclusivity Agreement. In addition, on the effective date of the Plan, the Company shall pay all outstanding reasonable and documented fees and expenses of the Convertible Notes Trustee (including the fees and expenses of its outside counsel and other professionals), regardless of whether such fees and expenses were incurred before or after the Petition Date.  

9.13 Disclosure Letter References.

The Parties agree that the disclosure set forth in any particular section or subsection of the disclosure schedules provided in connection with this Agreement and/or the Plan Funding Agreement (the “Disclosure Schedules”) or deemed disclosed as exceptions pursuant to the terms of the Plan Funding Agreement shall be deemed to be an exception to (or, as applicable, a disclosure for purposes of) (a) the representations and warranties (or covenants, as applicable) of the disclosing Party that are set forth in this Agreement or the Plan Funding Agreement; and (b) any other representations and warranties (or covenants, as applicable) of the disclosing party that are set forth in this Agreement or the Plan Funding Agreement.  

9.14 Severability of Provisions.

If any provision of this Agreement for any reason is held to be invalid, illegal or unenforceable in any respect, that provision shall not affect the validity, legality or enforceability of any other provision of this Agreement.  

9.15 Headings.

The headings of the sections, paragraphs and subsections of this Agreement are inserted for convenience only and shall not affect the interpretation hereof.

9.16 Certain Limitations.

Each of the Parties acknowledges and agrees that, notwithstanding anything in this Agreement to the contrary, it shall have no, and agrees not to pursue any, recourse against (a) Novelion for breaches or threatened breaches hereunder by the Company hereunder or thereunder, or (b) the Company for breaches or threatened breaches hereunder by Novelion hereunder or thereunder.  

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9.17 Subsidiaries Bound.

Novelion shall cause any and all of its subsidiaries (other than Aegerion and its subsidiaries) to comply with the terms of this Agreement and the other Definitive Documentation as if they were a party hereto and had the obligations of Novelion hereunder, and at the request of the Company, Novelion shall cause such subsidiaries (other than Aegerion and its subsidiaries) to sign reasonable documentation (including joinder agreements) as may be required to effect the foregoing.  

9.18 Notices.

Any notices required or elected to be given hereunder must be in writing and may be served in person or by overnight mail or by electronic mail upon the respective parties as follows (or to such other addresses as may hereafter be designated in accordance with the terms hereof):  

if to the Company:  

c/o Aegerion Pharmaceuticals, Inc. 

245 First Street

Riverview II, 18^th Floor

Cambridge, MA 02142 

Attention: John R. Castellano

Email: JCastellano@alixpartners.com

with a copy to:  

Willkie Farr & Gallagher LLP 

787 Seventh Avenue 

New York, NY 10019 

Attention: Russell L. Leaf, Esq.; Jared Fertman, Esq.; Paul V. Shalhoub, Esq.; and

 Andrew S. Mordkoff, Esq.

Email: rleaf@willkie.com; jfertman@willkie.com; pshalhoub@willkie.com;

 amordkoff@willkie.com

if to the Consenting Lenders:  

as set forth in each signature page

with a copy to:

(For Novelion)

Goodwin Procter LLP 

The New York Times Building 

620 Eighth Avenue 

New York, NY 10018 

Attention: Gregory Fox, Esq.; and Jacqueline Mercier, Esq.

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Email: GFox@goodwinlaw.com; JMercier@goodwinlaw.com

(For certain of the holders of loans under the Bridge Credit Agreement and/or the 

Convertible Notes Indenture that are Parties as of the date hereof)

Latham & Watkins LLP 

330 North Wabash Avenue, Suite 2800 

Chicago, IL 60611 

Attention: Richard A. Levy, Esq.

Email: Richard.Levy@lw.com

and

King & Spalding LLP 

444 West Lake Street 

Suite 1650 

Chicago, IL 60606 

Attention: Matthew L. Warren, Esq.

Email: mwarren@kslaw.com

if to the Plan Investor:

Amryt Pharma plc 

90 Harcourt Street 

Dublin 2, Ireland 

Attention: Joe Wiley

Email: joe.wiley@ amrytpharma.com

with a copy to:

Gibson, Dunn & Crutcher LLP 

200 Park Avenue 

New York, NY 10166 

Attention: George P. Stamas, Esq.; William B. Sorabella, Esq.; Matthew J. Williams,

Esq.; and Jason Zachary Goldstein, Esq.

Email: GStamas@gibsondunn.com; WSorabella@gibsondunn.com;

MJWilliams@gibsondunn.com; JGoldstein@gibsondunn.com

[Signature pages follow]

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IN WITNESS WHEREOF, the parties hereto have caused this Amendment to be executed and delivered as of the date first above written.        

AEGERION PHARMACEUTICALS, INC.
By:	/s/ John R. Castellano
	Name:	John R. Castellano
	Title:	Chief Restructuring Officer

AEGERION PHARMACEUTICALS HOLDINGS, INC.
By:	/s/ John R. Castellano
	Name:	John R. Castellano
	Title:	Chief Restructuring Officer

NOVELION THERAPEUTICS INC.
By:
	Name:
	Title:

AMRYT PHARMA PLC
By:
	Name:
	Title:

[Signature Page to Restructuring Support Agreement]

IN WITNESS WHEREOF, the parties hereto have caused this Agreement to be executed and delivered as of the date first above written.

AEGERION PHARMACEUTICALS, INC.
By:
	Name:	John R. Castellano
	Title:	Chief Restructuring Officer

AEGERION PHARMACEUTICALS HOLDINGS, INC.
By:
	Name:	John R. Castellano
	Title:	Chief Restructuring Officer

NOVELION THERAPEUTICS INC.
By:	/s/ Ben Harshbarger
	Name:	Ben Harshbarger
	Title:	Interim CEO

AMRYT PHARMA PLC
By:
	Name:
	Title:

[Signature Page to Restructuring Support Agreement]

IN WITNESS WHEREOF, the parties hereto have caused this Agreement to be executed and delivered as of the date first above written.

AEGERION PHARMACEUTICALS, INC.
By:
	Name:	John R. Castellano
	Title:	Chief Restructuring Officer

AEGERION PHARMACEUTICALS HOLDINGS, INC.
By:
	Name:	John R. Castellano
	Title:	Chief Restructuring Officer

NOVELION THERAPEUTICS INC.
By:
	Name:
	Title:

AMRYT PHARMA PLC
By:	/s/ Joe Wiley
	Name:	Joe Wiley
	Title:	CEO

[Signature Page to Restructuring Support Agreement]

ATHYRIUM OPPORTUNITIES II ACQUISITION LP
By: Athyrium Opportunities Associates II LP, its general partner
By: Athyrium GP Holdings LLC, its general partner
By:	/s/ Andrew C. Hyman
	Name:	Andrew C. Hyman
	Title:	Authorized Signatory

ATHYRIUM OPPORTUNITIES III ACQUISITION LP
By: Athyrium Opportunities Associates III LP, its general partner
By: Athyrium Opportunities Associates III GP LLC, its general partner
By:	/s/ Andrew C. Hyman
	Name:	Andrew C. Hyman
	Title:	Authorized Signatory

[Signature Page to Restructuring Support Agreement]

HIGHBRIDGE MSF INTERNATIONAL LTD.
By:	/s/ Jonathan Segal
	Name:	Jonathan Segal
	Title:	Managing Director

1992 TACTICAL CREDIT MASTER FUND, L.P.
By:	/s/ Jonathan Segal
	Name:	Jonathan Segal
	Title:	Managing Director

HIGHBRIDGE SCF SPECIAL SITUATIONS SPV, L.P.
By:	/s/ Jonathan Segal
	Name:	Jonathan Segal
	Title:	Managing Director

HIGHBRIDGE SCF LOAN SPV, L.P.
By:	/s/ Jonathan Segal
	Name:	Jonathan Segal
	Title:	Managing Director

[Signature Page to Restructuring Support Agreement]

Whitebox Relative Value Partners, LP
By:	/s/ Chris Hardy
	Name:	Chris Hardy
	Title:	Chief Compliance Officer

Whitebox GT Fund, LP
By:	/s/ Chris Hardy
	Name:	Chris Hardy
	Title:	Chief Compliance Officer

Whitebox Multi-Strategy Partners, LP
By:	/s/ Chris Hardy
	Name:	Chris Hardy
	Title:	Chief Compliance Officer

Pandora Select Partners, LP
By:	/s/ Chris Hardy
	Name:	Chris Hardy
	Title:	Chief Compliance Officer

[Signature Page to Restructuring Support Agreement]

NINETEEN77 GLOBAL MULTI-STRATEGY ALPHA MASTER LIMITED
By:	UBS O’Connor LLC, its investment adviser
By:	/s/ Andrew Hollenbeck
	Name:	Andrew Hollenbeck
	Title:	Managing Director

By:	/s/ James Del Medico
	Name:	James Del Medico
	Title:	Executive Director

[Signature Page to Restructuring Support Agreement]

NINETEEN77 GLOBAL CONVERTIBLE BOND MASTER LIMITED
By:	UBS O’Connor LLC, its investment adviser
By:	/s/ Andrew Hollenbeck
	Name:	Andrew Hollenbeck
	Title:	Managing Director

By:	/s/ James Del Medico
	Name:	James Del Medico
	Title:	Executive Director

[Signature Page to Restructuring Support Agreement]

SCHEDULE 6.4

Ownership Claims and Interests

Consenting Lender	Claim/Interest	Holdings (USD$) as of May 20, 2019
Athyrium Opportunities II Acquisition LP	Convertible notes (face)	95,400,000
Athyrium Opportunities II Acquisition LP	Roll-up (principal)	12,600,000
Athyrium Opportunities II Acquisition LP	Secured debt (principal)	1,700,000
Athyrium Opportunities III Acquisition LP	Convertible notes (face)	22,337,000
Athyrium Opportunities III Acquisition LP	Roll-up (principal)	3,000,000
Athyrium Opportunities III Acquisition LP	Secured debt (principal)	33,000,000
Highbridge MSF International  Ltd.	Roll-up	4,333,745.23
Highbridge MSF International  Ltd.	Convertible notes (face)	21,300,000.00
1992 Tactical Credit Master Fund, L.P.	Roll-up	2,260,404.09
1992 Tactical Credit Master Fund, L.P.	Convertible notes (face)	19,900,000.00
Highbridge SCF Special Situations SPV, L.P.	Convertible notes (face)	10,900,000.00
Highbridge SCF Loan SPV, L.P.	Secured debt	15,381,922.67
Nineteen77 Global Multi-Strategy Alpha Master Limited	Convertible notes (face)	25,000,000
Nineteen77 Global Convertible Bond Master Limited	Convertible notes (face)	1,000,000
Whitebox Relative Value Partners, LP	Convertible notes (face)	2,880,000.00
Whitebox GT Fund, LP	Convertible notes (face)	288,000.00
Whitebox Multi-Strategy  Partners, LP	Convertible notes (face)	2,808,000.00
Pandora Select Partners, LP	Convertible notes (face)	1,224,000.00

SCHEDULE 6.6(a)

NOT FOR RELEASE, PUBLICATION OR DISTRIBUTION, IN WHOLE OR IN PART, DIRECTLY OR INDIRECTLY, IN, INTO OR FROM THE UNITED STATES (INCLUDING ITS TERRITORIES AND POSSESSIONS, ANY STATE OF THE UNITED STATES OR THE DISTRICT OF COLUMBIA), AUSTRALIA, CANADA, JAPAN, THE REPUBLIC OF SOUTH AFRICA OR ANY OTHER JURISDICTION IN WHICH SUCH RELEASE, PUBLICATION OR DISTRIBUTION WOULD BE UNLAWFUL OR WOULD CONSTITUTE A VIOLATION OF THE RELEVANT LAWS OR REGULATIONS OF THAT JURISDICTION (“RESTRICTED JURISDICTIONS”). THIS ANNOUNCEMENT IS FOR INFORMATION PURPOSES ONLY AND DOES NOT ITSELF CONSTITUTE AN OFFER FOR SALE OR SUBSCRIPTION OF ANY SECURITIES IN THE COMPANY.

PLEASE SEE THE IMPORTANT NOTICE AT THE END OF THIS ANNOUNCEMENT.

This announcement contains inside information within the meaning of the EU Market Abuse Regulation 596/2014.

May 21 2019
AIM:AMYT
Euronext Growth: AYP

Amryt Pharma plc
(“Amryt” or the “Company”)

RECOMMENDED ACQUISITION OF AEGERION PHARMACEUTICALS

-     Creates a rare disease business with two approved products – lomitapide (Lojuxta^® I Juxtapid^®) and metreleptin (Myalept^® I Myalepta^®)

-     $136.5m of 2018 built-in revenues, multiple growth opportunities, and a robust pipeline for value creation

-     Reunites the lomitapide franchise and transforms Amryt into a global player in the orphan disease market

-     Capitalizes on Amryt management’s unique knowledge of Aegerion’s assets and European commercialization capabilities

-     Presents the opportunity for meaningful expense synergies - $25m-$40m in 2020

-     Pre-money implied transaction equity valuations: Amryt $120m and Aegerion $190.7m

-     Contingent Value Rights (“CVRs”) will be issued to Amryt stakeholders that could result in the payment of up to an additional $85m (settled in cash or stock) based on certain AP101 milestones being achieved

-     Amryt plans to raise $60m in equity concurrent with closing of the Transaction and certain Aegerion bondholders have agreed to backstop this equity raise

-     Establishes an appropriate capital structure and liquidity profile to drive growth and create value

-     Transaction already endorsed by 34.3% of Amryt’s shareholders and in excess of 67% of Aegerion’s bondholders

-     Management will host a conference call for analysts and investors today at 1330 BST (0830 EDT) – dial-in details below

Amryt, a biopharmaceutical company focused on rare and orphan diseases, today announces that it has reached agreement to acquire (the “Transaction”) Aegerion Pharmaceuticals (“Aegerion”), a subsidiary of Novelion Therapeutics Inc. - NASDAQ:NVLN - (“Novelion”). The Transaction has been unanimously approved and recommended by the Boards of Amryt, Aegerion and Novelion.

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Transaction Rationale

The Company has built a diversified portfolio of drugs to treat patients with rare and orphan diseases through the acquisition of its AP101 and AP103 product lines and through the in-licencing of the Lojuxta^® product line. The Transaction is in line with the Company’s strategy to expand its product portfolio to enhance shareholder value.

The Transaction will put Amryt on the path to creating a rare and orphan disease company with a diversified offering of multiple commercial and development stage assets and will provide it with scale to support further growth. The Transaction will give Amryt an expanded commercial footprint to market two US and EU approved products, lomitapide (Juxtapid^® (US/ROW) I Lojuxta^® (EU)) and metreleptin (Myalept^® (US) I Myalepta^® (EU)). Amryt’s leadership team already has a deep knowledge of both these products and since December 2016 has successfully commercialized Lojuxta^® across Europe and the Middle East.

Dr. Joe Wiley, Chief Executive Officer of Amryt, commented: “The acquisition of Aegerion accelerates our ambition to become a global leader in treating rare conditions to help improve the lives of patients where there is a high unmet medical need. By delivering two substantial revenue-generating products and an enhanced pipeline of promising development opportunities, this will significantly strengthen our growth in highly attractive markets globally. Amryt has a unique insight into both Aegerion and its products, through our commercial success with Lojuxta^® and given that many of our senior management team previously worked at Aegerion.”

“With this Transaction we can continue the strong growth trajectory already underway with Lojuxta^® in Europe on a global scale. It also delivers metreleptin, another highly compelling commercial rare disease product alongside an established commercial footprint in the US and internationally. This transformational deal provides Amryt with the financial flexibility to fully execute our medium-term growth plans, and is expected to deliver significant shareholder returns.”

Transaction Highlights:

• Amryt has agreed to acquire Aegerion in an all-paper transaction

• The combined group had 2018 pro-forma combined revenues of $136.5m

• Pre-money implied transaction equity valuations: Amryt $120m and Aegerion $190.7m

• Contingent Value Rights (“CVRs”) will be issued to Amryt stakeholders that could result in the payment of up to $85m (settled in cash or stock) based on certain AP101 milestones being achieved

• Amryt plans to raise $60m in equity concurrent with closing of the Transaction and certain Aegerion bondholders have agreed to backstop this equity raise

• This equity raise will be placed at a 20% discount to the implied transaction equity value

• Aegerion’s balance sheet is to be restructured through a US Chapter 11 process prior to Amryt acquiring Aegerion - Aegerion will continue to operate as usual during the Chapter 11 process

• New loan facilities for the combined group will be put in place, and the key terms of such facilities have been agreed - Amryt’s existing European Investment Bank facility is to be repaid

• The combined group’s global HQ will be in Dublin, Ireland with its US HQ in Boston, Massachusetts

• Enlarged group to be re-admitted to AIM and Euronext Growth on closing with a planned dual-listing on NASDAQ

• Transaction already endorsed by 34.3% of Amryt shareholders and in excess of 67% of Aegerion’s bondholders

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Rich Commercial Portfolio & Development Pipeline with a Global Footprint

• Amryt will have a differentiated, diverse, global offering of multiple commercial and development stage rare disease assets, including:

• Two high-value commercial assets with multiple development opportunities in complementary global markets

○ Lomitapide (Juxtapid^®(US)/Lojuxta^®(EU)) for the treatment of adult homozygous familial hypercholesterolemia (HoFH)

○ Metreleptin (Myalept^®(US)IMyalepta^® (EU)), a leptin hormone replacement therapy, approved in the US for Generalised Lipodystrophy (GL), and recently in Europe for GL and Partial Lipodystrophy (PL)

• Additional near-term potential commercial opportunities for a broadened Amryt portfolio of products

○ Metreleptin as a potential treatment for partial lipodystrophy (PL) in the US

○ Lomitapide (Juxtapid^®/Lojuxta^®) as a potential treatment for familial chylomicronemia syndrome (FCS)

○ A lead development asset (AP101) for Epidermolysis Bullosa (“EB”), a >$1bn market opportunity in a pivotal Phase 3 trial, which recently reported positive unblinded interim efficacy analysis results and is anticipated will be fully enrolled by end of H2 2019

○ Novel gene therapy platform (AP103) which offers a potential treatment for patients with EB and other topical indications

Value Creation

• Enhanced scale of combined group expected to drive revenue growth and future profitability

• Expected to deliver meaningful operational synergies over the medium term - the Directors believe, on the work undertaken to date, that the enlarged group can deliver operational synergies of between $25m and $40m in 2020, rising further in 2021

• Amryt’s deep knowledge of Aegerion products is key to driving growth

• Reunification of lomitapide brands provides potential to replicate success of Lojuxta^® in Europe with Juxtapid^® in the US

• Opportunity to grow Myalepta^® revenues with broader reach across EU to accelerate recent launch

• Delivers a ready-made commercial US infrastructure in advance of anticipated launch of AP101

• Recapitalized business well-positioned to drive pipeline value

• Planned NASDAQ listing to drive liquidity and investor reach

• Opportunity for corporate restructuring to drive additional value

Board & Management

• Team led by Dr Joe Wiley, CEO of Amryt

• Strong international management with significant industry experience

• Revised Board composition, on closing of the Transaction, consisting of CEO and six Non-Executive Directors

• New Board to be appointed on closing

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Ben Harshbarger, Novelion’s (parent company of Aegerion) Interim Chief Executive Officer, said,“The combination of Amryt and Aegerion will create a financially stronger and well-capitalized rare disease company with two commercial products and a pipeline of late stage rare disease products. Amryt’s executive management team has the depth of experience to commercialize Aegerion’s marketed products, as demonstrated by its ability to grow sales of Lojuxta^® in the European market, to develop and, if approved, commercialize Amryt’s late stage product candidate, AP101, and to pursue additional potential indications for metreleptin and lomitapide.”

The Transaction constitutes a reverse takeover of the Company under the Euronext Growth Rules and AIM Rules and requires shareholder approval and the publication of an AIM and Euronext Growth Admission Document (the “Admission Document”) with details of the Enlarged Group. Trading in Amryt’s shares will be suspended on both the AIM Market and the Euronext Growth Market with immediate effect until the Admission Document has been published. The Transaction is also conditional on the UK Takeover Panel waiving the obligation on certain lenders of Aegerion to make a general offer under Rule 9 of The UK Takeover Code, and on independent Amryt shareholder approval being obtained for such waiver and whitewash.

MTS Securities, LLC is serving as financial advisor and Gibson, Dunn & Crutcher LLP is serving as legal advisor to Amryt in this transaction. Shore Capital is acting as financial advisor, NOMAD and Joint Broker to Amryt. Stifel Nicolaus Europe Limited are Joint Broker to Amryt. Davy is acting as Euronext Growth Advisor and Joint Broker to Amryt. Moelis & Co LLC is serving as financial advisor to Aegerion.

Conference Call Details

Management will host a conference call for analysts today at 1330 BST (0830 EDT). Dial in details:

Conference ID: 3387304

From the UK/International: +44 (0) 2071 928000 I 0800 376 7922

From Ireland: (01) 431 9615 I 1800 936148

From the US: +1 631 510 7495 I 1 866 966 1396

A recording of the call will be available from 1830 (BST) today, please email ir@amrytpharma.com for access details. The presentation for today’s call will be available to download shortly before the call commences at https://www.amrytpharma.com/newsroom/

Enquiries:

Amryt Pharma plc	+353 (1) 518 0200
Dr. Joe Wiley, CEO
Rory Nealon, CFO/COO
Shore Capital	+44 (0) 20 7408 4090
Financial Advisor, NOMAD and Joint Broker
Edward Mansfield, Mark Percy, Daniel Bush
Stifel	+44 (0) 20 7710 7600
Joint Broker
Jonathan Senior, Ben Maddison
Davy	+353 (1) 679 6363
Euronext Growth Advisor and Joint Broker
John Frain, Daragh O’Reilly

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Consilium Strategic Communications	+44 (0) 20 3709 5700
Amber Fennell, Matthew Neal, David Daley

About Amryt

Amryt is a biopharmaceutical company focused on developing and delivering innovative new treatments to help improve the lives of patients with rare or orphan diseases.

Lojuxta^® is an approved treatment for adult patients with the rare cholesterol disorder - Homozygous Familial Hypercholesterolaemia (“HoFH”). This disorder impairs the body’s ability to remove low density lipoprotein (“LDL”) cholesterol (“bad” cholesterol) from the blood, typically leading to abnormally high blood LDL cholesterol levels in the body from before birth - often ten times more than people without HoFH - and subsequent aggressive and premature narrowing and blocking of blood vessels. Lojuxta^® is indicated as an adjunct to a low-fat diet and other lipid-lowering medicinal products with or without LDL apheresis in adult patients with HoFH.

Amryt is the marketing authorisation holder and has an exclusive licence to sell Lojuxta^® (lomitapide) across the European Economic Area, Middle East and North Africa, Switzerland, Turkey, Israel, Russia, the Commonwealth of Independent States and the non-EU Balkan states.

Amryt’s lead development candidate, AP101, is a potential treatment for Epidermolysis Bullosa (“EB”), a rare and distressing genetic skin disorder affecting young children and adults for which there is currently no treatment. It is currently in Phase 3 clinical trials and recently reported positive unblinded interim efficacy analysis results and is anticipated will be fully enrolled by end of H2 2019. The European and US market opportunity for EB is estimated to be in excess of $1 billion.

In March 2018, Amryt in-licenced a pre-clinical gene-therapy platform technology, AP103, which offers a potential treatment for patients with Recessive Dystrophic Epidermolysis Bullosa, a subset of EB, and is also potentially relevant to other genetic disorders.

For more information on Amryt, please visit www.amrytpharma.com.

About Novelion Therapeutics and Aegerion Pharmaceuticals

Novelion, through its subsidiary Aegerion Pharmaceuticals, is a global biopharmaceutical company dedicated to developing and commercializing therapies that deliver new standards of care for people living with rare diseases. With a global footprint and an established commercial portfolio, including Myalept/a^® (metreleptin) and Juxtapid^® (lomitapide), their business is supported by differentiated treatments that treat severe and rare diseases.

Description of Transaction

• Amryt has agreed to acquire Aegerion in an all-paper transaction. On closing, the implied equity valuations of Amryt and Aegerion will be $120m and $190.7m respectively. Amryt stakeholders will also receive a Contingent Value Right (“CVR”) of up to $85m, in cash or stock, at the election of its board, subject to certain regulatory approval and commercialization milestones of its late-stage development product candidate, AP101.

• Amryt plans to raise $60m in new equity concurrent with the Transaction closing at a 20% discount to the implied transaction valuations. The proceeds from this financing will be used to continue to develop the combined group’s pipeline, to develop potential new indications for Amryt’s late

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stage product candidates, and to be used for general corporate purposes. Certain Aegerion bondholders have agreed to backstop this capital raise.

• Amryt, Aegerion and Aegerion’s key stakeholders have entered into a “Restructuring Support Agreement” pursuant to which Aegerion has filed for Chapter 11 in the United States and seek to consummate the Transaction through a plan of reorganization that has garnered the support of Aegerion’s key creditors and stakeholders. Pursuant to the plan of reorganization, upon Bankruptcy Court approval, Amryt will acquire the reorganized Aegerion in exchange for Amryt stock, which stock will be distributed, together with other consideration in the form of new debt, to certain Aegerion secured and unsecured creditors, including Aegerion’s convertible bond holders, certain unsecured creditors and Novelion. As a result, Aegerion will emerge from Chapter 11 after having discharged substantial pre-transaction liabilities and with a reorganized and streamlined capital structure that materially reduces its debt obligations.

• To facilitate a smooth entry into Chapter 11, Aegerion has arranged for financing to allow it to operate uninterrupted during the Chapter 11 process, which financing will be repaid in cash pre-closing or otherwise exchanged into the new $125m convertible notes referred to below. Aegerion’s bondholders have agreed to support this transaction and oppose other potential transactions to acquire Aegerion.

• $125 million of new 5% convertible notes will be issued. The notes will mature 5.5 years from closing and be convertible into equity of Amryt at a 20% premium to the implied transaction valuation. Aegerion’s existing $50 million (in principal) secured loan, held by certain funds managed by Athyrium Capital Management and Highbridge Capital Management, as well as Amryt’s existing €20m (in principal) secured loan, will be converted and/or refinanced into new first-lien secured debt of the Amryt Group, which will have a cash interest rate of 6.5% per annum and an additional 6.5% PIK (“Payment-in-kind”) interest rate and will mature 5 years from closing.

• In connection with the Transaction, it is proposed that a corporate reorganization of Amryt will be undertaken by way of a scheme of arrangement, pursuant to which a new Irish incorporated public company will become the new ultimate holding company of the combined group.

Governance & Management

Amryt will continue to be listed on the London Stock Exchange’s Alternative Investment Market, Euronext Growth Market in Dublin and after the Transaction will pursue a dual-listing on NASDAQ. Following the Transaction, Amryt’s global headquarters will be in Dublin, Ireland and its US headquarters will be in Boston, Massachusetts.

Upon the closing of the Transaction, the Amryt board will consist of seven Directors including Dr. Joe Wiley (CEO). The six Non-Executive Directors will be proposed as follows – two by Amryt and four by Athyrium Capital Management and Highbridge Capital Management (current Aegerion bondholders). The Chairperson of the Board will be proposed by Amryt and will be unaffiliated with Amryt, Novelion or Aegerion. All board appointments will be made by mutual consent. Amryt will continue to be led by its executive team, which will be supplemented by certain Aegerion executives on both a transitional and permanent basis.

Conditions of the Transaction - Closing of the Transaction is conditional, inter alia, on:

• US Bankruptcy Court approval of the plan of reorganization and all conditions precedent to consummation of the plan of reorganization having been satisfied or waived;

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• the receipt of all necessary regulatory approvals and confirmation of no injunction preventing consummation of the Transaction;

• the passing of all resolutions necessary in connection with the Transaction by the shareholders of Amryt, such resolutions to be set out in the Admission Document to be published by Amryt including in relation to a scheme of arrangement in connection with a corporate reorganization required to be undertaken in connection with the Transaction and the issuance of the CVRs;

• a waiver being granted by The Panel on Takeovers and Mergers of the obligations which may otherwise arise pursuant to Rule 9 of the Takeover Code for certain lenders of Aegerion to make a general offer to the Company’s shareholders for all the issued ordinary shares in the capital of the Company as a result of the distribution of Amryt shares to such lenders following the issuance thereof to the Company as contemplated pursuant to the Transaction, and such waiver being approved by the Company’s shareholders by a resolution duly passed by the requisite majority of Company’s shareholders entitled to vote on such resolution pursuant to the Takeover Code and any requirement or direction issued by The Panel on Takeovers and Mergers in connection therewith;

• consummation of the backstopped equity raise of $60m;

• the Restructuring Support Agreement not having terminated and remaining in full force and effect;

• re-admission of the enlarged group to trading on AIM;

• completion of the agreed new term loan financing and the issuance of certain new convertible notes by the reorganized Amryt Group; and

• certain other customary closing conditions.

Indicative Timetable

• Announcement of Transaction - 21 May 2019

• Publication of Admission Document - Early August 2019

• Shareholder Meeting - Late August 2019

• Launch of the Equity Fundraise - September 2019

• Scheme of Arrangement Completion - September 2019

• Closing of Aegerion’s Chapter 11 Bankruptcy - Early Q4 2019

• Completion of the Transaction and Equity Fundraise and re-Admission - Early Q4 2019

The above dates are indicative only and are subject to change

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IMPORTANT NOTICE

THIS ANNOUNCEMENT IS RESTRICTED AND IS NOT FOR PUBLICATION, RELEASE OR DISTRIBUTION, DIRECTLY OR INDIRECTLY, IN WHOLE OR IN PART, IN OR INTO THE UNITED STATES, AUSTRALIA, CANADA, JAPAN, THE REPUBLIC OF SOUTH AFRICA OR ANY OTHER JURISDICTION IN WHICH SUCH PUBLICATION, RELEASE OR DISTRIBUTION WOULD BE UNLAWFUL (“RESTRICTED JURISDICTIONS”).

THIS ANNOUNCEMENT DOES NOT ITSELF CONSTITUTE AN OFFER FOR SALE OR SUBSCRIPTION OF ANY SECURITIES IN THE COMPANY IN ANY JURISDICTION INCLUDING IN THE UNITED STATES. DISTRIBUTION OF THIS ANNOUNCEMENT IN CERTAIN JURISDICTIONS MAY BE RESTRICTED OR PROHIBITED BY LAW. PERSONS DISTRIBUTING THIS ANNOUNCEMENT MUST SATISFY THEMSELVES THAT IT IS LAWFUL TO DO SO.

SECURITIES MAY NOT BE OFFERED OR SOLD IN THE UNITED STATES ABSENT REGISTRATION UNDER THE UNITED STATES SECURITIES ACT OF 1933, AS AMENDED (THE “SECURITIES ACT”), OR AN EXEMPTION FROM, OR IN A TRANSACTION NOT SUBJECT TO, REGISTRATION. THE COMPANY HAS NOT AND DOES NOT INTEND TO REGISTER ANY SECURITIES UNDER THE SECURITIES ACT, AND DOES NOT INTEND TO OFFER ANY SECURITIES TO THE PUBLIC IN THE UNITED STATES UNLESS REGISTERED UNDER THE SECURITIES ACT OR AN EXEMPTION FROM SUCH REGISTRATION IS AVAILABLE. NO PUBLIC OFFERING OF SECURITIES OF THE COMPANY IS BEING MADE IN THE UNITED STATES. NO COMMUNICATION OR INFORMATION RELATING TO THE ISSUE AND OFFERING OF SECURITIES MAY BE DISSEMINATED TO THE PUBLIC IN JURISDICTIONS OTHER THAN THE UK WHERE PRIOR REGISTRATION OR APPROVAL IS REQUIRED FOR THAT PURPOSE. NO ACTION HAS BEEN TAKEN THAT WOULD PERMIT AN OFFER OF SECURITIES IN ANY JURISDICTION WHERE ACTION FOR THAT PURPOSE IS REQUIRED, OTHER THAN IN THE UK.

References in this announcement and these terms and conditions to Davy refer to J&E Davy. References in these terms and conditions to Shore Capital refer to Shore Capital Stockbrokers Limited and/or Shore Capital and Corporate Limited as the context admits.

This announcement has been issued by and is the sole responsibility of the Company. No representation or warranty, express or implied, is or will be made as to, or in relation to, and no responsibility or liability is or will be accepted by MTS Securities LLC (“MTS”), Shore Capital or Davy or by any of their respective affiliates or agents as to or in relation to, the accuracy or completeness of this Announcement or any other written or oral information made available to or publicly available to any interested party or its advisers, and any liability therefore is expressly disclaimed.

The distribution of this announcement may be restricted by law in certain jurisdictions and persons into whose possession this announcement, or other information referred to herein, comes should inform themselves about and observe any such restriction. Any failure to comply with these restrictions may constitute a violation of the securities laws of any such jurisdiction. To the fullest extent permitted by applicable law, the companies and persons involved in the Transaction disclaim any responsibility or liability for the violation of such requirements by any person.

This announcement has been prepared for the purposes of complying with English law, the rules of AIM and Euronext Growth and the information disclosed may not be the same as that which would

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have been disclosed if this announcement had been prepared in accordance with the laws and regulations of any jurisdictions outside England and Wales.

Statements in this announcement with respect to each of Amryt’s and Aegerion’s business, strategies, projected financial figures, transaction synergies, earnings guidance, financial guidance, future dividends and beliefs and with respect to the Transaction, as well as other statements that are not historical facts are forward-looking statements involving risks and uncertainties which could cause the actual results to differ materially from such statements. Statements containing the words “expect”, “anticipate”, “intends”, “plan”, “estimate”, “aim”, “forecast”, “project” and similar expressions (or their negative) identify certain of these forward-looking statements. The forward-looking statements in this Announcement are based on numerous assumptions regarding the Transaction and each of Amryt’s and Aegerion’s present and future business strategies and the environment in which each of Amryt and Aegerion will operate in the future. Forward-looking statements involve inherent known and unknown risks, uncertainties and contingencies because they relate to events and depend on circumstances that may or may not occur in the future and may cause the actual results, performance or achievements to be materially different from those expressed or implied by such forward-looking statements. These statements are not guarantees of future performance or the ability to identify and consummate investments. Many of these risks and uncertainties relate to factors that are beyond each of Amryt’s and Aegerion’s ability to control or estimate precisely, such as future market conditions, currency fluctuations, the behaviour of other market participants, the actions of regulators and other factors such as each of Amryt’s and Aegerion’s ability to obtain financing, changes in the political, social and regulatory framework in which each of Amryt and Aegerion operates or in economic, technological or consumer trends or conditions. Past performance should not be taken as an indication or guarantee of future results, and no representation or warranty, express or implied, is made regarding future performance. No person is under any obligation to update or keep current the information contained in this Announcement or to provide the recipient of it with access to any additional relevant information that may arise in connection with it. Such forward-looking statements reflect the directors’ current beliefs and assumptions and are based on information currently available to management.

This announcement includes certain combined or pro forma financial information for Aegerion and Amryt. Such combined or pro forma financial information is preliminary in nature, only represents current estimates of the potential impact of the Transaction on Amryt, remains subject to change and is provided solely for illustrative purposes. No reliance should be placed on the combined or pro forma financial information contained in this Announcement.

No statement in this announcement is intended to be a profit forecast, and no statement in this announcement should be interpreted to mean that earnings per share of the Company for the current or future financial years would necessarily match or exceed the historical published earnings per share of the Company.

Shore Capital and Corporate Limited and Shore Capital Stockbrokers Limited are, authorised and regulated in the United Kingdom by the Financial Conduct Authority. Shore Capital and Corporate Limited acts as nominated adviser to the Company for the purposes of the AIM Rules. Shore Capital is acting exclusively for the Company and for no one else in connection with the Transaction and will not be responsible to anyone other than the Company for providing the protections afforded to clients of Shore Capital or for providing advice in relation to the Transaction, or any other matters referred to in this announcement.

Davy, which is regulated in Ireland by the Central Bank of Ireland, acts as the Euronext Growth adviser to the Company for the purposes of the Euronext Growth Rules. Davy is acting exclusively

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for the Company and for no one else in connection with the Transaction and will not be responsible to anyone other than the Company for providing the protections afforded to clients of Davy or for providing advice in relation to the Transaction, or any other matters referred to in this announcement. MTS is acting exclusively for the Company and for no one else in connection with the Transaction and will not be responsible to anyone other than the Company for providing the protections afforded to clients of MTS or for providing advice in relation to the Transaction, or any other matters referred to in this announcement.

Save for the responsibilities and liabilities, if any, of MTS, Shore Capital and Davy under relevant laws or in respect of fraudulent misrepresentation, no representation or warranty, express or implied, is or will be made as to, or in relation to, and no responsibility or liability is or will be accepted by or on behalf of MTS, Shore Capital, Davy or by their respective affiliates, agents, directors, officers and employees as to, or in relation to, the accuracy or completeness of this announcement or any other written or oral information made available to or publicly available to any interested party or its advisers, and any liability therefor is expressly disclaimed.

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Privileged & Confidential
Draft of 5/20/2019

Updated 7:45 pm EST

Novelion Therapeutics Announces Subsidiary Aegerion
Pharmaceuticals to Recapitalize Through Court Supervised
Process In Which Amryt Pharma Plc Will Acquire 100% of
Reorganized Stock of Aegerion

• Transaction is result of comprehensive capital structure and strategic review conducted independently by both Novelion’s and Aegerion’s Boards of Directors

• Aegerion will continue to make available to patients its two approved therapies, JUXTAPID^® and MYALEPT^®

• Novelion to receive approximately 10% of the equity of the combined company (subject to dilution) on account of its intercompany loan and cash payments from Aegerion related to past and future expenditures for shared services

Vancouver, BC, and Cambridge, MA, May 20, 2019 – Novelion Therapeutics Inc. (NASDAQ: NVLN) (Novelion), a biopharmaceutical company dedicated to developing new standards of care for individuals living with rare diseases, announced today that its wholly-owned subsidiary Aegerion Pharmaceuticals, Inc. (Aegerion) has entered into a plan funding agreement (PFA) and a restructuring support agreement(RSA) that will result in Aegerion selling 100% of its reorganized stock to, and becoming a wholly-ownedsubsidiary of, Dublin-based Amryt Pharma Plc (Amryt).

The agreements, which will result in a recapitalization of Aegerion (the Recapitalization), are the result of the previously announced capital structure and strategic review undertaken independently by the Boards of Directors of Novelion and Aegerion, and a broad marketing process. The Recapitalization of Aegerion pursuant to the PFA and a proposed Chapter 11 plan of reorganization (the Plan) has been approved by Aegerion’s board and approved and recommended by the independent restructuring committee of Aegerion’s board. Novelion’s board has approved Novelion’s entry into the RSA and support for Aegerion’s proposed Chapter 11 restructuring.

In conjunction with the Recapitalization, Aegerion has entered into the RSA with many of its key stakeholders, including Novelion, the holders of in excess of 67% of the 2.00% convertible notes issued by Aegerion due 2019 (Existing Convertible Notes) and the holders of 100% of the principal amount under Aegerion’s other indebtedness for borrowed money.

To facilitate the Recapitalization, Aegerion and its U.S. subsidiary Aegerion Pharmaceuticals Holdings, Inc. (the Debtors) have commenced cases in the United States Bankruptcy Court for the Southern District of New York (the Court) pursuant to Chapter 11 of the United States Code. Aegerion will

continue to operate in the ordinary course of business during the Chapter 11 process. The non-U.S. subsidiaries of Aegerion are not part of the Chapter 11 proceedings.

Certain Key Terms of the Recapitalization

The Recapitalization ascribes an enterprise value to Aegerion and Amryt of $395 million and $146 million, respectively, excluding cash and cash equivalents and subject to adjustment for accrued interest and certain payments that are due to the DOJ and the SEC. The key terms of the Recapitalization (the Restructuring Transactions), which are subject to Bankruptcy Court approval and other customary conditions, include the following:

• Amryt acquiring 100% of the outstanding new equity interests in recapitalized Aegerion;

• Ordinary equity of Amryt representing 61.4% of the outstanding ordinary equity of Amryt, after giving effect to the Restructuring Transactions but before giving effect to equity underlying the New Convertible Notes, the Deal Equity Raise (each as described below), ordinary shares that may be issuable in satisfaction of the CVR (described below) if the relevant milestones are achieved, and equity that is reserved for issuance under any management equity compensation plan adopted by Amryt, will be distributed to certain existing creditors of Aegerion in complete or partial satisfaction of their claims, including in partial satisfaction of the claims of the holders of the Existing Convertible Notes and in complete satisfaction of Novelion’s approximately $36 million claims on account of the Intercompany Loan;

• Pre-Recapitalization shareholders of Amryt continuing to own 38.6% of the outstanding ordinary equity of Amryt, after giving effect to the Restructuring Transactions but before giving effect to equity underlying the New Convertible Notes, the Deal Equity Raise, and any equity issued on account of the CVRs and under any management equity compensation plan adopted by Amryt;

• The equity interests of Aegerion held by Novelion being terminated;

• Aegerion issuing $125 million of new 5% convertible notes (the New Convertible Notes). The New Convertible Notes will be issued to certain existing creditors of Aegerion in satisfaction of their claims (and not for cash), including in satisfaction of a portion of the Existing Convertible Notes, the approximately $22 million of “Roll Up Debt” under the Aegerion’s existing bridge loan facility, and any amounts drawn down under Aegerion’s DIP Financing (defined below) that are not otherwise satisfied in cash at the closing of the Restructuring Transactions;

• Aegerion’s existing Bridge Loan in the original principal amount of $50 million, held by certain funds managed by Athyrium Capital Management, LP (Athyrium) and Highbridge Capital Management, LLC (Highbridge), as well as Amryt’s existing approximately €20

1 Note: The announcement will lead to suspension. Following publication of the admission document, the larger Amryt Group will be listed on the AIM market.

2

million (in principal) of secured debt, will be converted into new first-lien secured debt of Amryt and Aegerion, which will have a cash interest rate of 6.5% per annum and an additional 6.5% PIK (Paid in Kind) interest rate and mature five years from the closing date of the Restructuring Transactions;

• Amryt shareholders prior to the consummation of the Restructuring Transactions will receive a contingent value right (CVR) entitling them to receipt of proceeds of up to $85 million upon the occurrence of certain milestones related to the regulatory approval and commercialization of AP 101, its late-stage development product candidate, with such payments to be made in loan notes or ordinary shares, at the election of its board;

• In connection with the closing of the Restructuring Transactions, Amryt plans to raise $60 million through the issuance of new equity of Amryt (the Deal Equity Raise). The proceeds from the Deal Equity Raise will be used as provided in the Plan to pay certain expenses and for general corporate purposes. The new equity will be priced at a 20 percent discount to Amryt’s implied valuation pro forma to the Restructuring Transaction with $18 million of the new equity offered to certain Amryt investors and $42 million to certain creditors of Aegerion on a pro rata basis, including Novelion. Certain of Aegerion bondholders, including Athyrium, Highbridge, UBS and Whitebox, have agreed to purchase any unsubscribed portion of the new equity;

• Aegerion intends to, and the Plan provides that Aegerion will, continue to fully honor all obligations to the U.S. Department of Justice, the U.S. Securities and Exchange Commission and other U.S. and state government agencies and courts, which obligations will not be impaired by the Restructuring Transactions;

• Aegerion intends to continue to pay all trade and other ordinary operating expenses that arise during the course of the Chapter 11 cases and, upon consummation of the Restructuring Transactions, repay 100% of any allowed trade claims outstanding as of the Chapter 11 filing;

• Under the terms of the PFA, following the approval by the Court of certain provisions of the PFA, Aegerion and its advisors will have a 55-day period to solicit alternative transactions that are superior, from a financial point of view, to the Restructuring Transactions. Subject to the limitations of the PFA, Aegerion is also entitled to respond to unsolicited proposals if Aegerion determines that such proposals are reasonably likely to result in a superior transaction. Aegerion is entitled to terminate the PFA in order to enter into a superior transaction, provided that it reimburses Amryt for costs and expenses incurred in connection with the Restructuring Transactions (with a cap of $4,000,000) at the time of termination and pays a termination fee of $11,850,000 upon the consummation of the superior transaction. Approximately 34.3% of Amryt’s existing shareholders have committed to supporting the Restructuring Transactions through written undertakings.

1 Note: The announcement will lead to suspension. Following publication of the admission document, the larger Amryt Group will be listed on the AIM market.

3

• The Debtors expect to enter into a $20 million super-priority debtor-in-possession multi-draw term loan facility (the DIP Financing) with Athyrium and Highbridge on terms and conditions set forth in the DIP credit agreement and proposed DIP order filed with the Court. Upon approval by the Court and the satisfaction of the conditions set forth in the DIP credit agreement, the DIP Financing will provide the Debtors with liquidity that will be used to support the Restructuring Transactions. Any portion of the DIP Financing that is drawn and not repaid in cash upon the closing of the Restructuring Transactions will be converted into a portion of the $125 million of New Convertible Notes discussed above. The Debtors have also negotiated with their existing secured lenders the terms of consensual use of cash collateral during the pendency of the Chapter 11 cases.

The Recapitalization and business combination between Aegerion and Amryt is expected to create a global rare disease company with a diversified commercial and clinical-stage portfolio with growing commercial assets and multiple late stage product candidates. The development pipeline includes Amryt’s AP101 product candidate currently in Phase III development for epidermolysis bullosa (EB), as well as additional potential indications for Aegerion’s products, including metreleptin as a potential treatment for partial lipodystrophy (PL) in the U.S., which is already approved in Europe, and lomitapide as a potential treatment for familial chylomicronemia syndrome (FCS).

“The combination of Amryt and Aegerion will create a financially stronger and well-capitalized rare disease company with two commercial products and a pipeline of late stage rare disease products. Amryt’s executive management team has the depth of experience to commercialize Aegerion’s marketed products, as demonstrated by its ability to grow sales of LOJUXTA^® in the European market, to develop and, if approved, commercialize Amryt’s late stage product candidate, AP101, and to pursue additional potential indications for metreleptin and lomitapide,” said Ben Harshbarger, Novelion’s Interim Chief Executive Officer. “With the opportunity to leverage synergies between the two companies to reduce overlap in expenses and eliminate the intercompany royalties through the existing LOJUXTA licensing agreement among the two companies, we believe these transactions create a compelling growth story and value creation opportunity for Aegerion and its stakeholders, including Novelion.”

“The acquisition of Aegerion accelerates our ambition to become a global leader in treating rare conditions where there is a high unmet medical need,” commented Joe Wiley, Chief Executive Officer of Amryt. By delivering two substantial revenue-generating products and an enhanced pipeline of promising development opportunities, this will significantly strengthen our growth in highly attractive markets globally. Amryt has a unique insight into both Aegerion and its products, through our commercial success with LOJUXTA and given that many of our senior management team previously worked at Aegerion.”

1 Note: The announcement will lead to suspension. Following publication of the admission document, the larger Amryt Group will be listed on the AIM market.

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Impact on Novelion

Novelion has agreed to enter into the RSA and support Aegerion’s proposed Chapter 11 plan, which Novelion believes will avoid value destructive potential litigation with Aegerion, its other secured lenders and the majority holders of the Existing Convertible Notes, including as it may relate to challenges to Novelion’s intercompany secured loan and the terms that Aegerion could impose or “cram down” on Novelion through a Chapter 11 plan that Novelion did not support. Under the proposed plan, Novelion’s existing approximately $36 million intercompany secured loan to Aegerion (the Intercompany Loan) will be allowed in full and will receive a distribution of equity under Aegerion’s plan of reorganization representing approximately 10.1% equity ownership of Amryt on a pro forma basis, prior to any dilution from equity to be issued in connection with Deal Equity Raise, upon conversion of the New Convertible Notes, ordinary shares that may be issuable in satisfaction of the CVR if the relevant milestones are achieved, and equity that is reserved for issuance under any management equity compensation plan adopted by Amryt. After taking into account the new Amryt equity anticipated to be issued in connection with the Deal Equity Raise, Novelion is projected to own approximately 8.1% of Amryt. Novelion’s treatment under the plan on account of its intercompany loan represents an approximately 84% recovery and the equity received will be freely transferable. Also, Novelion has the right to subscribe to purchase its pro rata share of the $42 million of new equity being offered to Aegerion’s creditors, which are priced at a 20 percent discount to Amryt’s implied Recapitalization valuation. Due to Novelion’s liquidity position, however, it is unlikely that Novelion will exercise that right in full or at all.

In addition, the Debtors entered into shared services agreements with Novelion and Novelion Services USA, Inc., a subsidiary of Novelion, dated as of December 1, 2016, but effective as of November 29, 2016 (the Shared Services Agreements), pursuant to which the Debtors provide to Novelion and Novelion provides to the Debtors, certain services, including, but not limited to administrative support, human resources, information technology support, accounting, finance, and legal services. In connection with the execution of the RSA and to facilitate the restructuring, the Debtors and Novelion negotiated and executed an amendment to the Shared Services Agreements (together, the Amended Shared Services Agreements), which modified the Shared Services Agreements to provide, among other things, for Aegerion to make certain cash payments to Novelion on account of certain services Novelion provided or will provide to Aegerion. Pursuant to the Amended Shared Services Agreement, Aegerion has made a payment to Novelion of approximately $3.1 million and has committed to make additional cash payments of up to approximately $2 million. Amended Shared Services Agreements provide Novelion with greater and more certain recoveries from Aegerion for the critical shared services Novelion provides.

1 Note: The announcement will lead to suspension. Following publication of the admission document, the larger Amryt Group will be listed on the AIM market.

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Novelion will retain its existing cash balances, public listing and net operating loss (NOL) carryforwards (subject to applicable tax laws). The value, if any, of such listing and NOL carryforwards are unknown at this time.

As a result of the valuation of Aegerion and its outstanding debts, Novelion is not receiving any consideration under Aegerion’s plan on account of its equity in Aegerion. Those existing equity interests are being cancelled under Aegerion’s Chapter 11 plan and Aegerion is issuing new equity interests to Amryt in exchange for the consideration to be paid under the PFA. Because its equity interests are being cancelled for no consideration under the Chapter 11 plan, Novelion is deemed to reject the plan in its capacity as a shareholder. By operation of U.S. bankruptcy law, however, Aegerion’s plan may be confirmed and consummated notwithstanding the deemed rejection by Novelion as its sole equity holder.

In furtherance of its duty to maximize value for its shareholders, the board of directors of Novelion, together with its management team and legal and financial advisors, is evaluating post-closing plans with respect to Novelion, including a potential wind-up of Novelion and a distribution of assets to shareholders, and recommendations related to same will be communicated to shareholders in due course.

Aegerion Chapter 11 Cases

As described above, to facilitate the Recapitalization, concurrent with the PFA and RSA, the Debtors filed for Chapter 11 protection. Aegerion will continue to operate in the ordinary course of business during the Chapter 11 cases. Novelion and non-U.S. Aegerion subsidiaries are not debtors in these Chapter 11 cases.

Importantly, during the pendency of the Chapter 11 cases, Aegerion intends to:

• continue to make available to patients its two approved therapies, JUXTAPID and MYALEPT;

• continue to pay all trade and other ordinary course operating expenses during the course of the Chapter 11 cases and, upon consummation of the Recapitalization, repay 100% of any allowed trade claims; and

• continue to pay and provide all ordinary course compensation and benefits to its existing employees, without any impairment, delay, adjustment or changes.

Amryt Listing, Board of Directors and Management

1 Note: The announcement will lead to suspension. Following publication of the admission document, the larger Amryt Group will be listed on the AIM market.

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¹ Amryt will continue to be listed on the AIM market of the London Stock Exchange. Following the Recapitalization, Amryt’s global headquarters will be in Dublin, Ireland and its U.S. headquarters will be in the Cambridge, Massachusetts area.

Upon the closing of the Recapitalization, Amryt will designate three members to its board, including CEO Joe Wiley, and Athyrium and Highbridge will designate two members each to the board. The Chairperson of the Board will be appointed by Amryt and will be unaffiliated with Amryt, Novelion or Aegerion. Amryt will continue to be led by its executive team, which will be supplemented by certain Aegerion executives on both a transitional and permanent basis. Amryt executives have significant experience in the development and commercialization of rare disease products, including specific knowledge of Aegerion’s products through its licensing relationship for LOJUXTA^® in the EU. In addition, certain Amryt executives, including Chief Medical Officer Mark Sumeray and Chief Commercial Officer David Allmond, are former members of Aegerion’s executive team.

Closing Conditions and Timing

The consummation of the Recapitalization is subject to a number of closing conditions, including approval by Amryt’s shareholders, approval of the independent Amryt shareholders in connection with the whitewash waiver granted by the UK Panel on Takeovers and Mergers, re-admission of Amryt’s ordinary shares for trading on AIM, confirmation of the Aegerion plan of reorganization by the Bankruptcy Court, and other customary closing conditions.

The parties expect the transaction to close in the late third or early fourth calendar quarter of 2019.

Advisors

Evercore acted as financial advisor and Goodwin Procter LLP and Norton Rose Fulbright Canada LLP acted as legal advisors to Novelion. Moelis & Company LLC acted as financial and restructuring advisor, AP Services, LLC acted as financial advisor and chief restructuring officer, and Willkie Farr & Gallagher LLP acted as legal advisor to Aegerion. Ducera Partners LLC acted as financial advisor and Latham & Watkins LLP and King & Spalding LLP acted as legal advisors to the ad hoc group of convertible noteholders.

Additional details, including copies of the PFA, RSA and other agreements, will be contained in Current Report on Form 8-K that Novelion intends to file with the Securities and Exchange Commission (www.sec.gov). Investors are encouraged to read the Current Report on Form 8-K and the agreements filed therewith, and the foregoing summary of the Recapitalization is qualified in its entirety by reference thereto.

1 Note: The announcement will lead to suspension. Following publication of the admission document, the larger Amryt Group will be listed on the AIM market.

7

Conference Call Details

Amryt Management will host a conference call for analysts today at 1330 BST (0830 EDT). Dial in details:

Conference ID: 3387304

From the UK/International: +44 (0) 2071 928000 I 0800 376 7922

From Ireland: (01) 431 9615 I 1800 936148

From the US: +1 631 510 7495 I 1 866 966 1396

A recording of the call will be available from 18.30 (BST) today, please email ir@amrytpharma.com for access details.

About Novelion Therapeutics

Novelion, through its subsidiary Aegerion Pharmaceuticals, is a global biopharmaceutical company dedicated to developing and commercializing therapies that deliver new standards of care for people living with rare diseases. With a global footprint and an established commercial portfolio, including MYALEPT^® (metreleptin) and JUXTAPID^® (lomitapide), our business is supported by differentiated treatments that treat severe and rare diseases.

About Amryt

Amryt is a biopharmaceutical company focused on developing and delivering innovative new treatments to help improve the lives of patients with rare or orphan diseases.

LOJUXTA^® is an approved treatment for adult patients with the rare cholesterol disorder - Homozygous Familial Hypercholesterolaemia (“HoFH”). This disorder impairs the body’s ability to remove low density lipoprotein (“LDL”) cholesterol (“bad” cholesterol) from the blood, typically leading to abnormally high blood LDL cholesterol levels in the body from before birth - often ten times more than people without HoFH - and subsequent aggressive and premature narrowing and blocking of blood vessels. LOJUXTA^® is indicated as an adjunct to a low-fat diet and other lipid-lowering medicinal products with or without LDL apheresis in adult patients with HoFH.

Amryt is the marketing authorisation holder and has an exclusive license to sell LOJUXTA^® across the European Economic Area, Middle East and North Africa, Switzerland, Turkey, Israel, Russia, the Commonwealth of Independent States and the non-EU Balkan states.

Amryt’s lead development candidate, AP101, is a potential treatment for Epidermolysis Bullosa (“EB”), a rare and distressing genetic skin disorder affecting young children and adults for which there is currently no treatment. It is currently in Phase 3 clinical trials and recently reported positive unblinded interim efficacy analysis results and is anticipated will be fully enrolled by end of H2 2019. The European and US market opportunity for EB is estimated to be in excess of $1 billion.

1 Note: The announcement will lead to suspension. Following publication of the admission document, the larger Amryt Group will be listed on the AIM market.

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In March 2018, Amryt in-licenced a pre-clinical gene-therapy platform technology, AP103, which offers a potential treatment for patients with Recessive Dystrophic Epidermolysis Bullosa, a subset of EB, and is also potentially relevant to other genetic disorders.

For more information on Amryt, please visit www.amrytpharma.com.

Forward-Looking Statements and Risk Factors

Certain statements in this press release constitute “forward-looking statements” and “forward-looking information” within the meaning of applicable laws and regulations, including U.S. and Canadian securities laws. Any statements contained herein which do not describe historical facts, including, among others, statements regarding beliefs about, and expectations for, plans to undertake a comprehensive restructuring of Aegerion Pharmaceuticals, the proposed transaction between Aegerion Pharmaceuticals and Amryt, including the key terms, expected ownership, benefits of the proposed transaction to Novelion’s and Aegerion’s stakeholders, expected closing and performance of the combined company, and the RSA are forward-looking statements which involve risks and uncertainties that could cause actual results to differ materially from those discussed in such forward-looking statements.

Such risks and uncertainties include, among others, Novelion’s and Aegerion’s ability to meet immediate operational needs and obligations, as well as long-term obligations; Novelion’s and Aegerion’s ability to continue as a going concern; the possibility that the restrictions in and other terms of Aegerion’s loan arrangements could have a negative impact on Novelion’s business and its shareholders (whose interests may not be aligned, and may be in conflict, with those of Aegerion’s holders of convertible notes and other lenders); whether Aegerion will be able to successfully complete the Restructuring Transactions; that Novelion will not realize the benefits of the Restructuring Transactions; potential adverse effects of the Chapter 11 cases; the Debtors ability to obtain timely approval by the Court with respect to motions filed in the Chapter 11 cases; objections to the Restructuring Transactions, DIP Financing or other pleadings filed that could protract the Chapter 11 cases; the effects of the bankruptcy petitions on Novelion and on the interest of various constituents, including holders of Novelion’s common stock; the Court’s ruling in the Chapter 11 cases; risks associated with third party motions in the Chapter 11 cases; and increased administrative and legal costs related to the Chapter 11 process and other litigation and inherent risks involved in a bankruptcy process; Novelion’s ability to maintain its listing status on Nasdaq (the failure of which would constitute an event of default under Aegerion’s loan arrangements), as well as those risks identified in Novelion’s filings with the Commission, including under the heading “Risk Factors” in Novelion’s Annual Report on Form 10-K for the fiscal year ended December 31, 2018, and subsequent filings with the Commission, all of which are available on the Commission’s website at www.sec.gov.

We caution you not to place undue reliance on any forward-looking statements, which speak only as of the date they are made. Except as required by law, we undertake no obligation to update or revise the

1 Note: The announcement will lead to suspension. Following publication of the admission document, the larger Amryt Group will be listed on the AIM market.

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information contained in this press release, whether as a result of new information, future events or circumstances or otherwise. Given the uncertainties, assumptions and risk factors associated with this type of information, including those described above, investors are cautioned that the information may not be an appropriate subject of reliance for other purposes.

Investors and others should note that we communicate with our investors and the public using the Novelion website www.novelion.com, including, but not limited to, company disclosures, investor presentations and FAQs, Commission filings, press releases, public conference call transcripts and webcast transcripts. The information that we post on this website could be deemed to be material information. As a result, we encourage investors, the media and others interested to review the information that we post there on a regular basis. The contents of our website shall not be deemed incorporated by reference in any filing under the Securities Act of 1933, as amended.

CONTACT:

Amanda (Murphy) Cray, Director, Investor Relations & Corporate Communications

Novelion Therapeutics Inc.

857-242-5024

amanda.cray@novelion.com

1 Note: The announcement will lead to suspension. Following publication of the admission document, the larger Amryt Group will be listed on the AIM market.

10

EXHIBIT A

Plan

EXHIBIT B

Plan Funding Agreement

EXHIBIT C

DIP Credit Agreement

EXHIBIT D

Disclosure Statement

EXHIBIT E

Backstop Commitment Agreement

EXHIBIT F

Term Sheet for New Convertible Notes Indenture

EXHIBIT G

Term Sheet for New First Lien Secured Credit Facility

EX-10.3

Exhibit 10.3

EXECUTION VERSION

AMRYT PHARMA PLC

HIGHBRIDGE MSF INTERNATIONAL LTD.

HIGHBRIDGE SCF SPECIAL SITUATIONS SPV, L.P.

1992 TACTICAL CREDIT MASTER FUND, L.P.

ATHYRIUM OPPORTUNITIES II ACQUISITION 2 LP

ATHYRIUM OPPORTUNITIES III ACQUISITION 2 LP

WHITEBOX RELATIVE VALUE PARTNERS, LP

WHITEBOX GT FUND, LP

WHITEBOX MULTI-STRATEGY PARTNERS, LP

PANDORA SELECT PARTNERS, LP

NINETEEN77 GLOBAL MULTI-STRATEGY ALPHA MASTER LIMITED

AND

NINETEEN77 GLOBAL CONVERTIBLE BOND MASTER LIMITED

BACKSTOP SUBSCRIPTION AGREEMENT

 

99 Bishopsgate

London

EC2M3XF

www.1w.com

TABLE OF CONTENTS

Clause		Page
1.	DEFINITIONS AND INTERPRETATION	5
2.	CONDITIONS	8
3.	BACKSTOP SUBSCRIPTION OBLIGATION	9
4.	FEE	10
5.	BACK STOP PARTY WARRANTIES	10
6.	COMPANY WARRANTIES	10
7.	THIRD PARTY RIGHTS	11
8.	NOTICES	11
9.	TERMINATION	13
10.	MISCELLANEOUS	13
11.	ENTIRE AGREEMENT	14
12.	APPLICABLE LAW	14

This Agreement (the “Agreement”) is made on July 10, 2019

BETWEEN

(1) AMRYT PHARMA PLC, a company incorporated in England and Wales with the registered number 05316808 and registered address at Dept 920a 196 High Road, Wood Green, London, England, N22 8HH (the “Company”);

(2) HIGHBRIDGE MSF INTERNATIONAL LTD., an exempted company incorporated under the laws of the Cayman Islands with registered office at c/o Maples Corporate Services Limited, PO Box 309, Ugland House, Grand Cayman, KY1-1101, Cayman Islands (“MSF”);

(3) HIGHBRIDGE SCF SPECIAL SITUATIONS SPV, L.P., an exempted limited partnership formed under the laws of the Cayman Islands and registered office at c/o Maples Corporate Services Limited, PO Box 309, Ugland House, Grand Cayman, KY1-1101, Cayman Islands (“SCF”);

(4) 1992 TACTICAL CREDIT MASTER FUND, L.P., an exempted limited partnership organized under the laws of the Cayman Islands and registered office at c/o Maples Corporate Services Limited, PO Box 309, Ugland House, Grand Cayman, KY1-1101, Cayman Islands (“TCF”, and together with MSF and SCF, “Highbridge”);

(5) ATHYRIUM OPPORTUNITIES II ACQUISITION 2 LP, a limited partnership whose registered address is at 251 Little Falls Drive Wilmington, DE 19808-1674 (“Athyrium II”);

(6) ATHYRIUM OPPORTUNITIES III ACQUISITION 2 LP, a limited partnership whose registered address is at 251 Little Falls Drive Wilmington, DE 19808-1674 (“Athyrium III”, and together with Athyrium III, “Athyrium”);

(7) WHITEBOX RELATIVE VALUE PARTNERS, LP, a limited partnership whose registered address is at Jayla Place, Wickhams Cay 1, Road Town, Tortola, British Virgin Islands VG1110 (“Whitebox Relative”);

(8) WIDTEBOX GT FUND, LP, a limited partnership whose registered address is at 251 Little Falls Drive, Wilmington, DE 19808 (“Whitebox GT”);

(9) WHITEBOX MULTI-STRATEGY PARTNERS, LP, a limited partnership whose registered address is at Jayla Place, Wickhams Cay 1, Road Town, Tortola, British Virgin Islands VG1110 (“Whitebox Multi-Strategy”);

(10) PANDORA SELECT PARTNERS, LP, a limited partnership whose registered address is at Jayla Place, Wickhams Cay 1, Road Town, Tortola, British Virgin Islands VG1110 (“Pandora”, and together with Whitebox Relative, Whitebox GT and Whitebox Multi-Strategy, “Whitebox”);

(11) NINETEEN77 GLOBAL MULTI-STRATEGY ALPHA MASTER LIMITED, an exempted company incorporated under the laws of the Cayman Islands whose registered office address is at c/o Maples Corporate Services Limited, Ugland House, PO Box 309, Grand Cayman, KY1-1104, Cayman Islands (“Nineteen Multi-Strategy”); and

(12) NINETEEN77 GLOBAL CONVERTIBLE BOND MASTER LIMITED, an exempted company incorporated under the laws of the Cayman Islands whose registered office address is at c/o Maples Corporate Services Limited, Ugland House, PO Box 309, Grand Cayman, KY1-1104, Cayman Islands (“Nineteen Bond,” and together with Nineteen Multi-Strategy, “O’Connor”).

WHEREAS

(A) The Company was incorporated on 20 December 2004 and its shares are admitted to trading on AIM, a market operated by the London Stock Exchange plc (“AIM”) and the Euronext Growth Market, a market operated by Euronext Dublin (“Euronext”).

(B) The Company has entered into a restructuring support agreement (the “RSA”) and a plan funding agreement (the“Plan Funding Agreement”) pursuant to which, among others, it proposes to acquire the entire share capital of Aegerion Pharmaceuticals, Inc. (“Aegerion”).

(C) The Company proposes to effect a scheme of arrangement under Part 26 of the Companies Act pursuant to which the holders of Ordinary Shares (the “Company Shareholders”) and the holders of options or warrants over Ordinary Shares (the “Company Options”) (and the holders of the Company Options, the “Company Optionholders”) will exchange the Ordinary Shares held by such Company Shareholders (and the Company Options held by such Company Optionholders) for new ordinary shares (or new options or warrants over such ordinary shares) issued by a newly incorporated company (“New Amryt TopCo”), together with one (1) CVR security for each Company Share (or Company Option), whereupon the Company shall procure that the rights and obligations of the Company under this Agreement shall be assumed by New Amryt TopCo (such scheme of arrangement, the “Scheme”).

(D) In accordance with the RSA and subject to the terms and conditions set out in this Agreement, the Company proposes to raise $60 million (the “Capital Raise Amount”) by way of an offer of Ordinary Shares to Investors and Creditors at the Placing Price (the“Capital Raise”).

(E) Pursuant to the terms of the Capital Raise:

(i) the Creditor Placing Shares will be offered by the Company to the Creditors for an aggregate subscription price of $42 million at the Placing Price, and each Creditor will have the right, but not the obligation, to subscribe, for its pro rata share of the Creditor Placing Shares. To the extent that any Creditor Placing Shares remain unsubscribed, first, each Investor as determined by the Company may elect to subscribe for any unsubscribed Creditor Placing Shares and thereafter, to the extent that any Creditor Placing Shares remain unsubscribed, each Creditor who has previously subscribed for the Creditor Placing Shares will have the right, but not the obligation, to subscribe, for its pro rata share of the remaining Creditor Placing Shares (the “Creditor Placing”). To the extent that a subscription for Creditor Placing Shares by a Creditor is, or is deemed to be, an offer to purchase such Creditor Placing Shares and not an acceptance of an offer by the Company to purchase such Creditor Placing Shares, the Company agrees (1) to not unreasonably reject such offer/subscription and, in any event, (2) to treat each Creditor that offers to purchase Creditor Placing Shares, and each offer/subscription therefor, equally in all material respects; and

(ii) the Investor Placing Shares will be offered by the Company to the Investors for an aggregate subscription price of $18 million at the Placing Price (the “Investor Placing”).

(F) Each Back Stop Party will subscribe for, and the Company will allot and issue to each Back Stop Party, any Remaining Shares, if:

(i) the Creditor Placing has not been fully subscribed (as described in Recital (E)(i)); and

(ii) the Investor Placing has not been fully subscribed (as described in Recital (E)(ii)),

on such terms, in such proportions, and in such manner, as prescribed in this Agreement.

(G) An application will be made by the Company for all of the Placing Shares to be admitted to trading on AIM and Euronext.

IT IS AGREED THAT:

1. DEFINITIONS AND INTERPRETATION

1.1 In this Agreement (including the Recitals hereto), in addition to expressions already defined, the following expressions shall have the meanings set out below.

“Accredited Investors” has the meaning specified in Rule 501(a) of Regulation D under the Securities Act;

“Admission” means the admission of the Placing Shares to trading on AIM and Euronext becoming effective as provided under the AIM Rules and the Euronext Rules respectively;

“Affiliate” has the meaning specified in Rule 501(b) of Regulation D under the Securities Act;

“AIM” has the meaning set out in Recital (A);

“AIM Rules” means the rules and guidance notes for AIM Companies issued by the LSE from time to time related to AIM traded securities and the operation of AIM;

“Applications” means the applications made by (or on behalf of) the Company for Admission in the form prescribed by the LSE and the Euronext;

“Articles of Association” means the articles of association of the Company from time to time;

“Average USD to GBP Exchange Rate” means the average closing USD to GBP exchange rate as derived from the Bloomberg Composite Rate (taken each day as at 4 pm London time) over the five (5) Business Days falling immediately prior to the date of this Agreement;

“Back Stop Party” means each of MSF, TCF, Athyrium II, Athyrium III, Whitebox Relative, Whitebox GT, Whitebox Multi-Strategy, Pandora, Nineteen77 Multi-Strategy and Nineteen Bond, and collectively, the “Back Stop Parties”;

“Board” means the board of directors of the Company from time to time;

“Business Day” means any day other than a Saturday, Sunday or bank or public holiday in New York, London or Dublin;

“Capital Raise” has the meaning set out in Recital (D);

“Capital Raise Amount” has the meaning set out in Recital (D);

“Companies Act” means the Companies Act 2006;

“Company Options /Warrants” means any options or warrants issued by the Company which entitle the holder thereof to require the issuance of any Ordinary Shares by the Company on exercise of such options or warrants by the holder in accordance with the terms thereof;

“Company Warranties” means the warranties given by the Company pursuant to Clause 6;

“Conditions” has the meaning given in Clause 2.1;

“Creditors” means the publicly traded ultimate parent of Aegerion and, subject to all applicable regulatory restrictions and securities laws in any applicable jurisdiction, certain unsecured creditors of Aegerion;

“Creditor Placing” has the meaning set out in Recital (E)(i);

“Creditor Placing Shares” means Ordinary Shares offered pursuant to the Creditor Placing for an aggregate subscription price of $42 million;

“CREST” means the system for the paperless settlement of trades in securities and the holding of securities in uncertificated form of which Euroclear is the Operator (as defined in the CREST Regulations);

“CREST Regulations” means the Uncertificated Securities Regulations 2001 (SI 2001 No 3755), as amended;

“CVR Instrument” means the deed poll in the agreed form to be entered into by New Amryt TopCo constituting the CVR securities to be issued to the Company Shareholders and the Company Optionholders;

“Employee Share Option Plan” means the Company’s Employee Share Option Plan, as adopted 18 April 2016 and as amended 25 May 2017;

“Encumbrance” means any pledge, charge, lien, mortgage, debenture, hypothecation, security interest, pre-emption right, option, claim, equitable right, power of sale, pledge, retention of title, right of first refusal or other third party right or security interest of any kind or an agreement, arrangement or obligation to create any of the above;

“Euronext Rules” means the Euronext Growth Rules for Companies published by Euronext Dublin from time to time;

“FSMA” means the Financial Services and Markets Act 2000;

“Group” means in relation to each of the Back Stop Parties or the Company, as the context requires, any legal entity or person which from time to time controls, is controlled by or is under common control with that party, and “control”, in relation to a legal entity, means the power of a person to secure that the affairs of the legal entity are conducted in accordance with the wishes of that person (i) by means of the holding of shares, or the possession of voting power, in or in relation to that or any other legal entity; (ii) by means of the ability to direct the business of such legal entity (whether through its board or otherwise); or (iii) by virtue of any powers conferred by the constitutional or corporate documents, or any other document, regulating that or any other legal entity.

“Investor Placing” has the meaning set out in Recital (E)(ii);

“Investor Placing Shares” means Ordinary Shares offered pursuant to the Investor Placing for an aggregate subscription price of $18 million;

“Investors” means such persons (whether existing Company Shareholders or otherwise) as the Company may procure for the purposes of subscribing for the Investor Placing Shares or the unsubscribed portion of the Creditor Placing;

“Long-Stop Date” means the Outside Date as defined in the Plan Funding Agreement or any such later date as may be agreed between the parties in writing;

“LSE” means the London Stock Exchange plc;

“Notification Form” has the meaning set out in Clause 3.2;

“Ordinary Shares” means the ordinary shares in the capital of the Company in issue from time to time;

“Payment Amount” has the meaning given in Clause 3.3;

“Placing Price” means the placing price at which Placing Shares will be offered for subscription calculated in accordance with the following formula:

$60,000,000	x A
Placing Shares

where:

“A” means the Average USD to GBP Exchange Rate;

“Closing Shares” has the meaning given in the Plan Funding Agreement;

“Placing Shares” means such number of Ordinary Shares to be allotted and issued pursuant to the Capital Raise, calculated in accordance with the following formula:

Capital Raise Ownership Percentage x	Pre – Money Shares
	(1 – Capital Raise Ownership Percentage)

where:

“Capital Raise Ownership Percentage” is calculated using the following formula:

$60,000,000

(($190,700,000 + $120,000,000) X 0. 8 + $60,000,000)

“Pre-Money Shares” means the number of Ordinary Shares and Company Options and Warrants (other than Company Options issued pursuant to the Employee Share Option Plan) in issue immediately prior to the issue of the Placing Shares, including the Closing Shares;

where, for the avoidance of doubt, the number of Placing Shares shall be equal to the Capital Raise Ownership Percentage (which is approximately 19.4452%) of the sum of: (i) the Pre-Money Shares; and (ii) the Placing Shares.

“Regulation S” means Regulation S under the Securities Act;

“Relevant Percentage” means in the case of MSF, 10.5 per cent., in the case of TCF, 9.8 per cent., in the case of SCF, 5.4 per cent., in the case of Athyrium II, 47 per cent., in the case of Athyrium III, 11 per cent, in the case of Whitebox Relative 1.4 per cent., in the case of Whitebox GT 0.1 per cent., in the case of Whitebox Multi-Strategy 1.4 per cent., in the case of Pandora 0.6 per cent., in the case of Nineteen Multi-Strategy 12.3 per cent., and in the case of Nineteen Bond, 0.5 per cent.;

“Remaining Creditor Placing Shares” means such number of Creditor Placing Shares that have not been subscribed for pursuant to the Creditor Placing;

“Remaining Investor Placing Shares” means such number of Investor Placing Shares that have not been subscribed for pursuant to the Investor Placing;

“Remaining Shares” means the Remaining Investor Placing Shares and the Remaining Creditor Placing Shares;

“Scheme Document” means the document to be sent to the Company Shareholders containing, amongst other things, the Scheme and the notices convening the meetings of the the Company Shareholders to consider and, if thought fit, approve the Scheme;

“Securities Act” means the United States Securities Act of 1933, as amended;

“Subscription Date” means the date given for the allotment and issue of the Remaining Shares as notified to each Back Stop Party by the Company in the Notification Form, provided that this date shall be no earlier than two (2) Business Days following the date of the Notification Form; and

“Takeover Code” means the UK City Code on Takeovers and Mergers;

“United States” has the meaning assigned thereto in Regulation S under the Securities Act.

1.2 References in this Agreement to any act, statute or statutory provision include references to any such provision as amended, re-enacted or replaced from time to time and any former statutory provision replaced (with or without modification) by the provision referred to.

1.3 References in this Agreement to the singular include references to the plural and vice versa and references to any gender shall include references to all other genders.

1.4 Headings in this Agreement are inserted for convenience only and shall not affect the interpretation of this Agreement or any part thereof.

1.5 The expressions “subsidiary” shall have the meaning given by the Companies Act and the expression “subsidiary” shall be deemed to include “subsidiary undertakings” as defined by the Companies Act.

1.6 References to “$” or “US Dollars” are to the lawful currency of the United States.

1.7 References to “£” are to the lawful currency of the United Kingdom.

2. CONDITIONS

2.1 The obligations of each Back Stop Party under this Agreement are in all respects conditional on the satisfaction (or waiver, as the case may be) of the following (the “Conditions”):

(a) the Applications and all other documents required to be submitted with the Applications, being delivered to the LSE and Euronext Dublin respectively, no later than 8.00 am (London time) two (2) Business Days prior to the Subscription Date;

(b) Admission becoming effective by no later than 8.00 a.m. (London time) on the Subscription Date;

(c) the rights attaching to the Ordinary Shares under the articles of association of the Company as at the date of execution of the RSA not having been altered in any respect;

(d) the approval of the “Rule 9 whitewash waiver” shareholder resolution (the “whitewash Resolution”) by the independent Company Shareholders for the purposes of the Takeover Code;

(e) resolutions of the Company’s shareholders having been passed to authorise and approve the issue and allotment of the Placing Shares pursuant to the terms of this Agreement;

(f) the Company having fully performed its material obligations under this Agreement in all respects to the extent that such obligations are to be performed prior to Admission;

(g) none of the Company Warranties being untrue or inaccurate or misleading in any respect at any time between the date of this Agreement and Admission and no fact or circumstance having arisen which would render any of the Company Warranties untrue or inaccurate or misleading if repeated as at Admission;

(h) with respect to the Chapter 11 bankruptcy case for Aegerion, there having been entered a final order confirming the plan of reorganisation filed in such case (the “Plan”), in form and substance reasonably satisfactory to the Back Stop Parties and to the Company (in each case acting in good faith) and the “Effective Date”, as set forth in the Plan, having occurred, and all conditions precedent to the occurrence of such Effective Date having been satisfied (or waived by the Backstop Parties).

2.2 The Back Stop Parties, acting unanimously and in their sole discretion, may waive in whole or in part all or any of the Conditions by notice in writing given to the Company.

2.3 If any of the Conditions is not fulfilled or waived on or before the Long-Stop Date, the Back Stop Parties shall be entitled to treat this Agreement as terminated on the basis set out in Clause 9.

3.          BACKSTOP SUBSCRIPTION OBLIGATION

3.1 Each Back Stop Party hereby severally, and neither jointly, nor jointly and severally, undertakes to the Company to subscribe for itself (in its own name or as it may direct, provided that the subscription is compliant with the Whitewash Resolution) its Relevant Percentage of: (a) the Remaining Creditor Placing Shares; and (b) the Remaining Investor Placing Shares, in each case, at the Placing Price.

3.2 The Company shall notify each Back Stop Party of the number of Remaining Shares that they are required to subscribe for and the Subscription Date by giving notice in writing to the Back Stop Parties in the form appended hereto in the Schedule (the “Notification Form”), such Notification Form to be served not later than two (2) Business Days prior to the Subscription Date, and each Back Stop Party shall subscribe for such number of Securities in accordance with Clause 3.4.

3.3 Subject to Clause 4.2, if the Company requires the Back Stop Parties to subscribe for any Remaining Shares and delivers a Notification Form in accordance with Clause 3.2, each Back Stop Party shall pay to the Company an amount equal to the Placing Price multiplied by the number of Remaining Shares subscribed by such Back Stop Party in accordance with Clause 3.4 (the “Payment Amount”).

3.4 On the Subscription Date, each Back Stop Party shall subscribe for, and the Company shall allot and issue to the Back Stop Party with such rights as set out in the Articles of Association, fully paid, such number of Remaining Shares as is notified in the Notification Form, provided that the maximum number of Remaining Shares to be subscribed for by each Back Stop Party shall be subject to the limits set out in Clause 3.1.

3.5 Subject to clause 4.2, on the Subscription Date: (a) the Company shall procure that the Remaining Shares subscribed by each Back Stop Party are credited to the CREST stock accounts (or depositary accounts) notified by the Back Stop Parties in writing to the Company no later than one (1) Business Day prior to the Subscription Date; and (b) each Back Stop Party undertakes to pay in cash to the Company their respective Payment Amount.

3.6 Settlement of the issue of the Remaining Shares shall be performed on a delivery versus payment (DVP) basis.

4. FEE

4.1 In consideration of the undertakings given herein by the Back Stop Patties and conditional upon the subscription for the Placing Shares occurring on the Subscription Date, the Company shall pay to the Back Stop Parties by way of commission a fee equal to five (5) per cent. of the Capital Raise in an aggregate amount of $3,000,000 (the “Fee”). The Fee payable under this Clause 4.1 shall be paid in cash in same day available funds on or before the Subscription Date or, if there are no Remaining Shares, the Effective Date (as defined in the Plan Funding Agreement). The Fee shall be allocated and paid by the Company to each of the Back Stop Parties in accordance with each Back Stop Party’s Relevant Percentage.

4.2 Without prejudice to their right to receive the Fee directly from the Company, the Back Stop Parties shall be entitled and are authorised to deduct some or all of the amounts due and payable pursuant to Clause 4.1 from the Payment Amount payable under Clause 3.3, if the Company has not paid such amounts prior to the date on which each Back Stop Party is required to make such payment.

5. BACK STOP PARTY WARRANTIES

5.1 Each Back Stop Party severally, and not jointly, or jointly and severally, hereby warrants and undertakes to the Company on the date of this Agreement and on the Subscription Date that:

(a) it is an Accredited Investor;

(b) this Agreement has been duly authorised, executed and delivered by the Back Stop Party and constitutes a valid and legally binding agreement of the Back Stop Party enforceable against the Back Stop Patty in accordance with its terms; and

(c) it is authorised and entitled to subscribe for the Remaining Shares under the laws of all relevant jurisdictions that apply to it, has complied and will fully comply with all such laws, and has obtained all applicable consents which may be required, in relation to the subscription of the Remaining Shares.

6. COMPANY WARRANTIES

6.1 The Company hereby warrants and undertakes to the Back Stop Patties on the date of this Agreement and on the Subscription Date that:

(a) the Company is a company duly incorporated and validly existing under the laws of England and Wales;

(b) this Agreement has been duly authorised, executed and delivered by the Company and constitutes a valid and legally binding agreement of the Company enforceable against the Company in accordance with its terms;

(c) save for the Conditions referred to at Clauses 2.1(a) to (e), the execution, delivery and performance by the Company of its obligations under this Agreement will not require the Company to obtain any consent, waiver or approval of, or give any notice to or make

any registration or filing with, any governmental, regulatory, other authority or other person which has not been obtained or made at the date of this Agreement on a basis both unconditional and which cannot be revoked;

(d) the Remaining Shares will, when issued, have been validly authorised and issued, and, following payment of the Payment Amount (less any amounts deducted under Clause 4.2), be fully paid up and no further amounts will be payable to the Company in respect of their issue; and

(e) when issued, there will be no Encumbrance on, over or affecting any of the Remaining Shares nor will there be any commitment by it to give or create any such Encumbrance, and, so far as the Company is aware, no person has claimed to be entitled to any such Encumbrance.

6.2 Any warranties and representations given by the Company to the Investors and/or the Creditors as part of the Capital Raise shall also be deemed given to the Back Stop Parties mutatis mutandis in relation to the subscription of the Remaining Shares on the date of this Agreement and on the Subscription Date.

7. THIRD PARTY RIGHTS

7.1 Any person who is not a party to this Agreement shall have no right under the Contracts (Rights of Third Parties) Act 1999 to enforce any terms of this Agreement, but this does not affect any right or remedy of a third party which exists or is available apart from that act.

8. NOTICES

8.1 All notices given hereunder shall be in writing and signed by or on behalf of the party giving it and shall be served by delivering it by hand or sending it by pre-paid envelope by recorded delivery or registered post or by email to the party due to receive it, at its address or email address set out in this Agreement or to such other address or email address as are last notified in writing to the parties.

The Companv:

Amryt Pharma plc
90 Harcourt Street
Dublin 2
Ireland

For attention of: Joe Wiley

Email: joe.wiley@amrytpharma.com

With a copy to:

Gibson, Dunn & Crutcher LLP
200 Park Avenue
New York
NY 10166

For the attention of: George P.Stamas, Esq.; William B. Sorabella, Esq.; Robert Klyman, Esq.; and Mathhew J. Williams, Esq.

Email: GStamas@gibsondunn.com; WSorabella@gibsondunn.com;
RKlyman@gibsondunn.com; and MJWilliams@gibsondunn.com

Highbridge

Highbridge Capital Management LLC
40 W 57th St # E
New York, NY 10019
United States of America

For attention of: Damon Meyer and Jonathan Segal
Email: Damon.Meyer@highbridge.com; and Jonathan.Segal@highbridge.com

Athyrium

Athyrium Capital Management, LP
530 5th Ave,
New York, NY 10036

For attention of: Hondo Sen and Samuel Helfaer
Email: hsen@,athyrium.com; and SHelfaer@athyrium.com

Whitebox

Whitebox Advisors LLC
3033 Excelsior Boulevard, Suite 300
Minneapolis, MN 55416

For attention of: Ruth Andrews Scott Specken
Email: Aruth@whiteboxadvisors.com; and SSpecken@whiteboxadvisors.com

O’Connor

UBS O’Connor LLC
UBS Tower
1 North Wacker Drive, 32nd Floor
Chicago, IL 60606

For attention of: Andy Martin and Andrew Hollenbeck
Email: andy.martin@ubs.com; and andrew.hollenbeck@ubs.com

8.2 Such notice shall be deemed served, if delivered by hand, at the time of delivery, in the case of recorded delivery or registered post, two Business Days after posting, or if sent by email, at the time of transmission, provided in each case that the time of deemed service shall be a Business Day failing which the time of deemed service shall be the commencement of the next Business Day.

8.3 In proving service of any notice it shall be sufficient to prove:

(a) in the case of a letter, that the letter was delivered by hand or was properly stamped and addressed and sent by recorded delivery or registered post; and

(b) in the case of an email, that it was duly transmitted provided the sender obtains confirmation of transmission.

9. TERMINATION

9.1 This Agreement will terminate upon the earlier of:

(a) if the Conditions have not been fulfilled (or waived, as the case may be) by the Long-Stop Date, by either the Company or the Back Stop Parties giving notice in writing to the other parties on or after the Long-Stop Date;

(b) the termination of the RSA, other than any termination solely on account of the action or inaction of the Back Stop Parties in violation of the RSA; and

(c) the mutual written agreement between the Company, Highbridge, Athyrium, Whitebox, and O’Connor,

subject to the rights of the parties accrued at the date of such termination, which rights shall remain in force; provided, however, that the provisions of Clauses 6.2, 8, 9, 11, and 12 shall survive the termination of this Agreement.

9.2 In the event of termination under this Clause 9, all offers to subscribe any Remaining Shares shall be cancelled, and the Company shall have no obligation to issue and deliver, and the Back Stop Parties shall have no obligation to subscribe for, any Remaining Shares.

9.3 No party shall have any right to rescind this Agreement and the only termination rights shall be those set out in Clause 9.1.

10. MISCELLANEOUS

10.1 Time shall be of the essence of this Agreement with respect to all dates and time periods set forth or referred to in this Agreement.

10.2 No party to this Agreement may assign any of their respective rights under this Agreement to a party which is not a member of its Group without the prior written consent of the other parties. This Agreement shall be binding on and enure for the benefit of each parties’ successors in title. No provision of this Agreement may be varied without the prior written consent of all parties to this Agreement.

10.3 At any time after the date of this Agreement, each party shall, and shall use reasonable endeavours to procure that any necessary third party shall at the cost of that party, execute all such documents and do all such acts and things as the other party may reasonably require for the purpose of giving full effect to the provisions of this Agreement.

10.4 All payments by the Company under this Agreement shall be paid without set-off or counterclaim, and free and clear of and without deduction or withholding for or on account of, any present or future taxes.

10.5 All of the obligations of the Back Stop Parties under this Agreement are several and not joint or joint and several and none of the Back Stop Parties shall have any obligation or liability to any Party or any other person as a result of any failure or breach of the obligations of any other such person under this Agreement.

10.6 The Back Stop Parties irrevocably appoint GLAS Agency of 45 Ludgate Hill, London EC4M 7JU (Attention: Paul Cattennole, TES@glas.agency, +44 20 3597 2940) as their agent to receive on their behalf in England or Wales service of any proceedings under Clause 12.2. Such service shall be deemed completed on delivery to such agent (whether or not it is forwarded to and received by the Back Stop Parties) and shall be valid until such time as New Amryt Topco has received prior written notice that such agent has ceased to act as agent. If for any reason such

agent ceases to be able to act as agent or no longer has an address in England or Wales, the Back Stop Parties shall forthwith appoint a substitute acceptable to the Back Stop Parties and deliver to New Amryt Topco the new agent’s name and address within England and Wales.

10.7 The parties acknowledge and agree that on and from the effectiveness of the Scheme, New Amryt TopCo shall assume all rights and obligations of the Company hereunder, and shall be substituted for the Company as a party to this Agreement for all purposes hereunder, mutatis mutandis. The parties shall, and the Company shall procure that New Amryt TopCo shall, enter into all such agreements and execute all such further contracts as are required to give effect to this Clause 10.7. Any references in this Agreement to the Company Shareholders, the Company and its corporate details or place of incorporation shall be construed accordingly.

10.8 If any ruling is made by the UK Panel on Takeovers and Mergers (the “Panel”) that any provision of this Agreement is not permitted by the Takeover Code, such provision shall be given no effect. The parties shall use reasonable efforts to replace such provision with a valid and enforceable provision which is acceptable to the Panel and carries out, as closely as possible, the intentions of the patties.

11. ENTIRE AGREEMENT

11.1 This Agreement, and any other agreement entered into in connection with this Agreement, constitute the entire and only agreements between the parties relating to the subject matter of this Agreement.

11.2 If any provision of this Agreement shall be held to be illegal, invalid or unenforceable, in whole or in part, such provision or relevant part shall be deemed not to form part of this Agreement but the legality, validity and enforceability of the remainder of this Agreement shall not be affected.

12. APPLICABLE LAW

12.1 This Agreement shall be governed by and construed in accordance with English law.

12.2 Each of the parties hereby submits to the non-exclusive jurisdiction of the courts of England and Wales in relation to any matters arising out of this Agreement. This Clause 12 shall be without prejudice to the right of any patty to bring proceedings in any other jurisdiction for the purpose of enforcement or execution of any judgment or other settlement in any other court.

12.3 Notwithstanding Clause 12.2, each Back Stop Party shall retain the right to join the Company to proceedings in connection with the Capital Raise, the transactions contemplated by this Agreement or any dispute, to which such Back Stop Party is a party, in any other court (or courts) of competent jurisdiction.

IN WITNESS of which the parties have signed this Agreement on the date set out above.

AMRYT PHARMA PLC

By:	/s/ Rory Nealon
Name: Rory Nealon
Title: Director

ATHYRIUM OPPORTUNITIES II ACQUISTION 2 LP

By: Athyrium Opportunities Associates II LP, its general partner

By: Athyrium GP Holdings LLC, its general partner

By:	/s/ Andrew C. Hyman
Name:	Andrew C. Hyman
Title:	Authorized Signatory

ATHYRIUM OPPORTUNITIES III ACQUISTION 2 LP

By: Athyrium Opportunities Associates III LP, its general partner

By: Athyrium Opportunities Associates III GP LLC, its general partner

By:	/s/ Andrew C. Hyman
Name:	Andrew C. Hyman
Title:	Authorized Signatory

HIGHBRIDGE MSF INTERNATIONAL LTD.

By:	/s/ Jonathan Segal
Name: Jonathan Segal
Title: Managing Director

1992 TACTICAL CREDIT MASTER FUND, L.P.

By:	/s/ Jonathan Segal
Name: Jonathan Segal
Title: Managing Director

HIGHBRIDGE SCF SPECIAL SITUATIONS SPV, L.P.

By:	/s/ Jonathan Segal
Name: Jonathan Segal
Title: Managing Director

NINETEEN77 GLOBAL MULTI-STRATEGY ALPHA MASTER LIMITED

By: UBS O’Connor LLC, its investment adviser

By:	/s/ James Del Medico
Name: James Del Medico
Title: Executive Director

By:	/s/ Connor Burke
Name: Connor Burke
Title: Director

NINETEEN77 GLOBAL CONVERTIBLE BOND MASTER LIMITED

By: UBS O’Connor LLC, its investment adviser

By:	/s/ James Del Medico
Name: James Del Medico
Title: Executive Director

By:	/s/ Connor Burke
Name: Connor Burke
Title: Director

WHITEBOX RELATIVE VALUE PARTNERS, LP
By: Whitebox Advisors LLC its investment manager

By:	/s/ Luke Harris
Name: Luke Harris
Title: Deputy General Counsel

WHITEBOX GT FUND, LP
By: Whitebox Advisors LLC its investment manager

By:	/s/ Luke Harris
Name: Luke Harris
Title: Deputy General Counsel

WHITEBOX MULTI-STRATEGY PARTNERS, LP
By: Whitebox Advisors LLC its investment manager

By:	/s/ Luke Harris
Name: Luke Harris
Title: Deputy General Counsel

PANDORA SELECT PARTNERS, LP
By: Whitebox Advisors LL its investment manager

By:	/s/ Luke Harris
Name: Luke Harris
Title: Deputy General Counsel

SCHEDULE
FORM OF NOTIFICATION FORM

[Back Stop Party]
[Address]

(the “Back Stop Party”, “you”)

[l]

Dear Sirs

We refer to the backstop subscription agreement dated [l]2019, and any valid amendments thereto, between, inter alia, Amryt Pharma PLC and the Back Stop Party (the “Agreement”).

The Company hereby declares and undertakes in favour of the Back Stop Party that all of the Conditions have been satisfied or validly waived in accordance with the terms of the Agreement.

In accordance with the Agreement, we hereby notify you that you are required to subscribe for [l] Remaining Shares for an aggregate subscription price of $[l], and such Remaining Shares shall be issued to you on [l] (the “Subscription Date”).

Payment for, and the allotment and issue of, the [l] Remaining Shares will be effected in accordance with the provisions of Clauses 3 and 4.2 to the Agreement.

Defined terms used in this letter have the same meaning as set out in the Agreement unless defined herein.

Yours faithfully,

For and on behalf of
AMRYT PHARMA PLC

By:
Name:

EX-10.4

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.4

REGISTRATION RIGHTS AGREEMENT

This REGISTRATION RIGHTS AGREEMENT, dated as of September 25, 2019 (the “Effective Date”), is made by and among Amryt Pharma Holdings Plc (to be renamed Amryt Pharma plc), a company incorporated under the laws of England and Wales with the registered number 12107859 and registered address at Dept 920a, 196 High Road, London, N22 8HH (the “Company”), Highbridge MSF International Ltd., an exempted company incorporated under the laws of the Cayman Islands (“MSF”), Highbridge Tactical Credit Master Fund, L.P. (previously known as 1992 Tactical Credit Master Fund, L.P.), an exempted limited partnership organized under the laws of the Cayman Islands (“Tactical Credit Master Fund”) and Highbridge SCF Special Situations SPV, L.P., an exempted limited partnership formed under the laws of the Cayman Islands, (together with MSF and Tactical Credit Master Fund, “Highbridge”), Athyrium Opportunities II Acquisition 2 LP, a limited partnership formed under the laws of Delaware (“Athyrium II”), and Athyrium Opportunities III Acquisition 2 LP, a limited partnership formed under the laws of Delaware (together with Athyrium II, “Athyrium”, and together with Highbridge, the “Current Shareholders”), and any other Person who becomes a party to this Agreement pursuant to the provisions hereof (together with the Current Shareholders, each, individually, a “Shareholder” and, collectively, the “Shareholders”).

WHEREAS, Aegerion Pharmaceuticals, Inc., a Delaware corporation, the Company and the Current Shareholders entered into that certain Restructuring Support Agreement, dated as of May 20, 2019 (the “Restructuring Support Agreement”); and

WHEREAS, in connection with the Restructuring Support Agreement and the transactions contemplated thereby, the Company and each of the Shareholders desire, for their mutual benefit and protection, to enter into this Agreement to set forth their respective rights and obligations with respect to the affairs of the Company and the capital stock held by the Shareholders.

NOW, THEREFORE, in consideration of the recitals and the mutual premises, covenants and agreements contained herein and other good and valuable consideration, the receipt and sufficiency of which are hereby acknowledged, the parties hereto agree as follows:

Section 1.           Definitions; Rules of Construction.

(a)           For purposes of this Agreement, each of the following capitalized terms shall have the meaning ascribed to it in this Section 1:

“Affiliate” means as to any Person, any other Person directly or indirectly controlling or controlled by or under direct or indirect common control with such specified Person, including any Person advised by investment advisers under common control or any fund or account managed or advised by any Person, provided, however, that neither the Company nor any of its Subsidiaries shall be deemed an Affiliate of any of the Shareholders (and vice versa). For purposes of this definition, “control,” as used with respect to any Person, means the possession, directly or indirectly, of the power to direct or cause the direction of the management or policies of such Person, whether through the ownership of voting securities, by agreement or otherwise or by virtue of any powers conferred by the constitutional or corporate documents, or any other document, regulating that or any other Person. For purposes of this definition, the terms “controlling,” “controlled by” and “under common control with” have correlative meanings.

 

“Agreement” means this Registration Rights Agreement, as originally executed and as it may from time to time be supplemented or amended by one or more agreements supplemental hereto entered into pursuant to the applicable provisions hereof.

“AIM” means the Alternative Investment Market of the London Stock Exchange.

“AIM Rules” means the AIM Rules for Companies in force from time to time.

“Articles” means the articles of association of the Company from time to time.

“Athyrium” – as defined in the Preamble.

“Athyrium Directors” means the Directors designated for nomination to the Board by Athyrium in accordance with the terms of the Plan Funding Agreement.

“Athyrium II” – as defined in the Preamble.

“Board” means the board of directors of the Company.

“Business Day” means any day other than a Saturday, Sunday or other day in New York, London or Dublin on which banking institutions are authorized by law or regulations to close.

“Chairman” – as defined in Section 4(a).

“Companies Act” means The Companies Act 2006, as amended

“Company” – as defined in the Preamble.

“Company Directors” means the chief executive officer of the Company together with the directors designated for nomination to the Board by the chief executive officer of the Company in accordance with the terms of the Plan Funding Agreement.

“Current Shareholders” – as defined in the Preamble.

“Damages” – as defined in Section 3(i)(i).

“Demand Notice” – as defined in Section 3(a)(ii).

“Director” – as defined in Section 4(a)(i).

“Effective Date” – as defined in the Preamble.

“Euronext” means the Euronext Growth Market of Euronext Dublin.

2

“Euronext Advisor” means the nominated adviser from time to time of the Company for the purposes of the Euronext Rules.

“Euronext Rules” means the Euronext Growth Rules for Companies in force from time to time.

“Governmental Authority” means any regional, federal, state or local legislative, executive or judicial body or agency, any court of competent jurisdiction, any department, political subdivision or other governmental authority or instrumentality, or any arbitral authority, in each case, whether domestic or foreign.

“Highbridge” – as defined in the Preamble.

“Highbridge Directors” means the Directors designated for nomination to the Board by Highbridge in accordance with the terms of the Plan Funding Agreement.

“Independent” means a person who has been proposed as a Director of the Company who satisfies the criteria of an ‘independent director’ for the purposes of the NASDAQ Stock Market standards and the Quoted Companies Alliance corporate governance code.

“Initiating Holders” means, collectively, Shareholders who properly initiate a registration request under this Agreement.

“Maximum Offering Size” – as defined in Section 3(d)(ii).

“NOMAD” means the nominated adviser from time to time of the Company for the purposes of the AIM Rules.

“Ordinary Shares” means the ordinary shares of £0.01 each and the American Depositary Shares representing such shares in the share capital of the Company.

“Permitted Transfer” means one or more Transfers by a Shareholder made (a) to another Shareholder or (b) to one of its Affiliates. In addition, “Permitted Transfer” shall include one or more Transfers from a Person receiving Shares pursuant to the prior sentence to the Shareholder who originally transferred such Shares to such recipient.

“Permitted Transferee” means a Transferee receiving Shares pursuant to a Transfer made in accordance with the definition of Permitted Transfer.

“Person” means an individual, a company, a partnership, a joint venture, a limited liability company or limited liability partnership, an association, a trust, estate or other fiduciary, any other legal entity, and any Governmental Authority.

“Piggyback Registration” – as defined in Section 3(b)(i).

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“Plan Funding Agreement” means the plan funding agreement entered into between the Amryt Pharma PLC and Aegerion Pharmaceuticals, Inc, dated as of May 20, 2019.

“Public Offering” means any offering by the Company of its Ordinary Shares to the public pursuant to an effective registration statement under the Securities Act or any comparable statement under any comparable federal statute then in effect (other than any registration statement on Form S-8 or Form F-4 or any successor forms thereto).

“Registrable Securities” means, at any time, any Shares until (a) a registration statement covering such Shares has been declared effective by the SEC and such Shares have been disposed of pursuant to such effective registration statement, (b) such Shares are sold by such Shareholder under circumstances in which all of the applicable conditions of Rule 144 (or any similar provisions then in force) under the Securities Act are met or (c) such Shares may be resold without subsequent registration under the Securities Act in a single transaction under Rule 144 without regard to the volume, manner of sale and other requirements under Rule 144 applicable to an “affiliate” (as defined in Rule 144) of the Company and the Company has delivered a new certificate or other evidence of ownership for such Shares not bearing any legend.

“Registration Expenses” – as defined in Section 3(g).

“Restructuring Support Agreement” – as defined in the Recitals.

“SEC” means the Securities and Exchange Commission.

“Securities Act” means the Securities Act of 1933, as amended, or any successor federal statute, and the rules and regulations of the Securities and Exchange Commission thereunder, as the same shall be in effect from time to time.

“Selling Expenses” means all underwriting discounts, selling commissions, and stock transfer taxes applicable to the sale of Registrable Securities, and fees and disbursements of counsel for any Shareholder, except for the fees and disbursements of the Selling Holder Counsel borne and paid by the Company as provided in Section 3(g).

“Selling Shareholder Counsel” – as defined in Section 3(g).

“Shares” means all Ordinary Shares held by any Shareholder that is a party to this Agreement, whether now owned or hereafter acquired, including Ordinary Shares issued upon (i) conversion of those certain Convertible Senior Notes issued by the Company or (ii) exercise of any warrants issued by the Company.

“Shareholder(s)” – as defined in the Preamble.

“Shareholder Directors” means the Highbridge Directors and the Athyrium Directors, collectively.

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“Subsidiary” means as to a Person, any corporation, partnership, limited liability company or other organization, whether incorporated or unincorporated, of which at least a majority of the securities or other interests having by their terms voting power to elect a majority of the board of directors or others performing similar functions with respect to such corporation or other organization is directly or indirectly beneficially owned or controlled by such party or by any one or more of its subsidiaries, or by such party and one or more of its subsidiaries.

“Tactical Credit Master Fund” – as defined in the Preamble.

“Transaction Documents” means this Agreement, the Restructuring Support Agreement, the Plan Funding Agreement, the Voting Support Agreement, dated as of May 20, 2019, by and among certain stockholders of the Company, that certain Debtors’ Joint Chapter 11 Plan, dated as of May 20, and each other contract, exhibit, schedule, certificate and other document being delivered pursuant to, or in furtherance of the transactions contemplated by, this Agreement, the Restructuring Support Agreement or the Plan Funding Agreement.

“Transfer” means the direct or indirect (whether by act, omission or operation of law), sale, exchange, transfer, hypothecation, negotiation, gift, conveyance in trust, pledge, assignment, encumbrance, or other disposal of Ordinary Shares, or the assignment for the benefit of creditors, attachment, levy or other seizure by any creditor (whether or not pursuant to judicial process) of Ordinary Shares, or the passage or distribution of Ordinary Shares under judicial order or legal process.

“Transferee” means a Person to whom Ordinary Shares are Transferred.

(b)           The following provisions shall be applied wherever appropriate herein:

(i)          for purposes of this Agreement, the words “hereof,” “herein,” “hereby” and other words of similar import refer to this Agreement as a whole unless otherwise indicated. Whenever the singular is used herein, the same shall include the plural, and whenever the plural is used herein, the same shall include the singular, where appropriate. All terms defined herein in the singular shall have the same meaning when used in the plural; all terms defined herein in the plural shall have the same meaning when used in the singular;

(ii)         with regard to each and every term and condition of this Agreement, the parties hereto understand and agree that the same have or has been mutually negotiated, prepared and drafted, and that if at any time the parties hereto desire or are required to interpret or construe any such term or condition or any agreement or instrument subject hereto, no consideration shall be given to the issue of which party actually prepared, drafted or requested any term or condition of this Agreement;

(iii)        all references herein to Sections, subsections, paragraphs, subparagraphs and clauses shall be deemed references to such parts of this Agreement, unless the context shall otherwise require;

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(iv)       all pronouns and any variations thereof refer to the masculine, feminine or neuter, singular or plural, as the context may require;

(v)        the words “include” and “including” and variations thereof shall not be deemed terms of limitation, but rather shall be deemed to be followed by the words “without limitation”;

(vi)       when calculating the period of time before which, within which or following which any act is to be done or step taken pursuant to this Agreement, the date that is the reference date in calculating such period shall be excluded. If the last day of such period is a non-Business Day, the period in question shall end on the next succeeding Business Day;

(vii)      any accounting terms not specifically defined herein shall be construed in accordance with International Financial Reporting Standards as adopted by the European Union;

(viii)     the Exhibits and Schedules, if any, attached hereto are incorporated herein by reference and shall be considered part of this Agreement;

(ix)        any consent or approval rights of the Board or the Company contained herein shall be exercised in the sole and absolute discretion of the Board or the Company, as applicable, unless otherwise expressly set forth herein; and

(x)         all references to $, currency, monetary values and dollars set forth herein shall mean United States (U.S.) dollars.

Section 2.           Transfers. No person to whom a Shareholder transfers any Ordinary Shares shall be entitled to the rights and powers of this Agreement unless such Transferee is a Permitted Transferee, in which case such Permitted Transferee shall have all rights and powers, and shall be subject to the restrictions and liabilities, of a Shareholder under this Agreement. No Permitted Transfer may be made unless the Permitted Transferee agrees in writing to be bound by the provisions of this Agreement as though it were a Shareholder hereunder.

Section 3.           Registration Rights. The Company covenants and agrees as follows:

(a)           Listing; Demand Registration.

(i)         The Company shall make application to the NASDAQ Stock Market for the listing of the Ordinary Shares (including the Shares) and use all reasonable best efforts to cause such Ordinary Shares to be approved for listing on the NASDAQ Stock Market within ninety (90) days from the Effective Date; provided, that if, despite the Company’s reasonable best efforts, the Company is not listed on the NASDAQ Stock Market within such ninety (90) day period, the Company shall continue to use all reasonable best efforts to cause the Ordinary Shares to be listed on the NASDAQ Stock Market.

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(ii)        Beginning on the first date after the date on which the Ordinary Shares are approved for listing on the NASDAQ Stock Market, if at any time the Company receives a written request from Initiating Holders of at least [***] of the aggregate amount of Registrable Securities then outstanding that the Company file a registration statement under the Securities Act with respect to a specified amount of Registrable Securities held by such Initiating Holders, the Company shall (i) within [***] after the date such request is given, give notice thereof (the “Demand Notice”) to all Shareholders other than the Initiating Holders, after which such Shareholders shall have [***] from the date of the Demand Notice to request by written notice that a specified amount of Registrable Securities be included in any such registration statement; and (ii) as soon as practicable, and in any event within [***] after the date on which such request by the Initiating Holders is received, file a registration statement under the Securities Act covering all Registrable Securities requested to be included in such registration by such Initiating Holders and any other Shareholders, and in each case, subject to the limitations of Section 3(b)(ii) and Section 3(d).

(iii)       Notwithstanding the foregoing obligations, if the Company furnishes to Initiating Holders requesting a registration pursuant to this Section 3(a) a certificate signed by the Company’s chief executive officer stating that in the good faith judgment of the Board it would be materially detrimental to the Company and its shareholders for such registration statement to either become effective or remain effective for as long as such registration statement otherwise would be required to remain effective because such action would (i) materially interfere with a significant acquisition, contemplated financing, corporate reorganization, merger or other similar transaction involving the Company; (ii) require premature disclosure of material information that the Company has a bona fide business purpose for preserving as confidential; (iii) render the Company unable to comply with requirements under the Securities Act, Exchange Act, rules of the NASDAQ Stock Market, AIM or Euronext; or (iv) otherwise have a material adverse effect on the Company, then the Company shall have the right to defer taking action with respect to such filing for a period of not more than [***] after the request of the Initiating Holders is given; provided, however, that the Company may not invoke this right more than twice in any twelve (12) month period; and provided further that the Company shall not register any Ordinary Shares for its own account or that of any other Shareholder during such [***] period.

(iv)       The Company shall not be required to cause more than two (2) registrations pursuant to Section 3(a)(ii) to be effected within any twelve (12) month period.

(v)        The Company shall be liable for and pay all Registration Expenses in connection with any demand registration.

(vi)       A registration shall not be counted as “effected” for purposes of this Section 3(a) until such time as the applicable registration statement has been declared effective by the SEC, unless the Initiating Holders withdraw their request for such registration and forfeit their right to one demand registration statement, in which case such withdrawn registration statement shall be counted as “effected” for purposes of this

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Section 3(a); provided, that if such withdrawal is during a period the Company has deferred taking action pursuant to Section 3(a)(iii), then the Initiating Holders may withdraw their request for registration and such registration will not be counted as “effected” for purposes of this Section 3(a).

(b)           Piggyback Registration.

(i)         If the Company proposes to register any Ordinary Shares under the Securities Act (other than a registration on Form S-8, F-4 or any successor forms, relating to securities of the Company issuable upon exercise of employee stock options or in connection with any employee benefit or similar plan of the Company, in connection with a direct or indirect acquisition by the Company of another Person, or pursuant to Section 3(a) hereof), whether or not for sale for its own account, the Company shall each such time give prompt notice at least ten (10) Business Days prior to the anticipated filing date of the registration statement to each Shareholder, which notice shall set forth such Shareholder’s rights under this Section 3(b)(i) and shall offer such Shareholder the opportunity to include in such registration statement the amount of Registrable Securities of the same class or series as those proposed to be registered that such Shareholder may request (a “Piggyback Registration”), subject to the provisions of Section 3(b)(ii), Upon the request of any such Shareholder made within five (5) Business Days after the receipt of notice from the Company (which request shall specify the amount of Registrable Securities to be registered), the Company shall use reasonable best efforts to effect the registration under the Securities Act of all Registrable Securities so requested to register by all such Shareholders; provided that (A) if such registration involves a Public Offering, all such Shareholders must sell their Registrable Securities to the underwriter(s) selected on the same terms and conditions as apply to the Company, and (B) if, at any time after giving notice of its intention to register any Ordinary Shares pursuant to this Section 3(b)(1)and prior to the effective date of the registration statement filed in connection with such registration, the Company shall determine for any reason not to register such Ordinary Shares, the Company shall give notice to all Shareholders and, thereupon, shall be relieved of its obligation to use reasonable best efforts to register any Registrable Securities. No registration effected under this Section 3(b) shall relieve the Company of its obligations to effect a demand registration to the extent required by Section 3(a). The Company shall pay all Registration Expenses in connection with each Piggyback Registration.

(ii)        If a Piggyback Registration involves a Public Offering (other than any demand registration, in which case the provisions with respect to priority of inclusion in such offering set forth in Section 3(d)(i) shall apply) and the underwriter(s) advise the Company that, in its view, the number of Ordinary Shares of the Company that the Company and the selling Shareholders intend to include in such registration exceeds the Maximum Offering Size (as defined below), the Company shall include in such registration, in the following priority, up to the Maximum Offering Size:

A.         first, so much of the Ordinary Shares of the Company proposed to be registered for the account of the Company as would not cause the offering to exceed the Maximum Offering Size;

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B.       second, to the extent the Maximum Offering Size exceeds the amount of shares included pursuant to Section 3(b)(ii)A, all Registrable Securities requested to be included in such registration by any Shareholder (allocated, if necessary for the offering not to exceed the Maximum Offering Size, pro rata on the basis of the relative number of Registrable Securities so requested to be included in such registration by each such Shareholder); and

C.       third, any securities proposed to be registered for the account of any other Persons with such priorities among them as the Company shall determine.

(c)           Underwriting Requirements.

(i)        If, pursuant to Section 3(a), the Initiating Holders intend to distribute the Registrable Securities covered by their request by means of an underwriting, they shall so advise the Company as a part of their request made pursuant to Section 3(a), and the Company shall include such information in the Demand Notice. The underwriter(s) will be selected by the Board and shall be reasonably acceptable to a majority in interest of the Initiating Holders. In such event, the right of any Shareholder to include such Shareholder’s Registrable Securities in such registration shall be conditioned upon such Shareholder’s participation in such underwriting and the inclusion of such Shareholder’s Registrable Securities in the underwriting to the extent provided herein. All Shareholders proposing to distribute their Shares through such underwriting shall (together with the Company as provided in Section 3(d)(v)) enter into an underwriting agreement in customary form with the underwriter(s) selected for such underwriting. Notwithstanding any other provision of this Section 3(c) if the underwriter(s) advise(s) the Initiating Holders in writing that marketing factors require a limitation on the number of shares to be underwritten, then the Initiating Holders shall so advise all holders of Registrable Securities that otherwise would be underwritten pursuant hereto, and the number of Registrable Securities that may be included in the underwriting shall be allocated among such holders of Registrable Securities, including the Initiating Holders, in proportion (as nearly as practicable) to the number of Registrable Securities owned by each Shareholder or in such other proportion as shall mutually be agreed to by all such selling Shareholders; provided, however, that the number of Registrable Securities held by the Shareholders to be included in such underwriting shall not be reduced unless all other Shares are first entirely excluded from the underwriting.

(ii)       In connection with any offering involving an underwriting of shares of the Company pursuant to Section 3(b), the Company shall not be required to include any of the Shareholders’ Registrable Securities in such underwriting unless the Shareholders accept the terms of the underwriting as agreed upon between the Company and its underwriters, and then only in such quantity as the underwriters in their sole discretion determine will not jeopardize the success of the offering by the Company. If the total number of Registrable Securities requested by the Shareholder to be included in such offering causes the total amount of Ordinary Shares included in the offering to exceed the amount that the underwriters in their reasonable discretion determine is compatible with the success of the offering, then the Company shall be required to include in the offering

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only the amount of Registrable Securities, which the underwriters and the Company in their sole discretion determine will not jeopardize the success of the offering (the “Maximum Offering Size”). If the underwriters determine that less than all of the Registrable Securities requested to be registered can be included in such offering, then the Registrable Securities that are included in such offering shall be allocated among the selling Shareholders in accordance with the priority listed below, up to the Maximum Offering Size:

A.       first, all Registrable Securities requested to be registered by the selling Shareholders (allocated, if necessary for the offering not to exceed the Maximum Offering Size, pro rata on the basis of the relative number of Registrable Securities, or Ordinary Shares, as applicable, so requested to be included in such registration); and

B.       second, any Ordinary Shares proposed to be registered for the account of the Company or any other Persons with such priorities among them as the Company shall determine.

(d)          Obligations of the Company. When ever required under this Section 3 to effect the registration of any Registrable Securities, the Company shall, as expeditiously as reasonably possible: prepare and file with the SEC a registration statement with respect to such Registrable Securities and use its reasonable best efforts to cause such registration statement to become effective and, upon the request of the holders of a majority of the Registrable Securities registered thereunder, keep such registration statement effective for a period of up to one hundred twenty (120) days or, if earlier, until the distribution contemplated in the registration statement has been completed; provided, however, that (A) such one hundred twenty (120) day period shall be extended for a period of time equal to the period the Shareholder refrains, at the request of an underwriter of Ordinary Shares of the Company, from selling any Shares included in such registration, and (B) in the case of any registration of Registrable Securities on Form F-3 that are intended to be offered on a continuous or delayed basis, subject to compliance with applicable SEC rules, such one hundred twenty (120) day period shall be extended for up to one hundred eighty (180) days, if necessary, to keep the registration statement effective until all such Registrable Securities are sold;

(ii)          prepare and file with the SEC such amendments and supplements to such registration statement, and the prospectus used in connection with such registration statement, as may be necessary to comply with the Securities Act in order to enable the disposition of all Ordinary Shares covered by such registration statement;

(iii)         furnish to the selling Shareholders such numbers of copies of a prospectus, including a preliminary prospectus, as required by the Securities Act, and such other documents as such Shareholders may reasonably request in order to facilitate their disposition of their Registrable Securities;

(iv)         use its commercially reasonable efforts to register and qualify the Ordinary Shares covered by such registration statement under such other securities or

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blue-sky laws of such jurisdictions as shall be reasonably requested by the selling Shareholders; providedthat the Company shall not be required to qualify to do business or to file a general consent to service of process in any such states or jurisdictions, unless the Company is already subject to service in such jurisdiction and except as may be required by the Securities Act;

(v)          in the event of any underwritten Public Offering, enter into and perform its obligations under an underwriting agreement, in usual and customary form, with the underwriter(s) of such offering;

(vi)        use its commercially reasonable efforts to cause all such Registrable Securities covered by such registration statement to be listed on a national securities exchange or trading system and each securities exchange and trading system (if any) on which similar securities issued by the Company are then listed;

(vii)       provide a transfer agent and registrar for all Registrable Securities registered pursuant to this Agreement and provide a CUSIP number for all such Registrable Securities, in each case not later than the effective date of such registration;

(viii)       promptly make available for inspection by the selling Shareholders, any underwriter(s) participating in any disposition pursuant to such registration statement, and any attorney or accountant or other agent retained by any such underwriter, all financial and other records, pertinent corporate documents, and properties of the Company, and cause the Company’s officers, directors, employees, and independent accountants to supply all information reasonably requested by any such underwriter, attorney, accountant, or other agent, in each case, as necessary or advisable to verify the accuracy of the information in such registration statement and to conduct appropriate due diligence in connection therewith;

(ix)         notify each selling Shareholder, promptly after the Company receives notice thereof, of the time when such registration statement has been declared effective or a supplement to any prospectus forming a part of such registration statement has been filed; and

(x)          after such registration statement becomes effective, notify each selling Shareholders of any request by the SEC that the Company amend or supplement such registration statement or prospectus.

In addition, the Company shall ensure that, at all times after any registration statement covering a Public Offering shall have become effective, its insider trading policy shall provide that the Company’s directors may implement a trading program under Rule 10b5-l of the Exchange Act.

(e)          Furnish Information. It shall be a condition precedent to the obligations of the Company to take any action pursuant to this Section 3 with respect to the Registrable Securities of any selling Shareholders that such Shareholders shall furnish to the Company such information regarding itself, the Registrable Securities held by it, and the intended method of

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disposition of such Ordinary Shares as is reasonably required to effect the registration of such Shareholder’s Registrable Securities.

(f)         Expenses of Registration. All expenses (other than Selling Expenses) incurred in connection with registrations, filings, or qualifications pursuant to this Section 3, including all registration, filing, and qualification fees; printers’ and accounting fees; fees and disbursements of counsel for the Company (such expenses, “Registration Expenses”); and the reasonable fees and disbursements of one counsel for the selling Shareholders (“Selling Shareholder Counsel”), shall be borne and paid by the Company. All Selling Expenses relating to Registrable Securities registered pursuant to this Section 3 shall be borne and paid by the Shareholders pro rata on the basis of the number of Registrable Securities registered on their behalf.

(g)        Delay of Registration. No Shareholder shall have any right to obtain or seek an injunction restraining or otherwise delaying any registration pursuant to this Agreement as the result of any controversy that might arise with respect to the interpretation or implementation of this Section 3.

(h)        Indemnification. If any Registrable Securities are included in a registration statement under this Section 3:

(i)         The Company will indemnify and hold harmless each selling Shareholder, and the partners, members, officers, directors, and shareholders of each such Shareholder; legal counsel and accountants for each such Shareholder; any underwriter (as defined in the Securities Act) for each such Shareholder; and each Person, if any, who controls such Shareholder or underwriter within the meaning of the Securities Act or the Exchange Act, against any against any and all losses, claims, damages, liabilities and expenses (including reasonable expenses of investigation and reasonable attorneys’ fees and expenses) (“Damages”), caused by or relating to any untrue statement or alleged untrue statement of a material fact contained in any registration statement or prospectus relating to the Registrable Securities (as amended or supplemented if the Company shall have furnished any amendments or supplements thereto) or any preliminary prospectus, or caused by or relating to any omission or alleged omission to state therein a material fact required to be stated therein or necessary to make the statements therein not misleading, except insofar as such Damages are caused by or related to any such untrue statement or omission or alleged untrue statement or omission so made based upon information furnished in writing to the Company by such selling Shareholder or on such Shareholder’s behalf expressly for use therein; provided that, with respect to any untrue statement or omission or alleged untrue statement or omission made in any preliminary prospectus, or in any prospectus, as the case may be, the indemnity agreement contained in this Section 3(h)(i) shall not apply to the extent that any Damages result from the fact that a current copy of the prospectus (or such amended or supplemented prospectus, as the case may be) was not sent or given to the Person asserting any such Damages at or prior to the written confirmation of the sale of the Registrable Securities concerned to such Person if it is determined that the Company has provided such prospectus to such selling Shareholder and it was the responsibility of such selling Shareholder to provide such Person with a current copy of the prospectus (or such amended or supplemented prospectus, as the case may be) and such current copy of the prospectus (or such

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amended or supplemented prospectus, as the case may be) would have cured the defect giving rise to such Damages. The Company also agrees to indemnify any underwriter(s) of the Registrable Securities, their officers and directors and each Person who controls such underwriter(s) within the meaning of Section 15 of the Securities Act or Section 20 of the Exchange Act on substantially the same basis as that of the indemnification of the selling Shareholders provided in this Section 3(h)(i).

(ii)        Each selling Shareholder, severally and not jointly, will indemnify and hold harmless the Company, and each of its directors, each of its officers who has signed any registration statement, each Person (if any), who controls the Company within the meaning of the Securities Act, legal counsel and accountants for the Company, any underwriter (as defined in the Securities Act), any other Shareholders selling Shares in any registration statement, and any controlling Person of any such underwriter or other Shareholder, against any Damages, in each case only to the extent that such Damages arise out of or are based upon actions or omissions made in reliance upon and in conformity with written information furnished by or on behalf of such selling Shareholder, but only (i) with respect to information furnished in writing by such selling Shareholder or on such selling Shareholder’s behalf expressly for use in any registration statement or prospectus relating to the Registrable Securities, or any amendment or supplement thereto, or any preliminary prospectus or (ii) to the extent that any Damages result from the fact that a current copy of the prospectus (or such amended or supplemented prospectus, as the case may be) was not sent or given to the Person asserting any such Damages at or prior to the written confirmation of the sale of the Registrable Securities concerned to such Person if it is determined that it was the responsibility of such selling Shareholder to provide such Person with a current copy of the prospectus (or such amended or. supplemented prospectus, as the case may be) and such current copy of the prospectus (or such amended or supplemented prospectus, as the case may be) would have cured the defect giving rise to such loss, claim, damage, liability or expense.

(iii)       Promptly after receipt by an indemnified party under this Section 3(h) of notice of the commencement of any action (including any governmental action) for which a party may be entitled to indemnification hereunder, such indemnified party will, if a claim in respect thereof is to be made against any indemnifying party under this Section 3(h), give the indemnifying party notice of the commencement thereof. The indemnifying party shall have the right to participate in such action and, to the extent the indemnifying party so desires, participate jointly with any other indemnifying party to which notice has been given, and to assume the defense thereof with counsel mutually satisfactory to the parties; provided, however, that an indemnified party (together with all other indemnified parties that may be represented without conflict by one counsel) shall have the right to retain one separate counsel, with the fees and expenses to be paid by the indemnifying party, if representation of such indemnified party by the counsel retained by the indemnifying party would be inappropriate due to actual or potential differing interests between such indemnified party and any other party represented by such counsel in such action.

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(iv)       To provide for just and equitable contribution to joint liability under the Securities Act in any case in which either: (i) any party otherwise entitled to indemnification hereunder makes a claim for indemnification pursuant to this Section 3(h) but it is judicially determined (by the entry of a final judgment or decree by a court of competent jurisdiction and the expiration of time to appeal or the denial of the last right of appeal) that such indemnification may not be enforced in such case, notwithstanding the fact that this Section 3(h) provides for indemnification in such case, or (ii) contribution under the Securities Act may be required on the part of any party hereto for which indemnification is provided under this Section 3(h), then, and in each such case, such parties will contribute to the aggregate losses, claims, damages, liabilities, or expenses to which they may be subject (after contribution from others) in such proportion as is appropriate to reflect the relative fault of each of the indemnifying party and the indemnified party in connection with the statements, omissions, or other actions that resulted in such loss, claim, damage, liability, or expense, as well as to reflect any other relevant equitable considerations. The relative fault of the indemnifying party and of the indemnified party shall be determined by reference to, among other things, whether the untrue or allegedly untrue statement of a material fact, or the omission or alleged omission of a material fact, relates to information supplied by the indemnifying party or by the indemnified party and the parties’ relative intent, knowledge, access to information, and opportunity to correct or prevent such statement or omission; provided, however, that, in any such case (x) no Shareholder will be required to contribute any amount in excess of the proceeds from the offering received by such Shareholder (net of any Selling Expenses paid by such Shareholder) (y) no Person guilty of fraudulent misrepresentation (within the meaning of Section 11(f) of the Securities Act) will be entitled to contribution from any Person who was not guilty of such fraudulent misrepresentation; and provided further that in no event shall a Shareholder’s liability pursuant to this Section 3(h)(iv), when combined with the amounts paid or payable by such Shareholder pursuant to Section 3(h)(ii), exceed the proceeds from the offering received by such Shareholder (net of any Selling Expenses paid by such Shareholder), except in the case of willful misconduct or fraud by such Shareholder.

(v)        Notwithstanding the foregoing, to the extent that the provisions on indemnification and contribution contained in the underwriting agreement entered into in connection with the underwritten public offering are in conflict with the foregoing provisions, the provisions in the underwriting agreement shall control.

(vi)       Unless otherwise superseded by an underwriting agreement entered into in connection with the underwritten public offering, the obligations of the Company and Shareholders under this Section 3(h) shall survive the completion of any offering of Registrable Securities in a registration under this Section 3, and otherwise shall survive the termination of this Agreement.

(i)         Reports Under Exchange Act. With a view to making available to the Shareholders the benefits of SEC Rule 144 and any other rule or regulation of the SEC that may at any time permit a Shareholder to sell Shares of the Company to the public without registration or pursuant to a registration on Form F-3, the Company shall:

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(i)        make and keep available adequate current public information, as those terms are understood and defined in SEC Rule 144;

(ii)        use commercially reasonable efforts to file with the SEC in a timely manner all reports and other documents required of the Company under the Securities Act and the Exchange Act (at any time after the Company has become subject to such reporting requirements); and

(iii)       furnish to any Shareholder, so long as the Shareholder owns any Registrable Securities, forthwith upon request (i) to the extent accurate, a written statement by the Company that it has complied with the reporting requirements of SEC Rule 144, the Securities Act, and the Exchange Act, or that it qualifies as a registrant whose Shares may be resold pursuant to Form F-3 and (ii) such other information as may be reasonably requested in availing any Shareholder of any rule or regulation of the SEC that permits the selling of any such Shares without registration or pursuant to Form F-3.

(j)         Limitations on Subsequent Registration Rights. From and after the date of this Agreement, the Company shall not, without the prior written consent of the Shareholders holding at least forty-five percent (45%) of the Registrable Securities then outstanding, enter into any agreement with any Shareholder or prospective holder of any Ordinary Shares of the Company that would provide to such holder or prospective holder the right to include Ordinary Shares in any registration on other than either (i) a pro rata basis with respect to the Registrable Securities or (ii) on a subordinate basis after all Shareholders have had the opportunity to include in the registration and offering all shares of Registrable Securities that they wish to so include.

Section 4.      Board of Directors.

(a)        Composition of the Board; Vacancies; Removal; Nominations.

 (i)         The initial Board of the Company shall be constituted in accordance with the terms of the Plan Funding Agreement, comprising: (A) one non-independent executive director, being Joe Wiley as the chief executive officer; (B) one independent Chairman proposed by the Company; (C) one independent director proposed by the Company; (D) two independent directors proposed by Highbridge; (E) one independent director proposed by Athyrium; and (F) one non-independent director proposed by Athyrium (the “Shareholder Representative”). All directors proposed must be mutually agreed by the parties prior to appointment. Highbridge may subsequently select a non-independent director, to replace one of its independent appointments, subject to the shareholding requirements noted below in which case this director shall also be a “Shareholder Representative” under this Agreement.

 (ii)        The chief executive officer, as at Effective Date, shall be Joe Wiley and he shall continue as the chief executive officer of the Company for not less than the two (2) years after the Closing (as defined in the Plan Funding Agreement), unless he (A) resigns, (B) is otherwise removed from the position by a vote of a majority of the Board, which majority vote must include the vote of at least one director appointed pursuant to

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Section 4(a)(i)(B) or Section 4(a)(i)(C), or (C) is removed for Cause (as defined in his Employment Agreement).

(iii)       The Board so constituted as described in Section 4(a)(i) shall be subject to compulsory retirement and put for re-election at the annual general meeting of the Company which is first held at least twenty-four (24) months after Closing occurs under the Plan Funding Agreement, and thereafter the composition of the Board shall be determined in accordance with the Articles, the Companies Act and/or applicable law, subject to the remainder of this Section 4(a).

(iv)       The number of Directors serving on the Board at any time shall be limited by the Articles to a maximum of seven (7), unless determined otherwise by special resolution of the Company.

(v)        For so long as Athyrium (including all of its Affiliates) continue to hold at least ten percent (10%) of the outstanding Ordinary Shares of the Company, or for so long as Highbridge (including all of its Affiliates) continue to hold at least ten percent (10%) of the outstanding Ordinary Shares of the Company, Athyrium or Highbridge as the case may be (and only Athyrium or Highbridge) shall be entitled to nominate any replacement of their respective Shareholder Representatives following the retirement (whether by rotation or otherwise) or resignation of the Shareholder Representative, and the Board shall appoint such replacement to serve as a Director until the next planned shareholders general meeting where such Director shall resign and his or her appointment to the Board shall then be subject to approval by a general resolution of the shareholders of the Company.

(vi)       Other than any Shareholder Representative subject to Section 4(a)(v), all other Directors of the Company shall hold office subject to the Articles of association of the Company, and shall be required to retire, entitled to resign, and entitled to be nominated for re-election, in accordance therewith.

(b)       Appointment Process.

(i)         For so long as the Company is listed on AIM and/or Euronext, each person proposed for appointment as a Director shall, as a condition to his or her appointment, be required to provide the NOMAD and/or the Euronext Advisor (as applicable) with such information and certifications as the NOMAD and/or the Euronext Advisor may reasonably require to enable them to satisfy themselves as to the suitability of such person to serve as a director of a company listed on AIM and/or Euronext.

(ii)       Any nomination for appointment or reappointment of a Shareholder Representative by either Highbridge or Athyrium or their respective Affiliates, pursuant to Section 4(a)(v), shall be made by written notice to the Company. The Company shall, subject to prior compliance with Section 4(b)(i), within fifteen (15) Business Days after the date of the notice from Highbridge or Athyrium, ensure that the person so nominated is proposed for appointment or reappointment (as the case may be) as a Director, at a meeting of the Board by way of resolution of the Board or by a written resolution of the

16

Directors and that such appointment is approved at the next meeting of the shareholders of the Company.

(iii)       For a period of two (2) years from the Effective Date, the chief executive officer of the Company shall have the right to nominate for appointment, subject to the prior unanimous approval of the Current Shareholders, which approval shall not be unreasonably withheld, the chairman of the Board (the “Chairman”). The Chairman shall not be affiliated with the Company. Following the second anniversary of the Effective Date, the Chairman shall be appointed by a majority vote of the Directors.

(c)       Quorum. Quorum for any meeting of the Board will require the attendance (telephonically or in person) of a majority of the full Board, including in each case any Shareholder Representative and the chief executive officer of the Company. Each of the Directors shall be provided with reasonable advance notice (not less than 72 hours) of any meeting of the Board and a reasonable number of options of reasonable times and dates for such meeting to be held.

(d)       Committees of the Board. The chief executive officer shall be entitled, where appropriate, to be appointed to serve on each duly constituted committee of the Board. Until the second anniversary of the Effective Date, the majority of each duly constituted committee shall be comprised of Directors appointed by Sections 4(a)(i)(D), 4(a)(i)(E) and 4(a)(i)(F) hereof.

(e)       Compensation. Each member of the Board designated pursuant to Section 4(a)above shall be entitled to reimbursement from the Company for his or her reasonable out of pocket expenses (including travel) incurred in attending any meeting of the Board or Subsidiary board of directors or any committee thereof, pursuant to Company policy.

(f)        Termination of Rights. Each Shareholder shall have the right upon written notice to the Company to terminate its rights and obligations pursuant to this Section 4.

Section 5.          D&O Insurance. Subject to the Companies Act and applicable law, the Company shall maintain directors’ and officers’ liability insurance and fiduciary liability insurance for all directors with insurers of recognized financial responsibility in such amounts as the Board determines to be prudent and customary for the Company’s business and operations. The Company and the Shareholders shall take all necessary action so that each of the directors shall be entitled to indemnification and advancement of expenses to the maximum extent available under applicable law.

Section 6.          Board Observers. The Current Shareholders shall have the right to designate a Board observer to attend meetings of the Board under the Senior Secured Credit Facility, dated on or around the date of this agreement, by and among Aegerion Pharmaceuticals, Inc., either of the Company or Amryt Pharmaceuticals DAC, Cantor Fitzgerald Securities, as Administrative Agent, and the Current Shareholders; provided, however, each Current Shareholder shall have the right upon written notice to the Company to terminate its rights pursuant to this Section 6; provided, further, such right shall terminate at such time as the Senior Secured Credit Facility is either refinanced or paid off in full. The Company shall have the right to grant such Board observer rights to one Shareholder holding at least 10% of the Ordinary

17

Shares outstanding; provided, however, that the Company reserves the right to exclude such Board observers from access to any material or meeting or portion thereof if (A) the Company believes upon advice of counsel that such exclusion is reasonably necessary to preserve the attorney-client privilege or (B) the Board believes that such exclusion would be in the best interests of the Company. Each such Board observer shall be entitled to notice of all meetings of the Board and, except with respect to information which is, upon the advice of counsel, the subject of attorney-client privilege, to information provided to any Director. Each such Board observer may be required to execute a confidentiality agreement reasonably acceptable to the Company prior to attending such meetings or receiving any written materials to be discussed at such meetings. The rights afforded to Current Shareholders pursuant to this Section 6 shall not be transferable.

Section 7.          Representations and Warranties. Each party hereto represents and warrants, as of the date hereof, to the other parties hereto as follows:

(a)       Such party is duly organized, validly existing and, if applicable, in good standing under the laws of the jurisdiction of its organization.

(b)       Such party, as appropriate, has the full power, right and authority to enter into this Agreement, to perform, observe and comply with all of such party’s agreements and obligations hereunder, and to consummate the transactions contemplated hereby. If an entity, such party has taken all action required to be taken by it with respect to the execution and delivery of this Agreement, and the consummation of the transactions contemplated hereby.

(c)       This Agreement has been duly and validly executed by such party and, upon delivery thereof by such party, will constitute a legally valid and binding obligation of such party, enforceable against such party in accordance with its terms, except as enforceability thereof may be limited by applicable bankruptcy, reorganization, insolvency or other similar laws affecting creditors’ rights generally or by general principles of equity.

(d)       The execution, delivery and performance by such party of this Agreement does not and will not, and the consummation of the transactions contemplated hereby in compliance with the terms and provisions hereof will not, to the best knowledge of such party, with or without the giving of notice, the passage of time, or both, conflict with, result in a beach of, or constitute a violation or default of or give any third party the right to terminate, accelerate or modify any obligation under (i) any material agreement or other document or instrument to which such party is a party or by which such party is bound or affected, (ii) if an entity, the organizational documents of such party, or (iii) any law, statute, rule, regulation, ordinance, writ, order or judgment to which such party is bound or affected.

The representations and warranties contained in this Agreement shall survive the execution of this Agreement and continue in full force and effect indefinitely.

Section 8.           Certain Events. In the event of any stock split, stock dividend, merger, reorganization, recapitalization or other change in the capital structure of the Company affecting the Shares, (i) the type and number of Shares shall be adjusted appropriately and (ii) this

18

Agreement and the obligations hereunder shall automatically attach to any additional Shares issued to or acquired by a Shareholder.

Section 9.           Notices. All notices, requests, claims, demands and other communications under this Agreement shall be in writing and shall be deemed to have been duly given (a) when delivered in person, (b) three (3) days after posting in the United States mail having been sent registered or certified mail return receipt requested, (c) when delivered by FedEx or other internationally recognized overnight delivery service or (d) when delivered by facsimile or electronic mail communication, in each case, addressed to the other parties at the following addresses (or at such other address for a party as shall be specified by like notice):

if to the Company:

Amryt Pharma Holdings plc 

Dept 920a, 196 High Road, London, N22 8HH 

Attention: Joe Wiley 

Email: [***]

with a copy (which shall not constitute notice) to:

Gibson, Dunn & Crutcher LLP 

200 Park Avenue

New York, NY 10166

Attention:	George P. Stamas, Esq.; William B. Sorabella, Esq.; Robert Klyman, Esq.;
	and Matthew J. Williams, Esq.

Email:	[***];
	[***]

if to Highbridge:

Highbridge Capital Management LLC
40 W 57th St # E

New York, NY 10019
Attention: Damon Meyer and Jonathan Segal
Email: [***]

with a copy (which shall not constitute notice) to:

Latham & Watkins LLP
330 North Wabash Avenue, Suite 2800
Chicago, IL 60611
Attention: Richard A. Levy
Email: [***]

if to Athyrium:

Athyrium Capital Management, LP 

505 Fifth Avenue, Floor 18

19

New York, NY 10017
Attention: Hondo Sen and Samuel Helfaer
Email: [***]

with a copy (which shall not constitute notice) to:

Latham & Watkins LLP
330 North Wabash Avenue, Suite 2800
Chicago, IL 60611
Attention: Richard A. Levy
Email: [***]

Section 10.            Governing Law; Consent to Jurisdiction; Waiver of Trial By Jury.

(a)       This Agreement shall be governed, construed and enforced in accordance with the Laws of the State of New York, without regard to the conflict of law principles that would result in the application of the Law of any other jurisdiction.

(b)       Each party hereto irrevocably and unconditionally submits, for itself and its property, to the exclusive jurisdiction of any state or federal court of competent jurisdiction in New York County, State of New York, and any appellate court from any thereof, in any action or proceeding arising out of or relating to this Agreement or the transactions contemplated hereby or for recognition or enforcement of any judgment relating thereof, and each of the parties hereby irrevocably and unconditionally (i) agrees not to commence any such action or proceeding except in such courts, (ii) agrees that any claim in respect of any such action or proceeding may be heard and determined in such court or, to the extent permitted by law, in such federal court, (iii) waives, to the fullest extent it may legally and effectively do so, any objection which it may now or hereafter have to the laying of venue of any such action or proceeding in any such state or federal court, and (iv) waives, to the fullest extent permitted by law, the defense of an inconvenient forum to the maintenance of such action or proceeding in any such state or federal court. Each party agrees that a final judgment in any such action or proceeding shall be conclusive and may be enforced in other jurisdictions by suit on the judgment or in any other manner provided by law. Each party irrevocably consents to service of process in the manner provided for notices in Section 9. Nothing in this Agreement will affect the right of any party to this Agreement to serve process in any other manner permitted by law.

(c)       EACH PARTY HERETO ACKNOWLEDGES AND AGREES THAT ANY CONTROVERSY WHICH MAY ARISE UNDER THIS AGREEMENT IS LIKELY TO INVOLVE COMPLICATED AND DIFFICULT ISSUES; AND THEREFORE IT HEREBY IRREVOCABLY AND UNCONDITIONALLY WAIVES ANY RIGHT IT MAY HAVE TO A TRIAL BY JURY IN RESPECT OF ANY LITIGATION DIRECTLY OR INDIRECTLY ARISING OUT OF OR RELATING TO THIS AGREEMENT OR THE TRANSACTIONS CONTEMPLATED HEREBY. EACH PARTY CERTIFIES AND ACKNOWLEDGES THAT (I) NO REPRESENTATIVE, AGENT OR ATTORNEY OF ANY OTHER PARTY HAS REPRESENTED, EXPRESSLY OR OTHERWISE, THAT SUCH OTHER PARTY WOULD NOT, IN THE EVENT OF LITIGATION, SEEK TO ENFORCE EITHER OF SUCH WAIVERS, (II) IT UNDERSTANDS AND HAS CONSIDERED THE IMPLICATIONS OF

20

SUCH WAIVERS, (III) IT MAKES SUCH WAIVERS VOLUNTARILY, AND (IV) IT HAS BEEN INDUCED TO ENTER INTO THIS AGREEMENT BY, AMONG OTHER THINGS, THE MUTUAL WAIVERS AND CERTIFICATIONS IN THIS SECTION 10.

Section 11.           Successors and Assigns. The provisions of this Agreement shall be binding upon and accrue to the benefit of the parties hereto and their respective permitted successors and assigns. No party may assign any of its rights or obligations under this Agreement or any part hereof except as expressly set forth herein.

Section 12.           No Other Relationships.

(a)       Nothing contained herein or in any other agreement delivered pursuant hereto or thereto shall be construed to create any agency relationship among the Shareholders. No Shareholder shall owe any fiduciary duties to the Company or to any other Shareholder by virtue of this Agreement. To the extent that at law or in equity, a Shareholder has duties (including fiduciary duties) and liabilities relating thereto to the Company or to any other Shareholder, a Shareholder acting under this Agreement shall not be liable to the Company or to any Shareholder for its good faith reliance on the provisions of this Agreement.

(b)       To the maximum extent permitted under applicable law, the Company renounces any interest or expectancy of the Company in, or in being offered an opportunity to participate in, any and all business opportunities that are presented to any of the Shareholders or Directors (other than in their capacity as a Director and other than those Directors who are employees of the Company). Without limiting the foregoing renunciation, the Company acknowledges that certain of the Shareholders are in the business of making investments in, and have investments in, other businesses similar to and that may be competitors of the Company, and agrees that each such Shareholder shall have the right to make additional investments in or have relationships with such competitor independent of its investment in the Company.

(c)       No Director or Shareholder shall be obligated to present to the Company any particular investment opportunity that such Director or Shareholder gains access to, other than by reason of such Director’s status as a Director (and other than those directors who are employees of the Company), even if such opportunity is of a character that, if presented to the Company or one of its Subsidiaries, could be taken by the Company or such Subsidiary, and such Director or Shareholder shall continue to have the right to take for such Director’s or Shareholder’s own respective account or to recommend to others any such particular investment opportunity.

(d)       The provisions of this Section 12 shall in no way limit or eliminate any such Shareholder’s or their direct or indirect equityholders’ duties, responsibilities and obligations with respect to the protection of any proprietary information of the Company and any of its Subsidiaries, including any applicable duty not to disclose or use such proprietary information improperly or to obtain therefrom an improper personal benefit. No amendment or repeal of this Section 12 shall apply to or have any effect on the liability or alleged liability of any Director of the Company for or with respect to opportunities of which such Director becomes aware prior to such amendment or repeal.

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Section 13.          Severability. If any provision of this Agreement (or any portion thereof) or the application of any such provision (or any portion thereof) to any Person or circumstance shall be held invalid, illegal or unenforceable in any respect by a Governmental Authority, such invalidity, illegality or unenforceability shall not affect any other provision hereof (or the remaining portion thereof) or the application of such provision to any other persons or circumstances, so long as the economic or legal substance of the transactions contemplated hereby is not affected in any manner materially adverse to any party. Upon such determination that any provision of this Agreement (or any portion thereof) or the application of any such provision (or any portion thereof) to any Person or circumstance is invalid, illegal or unenforceable, the parties hereto shall negotiate in good faith to modify this Agreement so as to effect the original intent of the parties hereto as closely as possible in an acceptable manner to the end that the transactions contemplated hereby are fulfilled to the extent possible.

Section 14.          Expenses. Except as otherwise provided herein, each party hereto shall bear its own expenses incurred in connection with this Agreement and the transactions contemplated hereby.

Section 15.          Remedies. Each of the parties to this Agreement will be entitled to enforce its rights under this Agreement specifically, to recover damages by reason of any breach of any provision of this Agreement and to exercise all other rights existing in its favor. The parties hereto agree and acknowledge that money damages may not be an adequate remedy for any breach of the provisions of this Agreement and that any party shall be entitled to immediate injunctive relief or specific performance without bond or the necessity of showing actual monetary damages in order to enforce or prevent any violations of the provisions of this Agreement.

Section 16.          Counterparts; Effectiveness. This Agreement may be executed in one or more counterparts, all of which shall be considered one and the same agreement and shall become effective when one or more counterparts have been signed by each of the parties and delivered to the other parties. The exchange of a fully executed Agreement (in counterparts or otherwise) by facsimile or by electronic delivery in .pdf format shall be sufficient to bind the parties to the terms and conditions of this Agreement.

Section 17.          No Recourse. Notwithstanding anything that may be expressed or implied in this Agreement, and notwithstanding the fact that certain of the parties hereto may be corporations, partnerships, limited liability companies or trusts, each party to this Agreement covenants, agrees and acknowledges that no recourse under this Agreement or any documents or instruments delivered in connection with this Agreement shall be had against any current or future director, officer, employee, general or limited partner, member, manager or trustee of any Shareholder or of any partner, member, manager, trustee, Affiliate or assignee thereof, as such, whether by the enforcement of any assessment or by any legal or equitable proceeding, or by virtue of any statute, regulation or other applicable law, it being expressly agreed and acknowledged that no personal liability whatsoever shall attach to, be imposed on or otherwise be incurred by any current or future officer, agent or employee of any Shareholder or any current or future member of any Shareholder or any current or future director, officer, employee, partner, member, manager or trustee of any Shareholder or of any Affiliate or assignee thereof, as such, for any obligation of any Shareholder under this Agreement or any documents or instruments

22

delivered in connection with this Agreement for any claim based on, in respect of or by reason of such obligations or their creation.

Section 18.          Aggregation. All Shares held by any Affiliates of any Shareholder shall be aggregated together with the Shares held by such Shareholder for the purposes of determining availability of rights and application of obligations of such Shareholder under this Agreement.

Section 19.          Entire Agreement. This Agreement, together with the Restructuring Support Agreement and the other Transaction Documents, constitutes the entire agreement of the parties with respect to the subject matter hereof and supersedes and shall supersede all prior agreements and understandings (whether written or oral) between the Company and the Shareholders, or any of them, with respect to the subject matter hereof. No representations, warranties, covenants, understandings or agreements, oral or otherwise, relating to the transactions contemplated by this Agreement exist between any of the parties hereto except as expressly set forth in this Agreement and other documents contemplated hereby.

Section 20.          Informed Decision; Advice of Counsel. Each party hereto hereby acknowledges and agrees that (a) this Agreement, including all Schedules and Exhibits hereto, have been or will be executed and delivered, as appropriate, following arm’s length negotiations between and among the parties; and (b) such party’s informed decision to execute, deliver and perform this Agreement, (i) was made on the basis of legal, tax, financial and other advice from professionals acting on behalf of such party or on the basis of such party having had the opportunity to engage legal, tax, financial and other advice from professionals, acting on behalf of such party, (ii) was voluntary, and (iii) was not based on any representations, warranties, covenants and/or agreements of any party or other Person not expressly provided for in this Agreement.

Section 21.          Amendment and Waiver. This Agreement may be amended, superseded, canceled, renewed or extended, and the terms hereof may be waived, only by a written instrument signed by the Company and the Shareholders, or, in the case of a waiver, by the party waiving compliance. No delay on the part of any party on exercising any right, power or privileges hereunder shall operate as a waiver thereof, nor shall any waiver on the part of any party of any right, power or privilege, or any single or partial exercise of any such right, power or privilege, preclude any further exercise thereof or the exercise of any other such right, power or privilege.

Section 22.          Rights of Third Parties. Except as otherwise expressly provided herein, this Agreement is intended to be solely for the benefit of the parties hereto and is not intended to confer any benefits upon, or create any rights in favor of, any Person other than the parties hereto.

Section 23.           Waiver of Certain Damages. To the extent permitted by applicable law, each party hereto agrees not to assert, and hereby waives, any claim against any other party hereto, on any theory of liability, for special, indirect, consequential or punitive damages (as opposed to direct or actual damages) arising out of, in connection with, or as a result of, this Agreement or any of the transactions contemplated hereby.

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Section 24.           Termination. This Agreement shall terminate and be of no further force and effect with respect to any Shareholder, on the first date when such Shareholder holds less than [***]% of the outstanding Ordinary Shares of the Company. Notwithstanding anything to the contrary, the obligations of the parties under Section 9, Section 10, Section 14, Section 15, Section 18, Section 24 and Section 25 shall survive termination and shall be enforceable hereunder.

Section 25.           Inconsistent Provisions. In the event that any provision of this Agreement is or becomes inconsistent with the Articles, the Shareholders shall take all actions necessary to amend the Company’s bylaws such that the Company’s bylaws are not inconsistent with and do not conflict with this Agreement.

[Signature pages follow.]

24

IN WITNESS WHEREOF, the parties hereto have duly executed this Registration Rights Agreement as of the date first above written.

THE COMPANY:
AMRYT PHARMA HOLDINGS PLC
By:	/s/ Joe Wiley
	Name: Joe Wiley
	Title: Director

SHAREHOLDERS:
HIGHBRlDGE MSF INTERNATIONAL LTD.
By:	/s/ Jonathan Segal
	Name: Jonathan Segal
	Title: Managing Director

HIGHBRIDGE TACTICAL CREDIT MASTER FUND, L.P.
By:	/s/ Jonathan Segal
	Name: Jonathan Segal
	Title: Managing Director

HIGHBRIDGE SCF SPECIAL SITUATIONS SPV, L.P.
By:	/s/ Jonathan Segal
	Name: Jonathan Segal
	Title: Managing Director

25

ATHYRIUM OPPORTUNITIES II ACQUISITION 2 LP
By: Athyrium Opportunities Associates II LP, its general partner
By: Athyrium GP Holdings LLC, its general partner
By:	/s/ Andrew C. Hyman
	Name: Andrew C. Hyman
	Title: Authorized Signatory

ATHYRIUM OPPORTUNITIES III ACQUISITION 2 LP
By: Athyrium Opportunities Associates III LP, its general partner
By: Athyrium Opportunities Associates III GP LLC, its general partner
By:	/s/ Andrew C. Hyman
	Name: Andrew C. Hyman
	Title: Authorized Signatory

EX-10.5

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibitÛecause it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.5

Execution Version

CREDIT AGREEMENT

Dated as of September 24, 2019

Among

AEGERION PHARMACEUTICALS, INC.,
as Borrower

AMRYT PHARMA PLC,
as Parent

THE LENDERS PARTY HERETO

and

CANTOR FITZGERALD SECURITIES,
as Administrative Agent

TABLE OF CONTENTS
		Page
ARTICLE I
DEFINITIONS AND ACCOUNTING TERMS
Section 1.01	Defined Terms	2
Section 1.02	Other Interpretive Provisions	26
Section 1.03	Accounting Terms	26
Section 1.04	References to Agreements, Laws, Etc.	27
Section 1.05	Times of Day	27
Section 1.06	Timing of Payment or Performance	27
ARTICLE II
THE COMMITMENTS AND THE LOANS
Section 2.01	The Commitments and the Loans	27
Section 2.02	Prepayments	28
Section 2.03	Repayment of Loans	30
Section 2.04	Interest	30
Section 2.05	Fees	31
Section 2.06	Computation of Interest and Fees	31
Section 2.07	Evidence of Indebtedness	31
Section 2.08	Payments Generally	32
Section 2.09	Sharing of Payments	33
ARTICLE III
TAXES, INCREASED COSTS PROTECTION AND ILLEGALITY
Section 3.01	Taxes	34
Section 3.02	Increased Cost and Reduced Return; Capital and Liquidity Requirements	38
Section 3.03	Matters Applicable to All Requests for Compensation	39
Section 3.04	Mitigation Obligations; Replacement of Lenders under Certain Circumstances	39
Section 3.05	Survival	40
ARTICLE IV
CONDITIONS PRECEDENT TO LOANS
Section 4.01	Conditions to Loans	40

i

ARTICLE V
REPRESENTATIONS AND WARRANTIES
Section 5.01	Existence, Qualification and Power; Compliance with Laws	43
Section 5.02	Authorization; No Contravention	43
Section 5.03	Governmental Authorization; Other Consents	43
Section 5.04	Binding Effect	43
Section 5.05	[Reserved]	44
Section 5.06	Litigation	44
Section 5.07	Ownership of Property; Liens	44
Section 5.08	Enforceable Obligations	44
Section 5.09	Environmental Compliance	44
Section 5.10	Taxes	46
Section 5.11	Compliance with ERISA	46
Section 5.12	Labor Matters	46
Section 5.13	Insurance	47
Section 5.14	Subsidiaries; Equity Interests	47
Section 5.15	Margin Regulations; Investment Company Act; Anti-Terrorism Laws; Sanctions and Other Regulations	47
Section 5.16	Disclosure	48
Section 5.17	Intellectual Property	48
Section 5.18	Plan Funding Agreement	49
Section 5.19	EEA Financial Institution	49
Section 5.20	Contractual Obligations	49
ARTICLE VI
AFFIRMATIVE COVENANTS
Section 6.01	Financial Statements	49
Section 6.02	Certificates; Reports; Other Information	50
Section 6.03	Notice Requirements; Other Information	51
Section 6.04	Environmental Matters	52
Section 6.05	Maintenance of Existence	54
Section 6.06	Maintenance of Properties	54
Section 6.07	Maintenance of Insurance	54
Section 6.08	Compliance with Laws	55
Section 6.09	Books and Records	55
Section 6.10	Inspection Rights; Lender Calls	55
Section 6.11	Additional Guarantors	56
Section 6.12	Use of Proceeds	56
Section 6.13	Anti-Corruption and Sanctions Laws	56
Section 6.14	Taxes	56
Section 6.15	End of Fiscal Years; Fiscal Quarters	56
Section 6.16	ERISA	57

ii

Section 6.17	Further Assurances	57
Section 6.18	Business	58
Section 6.19	Landlord Agreements	58
Section 6.20	Post-Closing Matters	58
ARTICLE VII
NEGATIVE COVENANTS
Section 7.01	Liens	58
Section 7.02	Investments	61
Section 7.03	Indebtedness	62
Section 7.04	Fundamental Changes	63
Section 7.05	Dispositions	63
Section 7.06	Restricted Payments	65
Section 7.07	Change in Nature of Business	65
Section 7.08	Transactions with Affiliates	65
Section 7.09	Prepayments and Modifications of Certain Agreements	66
Section 7.10	Negative Pledge	66
Section 7.11	Amendments to Organization Documents	67
Section 7.12	Use of Proceeds	67
Section 7.13	Accounting Changes	67
Section 7.14	OFAC	67
Section 7.15	Ownership of Subsidiaries	67
Section 7.16	Compliance With Certain Laws	67
Section 7.17	Minimum Liquidity	68
Section 7.18	Immaterial Subsidiaries	68
ARTICLE VIII
EVENTS OF DEFAULT AND REMEDIES
Section 8.01	Events of Default	68
Section 8.02	Remedies Upon Event of Default	71
Section 8.03	Application of Funds	72
ARTICLE IX
ADMINISTRATIVE AGENT AND OTHER AGENTS
Section 9.01	Appointment and Authorization	73
Section 9.02	Delegation of Duties	74
Section 9.03	Liability of the Administrative Agent	75
Section 9.04	Reliance by the Administrative Agent	75
Section 9.05	Notice of Default	76

iii

Section 9.06	Credit Decision; Disclosure of Information by the Administrative Agent	76
Section 9.07	Indemnification of the Administrative Agent	77
Section 9.08	The Administrative Agent in its Individual Capacity	77
Section 9.09	Successor Agents	78
Section 9.10	Administrative Agent May File Proofs of Claim	78
Section 9.11	Release of Collateral and Guarantee	79
Section 9.12	Other Agents; Arrangers and Managers	80
Section 9.13	Appointment of Supplemental Administrative Agent	80
ARTICLE X
MISCELLANEOUS
Section 10.01	Amendments, Etc.	81
Section 10.02	Notices and Other Communications; Facsimile and Electronic Copies	82
Section 10.03	No Waiver; Cumulative Remedies	86
Section 10.04	Costs and Expenses	87
Section 10.05	Indemnification by the Borrower	87
Section 10.06	Payments Set Aside	89
Section 10.07	Successors and Assigns	89
Section 10.08	Confidentiality	93
Section 10.09	Setoff	94
Section 10.10	Counterparts	94
Section 10.11	Integration	95
Section 10.12	Survival of Representations and Warranties	95
Section 10.13	Severability	95
Section 10.14	GOVERNING LAW	95
Section 10.15	WAIVER OF RIGHT TO TRIAL BY JURY	96
Section 10.16	Binding Effect	96
Section 10.17	Lender Action	96
Section 10.18	USA PATRIOT Act	96
Section 10.19	No Advisory or Fiduciary Responsibility	96
Section 10.20	Acknowledgement and Consent to Bail-In of EEA Financial Institutions	97

iv

SCHEDULES
Schedule 1	-	Closing Checklist
Schedule 2	-	Subsidiary Guarantors
Schedule 2.01	-	Commitments and Rollover Loans
Schedule 3	-	Immaterial Subsidiaries and Asset Levels
Schedule 5.01	-	Existence, Qualification and Power; Compliance with Laws
Schedule 5.02	-	Authorizations; No Contravention
Schedule 5.06	-	Litigation
Schedule 5.07(b)	-	Real Property
Schedule 5.09	-	Environmental Compliance
Schedule 5.10	-	Taxes
Schedule 5.14	-	Subsidiaries and Other Equity Investments
Schedule 5.17	-	Intellectual Property, Licenses
Schedule 5.20	-	Material Contracts
Schedule 6.20	-	Post-Closing Matters
Schedule 7.01(b)	-	Existing Liens
Schedule 7.02(c)	-	Existing Investments
Schedule 7.03(b)	-	Surviving Indebtedness
Schedule 7.08(c)	-	Existing Affiliate Transactions
Schedule 10.02	-	Administrative Agent’s Office, Certain Addresses for Notices

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EXHIBITS
Exhibit A-1	--	Form of Committed Loan Notice
Exhibit A-2	--	Form of Prepayment Notice
Exhibit A-3	--	Form of PIK Election Request
Exhibit B	--	Form of Note
Exhibit C	--	Form of Compliance Certificate
Exhibit D	--	Form of Assignment and Assumption
Exhibit E-l	--	Form of Guarantee and Collateral Agreement
Exhibit E-2	--	Form of Pledge Agreement
Exhibit F	--	Form of Officer’s Certificate
Exhibit G	--	Form of Administrative Questionnaire
Exhibits H-l to H-4	--	Forms of U.S. Tax Compliance Certificate

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CREDIT AGREEMENT

This CREDIT AGREEMENT (this “Agreement”) is entered into as of September 24, 2019 among AEGERION PHARMACEUTICALS, INC., a Delaware corporation (the “Borrower”), AMRYT PHARMA HOLDINGS PLC, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around the date hereof as AMRYT PHARMA PLC) (the “Parent”), each Lender (as hereinafter defined) from time to time party hereto and CANTOR FITZGERALD SECURITIES, as administrative agent and collateral agent for the Lenders (in such capacities, together with any successor administrative agent and collateral agent, the “Administrative Agent”).

PRELIMINARY STATEMENTS

1.           On May 20, 2019 (the “Petition Date”), the Borrower and certain of its Subsidiaries (collectively, the “Debtors”) filed in the United States Bankruptcy Court for the Southern District of New York (the “Bankruptcy Court”) a voluntary petition for relief under Chapter 11 of the Bankruptcy Code, and such reorganization, case was jointly administered under the Case Number 19-11632 (the “Chapter 11 Case”).

2.           The Debtors will emerge from bankruptcy on the date hereof upon the effectiveness of the Debtors’ Joint Plan of Reorganization Under Chapter 11 of the Bankruptcy Code (the “Reorganization Plan”), which Reorganization Plan was confirmed by the Bankruptcy Court on September 10, 2019.

3.           Pursuant to the terms of the Reorganization Plan and subject to the terms and conditions set forth herein, the Lenders have agreed to make available to the Borrower a senior secured term loan facility in an aggregate amount not to exceed $81,020,618.73 consisting of (a) $[***] in principal amount of new money term loans advanced on the Closing Date, the proceeds of which the Borrower may use for the purposes permitted hereunder, and (b) $[***] in rolled up “New Money Loans” under, and as defined in, the Existing Bridge Credit Agreement (as hereinafter defined), including accrued fees and interest thereon, owed to the Lenders on the Closing Date, which shall be deemed to constitute Rollover Loans hereunder pursuant to the terms hereof.

4.            The Guarantors (as hereinafter defined) have agreed to guarantee the obligations of the Borrower hereunder and the Borrower and the Guarantors have agreed to secure their respective Obligations by granting to the Administrative Agent, for the benefit of the Secured Parties (as hereinafter defined), a lien on substantially all of their respective assets, in accordance with the priorities provided in the Loan Documents (as hereinafter defined), except as otherwise may be set forth in such Loan Documents.

Subject to and upon the terms and conditions set forth herein, the Lenders are willing to make available to the Borrower the senior secured term loan facility provided for herein:

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ARTICLE I

DEFINITIONS AND ACCOUNTING TERMS

Section 1.01 Defined Terms. As used in this Agreement, the following terms shall have the meanings set forth below:

“Accounting Changes” means changes in accounting principles required by the promulgation of any rule, regulation, pronouncement or opinion by the Financial Accounting Standards Board of the American Institute of Certified Public Accountants (or successor thereto or any agency with similar functions).

“Acquisition” has the meaning specified in Section 4.01(e).

“Administrative Agent” has the meaning specified in the first paragraph of this Agreement and shall include any successor administrative agent appointed in accordance with Section 9.09.

“Administrative Agent’s Office” means, the Administrative Agent’s address and, as appropriate, account as set forth on Schedule 10.02. or such other address or account as the Administrative Agent may from time to time notify the Borrower and the Lenders.

“Administrative Questionnaire” means an Administrative Questionnaire substantially in the form of Exhibit G.

“Affiliate” means, in respect of any Person:

(a)          any Person which, directly or indirectly, controls, is controlled by or is under common control with such Person; and for the purposes of this definition, “control” (including, with correlative meanings, the terms “controlled by” or “under common control with”) means the power to direct or cause the direction of the management and policies of any Person, whether through the ownership of voting Equity Interests or by contract or otherwise;

(b)          any Person who beneficially owns or holds 10% or more of any class of shares (or, in the case of a Person that is not a corporation, 10% or more of the partnership or other Equity Interests) of such Person; or

(c)          any Person, 10% or more of any class of shares (or in the case of a Person that is not a corporation, 10% or more of the partnership or other Equity Interests) of which is beneficially owned or held by such Person or a Subsidiary of such Person.

Notwithstanding the foregoing, the Permitted Holders shall not be deemed to be Affiliates of the Loan Parties for purposes of this Agreement and the other Loan Documents.

“Agent Parties” has the meaning specified in Section 10.02(f).

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“Agent-Related Persons” means the Administrative Agent, together with its Affiliates, and the officers, directors, employees, agents and attorneys-in-fact of such Persons and Affiliates.

“Aggregate Commitments” means the Commitments of all the Lenders. As of the Closing Date, the amount of the Aggregate Commitments is $[***].

“Agreement” has the meaning specified in the introductory paragraph hereto.

“Amryt Finance Contract” means that certain Finance Contract dated as of December 1, 2016 between Amryt Pharmaceuticals DAC and European Investment Bank, as the same may be amended, restated, supplemented or otherwise modified from time to time.

“Anti-Corruption Laws” has the meaning specified in Section 5.15(f).

“Anti-Terrorism Law” means any Requirement of Law related to money laundering or financing terrorism, including the USA PATRIOT Act, and its implementing regulations, The Currency and Foreign Transactions Reporting Act (also known as the Bank Secrecy Act, 31 U.S.C. §§ 5311-5330 and 12 U.S.C. §§ 1818(s), 1820(b) and 1951-1959), the Trading With the Enemy Act (50 U.S.C. § 1 et seq., as amended), Executive Order 13224 (effective September 24, 2001) and the Money Laundering Control Act of 1986 (18 U.S.C. §§1956 and 1957).

“Applicable Lending Office” means for any Lender, such Lender’s office, branch or affiliate designated for the Loans, as notified to the Administrative Agent and the Borrower or as otherwise specified in the Assignment and Assumption pursuant to which such Lender became a party hereto, any of which offices may, subject to the applicable provisions of Article III, be changed by such Lender upon 10 days’ prior written notice to the Administrative Agent and the Borrower; provided that for the purposes of the definition of “Excluded Taxes” and Section 3.01, any such change shall be deemed an assignment made pursuant to an Assignment and Assumption.

“Applicable Rate” means a percentage per annum equal to, at the Borrower’s election in accordance with Section 2.04, either (x) 11.0% paid in cash or (y) (i) 6.5% paid in cash plus (ii) 6.5% paid in kind.

“Approved Fund” means any Fund that is administered, advised or managed by (a) a Lender, (b) an Affiliate of a Lender, or (c) an entity or Affiliate of an entity that administers, advises or manages a Lender.

“Assignment and Assumption” means an Assignment and Assumption substantially in the form of Exhibit D.

“Attorney Costs” means and includes all reasonable and documented fees, out-of-pocket expenses and actual disbursements of any law firm or other external legal counsel.

“Attributable Indebtedness” means, at any date, (a) in respect of any Capital Lease Obligation (other than a lease resulting from a Sale Leaseback) of any Person, the

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capitalized amount thereof that would appear on a balance sheet of such Person prepared as of such date in accordance with IFRS, and (b) in respect of any Sale Leaseback, the present value, discounted in accordance with IFRS, at the interest rate implicit in the related lease, of the obligations of the lessee for net rental payments over the remaining term of such lease (including any period for which such lease has been extended or may, at the option of the lessor be extended).

“Backstop Subscription Agreement” means that certain Backstop Subscription Agreement dated as of July 10, 2019 by and among the Intermediate Parent, Highbridge MSF International Ltd., Highbridge SCF Special Situations SPV, L.P., Highbridge Tactical Credit Master Fund, L.P., Athyrium Opportunities II Acquisition 2 LP, Athyrium Opportunities III Acquisition 2 LP, Whitebox Relative Value Partners, LP, Whitebox GT Fund, LP, Whitebox Multi-strategy Partners, LP, Pandora Select Partners, LP, Nineteen77 Global Multi-Strategy Alpha Master Limited, and Nineteen77 Global Convertible Bond Master Limited funds party thereto, as the same may be amended, restated, supplemented or otherwise modified from time to time.

“Bail-In Action” means the exercise of any Write-Down and Conversion Powers by the applicable EEA Resolution Authority in respect of any liability of an EEA Financial Institution.

“Bail-In Legislation” means, with respect to any EEA Member Country implementing Article 55 of Directive 2014/59/EU of the European Parliament and of the Council of the European Union, the implementing law for such EEA Member Country from time to time which is described in the EU Bail-In Legislation Schedule.

“Bankruptcy Code” means Title 11 of the United States Code entitled “Bankruptcy,” as now or hereafter in effect, or any successor thereto.

“Bankruptcy Court” has the meaning specified in the Preliminary Statements hereto.

“Borrower” has the meaning specified in the introductory paragraph hereto.

“Budget” has the meaning specified in Section 6.01(c).

“Business Day” means any day other than a Saturday, Sunday or other day on which commercial banks are authorized or required to close under the Laws of, or are in fact closed in, the State of New York or the United Kingdom, generally, or London, England specifically.

“Capital Lease” means, with respect to any Person, any leasing or similar arrangement conveying the right to use any property, whether real or personal property, or a combination thereof, by that Person as lessee that, in conformity with IFRS, is required to be accounted for as a capital lease on the balance sheet of such Person.

“Capital Lease Obligation” means, with respect to any Person, all monetary or financial obligations of such Person and its Subsidiaries under any Capital Leases, and the

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amount of such obligations shall be the capitalized amount thereof determined in accordance with IFRS, and the stated maturity thereof shall be the date of the last payment of rent or any other amount due under such lease prior to the first date on which such lease may be terminated by the lessee without payment of a penalty; provided that any obligations that were not required to be included on the balance sheet of such Person as capital lease obligations when incurred but are subsequently re-characterized as capital lease obligations due to a change in accounting rules after the Closing Date shall for all purposes hereunder not be treated as a Capital Lease Obligation.

“Cash Equivalents” means any of the following: (a) readily marketable direct obligations of the Government of the United States or any agency or instrumentality thereof or obligations unconditionally guaranteed by the full faith and credit of the Government of the United States, (b) insured certificates of deposit of or time deposits with any commercial bank that is a Lender or any other domestic commercial bank having capital and surplus in excess of $500,000,000 maturing not more than one year after the date of issuance, (c) repurchase obligations of any Lender or of any commercial bank satisfying the requirements of clause (b) of this definition, having a term of not more than 30 days, with respect to securities issued or fully guaranteed or insured by the Government of the United States, (d) securities with maturities of 365 days or less from the date of acquisition that are issued or fully guaranteed by any state, district or territory of the United States, by any political subdivision or taxing authority of any such state, district or territory or by any foreign government, the securities of which state, district or territory, taxing authority or foreign government (as the case may be) are rated at least A by S&P or A by Moody’s, (e) commercial paper maturing not more than two hundred and seventy (270) days from the date of issue and issued by a corporation (other than an Affiliate of any Loan Party) organized under the laws of any state of the United States of America or of the District of Columbia and, at the time of acquisition thereof, rated A 2 or higher by S&P, P 2 or higher by Moody’s or F2 or higher by Fitch, (f) money market mutual or similar funds that invest substantially all of their assets in one or more type of securities satisfying the requirements of clauses (a) through (e) of this definition, (g) Investments, classified in accordance with IFRS as current assets of the Borrower or any of its Subsidiaries, in money market investment programs registered under the Investment Company Act of 1940, as amended, which are administered by financial institutions having capital of at least $500,000,000, and the portfolios of which are limited solely to Investments of the character, quality and maturity described in clauses (a) and (b) of this definition, (h) agencies (LSE’s), State (municipal bonds), or corporate bonds having a long term rating of at least A- or A3 from S&P, Moody’s or Fitch, having maturities of not more than fifteen (15) months from the date of acquisition and (i) money market funds having a rating of AAAm/Aaa or better from S&P, Moody’s or Fitch.

“Casualty Event” means any casualty, loss, damage, destruction or other similar loss with respect to real or personal property or improvements.

“CERCLA” means the Comprehensive Environmental Response, Compensation and Liability Act of 1980, as amended from time to time.

“CERCLIS” means the Comprehensive Environmental Response, Compensation and Liability Information System maintained by the U.S. Environmental Protection Agency.

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“Change in Law” means (a) the adoption of any law, treaty, order, policy, rule or regulation after the date of this Agreement, (b) any change in any law, treaty, order, policy, rule or regulation or in the interpretation or application thereof by any Governmental Authority after the date of this Agreement or (c) the making or issuance of any guideline, request or directive issued or made after the date hereof by any central bank or other Governmental Authority (whether or not having the force of law); provided that notwithstanding anything herein to the contrary, (x) the Dodd-Frank Wall Street Reform and Consumer Protection Act and all requests, rules, guidelines, requirements or directives thereunder or issued in connection therewith or in implementation thereof and (y) all requests, rules, guidelines, requirements or directives promulgated by the Bank for International Settlements, the Basel Committee on Banking Supervision (or any successor or similar authority) or the United States regulatory authorities, in each case pursuant to Basel III, shall in each case be deemed to be a “Change in Law”, regardless of the date enacted, adopted, issued or implemented; provided that increased costs as a result of a Change in Law pursuant to clauses (x) and (y) above shall only be reimbursable by the Borrower to a Lender to the extent such Lender is requiring reimbursement therefor generally from similarly situated borrowers under comparable credit facilities.

“Change of Control” means the occurrence of any of the following events:

(a)          any Wholly-owned direct or indirect Subsidiary of the Parent on the Closing Date shall cease to be a Wholly-owned direct or indirect Subsidiary of the Parent except as provided in Section 7.04;

(b)         the Borrower shall cease to be either (i) a Wholly-owned direct Subsidiary of the Intermediate Parent, which shall in turn be a Wholly-owned direct Subsidiary of the Parent or (ii) a Wholly-owned direct Subsidiary of the Parent;

(c)          any Person or “group” (within the meaning of Rules 13d-3 and 13d-5 under the Exchange Act) other than the Permitted Holders shall have (x) acquired beneficial ownership or control of 25% or more on a fully diluted basis of the voting and/or economic interest in the Equity Interests of the Parent; or (y) obtained the power (whether or not exercised) to elect a majority of the members of the board of directors (or similar governing body) of the Parent; or

(d)          those individuals who are members of the board of directors (or similar governing body) of the Parent on the Closing Date (together with any new or replacement directors whose initial nomination for election was approved by a majority of the directors who were either directors on the Closing Date or previously so approved) shall fail to constitute a majority of the board of directors (or similar governing body) of the Parent.

“Chapter 11 Case” has the meaning specified in the Preliminary Statements hereto.

“Closing Date” means the date on which all the conditions precedent in Section 4.01 are satisfied or waived in accordance with Section 10.01 and the Loans are made.

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“Code” means the U.S. Internal Revenue Code of 1986, as amended from time to time.

“Collateral” means a collective reference to all real and personal property required to be pledged to the Administrative Agent, for the benefit of the Secured Parties, to secure all or part of the Obligations pursuant to the Collateral Documents.

“Collateral Documents” means, collectively, the Guarantee and Collateral Agreement, the Pledge Agreement, the UK Security Documents, the Irish Security Documents, and, to the extent required hereunder or reasonably requested by the Administrative Agent and the Lenders, any Guarantee and Collateral Agreement Supplement, any Mortgages, any collateral assignments, any security agreements, pledge agreements, control agreements or other similar agreements, or any supplements to any of the foregoing, in each case delivered to the Administrative Agent and the Lenders in connection with this Agreement or any other Loan Document or any transaction contemplated hereby or thereby to secure or guarantee the payment of any part of the Obligations or the performance of any Loan Party’s other duties and obligations under the Loan Documents.

“Commitment” means, as to each Lender, its obligations to make New Money Loans pursuant to Section 2.01(a) in an aggregate principal amount not to exceed the amount set forth opposite such Lender’s name on Schedule 2.01 hereto under the caption “Commitment”. Commitments will terminate once advanced.

“Committed Loan Notice” means a notice of borrowing substantially in the form of Exhibit A-1.

“Communications” has the meaning specified in Section 10.02(e).

“Compliance Certificate” means a certificate substantially in the form of Exhibit C.

“Connection Income Taxes” means Other Connection Taxes that are imposed on or measured by net income (however denominated) or that are franchise Taxes or branch profits Taxes.

“Contractual Obligation” means, as to any Person, any provision of any security issued by such Person or of any agreement, instrument or other undertaking to which such Person is a party or by which it or any of its property is bound.

“Convertible Notes” means Indebtedness evidenced by the 5.00% convertible senior notes due 2025 issued under that certain Indenture dated as of September 24, 2019 between the Borrower and GLAS Trust Company LLC, as trustee thereunder, as the same may be amended, restated, supplemented or otherwise modified from time to time.

“Debtor Relief Laws” means the Bankruptcy Code, and all other liquidation, conservatorship, bankruptcy, Irish law examinership, assignment for the benefit of creditors, moratorium, rearrangement, receivership, insolvency, fraudulent transfer, reorganization, or

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similar debtor relief Laws of the United States or any similar foreign, federal or state law for the relief of debtors from time to time in effect and affecting the rights of creditors generally.

“Debtors” has the meaning specified in the Preliminary Statements hereto.

“Default” means any event or condition that constitutes an Event of Default or that, with the giving of any notice, the passage of time, or both, would be an Event of Default.

“Default Rate” means an interest rate equal to the Applicable Rate under clause (x) of such definition plus 2.0% per annum.

“Disposition” or “Dispose” means the sale, transfer, license, lease or other disposition of any asset or property by a Loan Party or any of its Subsidiaries (including any Sale Leaseback and any sale of Equity Interests, but excluding any issuance by a Loan Party of its own Equity Interests); provided that none of the foregoing shall be considered a “Disposition” for purpose of Section 7.05 if and only if the aggregate value of the assets or property that are the subject of such transaction is less than $100,000 in the aggregate during the term of this Agreement.

“Disqualified Equity Interests” means, with respect to any Person, any Equity Interest of such Person which, by its terms, or by the terms of any security or other Equity Interests into which it is convertible or for which it is exchangeable, or upon the happening of any event or condition, (a) matures or is mandatorily redeemable (other than solely for Qualified Equity Interests), pursuant to a sinking fund obligation or otherwise (except as a result of a change of control or asset sale so long as any rights of the holders thereof upon the occurrence of a change of control or asset sale event shall be subject to the prior repayment in full of the Loans and all other Obligations that are accrued and payable and the termination of the Commitments), (b) is redeemable at the option of the holder thereof (other than solely for Qualified Equity Interests), in whole or in part, (c) provides for the scheduled payments of dividends in cash, or (d) is or becomes convertible into or exchangeable for Indebtedness or any other Equity Interests that would constitute Disqualified Equity Interests, in each case, prior to the date that is ninety one (91) days after the Maturity Date then in effect; provided that, if such Equity Interests are issued pursuant to a plan for the benefit of employees of the Borrower or any of its Subsidiaries or by any such plan to such employees, such Equity Interests shall not constitute Disqualified Equity Interests solely because they may be required to be repurchased by the Borrower or any of its Subsidiaries in order to satisfy applicable statutory or regulatory obligations.

“Disqualified Person” means any holder of any Indebtedness under the Convertible Notes or any direct competitor of the Parent or its Subsidiaries to the extent that all such Disqualified Persons have been listed on a schedule provided to the Lenders and the Administrative Agent prior to the Closing Date.

“Dollars” means lawful money of the United States.

“EEA Financial Institution” means (a) any credit institution or investment firm established in any EEA Member Country which is subject to the supervision of an EEA Resolution Authority, (b) any entity established in an EEA Member Country which is a parent of

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an institution described in clause (a) of this definition, or (c) any financial institution established in an EEA Member Country which is a subsidiary of an institution described in clauses (a) or (b) of this definition and is subject to consolidated supervision with its parent.

“EEA Member Country” means any of the member states of the European Union, Iceland, Liechtenstein, and Norway.

“EEA Resolution Authority” means any public administrative authority or any person entrusted with public administrative authority of any EEA Member Country (including any delegee) having responsibility for the resolution of any EEA Financial Institution.

“Eligible Assignee” means any Person that meets the requirements to be an assignee under Section 10.07(b)(iii), (v) and (vi) (subject to such consents, if any, as may be required under Section 10.07(b)(iii)).

“Environmental Action” means any action, suit, demand, demand letter, claim, notice of non-compliance or violation, notice of liability or potential liability, investigation, proceeding, consent order or consent agreement relating to any Environmental Law, any Environmental Permit or Hazardous Material or arising from alleged injury or threat to health and safety as it relates to any Hazardous Material or the environment, including, without limitation, (a) by any Governmental Authority for enforcement, cleanup, removal, response, remedial or other actions or damages relating to Releases of Hazardous Materials or actual or alleged violations of Environmental Laws and (b) by any Governmental Authority or third party for damages, contribution, indemnification, cost recovery, compensation or injunctive relief.

“Environmental Laws” means any and all federal, provincial, local and foreign statutes, laws, regulations, ordinances, rules, decrees or other governmental restrictions of legal effect relating to the environment, to the release of any Hazardous Materials into the environment or to the manufacture, processing, distribution, use, treatment, storage, disposal, transport or handling of Hazardous Materials but only to the extent such Environmental Laws are legally applicable to any Loan Party pursuant to any Environmental Law.

“Environmental Liability” in respect of any Person, any and all legal obligations and liabilities under Environmental Laws for any Release caused by such Person or which is discovered or uncovered during the ownership or control of any real property by such Person and which adversely impacts any Person, property or the environment whether or not caused by a breach of applicable laws (including Environmental Laws).

“Environmental Permit” means any permit, approval, hazardous waste identification number, license or other authorization issued by or submitted to a Governmental Authority required under any Environmental Law.

“Equity Interests” means, with respect to any Person, all of the shares of capital stock of (or other ownership or profit interests in) such Person, all of the warrants, options or other rights for the purchase or acquisition from such Person of shares of capital stock of (or other ownership or profit interests in) such Person, all of the securities convertible into or exchangeable for shares of capital stock of (or other ownership or profit interests in) such Person or warrants, rights or options for the purchase or acquisition from such Person of such shares (or

9

such other interests), and all of the other ownership or profit interests in such Person (including partnership, member or trust interests therein), whether voting or nonvoting.

“ERISA” means the Employee Retirement Income Security Act of 1974, as amended from time to time and Treasury regulations promulgated and rulings issued thereunder.

“ERISA Affiliate” means any trade or business (whether or not incorporated) that is under common control with any Loan Party and is treated as a single employer within the meaning of Section 414 of the Code or Section 4001 of ERISA.

“ERISA Event” means (a) a Reportable Event with respect to a Pension Plan; (b) a withdrawal by any Loan Party or any ERISA Affiliate from a Pension Plan subject to Section 4063 of ERISA during a plan year in which it was a substantial employer (as defined in Section 4001(a)(2) of ERISA) or a cessation of operations at any facility of any Loan Party or ERISA Affiliate as described in Section 4062(e) of ERISA; (c) a complete or partial withdrawal by any Loan Party or any ERISA Affiliate from a Multiemployer Plan, notification of any Loan Party or ERISA Affiliate concerning the imposition of withdrawal liability or notification that a Multiemployer Plan is insolvent or is in reorganization within the meaning of Title IV of ERISA (or that is in endangered or critical status, within the meaning of Section 305 of ERISA); (d) the filing of a notice of intent to terminate, the treatment of a Pension Plan amendment as a termination under Sections 4041 or 4041A of ERISA, or the commencement of proceedings by the PBGC to terminate a Pension Plan or Multiemployer Plan; (e) an event or condition which constitutes grounds under Section 4042 of ERISA for the termination of, or the appointment of a trustee to administer, any Pension Plan or Multiemployer Plan; (f) the imposition of any liability under Title IV of ERISA, other than for PBGC premiums due but not delinquent under Section 4007 of ERISA, upon any Loan Party or any ERISA Affiliate; (g) a determination that any Pension Plan is, or is expected to be, in “at-risk” status (within the meaning of Section 303(i)(4) of ERISA or Section 430(i)(4) of the Code); or (h) the conditions for imposition of a lien under Section 303(k) of ERISA shall have been met with respect to any Pension Plan.

“EU Bail-In Legislation Schedule” means the EU Bail-In Legislation Schedule published by the Loan Market Association (or any successor person), as in effect from time to time.

“Event of Default” has the meaning specified in Section 8.01.

“Exchange Act” means the Securities Exchange Act of 1934, as amended from time to time.

“Excluded Taxes” means any of the following Taxes imposed on or with respect to a Recipient or required to be withheld or deducted from a payment to a Recipient, (a) Taxes imposed on or measured by net income (however denominated), franchise Taxes, and branch profits Taxes, in each case, (i) imposed as a result of such Recipient being organized under the laws of, or having its principal office or, in the case of any Lender, its Applicable Lending Office located in, the jurisdiction imposing such Tax (or any political subdivision thereof) or (ii) that are Other Connection Taxes, (b) in the case of a Lender, U.S. federal withholding Taxes imposed on

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amounts payable to or for the account of such Lender with respect to an applicable interest in a Loan or Commitment pursuant to a law in effect on the date on which (i) such Lender acquires such interest in the Loan or Commitment (other than pursuant to an assignment request by the Borrower under Section 3.04(b)) or (ii) such Lender changes its lending office, except in each case to the extent that, pursuant to Section 3.0.1, amounts with respect to such Taxes were payable either to such Lender’s assignor immediately before such Lender became a party hereto or to such Lender immediately before it changed its lending office, (c) Taxes attributable to such Recipient’s failure to comply with Section 3.01(g) and (d) any Taxes imposed under FATCA.

“Existing Bridge Credit Agreement” means that certain Bridge Credit Agreement dated as of November 8, 2018 among the Borrower, the lenders party thereto and Cantor Fitzgerald Securities, as administrative agent, as the same may be amended, restated, supplemented or otherwise modified from time to time.

“Existing Convertible Notes” means Indebtedness evidenced by the 2.00% convertible senior notes due 2019 issued under that certain Indenture dated as of August 15, 2014 between the Borrower and The Bank of New York Mellon Trust Company, N.A., as trustee thereunder, as the same may be amended, restated, supplemented or otherwise modified from time to time.

“Existing DIP Credit Agreement” means that certain Debtor-in-Possession Credit Agreement dated as of June 28, 2019 among the Borrower, the lenders party thereto and Cantor Fitzgerald Securities, as administrative agent, as the same may be amended, restated, supplemented or otherwise modified from time to time.

“FATCA” means Sections 1471 through 1474 of the Code, as of the date of this Agreement (or any amended or successor version that is substantively comparable and not materially more onerous to comply with), any current or future regulations or official interpretations thereof, any agreement entered into pursuant to Section 1471(b)(1) of the Code and any fiscal or regulatory legislation, rules or practices adopted pursuant to any intergovernmental agreement, treaty or convention among Governmental Authorities implementing such Sections of the Code.

“Federal Funds Rate” means, for any day, the rate per annum equal to the weighted average of the rates on overnight federal funds transactions with members of the Federal Reserve System arranged by federal funds brokers on such day, as published by the Federal Reserve Bank on the Business Day next succeeding such day; provided that (a) if such day is not a Business Day, the Federal Funds Rate for such day shall be such rate on such transactions on the next preceding Business Day as so published on the next succeeding Business Day and (b) if no such rate is so published on such next succeeding Business Day, the Federal Funds Rate for such day shall be the average rate of the quotations for such day on such transactions received by the Administrative Agent from three Federal funds brokers of recognized standing selected by it.

“Financial Advisor” means Ducera Partners LLC, in its capacity as financial advisor to the Lenders and their counsel solely with respect to the Loan Documents.

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“Fiscal Year” means the fiscal year of the Parent and its Subsidiaries, ending on December 31 of each calendar year.

“Fitch” means Fitch Ratings, Inc. and its successors.

“Foreign Lender” means (a) if the borrower is a U.S. Person, a Lender that is not a U.S. Person, and (b) if the Borrower is not a U.S. Person, a Lender that is a resident or organized under the laws of a jurisdiction other than that in which the Borrower is a resident for tax purposes.

“Foreign Subsidiary” means any direct or indirect Subsidiary of the Parent organized outside the United States.

“FRB” means the Board of Governors of the Federal Reserve System of the United States.

“Fund” means any Person (other than an individual) that is or will be engaged in making, purchasing, holding or otherwise investing in commercial loans and similar extensions of credit in the ordinary course.

“Governmental Authority” means any nation or government, any provincial, state, local, municipal or other political subdivision thereof, and any entity exercising executive, legislative, judicial, taxing, regulatory or administrative powers or functions of or pertaining to government.

“Governmental Authorization” means any authorization, approval, consent, franchise, license, covenant, order, ruling, permit, certification, exemption, notice, declaration or similar right, undertaking or other action of, to or by, or any filing, qualification or registration with, any Governmental Authority.

“Granting Lender” has the meaning specified in Section 10.07(f).

“Guarantee and Collateral Agreement” means, collectively, (a) the Guarantee and Collateral Agreement executed by the Loan Parties and the Administrative Agent substantially in the form of Exhibit E-l (as such agreement may be amended, restated, supplemented or otherwise modified from time to time) and (b) each Guarantee and Collateral Agreement Supplement executed and delivered pursuant to the provisions of Section 6.11.

“Guarantee and Collateral Agreement Supplement” means a supplement to the Guarantee and Collateral Agreement, in form reasonably satisfactory to the Required Lenders, executed and delivered to the Administrative Agent pursuant to the provisions of Section 6.11.

“Guarantee Obligations” means, with respect to any Person, any obligation or arrangement of such Person to guarantee or intended to guarantee any Indebtedness or other payment obligations (“primary obligations”) of any other Person (the “primary obligor”) in any manner, whether directly or indirectly, including, without limitation, (a) the direct or indirect guarantee, endorsement (other than for collection or deposit in the ordinary course of business), co-making, discounting with recourse or sale with recourse by such Person of the Obligation of a

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primary obligor, (b) the obligation to make take-or-pay or similar payments, if required, regardless of non-performance by any other party or parties to an agreement or (c) any obligation of such Person, whether or not contingent, (i) to purchase any such primary obligation or any property constituting direct or indirect security therefor, (ii) to advance or supply funds (A) for the purchase or payment of any such primary obligation or (B) to maintain working capital or equity capital of the primary obligor or otherwise to maintain the net worth or solvency of the primary obligor, (iii) to purchase property, assets, securities or services primarily for the purpose of assuring the owner of any such primary obligation of the ability of the primary obligor to make payment of such primary obligation or (iv) otherwise to assure or hold harmless the holder of such primary obligation against loss in respect thereof. The amount of any Guarantee Obligation shall be deemed to be an amount equal to the stated or determinable amount of the primary obligation in respect of which such Guarantee Obligation is made (or, if less, the maximum amount of such primary obligation for which such Person may be liable pursuant to the terms of the instrument evidencing such Guarantee Obligation) or, if not stated or determinable, the maximum reasonably anticipated liability in respect thereof (assuming such Person is required to perform thereunder), as determined by such Person in good faith.

“Guarantors” means the Parent and the Subsidiary Guarantors.

“Hazardous Materials” means any material, substance or waste that is regulated, classified, or otherwise characterized under or pursuant to any Environmental Law as “hazardous”, “toxic”, a “pollutant”, a “Contaminant”, a “deleterious substance”, “dangerous goods”, “radioactive” or words of similar meaning or effect, including petroleum and its by-products, asbestos, polychlorinated biphenyls, radon, greenhouse gases, mold, urea formaldehyde insulation, chlorofluorocarbons and all other ozone-depleting substances.

“IFRS” means international financial reporting standards, as in effect from time to time.

“Immaterial Subsidiaries” means (a) those Foreign Subsidiaries of the Parent listed on Schedule 3 hereto as of the Closing Date (setting forth the approximate asset values for each such Foreign Subsidiary as of the Closing Date), (b) any other Foreign Subsidiary of Parent formed or acquired after the Closing Date that has assets with a fair market value of $250,000 or less and annual revenues of $250,000 or less (excluding intercompany accounts and intercompany revenues respectively) and (c) SomTherapeutics Corp., a Florida corporation, so long as all Immaterial Subsidiaries taken together under clauses (a), (b) and (c) shall have assets with a fair market value of $3,000,000 or less and annual revenues of $3,000,000 or less (excluding intercompany accounts and intercompany revenues respectively) in the aggregate at all times; provided, that any such Immaterial Subsidiary under clause (a), (b) or (c) shall no longer constitute an Immaterial Subsidiary to the extent provided in Section 7.18.

“Indebtedness” of any Person means, without duplication, (a) all obligations of such Person for borrowed money, (b) all obligations of such Person evidenced by bonds, debentures, notes or similar instruments, (c) all obligations of such Person under conditional sale or other title retention agreements relating to property acquired by such Person, (d) all obligations of such Person in respect of the deferred purchase price of property or services (excluding (i) accounts payable and other accrued liabilities incurred in the ordinary course of

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business not past due for more than 120 days after its stated due date (except for accounts payable contested in good faith), (ii) any earn-out obligation until such obligation is both required to be reflected as a liability on the balance sheet of such Person in accordance with IFRS and not paid after becoming due and payable, (iii) deferred or equity compensation arrangements entered into in the ordinary course of business and payable to directors, officers or employees and (iv) milestone payments due to Software AG Stiftung in connection with Birkin AG in an aggregate amount not to exceed 38,000,000 Euros which shall be payable solely on the basis of the criteria disclosed to Lenders prior to the Closing Date), (e) all Indebtedness (excluding prepaid interest thereon) of others secured by (or for which the holder of such Indebtedness has an existing right, contingent or otherwise, to be secured by) any Lien on property owned or acquired by such Person, whether or not the Indebtedness secured thereby has been assumed but, in the case of Indebtedness which is not assumed by such Person, limited to the lesser of (x) the amount of such Indebtedness and (y) the fair market value of such property, (f) all guarantees by such Person of Indebtedness of others, (g) all Attributable Indebtedness of such Person, (h) all obligations, contingent or otherwise, of such Person as an account party in respect of letters of credit and letters of guaranty (excluding the portion thereof that has been fully cash collateralized in a manner permitted by this Agreement), (i) all obligations, contingent or otherwise, of such Person in respect of bankers’ acceptances, Surety bonds and performance bonds, whether or not matured and (j) all obligations of such Person in respect of Disqualified Equity Interests. The Indebtedness of any Person shall include the Indebtedness of any other entity (including any partnership in which such Person is a general partner) to the extent such Person is directly liable therefor as a result of such Person’s ownership interest in or other relationship with such entity, except to the extent the terms of such Indebtedness provide that such Person is not liable therefor. Anything herein to the contrary notwithstanding, obligations in respect of any Indebtedness that has been irrevocably defeased (either covenant or legal) or satisfied and discharged pursuant to the terms of the instrument creating or governing such Indebtedness shall not constitute Indebtedness.

“Indemnified Liabilities” has the meaning specified in Section 10.05(a).

“Indemnified Taxes” means (a) Taxes, other than Excluded Taxes, imposed on or with respect to any payment made by or on account of any obligation of any Loan Party under any Loan Document and (b) to the extent not otherwise described in (a), Other Taxes.

“Indemnitees” has the meaning specified in Section 10.05(a).

“Information” has the meaning specified in Section 10.08.

“Intellectual Property” has the meaning specified in Section 5.17.

“Interest Payment Date” means March 31, June 30, September 30 and December 31 of each year and the Maturity Date.

“Intermediate Parent” means Amryt Pharma plc, a company incorporated in England and Wales with company number 05316808 (to be renamed and re-registered on or around the Closing Date as Amryt Pharma Holdings Limited).

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“Investment” in any Person, means any loan or advance to such Person, any purchase or other acquisition of any voting Equity Interests or other Equity Interests or Indebtedness or the assets comprising a division or business unit or a substantial part or all of the business of such Person, any capital contribution to such Person or any other direct or indirect investment in such Person.

“Irish Law Account Charge” means an Irish law charge and assignment to be entered into on or about the date hereof between the Parent (company number: 12107859) and the Administrative Agent in respect of its Irish bank accounts.

“Irish Law Debenture” means an Irish Law Debenture to be entered into on or about the date hereof between the Irish Loan Parties and the Administrative Agent whereby each of the Irish Loan Parties shall provide fixed and floating security over all of their assets.

“Irish Law Share Charge” means an Irish law Share Charge to be entered into on or about the date hereof between the Intermediate Parent (company number: 05316808) and the Administrative Agent in respect of the shares of Amryt Pharmaceuticals DAC.

“Irish Loan Parties” means each of Amryt Pharmaceuticals DAC, Amryt Research Limited, Amryt Genetics Limited, Amryt Lipidology Limited and Amryt Endocrinology Limited.

“Irish Security Documents” means the Irish Law Debenture, the Irish Law Account Charge and Irish law Share Charge.

“Laws” means, collectively, all international, foreign, federal, state, provincial and local statutes, treaties, rules, guidelines, regulations, ordinances, codes and administrative or judicial precedents or authorities, including the interpretation or administration thereof by any Governmental Authority charged with the enforcement, interpretation or administration thereof, and all applicable administrative orders, directed duties, requests, licenses, authorizations and permits of, and agreements with, any Governmental Authority.

“Lender” means any Lender that may be a party to this Agreement from time to time, including its successors and assigns as permitted hereunder (each of which is referred to herein as a “Lender”).

“Lien” means any assignment, mortgage, charge, pledge, lien, encumbrance, title retention agreement (including Capital Leases but excluding operating leases) or any other security interest whatsoever, howsoever created or arising, whether fixed or floating, legal or equitable, perfected or not, but specifically excludes any legal, contractual or equitable right of set-off.

“Liquidity Amount” means, as of any date, an amount equal to the sum of (i) the aggregate amount of unrestricted cash and Cash Equivalents of the Loan Parties on a consolidated basis as of such date that are free and clear of all Liens other than Liens in favor of the Administrative Agent for the benefit of the Secured Parties and nonconsensual statutory Liens permitted by Section 7.01 plus (ii) any cash expenditures made to French governmental pricing authorities as of such date in connection with the pricing and reimbursement approval of

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the MYALEPTA product in France to the extent representing the difference between the approved price of the MYALEPTA product and the price of the MYALEPTA product under the existing cohort ATU in France.

“Loan” means each New Money Loan and each Rollover Loan.

“Loan Documents” means, collectively, (i) this Agreement, (ii) the Notes, (iii) the Collateral Documents, (iv) any agency fee letter entered into between the Borrower and the Administrative Agent in connection with this Agreement and the other Loan Documents and (v) all other instruments and documents delivered from time to time by or on behalf of the Loan Parties or any of their Subsidiaries in connection herewith or therewith.

“Loan Parties” or “Loan Party” means, collectively or individually as the context may require, the Borrower and each Guarantor.

“Make-Whole Premium” has the meaning specified in Section 2.02(e).

“Material Adverse Effect” means (a) a material adverse change in, or a material adverse effect upon, the operations, business, properties, assets, liabilities (actual or contingent), financial condition of the Parent and its Subsidiaries, taken as a whole; (b) a material impairment of the ability of any Loan Party to perform its material obligations under any Loan Document to which it is a party; (c) a material adverse effect upon the legality, validity, binding effect or enforceability against any Loan Party of any Loan Document to which it is a party; or (d) a material impairment of the Administrative Agent’s or the Lenders’ ability to enforce the Obligations or realize upon the Collateral.

“Material Contracts” means any Contractual Obligation of any Loan Party or any of its Subsidiaries the failure to comply with which, or the termination (without contemporaneous replacement) of which, could reasonably be expected to have a Material Adverse Effect or otherwise result in liabilities in excess of $500,000.

“Maturity Date” means, the earlier to occur of (i) September 24, 2024 and (ii) the date on which the Loans and other Obligations hereunder are accelerated and become due and payable following the occurrence of an Event of Default, in each case, pursuant to Section 8.02.

“Moody’s” means Moody’s Investors Service, Inc. and its successors.

“Mortgage” means collectively, the deeds of trust, trust deeds, deeds to secure debt and mortgages creating and evidencing a Lien on real property granted by the Loan Parties in favor or for the benefit of the Administrative Agent, on behalf of the Secured Parties, in form and substance reasonably satisfactory to the Required Lenders and their counsel to account for local law matters) and otherwise in form and substance reasonably satisfactory to the Required Lenders, executed and delivered pursuant to the terms of this Agreement.

“Mortgaged Property” means any real property of a Loan Party that is subject to a Mortgage.

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“Multiemployer Plan” means a multiemployer plan, as defined in Section 4001(a)(3) of ERISA, to which any Loan Party or any ERISA Affiliate is making or accruing an obligation to make contributions, or has within any of the preceding five plan years made or accrued an obligation to make contributions.

“Net Cash Proceeds” means:

(a)           with respect to the Disposition of any asset by any Loan Party or any of its Subsidiaries or any Casualty Event the excess, if any, of (i) the sum of cash and Cash Equivalents received in connection with such Disposition or Casualty Event (including any cash or Cash Equivalents received by way of deferred payment pursuant to, or by monetization of, a note receivable or otherwise, but only as and when so received and, with respect to any Casualty Event, any insurance proceeds or condemnation awards in respect of such Casualty Event actually received by or paid to or for the account of the Parent or any of its Subsidiaries) over (ii) the sum of (A) the principal amount of any Indebtedness permitted by this Agreement that is secured by a lien (other than a Lien on the Collateral that is subordinated or junior to the Liens securing the Obligations) by the asset subject to such Disposition or Casualty Event and that is repaid (and is timely repaid) in connection therewith (other than Indebtedness under the Loan Documents), (B) the reasonable out-of-pocket expenses actually incurred and paid by the Parent or any of its Subsidiaries in connection with such Disposition or Casualty Event (including, reasonable attorney’s, accountant’s and other similar professional advisor’s fees, investment banking fees, survey costs, title insurance premiums, and related search and recording charges, transfer taxes, deed or mortgage recording taxes, other customary expenses and brokerage, consultant, and other customary fees) to third parties (other than the Loan Parties or any of their Affiliates), (C) taxes paid or reasonably estimated to be actually payable or that are actually accrued in connection therewith with respect to the current tax year as a result of any gain recognized in connection therewith by such Person or any of the direct or indirect stockholders thereof and attributable to such Disposition or Casualty Event; provided that, if the amount of any estimated taxes pursuant to this subclause (C) exceeds the amount of taxes actually required to be paid in cash, the aggregate amount of such excess shall constitute Net Cash Proceeds and (D) any reasonable reserve actually maintained in respect of (x) the sale price of such asset or assets established in accordance with IFRS and (y) any liabilities associated with such asset or assets and retained by the Borrower or any of its Subsidiaries after such sale or other Disposition thereof, including pension and other post-employment benefit liabilities and liabilities related against any indemnification obligations associated with such transaction and it being understood that “Net Cash Proceeds” shall include any cash or Cash Equivalents (1) received upon the Disposition of any non-cash consideration received by such Person in any such Disposition, and (2) received upon the reversal (without the satisfaction of any applicable liabilities in cash in a corresponding amount) of any reserve described in subclause (D) above or, if such liabilities have not been satisfied in cash and such reserve not reversed within two years after such Disposition or Casualty Event, the amount of such reserve; and

(b)          with respect to the incurrence or issuance of any Indebtedness by any Loan Party or any of its Subsidiaries not permitted under Section 7.03, the excess, if any,

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of (i) the sum of the cash received in connection with such incurrence or issuance over (ii) the investment banking fees, underwriting discounts, commissions, costs and other out-of-pocket expenses and other customary expenses (including reasonable attorney’s, accountant’s and other similar professional advisor’s fees), incurred by such Person in connection with such incurrence or issuance to third parties (other than the Loan Parties or any of their Affiliates).

“New Money Loans” means the Loans made pursuant to Section 2.01(a) and, for the avoidance of doubt, excludes the Rollover Loans.

“Non-Consenting Lender” has the meaning specified in Section 3.04(c).

“Note” means a promissory note of the Borrower payable to a Lender or its assigns, substantially in the form of Exhibit B hereto, evidencing the aggregate Indebtedness of the Borrower owing to such Lender resulting from the Loans made by such Lender.

“NPL” means the National Priorities List under CERCLA.

“Obligations” means all advances to, and debts, liabilities, guarantees, obligations, covenants and duties of, any Loan Party to the Secured Parties arising under any Loan Document, whether direct or indirect (including those acquired by assumption), absolute or contingent, due or to become due, now existing or hereafter arising, including after the commencement of any Debtor Relief Laws and regardless of whether allowed or allowable as a claim in any proceeding under such Debtor Relief Laws. Without limiting the generality of the foregoing, the Obligations of the Loan Parties under the Loan Documents include the obligation (including Guarantee Obligations) to pay principal, interest, reimbursement obligations, charges, expenses, fees, Attorney Costs, indemnities and other amounts payable by any Loan Party under any Loan Document.

“OFAC” means the Office of Foreign Assets Control of the U.S. Department of the Treasury.

“Organization Documents” means (a) with respect to any corporation, the certificate or articles of incorporation and the bylaws; (b) with respect to any limited liability company, the certificate or articles of formation or constitution or association or organization and operating agreement; and (c) with respect to any partnership, joint venture, trust or other form of business entity, the partnership, joint venture or other applicable agreement of formation or organization and any agreement, declaration, instrument, filing or notice with respect thereto filed in connection with its formation or organization with the applicable Governmental Authority in the jurisdiction of its formation or organization and, if applicable, any certificate or articles of formation or organization of such entity.

“Other Connection Taxes” means, with respect to any Recipient, Taxes imposed as a result of a present or former connection between such Recipient and the jurisdiction imposing such Tax (other than connections arising from such Recipient having executed, delivered, become a party to, performed its obligations under, received payments under, received or perfected a security interest under, engaged in any other transaction pursuant to or enforced any Loan Document, or sold or assigned an interest in any Loan or Loan Document).

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“Other Taxes” means all present or future stamp, court or documentary, intangible, recording, filing or similar Taxes that arise from any payment made under, from the execution, delivery, performance, enforcement or registration of, from the receipt or perfection of a security interest under, or otherwise with respect to, any Loan Document, except any such Taxes that are Other Connection Taxes imposed with respect to an assignment.

“Parent” has the meaning specified in the introductory paragraph hereto.

“Participant” has the meaning specified in Section 10.07(d).

“Participant Register” has the meaning specified in Section 10.07(d).

“PBGC” means the Pension Benefit Guaranty Corporation (or any successor thereof).

“Pension Plan” means any “employee pension benefit plan” (as such term is defined in Section 3(2) of ERISA) other than a Multiemployer Plan, that is subject to Title IV of ERISA and is sponsored or maintained by any Loan Party or any ERISA Affiliate or to which any Loan Party or any ERISA Affiliate contributes or has an obligation to contribute, or in the case of a multiple employer or other plan described in Section 4064(a) of ERISA, has made contributions at any time since January 1, 2003.

“Permitted AP License” means any license of AP101 and AP103 assets outside of the United States and Europe.

“Permitted Holders” means those affiliates of, or investment funds managed or advised by either (a) Athyrium Capital Management, LP and its affiliates and the investment funds managed or advised by any of the foregoing or (b) Highbridge MSF International Ltd., Highbridge Tactical Credit Master Fund, L.P., Highbridge SCF Special Situations SPV, L.P., and Highbridge SCF Loan SPV, L.P. and its affiliates.

“Permitted Liens” has the meaning specified in Section 7.01.

“Permitted Uses” means collectively: (a) on the Closing Date, funding of an aggregate amount not to exceed $26,552,201.50 to be simultaneously applied to the repayment of all obligations of Amryt Pharmaceuticals DAC owing in respect of the Amryt Finance Contract; (b) conversion of the “New Money Loans” (and accrued fees and interest thereon) under, and as defined in, the Existing Bridge Credit Agreement and (c) working capital and general corporate expenses.

“Person” means any individual, sole proprietorship, partnership, limited liability company, joint venture, company, trust, unincorporated organization, association, corporation, institution, public benefit corporation, firm, joint stock company, estate, entity or government agency.

“Petition Date” has the meaning specified in the Preliminary Statements hereto.

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“PIK Election Request” means a PIK Election Request substantially in the form of Exhibit A-3.

“PIK Interest” has the meaning specified in Section 2.04(a).

“Plan” means any “employee benefit plan” (as such term is defined in Section 3(3) of ERISA) established by any Loan Party or, with respect to any such plan that is subject to Section 412 of the Code or Title IV of ERISA, any ERISA Affiliate. ,

“Plan Funding Agreement” means that certain Plan Funding Agreement dated as of May 20, 2019 by and between the Borrower and the Intermediate Parent, as the same may be amended, restated, supplemented or otherwise modified from time to time, in each case with the prior written consent of the Lenders.

“Platform” has the meaning specified in Section 10.02(e).

“Pledge Agreement” means the Pledge Agreement executed by the Intermediate Parent and the Administrative Agent substantially in the form of Exhibit E-2 (as such agreement may be amended, restated, supplemented or otherwise modified from time to time).

“Prepayment Notice” means a notice of prepayment in respect of any voluntary or mandatory prepayment in substantially the form of Exhibit A-2.

“Pro Rata Share” means, with respect to each Lender at any time a fraction (expressed as a percentage, carried out to the ninth decimal place), the numerator of which is the amount of the Commitment of such Lender at such time and the denominator of which is the amount of the Aggregate Commitments at such time; provided that if the Aggregate Commitments have been terminated, then the Pro Rata Share of each Lender shall be determined based on the outstanding principal amount of the Loans held by such Lender divided by the aggregate principal amount of all outstanding Loans held by all Lenders.

“Proceeding” has the meaning specified in Section 10.05(a).

“Public Lender” has the meaning specified in Section 10.02(h).

“Qualified Equity Interests” means any Equity Interests that are not Disqualified Equity Interests.

“Recipient” means the Administrative Agent or any Lender, as applicable.

“Refinancing Indebtedness” means refinancings, renewals, or extensions of Indebtedness, so long as:

(a)          such refinancings, renewals, or extensions do not result in an increase in the principal amount of the Indebtedness as of the time it is so refinanced, renewed, or extended (other than by the amount of the fees and expenses incurred in connection therewith);

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(b)          such refinancings, renewals, or extensions do not result in a shortening of the average weighted maturity (measured as of the refinancing, renewal, or extension) of the Indebtedness so refinanced, renewed, or extended;

(c)          if the Indebtedness that is refinanced, renewed, or extended was subordinated in right of payment to the Obligations, then the terms and conditions of the refinancing, renewal, or extension must include subordination terms and conditions that are at least as favorable to the Lenders as those that were applicable to the refinanced, renewed, or extended Indebtedness; and

(d)         the Indebtedness that is refinanced, renewed, or extended is not recourse to any Person that is liable on account of the Obligations other than those Persons which were obligated with respect to the Indebtedness that was refinanced, renewed, or extended.

“Register” has the meaning specified in Section 10.07(c).

“Registered” means, with respect to Intellectual Property, issued by, registered with, renewed by or the subject of a pending application before any Governmental Authority or Internet domain name registrar.

“Related Parties” means, with respect to any Person, such Person’s Affiliates and the partners, directors, officers, employees, agents, trustees, administrators, managers, advisors and representatives of such Person and of such Person’s Affiliates.

“Release” means any release, spill, emission, leaking, pumping, pouring, injection, escaping, deposit, disposal, discharge, leeching or migration of any Hazardous Material in or into the environment (including the abandonment or disposal of any barrels, tanks, containers or receptacles containing any Hazardous Material), or out of any vessel or facility, including the movement of any Hazardous Material through the air, soil, subsoil, surface, water, ground water, rock formation or otherwise.

“Reorganization Plan” has the meaning specified in the Preliminary Statements hereto.

“Reportable Event” means with respect to any Plan any of the events set forth in Section 4043(c) of ERISA or the regulations issued thereunder, other than events for which the thirty (30) day notice period has been waived.

“Required Lenders” means, as of any date of determination, Lenders holding more than 50% of the aggregate principal amount of all outstanding Loans at such time; provided that if there are two (2) or more Lenders that are not Affiliates, then Required Lenders shall require at least two (2) Lenders that are not Affiliates holding more than 50% of the aggregate principal amount of all outstanding Loans at such time.

“Requirement of Law” means, as to any Person, any law (including common law), statute, ordinance, treaty, rule, regulation, order, decree, judgment, writ, injunction or settlement agreement, requirement or determination of an arbitrator or a court or other

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Governmental Authority, in each case applicable to or binding upon such Person or any of its property or to which such Person or any of its property is subject.

“Responsible Officer” means the chief executive officer, director, president, chief financial officer, treasurer or, except for purposes of Sections 6.02 or 6.03, any other similar officer or a Person performing similar functions of a Loan Party (and, as to any document delivered on the Closing Date, to the extent permitted or required by the terms of this Agreement, any secretary or assistant secretary of a Loan Party). Any document delivered hereunder that is signed by a Responsible Officer of a Loan Party shall be conclusively presumed to have been authorized by all necessary corporate, partnership and/or other action on the part of such Loan Party and such Responsible Officer shall be conclusively presumed to have acted on behalf of such Loan Party.

“Restricted Payment” means any:

(a)          dividend or other distribution (whether in cash, securities or other property) or any payment (whether in cash, securities or other property), in each case, with respect to any capital stock or other Equity Interest of any Person or any of its Subsidiaries, including any sinking fund or similar deposit, on account of the purchase, retraction, redemption, retirement, defeasance, acquisition, cancellation or termination of any such capital stock or other Equity Interest, or on account of any return of capital to any Person’s stockholders, partners or members (or the equivalent of any thereof and including any thereof acquired through the exercise of warrants or rights of conversion, exchange or purchase); and

(b)          payment of any management or similar type fees by a Loan Party to any Affiliate thereof.

“Restricting Information” has the meaning assigned to such term in Section 10.02(f).

“Rollover Loans” has the meaning assigned to such term in Section 2.01(b).

“S&P” means Standard & Poor’s Ratings Services LLC, a Standard & Poor’s Financial Services LLC business, and its successors.

“Sale Leaseback” means any transaction or series of related transactions pursuant to which any Loan Party or any of its Subsidiaries (a) sells, transfers or otherwise disposes of any property, real or personal, whether now owned or hereafter acquired, and (b) as part of such transaction, thereafter rents or leases such property or other property that it intends to use for substantially the same purpose or purposes as the property being sold, transferred or disposed.

“Sanctions” means economic or financial sanctions or trade embargos imposed, administered or enforced from time to time by (a) the U.S. government, including those administered by OFAC or the U.S. Department of State, or (b) the United Nations Security Council, the European Union, Canada, any European Union member state or Her Majesty’s Treasury of the United Kingdom.

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“Sanctioned Country” means, at any time, a country or territory which is itself the subject or target of any Sanctions (at the time of this Agreement, Cuba, Iran, North Korea, Syria, and the Crimea region of Ukraine).

“Sanctioned Person” means any individual or entity, at any time, that is the subject or target or Sanctions, including (a) any individual or entity listed in any Sanctions related list of designated Persons maintained by OFAC or the U.S. Department of State, the United Nations Security Council, the European Union, Canada, any Member State of the European Union, or the United Kingdom, (b) any individual or entity operating, organized or resident in a Sanctioned Country or (c) any entity that is, in the aggregate, 50 percent or greater owned, directly or indirectly or otherwise, or where relevant under Sanctions, controlled by any such person or entity described in clause (a).

“SEC” means the Securities and Exchange Commission, or any Governmental Authority succeeding to any of its principal functions.

“Secured Parties” means, collectively, the Administrative Agent, the Lenders and each Supplemental Administrative Agent.

“SPC” has the meaning specified in Section 10.07(f).

“Subsidiary” is, as to any Person, a corporation, partnership, limited liability company or other entity of which shares of stock or other ownership interests having ordinary voting power (other than stock or such other ownership interests having such power only by reason of the happening of a contingency) to elect a majority of the board of directors or other managers of such corporation, partnership or other entity are at the time owned, or the management of which is otherwise controlled, directly or indirectly through one or more intermediaries, or both, by such Person. Unless the context otherwise requires, each reference to a Subsidiary herein shall be a reference to a Subsidiary of the Parent.

“Subsidiary Guarantor” means (a) each Subsidiary of the Parent, other than the Borrower, Aegerion Securities (Corporation, a Massachusetts corporation, the Immaterial Subsidiaries and any Subsidiary organized in France, but including each Subsidiary listed under the heading “Subsidiary Guarantors” on Schedule 2, and (b) each other Subsidiary of the Parent that becomes a Guarantor after the Closing Date pursuant to a Guarantee and Collateral Agreement Supplement or other documentation in form and substance reasonably satisfactory to the Required Lenders (it being understood that the Subsidiary Guarantors shall not include certain Subsidiaries, as agreed by the Parent and the Required Lenders to the extent the Parent and the Required Lenders determine that including such Subsidiaries as Subsidiary Guarantors will result in material adverse tax consequences to the Loan Parties and their Subsidiaries or determine that it would violate applicable law in any material respect).

“Supplemental Administrative Agent” has the meaning specified in Section 9.13(a) and “Supplemental Administrative Agents” shall have the corresponding meaning.

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“Taxes” means any and all present or future taxes, duties, levies, imposts, deductions, assessments, fees, stamp taxes, withholdings or other charges imposed by any Governmental Authority (including additions to tax, penalties and interest with respect thereto).

“Termination Date” has the meaning specified in Section 9.11 (a)(i).

“Threshold Amount” means $5,000,000.

“Trade Date” has the meaning specified in Section 10.07(h).

“UK Collateral” shall mean all property (whether real, personal or otherwise) with respect to which any security interests have been granted (or purported to be granted) by the UK Loan Parties or will be granted in accordance with this Agreement.

“UK Debenture” means an English law debenture to be entered into on or about the date hereof between the UK Loan Parties and the Administrative Agent whereby each of the UK Loan Parties provide fixed and floating security over all or substantially all of their assets.

“UK Loan Parties” means each of (a) Aegerion Pharmaceuticals Limited (company number: 08114919), (b) Amryt Pharma (UK) Limited (company number: 10463152), (c) the Intermediate Parent (company number: 05316808) and (d) the Parent (company number: 12107859). 

“UK Insolvency Event” shall mean any corporate action, legal proceedings or other procedure or step is taken in relation to:

(a)          the suspension of payments, a moratorium of any indebtedness (provided the ending of such moratorium will not remedy any Event of Default caused by such moratorium), winding-up, dissolution, administration or reorganization (by way of voluntary arrangement, scheme of arrangement or otherwise) of any UK Loan Party;

(b)          a composition, compromise, assignment or arrangement with any creditor of any UK Loan Party in connection with or as a result of any financial difficulty on the part of any UK Loan Party;

(c)          the appointment of a liquidator, receiver, administrative receiver, administrator, compulsory manager or other similar officer in respect of any UK Loan Party, or any of its assets; or

(d)          any UK Loan Party (i) is unable or admits generally an inability to pay its debts as they fall due, or (ii) suspends or threatens generally to suspend making payments on any of its debts,

or any analogous procedure or step is taken in any jurisdiction provided that clauses (a) to (c) above shall not apply to (i) any winding-up petition which is frivolous or vexatious or which is discharged, stayed or dismissed within 20 Business Days of commencement, (ii) the appointment of an administrator (or any procedure or step in relation to

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such appointment) which the Administrative Agent is satisfied will be withdrawn and unsuccessful or (iii) any actions expressly permitted by the Credit Agreement.

“UK Security Documents” means the UK Debenture, the UK Share Charge 1 and the UK Share Charge 2.

“UK Share Charge 1” means the English law share charge to be entered into on or about the date hereof between Amryt Pharmaceuticals DAC and the Administrative Agent.

“UK Share Charge 2” means the English law share charge to be entered into no later than the date set forth in Section 6.20 between Aegerion Pharmaceuticals Limited and the Administrative Agent.

“Uniform Commercial Code” means the Uniform Commercial Code as the same may from time to time be in effect in the State of New York or the Uniform Commercial Code (or similar code or statute) of another jurisdiction, to the extent it may be required to apply to any security interest in any item or items of Collateral.

“United States” and “U.S.” mean the United States of America.

“U.S. Person” means any Person that is a “United States Person” as defined in Section 7701(a)(30) of the Code.

“U.S. Tax Compliance Certificate” has the meaning specified in Section 3.01 (g)(ii)(B)(3).

“USA PATRIOT Act” means the Uniting and Strengthening America by Providing Appropriate Tools Required to Intercept and Obstruct Terrorism Act of 2001 (Title III of Pub. L. 107-56 (signed into law October 26, 2001)), as the same may be amended, supplemented, modified, replaced or otherwise in effect from time to time.

“Wholly-owned” means, with respect to a Subsidiary of a Person, a Subsidiary of such Person all of the outstanding Equity Interests of which (other than (x) director’s qualifying shares and (y) shares issued to foreign nationals to the extent required by applicable Law) are owned by such Person and/or by one or more wholly-owned Subsidiaries of such Person.

“Withdrawal Liability” means the liability of a Multiemployer Plan as a result of a complete or partial withdrawal from such Multiemployer Plan, as such terms are defined in Part I of Subtitle E of Title IV of ERISA.

“Withholding Agent” means any Loan Party and the Administrative Agent.

“Write-Down and Conversion Powers” means, with respect to any EEA Resolution Authority, the write-down and conversion powers of such EEA Resolution Authority from time to time under the Bail-In Legislation for the applicable EEA Member Country, which write-down and conversion powers are described in the EU Bail-In Legislation Schedule.

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Section 1.02 Other Interpretive Provisions. With reference to this Agreement and each other Loan Document, unless otherwise specified herein or in such other Loan Document:

(a)          The meanings of defined terms are equally applicable to the singular and plural forms of the defined terms.

(b)          The words “herein”, “hereto”, “hereof and “hereunder” and words of similar import when used in any Loan Document shall refer to such Loan Document as a whole and not to any particular provision thereof.

(c)          Article, Section, paragraph, clause, subclause, Exhibit and Schedule references are to the Loan Document in which such reference appears.

(d)          The term “including” is by way of example and not limitation.

(e)          The term “documents” includes any and all instruments, documents, agreements, certificates, notices, reports, financial statements and other writings, however evidenced, whether in physical or electronic form.

(f)           In the computation of periods of time from a specified date to a later specified date, the word “from” means “from and including”; the words “to” and “until” each mean “to but excluding”; and the word “through” means “to and including”.

(g)          Section headings herein and in the other Loan Documents are included for convenience of reference only and shall not affect the interpretation of this Agreement or any other Loan Document.

(h)          Whenever the context may require, any pronoun shall include the corresponding masculine, feminine or neuter forms.

Section 1.03 Accounting Terms. (a) All accounting terms not specifically or completely defined herein shall be construed in conformity with, and all financial data (including financial ratios and other financial calculations) required to be submitted pursuant to this Agreement shall be prepared in conformity with IFRS, consistently applied, except as otherwise specifically prescribed herein; provided, however, that if the Borrower notifies the Administrative Agent that the Borrower requests an amendment to any provision hereof to eliminate the effect of any Accounting Change occurring after the Closing Date or in the application thereof on the operation of such provision, regardless of whether any such notice is given before or after such Accounting Change or in the application thereof, then the Lenders and the Borrower agree that they will negotiate in good faith amendments to the provisions of this Agreement that are directly affected by such Accounting Change with the intent of having the respective positions of the Lenders and the Borrower after such Accounting Change conform as nearly as possible to their respective positions as of the date of this Agreement and, until any such amendments have been agreed upon, (i) the provisions in this Agreement shall be calculated as if no such Accounting Change had occurred and (ii) the Borrower shall provide to the Administrative Agent and the Lenders a written reconciliation in form and substance reasonably

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satisfactory to the Required Lenders, between calculations of any baskets and other requirements hereunder before and after giving effect to such Accounting Change.

(b)          Where reference is made to a Person “and its Subsidiaries on a consolidated basis” or similar language, such consolidation shall not include any subsidiaries other than Subsidiaries.

Section 1.04 References to Agreements, Laws, Etc. Unless otherwise expressly provided herein, (a) references to documents, agreements (including the Loan Documents) and other contractual instruments shall be deemed to include all subsequent amendments, restatements, amendments and restatements, extensions, supplements and other modifications thereto, but only to the extent that such amendments, restatements, amendments and restatements, extensions, supplements and other modifications are not prohibited by any Loan Document; and (b) references to any Law shall include all statutory and regulatory provisions consolidating, amending, replacing, supplementing or interpreting such Law.

Section 1.05 Times of Day. Unless otherwise specified, all references herein to times of day shall be references to Eastern time (daylight or standard, as applicable).

Section 1.06 Timing of Payment or Performance. When the payment of any obligation or the performance of any covenant, duty or obligation is stated to be due or performance required on a day which is not a Business Day, the date of such payment or performance shall extend to the immediately succeeding Business Day.

ARTICLE II

THE COMMITMENTS AND THE LOANS

Section 2.01 The Commitments and the Loans.

(a)          Subject to the terms and conditions set forth herein, each Lender severally agrees to make (or cause its Applicable Lending Office to make), to the Borrower, subject to the terms and conditions hereof, on the Closing Date, term loans in one drawing in an aggregate principal amount not to exceed such Lender’s Commitment; provided that the loans made by all Lenders under this Section 2.01(a) shall not exceed in the aggregate $[***]. Amounts paid or prepaid in respect of New Money Loans may not be reborrowed.

(b)          On the Closing Date, the outstanding principal amount of the “New Money Loans” (and accrued fees and interest thereon) under, and as defined in, the Existing Bridge Credit Agreement shall be converted into, and shall be deemed to constitute, additional loans under this Agreement (in addition to those advanced under Section 2.01(a) above). On the Closing Date, $[***] in principal amount of such additional loans shall be deemed to have been advanced by Lenders to Borrower under this Agreement in the amounts set forth for each Lender on Schedule 2.01 under the caption “Rollover Loans” opposite such Lender’s name (such loans, the “Rollover Loans”), in full satisfaction by conversion of the “New Money Loans” (and accrued fees and interest thereon) under, and as defined in, the Existing Bridge Credit Agreement. The Rollover Loans shall be deemed to be Loans outstanding for all purposes under this Agreement owed by the Borrower to such Lenders in the aggregate principal amount of

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$[***]. Amounts paid or prepaid in respect of Rollover Loans may not be reborrowed. Once borrowed (or deemed borrowed), the New Money Loans and the Rollover Loans shall constitute a single tranche of Loans for all purposes under this Agreement.

Section 2.02 Prepayments. (a) Optional Prepayments. The Borrower may, upon delivery of a Prepayment Notice to the Administrative Agent, at any time or from time to time voluntarily prepay Loans, in whole or in part subject to payment of the Make-Whole Premium, if applicable, at the time of such prepayment; provided that (1) such notice must be received by the Administrative Agent not later than 12:00 noon (New York, New York time) two (2) Business Days prior to any date of prepayment of Loans; and (2) any prepayment of Loans shall be in a principal amount of $5,000,000 or a whole multiple of $1,000,000 in excess thereof or, in each case, if less, the entire principal amount thereof then outstanding, in each case, with accrued and unpaid interest on the Loans to be repaid. Each such notice shall specify the date and amount of such prepayment. The Administrative Agent will promptly notify each Lender of its receipt of each such notice, and of the amount of such Lender’s Pro Rata Share of such prepayment. If such notice is given by the Borrower, the Borrower shall make such prepayment and the payment amount specified in such notice shall be due and payable on the date specified therein; provided, that if such prepayment is conditioned upon the occurrence of some other event, such notice may be revoked if such condition is not satisfied. Each prepayment of Loans pursuant to this Section 2.02(a) shall be paid to the Lenders in accordance with their respective Pro Rata Shares. For the avoidance of doubt, (x) the outstanding Loan amount shall include accrued interest and capitalized interest already added to the Loan balance and (y) any prepayment shall be applied in accordance with Section 8.03 if then applicable.

(b)          Mandatory Prepayments. (i) If any Loan Party or any of its Subsidiaries receives any Net Cash Proceeds from any Disposition (other than any Disposition permitted under Sections 7.05(b), 7.05(c), 7.05(e), 7.05(f) 7.05(h) or 7.05(i) (which proceeds in the case of Section 7.05(i) shall be subject to a partial reinvestment right in accordance with the second proviso to Section 7.05 addressing Section 7.05(i)) and Dispositions not to exceed $25,000 individually and $1,000,000 in the aggregate during the term of this Agreement), the Borrower shall, subject to Sections 2.02(b)(vi) and 2.02(c), cause to be prepaid an aggregate principal amount of the Loans equal to 100% of all Net Cash Proceeds received therefrom on the date which is six (6) Business Days after the receipt of such Net Cash Proceeds.

(ii)          If any Loan Party or any of its Subsidiaries receives any Net Cash Proceeds from any Casualty Event, the Borrower shall, subject to Sections 2.02(b)(vi) and 2.02(c), cause to be prepaid an aggregate principal amount of the Loans equal to 100% of all Net Cash Proceeds received therefrom on the date which is six (6) Business Days after the receipt of such Net Cash Proceeds; provided, that so long as no Event of Default shall have occurred and be continuing and the aggregate Net Cash Proceeds from all Casualty Events during the term of this Agreement does not exceed $10,000,000, the Borrower may elect to have the applicable Loan Party or Subsidiary reinvest all or a portion of such Net Cash Proceeds in replacement assets within 360 days following receipt of such Net Cash Proceeds; provided, further, that if any such Net Cash Proceeds are no longer intended to be so reinvested or are not reinvested by the end of such period, an amount equal to any such Net Cash Proceeds shall be promptly applied to prepayment of the Loans.

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(iii)         If any Loan Party or any of its Subsidiaries incurs or issues any Indebtedness not expressly permitted to be incurred or issued pursuant to Section 7.03, the Borrower shall, subject to Section 2.02(b)(vi) cause to be prepaid an aggregate principal amount of the Loans equal to 100% of all Net Cash Proceeds received therefrom on the date which is six (6) Business Days after the receipt of such Net Cash Proceeds, plus the Make-Whole Premium, if applicable.

(iv)         [Reserved].

(v)          The Borrower shall notify the Administrative Agent in writing of any mandatory prepayment of Loans required to be made pursuant to clauses (i) through (iii) of this Section 2.02(b) within one (1) Business Day of receipt of the applicable Net Cash Proceeds pursuant to a Prepayment Notice. Each such notice shall specify the date of such prepayment (which notice shall be at least five (5) Business Days prior to the date of prepayment) and provide a reasonably detailed calculation of the amount of such prepayment. The Administrative Agent will promptly notify each Lender of the contents of the Borrower’s Prepayment Notice and of such Lender’s Pro Rata Share of the prepayment, in each case, with accrued and unpaid interest on the Loans to be repaid and the Make-Whole Premium, if any, with respect to such Loans. For the avoidance of doubt, (x) the outstanding Loan amount shall include accrued interest and capitalized interest already added to the Loan balance and (y) any prepayment shall be applied in accordance with Section 8.03 if then applicable.

(vi)         Notwithstanding the foregoing, each Lender may decline all or a portion of its Pro Rata Share of any mandatory prepayment pursuant to this Section 2.02(b) (such declined amounts, the “Declined Proceeds’’) by providing written notice (each, a “Rejection Notice”) to the Administrative Agent and the Borrower no later than 5:00 p.m. (New York, New York time) on the date that is three (3) Business Days after the date of such Lender’s receipt of notice from the Administrative Agent regarding such prepayment. Each Rejection Notice from a Lender shall specify the principal amount of the mandatory prepayment to be declined by such Lender. If a Lender fails to deliver a’ Rejection Notice to the Administrative Agent within the time frame specified above, or such Rejection Notice fails to specify the principal amount of the mandatory prepayment to be declined, then such Lender shall be deemed to have accepted the total amount of such mandatory prepayment. Any Declined Proceeds may be retained by the Borrower and used for general corporate purposes and working capital.

(c)          Restrictions. Notwithstanding the foregoing, to the extent any or all of the Net Cash Proceeds of any Disposition by, or Casualty Event of, a Foreign Subsidiary that is not a Guarantor otherwise giving rise to a prepayment pursuant to Section 2.02(b) is prohibited or delayed by any applicable local Requirements of Law from being applied to the Loans or otherwise repatriated to the Borrower including through the repayment of intercompany Indebtedness (each, a “Repatriation”; with “Repatriated” having a correlative meaning) (the Parent hereby agreeing to use reasonable efforts to cause the applicable Foreign Subsidiary to take promptly all actions reasonably required by such Requirements of Law to permit such Repatriation), or if the Parent and the Required Lenders reasonably determine in good faith that Repatriation of any such amount would reasonably be expected to have material adverse tax consequences with respect to its Subsidiaries, after taking into account any foreign tax credit or benefit actually received in connection with such Repatriation, the portion of such Net Cash

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Proceeds so affected (such amount, the “Excluded Prepayment Amount”) will not be required to be applied to prepay Loans at the times provided in this Section 2.02; provided, that if and to the extent any such Repatriation ceases to be prohibited or delayed by applicable local Requirements of Law at any time immediately following the date on which the applicable mandatory prepayment pursuant to this Section 2.02(b) was required to be made, the Parent shall reasonably promptly Repatriate, or cause to be Repatriated, an amount equal to such portion of the Excluded Prepayment Amount, and the Borrower shall, or the Parent shall cause the applicable Loan Party or its Subsidiary to, reasonably promptly pay such portion of the Excluded Prepayment Amount to the Lenders, which payment shall be applied in accordance with this Section 2.02. For the avoidance of doubt, the non-application of any Excluded Prepayment Amount pursuant to this Section 2.02 shall not constitute a Default or an Event of Default. For the avoidance of doubt, this Section 2.02(c) shall not apply to any Disposition consisting of a Permitted AP License.

(d)          Interest. All prepayments under this Section 2.02 shall be accompanied by all accrued interest thereon.

(e)          Make-Whole Premium. In the event that all or any portion of the Loans is repaid (or prepaid) or accelerated prior to the date set forth in clause (i) of the definition of “Maturity Date” for any reason (including, without limitation, automatic acceleration upon an Event of Default under Section 8.01(f) or upon any redemption or buyback (including upon any Change of Control) but excluding mandatory prepayments under Section 2.02(b)(ii)), such repayment shall be made at (i) 105% of the amount then payable plus interest that would have accrued on such amount through the second anniversary of the Closing Date discounted at a rate equal to the yield on U.S. Treasury notes with a maturity closest to the second anniversary of the Closing Date plus 50 basis points if such repayment or acceleration occurs prior to the second anniversary of the Closing Date, (ii) 105% of the amount then payable if such repayment or acceleration occurs on or after the second anniversary of the Closing Date but prior to the third anniversary of the Closing Date, (iii) 101% of the amount then payable if such repayment or acceleration occurs on or after the third anniversary of the Closing Date but prior to the fourth anniversary of the Closing Date and (iv) 100% of the amount then payable if such repayment or acceleration occurs at any time thereafter (the “Make-Whole Premium”). Any Make-Whole Premium required to be made ‘pursuant to the foregoing clause (i) after acceleration shall be reduced by the amount of interest accruing following such acceleration through such repayment, provided that such interest shall be paid as part of the accrued interest that accompanies the principal amount of the Loans. No Make-Whole Premium shall be required in connection with any mandatory prepayment under Section 2.02(b)(i) with respect to Dispositions permitted under Section 7.05. The Borrower and the Lenders (as opposed to the Administrative Agent) shall be responsible for calculating the Make-Whole Premium.

Section 2.03 Repayment of Loans. The Borrower shall repay on the Maturity Date to the Administrative Agent (for the ratable account of the Lenders) the aggregate principal amount of all Loans, together with all accrued and capitalized interest (including interest paid in kind) and fees thereon (including the Make-Whole Premium, if any, and all other outstanding Obligations), outstanding on such date.

Section 2.04 Interest. (a) Subject to the provisions of Section 2.06(b), each Loan shall bear interest on the outstanding principal amount thereof at a rate per annum equal to

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the Applicable Rate and the accrued interest shall be due and payable in cash on each Interest Payment Date. Notwithstanding the foregoing, at the Borrower’s election of the interest option set forth in clause (y) of the definition of “Applicable Rate”, the portion of interest set forth in clause (ii) thereof in respect of the Loans shall be capitalized, compounded and added to the principal amount of the Loans outstanding on each Interest Payment Date (such capitalized interest, “PIK Interest”); provided that PIK Interest shall be treated as Loans for purposes of this Agreement and shall bear interest in accordance with this Section 2.04. To make an election pursuant to this Section 2.04(a), the Borrower shall notify the Administrative Agent of such election in writing by delivery to the Administrative Agent of an executed PIK Election Request at least five (5) Business Days prior to each Interest Payment Date indicating the amount of interest to be paid in kind and the amount of interest to be paid in cash in respect of the Loans on such Interest Payment Date.

(b)          Commencing (x) upon the occurrence and during the continuance of any Event of Default at the request of the Administrative Agent (upon the instruction of the Required Lenders), or automatically upon the occurrence of an Event of Default under Section 8.01(a) or 8.01(f), the Borrower shall pay interest on (i) the principal amount of the Loans and (ii) to the extent then due and payable all other outstanding Obligations hereunder, in each case under clauses (i) and (ii), at an interest rate per annum at all times equal to the Default Rate to the fullest extent permitted by applicable Laws. Accrued interest at the Default Rate and all other accrued and unpaid interest on past due amounts (including interest on past due interest to the fullest extent permitted by applicable Laws) shall be due and payable upon demand in cash.

(c)          Interest on each Loan shall be due and payable in arrears on each Interest Payment Date applicable thereto in accordance with Section 2.04(a) and at such other times as may be specified herein. Interest hereunder shall be due and payable in accordance with the terms hereof before and after any judgment.

Section 2.05 Fees. The Borrower shall pay to the Administrative Agent, for its own account, the fees set forth in the separate fee letter as between the Borrower and the Administrative Agent.

Section 2.06 Computation of Interest and Fees. All computations of fees and interest shall be made on the basis of a three hundred and sixty (360) day year and actual days elapsed. Interest shall accrue on each Loan for the day on which such Loan is made, and shall not accrue on such Loan, or any portion thereof, for the day on which such Loan or such portion is paid; provided that any such Loan that is repaid on the same day on which it is made shall, subject to Section 2.08(a), bear interest for one (1) day. Each determination by the Administrative Agent, the Borrower or the Lenders, as applicable, of an interest rate or fee hereunder shall be conclusive and binding for all purposes, absent manifest error.

Section 2.07 Evidence of Indebtedness, (a) The Loans made by each Lender shall be evidenced by one or more accounts or records maintained by such Lender and evidenced by one or more entries in the Register maintained by the Administrative Agent, as agent for the Borrower, in each case in the ordinary course of business. The accounts or records maintained by the Administrative Agent and each Lender shall be prima facie evidence absent manifest error of the amount of the Loans made by the Lenders to the Borrower and the interest and payments

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thereon. Any failure to so record or any error in doing so shall not, however, limit or otherwise affect the obligation of the Borrower hereunder to pay any amount owing with respect to the Obligations. In the event of any conflict between the accounts and records maintained by any Lender and the accounts and records of the Administrative Agent in respect of such matters, the accounts and records of the Administrative Agent shall control in the absence of manifest error. Upon the request of any Lender, the Borrower shall execute and deliver to such Lender a Note payable to such Lender, which shall evidence such Lender’s Loans in addition to such accounts or records. Each Lender may attach schedules to its Note and endorse thereon the date, amount and maturity of its Loans and payments with respect thereto.

(b)          In addition to the accounts and records referred to in Section 2.07(c), each Lender and the Administrative Agent shall maintain in accordance with its usual practice accounts or records. In the event of any conflict between the accounts and records maintained by the Administrative Agent and the accounts and records of any Lender in respect of such matters, the accounts and records of the Administrative Agent shall control in the absence of manifest error.

(c)          Entries made in good faith by the Administrative Agent in the Register pursuant to Section 2.07(a), and by each Lender in its account or accounts pursuant to Section 2.07(a), shall be prima facie evidence of the amount of principal and interest due and payable or to become due and payable from the Borrower to, in the case of the Register, each Lender and, in the case of such account or accounts, such Lender, under this Agreement and the other Loan Documents, absent manifest error; provided that the failure of the Administrative Agent or such Lender to make an entry, or any finding that an entry is incorrect, in the Register or such account or accounts shall not limit or otherwise affect the obligations of the Borrower under this Agreement and the other Loan Documents.

Section 2.08 Payments Generally. (a) All payments to be made by the Borrower shall be made without condition or deduction for any counterclaim, defense, recoupment or setoff. Except as otherwise expressly provided herein, all payments by the Borrower hereunder shall be made to the Administrative Agent, for the account of the respective Lenders to which such payment is owed, at the applicable Administrative Agent’s Office and in immediately available funds not later than 2:00 p.m. (New York, New York time) on the date specified herein. The Administrative Agent will promptly distribute to each Lender its Pro Rata Share (or other applicable share as provided herein) of such payment in like funds as received by wire transfer to such Lender’s Applicable Lending Office. All payments received by the Administrative Agent after 2:00 p.m. (New York, New York time) shall be deemed received on the next succeeding Business Day and any applicable interest or fee shall continue to accrue.

(b)          If any payment to be made by the Borrower shall come due on a day other than a Business Day, payment shall be made on the next following Business Day, and such extension of time shall be reflected in computing interest or fees, as the case may be.

(c)          If any Lender makes available to the Administrative Agent funds for any Loan to be made by such Lender as provided in the foregoing provisions of this Article II, and such funds are not made available to the Borrower by the Administrative Agent because the conditions (if any) to the Loan set forth in Article IV are not satisfied or waived in accordance

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with the terms hereof, the Administrative Agent shall return such funds (in like funds as received from such Lender) to such Lender, without interest.

(d)         The obligations of the Lenders hereunder to make Loans are several and not joint. The failure of any Lender to make any Loan on any date required hereunder shall not relieve any other Lender of its corresponding obligation to do so on such date, and neither the Administrative Agent nor any Lender shall be responsible for the failure of any other Lender to make its Loan.

(e)          Nothing herein shall be deemed to obligate any Lender to obtain the funds for any Loan in any particular place or manner or to constitute a representation by any Lender that it has obtained or will obtain the funds for any Loan in any particular place or manner.

(f)           Whenever any payment received by the Administrative Agent under this Agreement or any of the other Loan Documents is insufficient to pay in full all amounts due and payable to the Administrative Agent and the Lenders under or in respect of this Agreement and the other Loan Documents on any date, such payment shall be distributed by the Administrative Agent and applied by the Administrative Agent and the Lenders in the order of priority set forth in Section 8.03. If the Administrative Agent receives funds for application to the Obligations of the Loan Parties under or in respect of the Loan Documents under circumstances for which the Loan Documents do not specify the manner in which such funds are to be applied, the Administrative Agent may, but shall not be obligated to, elect to distribute such funds to each of the Lenders in accordance with such Lender’s Pro Rata Share of the aggregate principal amount of all Loans outstanding at such time.

Section 2.09 Sharing of Payments. If, other than as expressly provided elsewhere herein (including, without limitation, pursuant to Section 2.02(b)(vi) or in Section 10.07), any Lender shall obtain on account of the Loans made by it in excess of its ratable share (or other share contemplated hereunder subject to the priorities set forth herein) thereof, such Lender shall immediately (a) notify the Administrative Agent of such fact and (b) purchase from the other Lenders such participations in the Loans made by them as shall be necessary to cause such purchasing Lender to share the excess payment in respect of such Loans pro rata with each of them; provided that if all or any portion of such excess payment is thereafter recovered from the purchasing Lender under any of the circumstances described in Section 10.06 (including pursuant to any settlement entered into by the purchasing Lender in its discretion), such purchase shall to that extent be rescinded and each other Lender shall repay to the purchasing Lender the purchase price paid therefor, together with an amount equal to such paying Lender’s ratable share (according to the proportion of (i) the amount of such paying Lender’s required repayment to (ii) the total amount so recovered from the purchasing Lender) of any interest or other amount paid or payable by the purchasing Lender in respect of the total amount so recovered, without further interest thereon. The Borrower agrees that any Lender so purchasing a participation from another Lender may, to the fullest extent permitted by applicable Law, exercise all its rights of payment (including the right of setoff, but subject to Section 10.09) with respect to such participation as fully as if such Lender were the direct creditor of the Borrower in the amount of such participation. The Administrative Agent will keep records (which shall be conclusive and binding in the absence of manifest error) of participations purchased under this Section 2.09 and will in each case notify the Lenders following any such

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purchases or repayments. Each Lender that purchases a participation pursuant to this Section 2.09 shall from and after such purchase have the right to give all notices, requests, demands, directions and other communications under this Agreement with respect to the portion of the Obligations purchased to the same extent as though the purchasing Lender were the original owner of the Obligations purchased.

ARTICLE III

TAXES, INCREASED COSTS PROTECTION AND ILLEGALITY

Section 3.01 Taxes.

(a)          Defined Terms. For purposes of this Section 3.01, the term “applicable law” includes FATCA.

(b)          Payments Free of Taxes. Any and all payments by or on account of any obligation of any Loan Party under any Loan Document shall be made without deduction or withholding for any Taxes, except as required by applicable law. If any applicable law (as determined in the good faith discretion of an applicable Withholding Agent) requires the deduction or withholding of any Tax from any such payment by a Withholding Agent, then the applicable Withholding Agent shall be entitled to make such deduction or withholding and shall timely pay the full amount deducted or withheld to the relevant Governmental Authority in accordance with applicable law and, if such Tax is an Indemnified Tax, then the sum payable by the applicable Loan Party shall be increased as necessary so that after such deduction or withholding has been made (including such deductions and withholdings applicable to additional sums payable under this Section) the applicable Recipient receives an amount equal to the sum it would have received had no such deduction or withholding been made.

(c)          Payment of Other Taxes by the Loan Parties. The Loan Parties shall timely pay to the relevant Governmental Authority in accordance with applicable law, or at the option of the Administrative Agent timely reimburse it for the payment of, any Other Taxes.

(d)         Indemnification by the Loan Parties. The Loan Parties shall jointly and severally indemnify each Recipient, within 10 days after demand therefor, for the full amount of any Indemnified Taxes (including Indemnified Taxes imposed or asserted on or attributable to amounts payable under this Section) payable or paid by such Recipient or required to be withheld or deducted from a payment to such Recipient and any reasonable expenses arising therefrom or with respect thereto, whether or not such Indemnified Taxes were correctly or legally imposed or asserted by the relevant Governmental Authority. A certificate as to the amount of such payment or liability delivered to the Borrower by a Lender (with a copy to the Administrative Agent), or by the Administrative Agent on its own behalf, shall be conclusive absent manifest error.

(e)          Indemnification by the Lenders. Each Lender shall severally indemnify the Administrative Agent, within 10 days after demand therefor, for (i) any Indemnified Taxes attributable to such Lender (but only to the extent that any Loan Party has not already indemnified the Administrative Agent for such Indemnified Taxes and without limiting the

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obligation of the Loan Parties to do so), (ii) any Taxes attributable to such Lender’s failure to comply with the provisions of Section 10.07(c) relating to the maintenance of a Participant Register and (iii) any Excluded Taxes attributable to such Lender, in each case, that are payable or paid by the Administrative Agent in connection with any Loan Document, and any reasonable expenses arising therefrom or with respect thereto, whether or not such Taxes were correctly or legally imposed or asserted by the relevant Governmental Authority. A certificate as to the amount of such payment or liability delivered to any Lender by the Administrative Agent shall be conclusive absent manifest error. Each Lender hereby authorizes the Administrative Agent to set off and apply any and all amounts at any time owing to such Lender under any Loan Document or otherwise payable by the Administrative Agent to the Lender from any other source against any amount due to the Administrative Agent under this paragraph (e).

(f)           Evidence of Payments. As soon as practicable after any payment of Taxes by any Loan Party to a Governmental Authority pursuant to this Section 3.01, such Loan Party shall deliver to the Administrative Agent the original or a certified copy of a receipt issued by such Governmental Authority evidencing such payment, a copy of the return reporting such payment or other evidence of such payment reasonably satisfactory to the Administrative Agent.

(g)         Status of Lenders. (i) Any Lender that is entitled to an exemption from or reduction of withholding Tax with respect to payments made under any Loan Document shall deliver to the Borrower and the Administrative Agent, at the time or times reasonably requested by the Borrower or the Administrative Agent, such properly completed and executed documentation reasonably requested by the Borrower or the Administrative Agent as will permit such payments to be made without withholding or at a reduced rate of withholding. In addition, any Lender, if reasonably requested by the Borrower or the Administrative Agent, shall deliver such other documentation prescribed by applicable law or reasonably requested by the Borrower or the Administrative Agent as will enable the Borrower or the Administrative Agent to determine whether or not such Lender is subject to backup withholding or information reporting requirements. Notwithstanding anything to the contrary in the preceding two sentences, the completion, execution and submission of such documentation (other than such documentation set forth in Section 3.01 (g)(ii)(A), (ii)(B) and (ii)(D) below) shall not be required if in the Lender’s reasonable judgment such completion, execution or submission would subject such Lender to any material unreimbursed cost or expense or would materially prejudice the legal or commercial position of such Lender.

(ii)          Without limiting the generality of the foregoing, in the event that the Borrower is a U.S. Borrower,

(A)       any Lender that is a U.S. Person shall deliver to the Borrower and the Administrative Agent on or prior to the date on which such Lender becomes a Lender under this Agreement (and from time to time thereafter upon the reasonable request of the Borrower or the Administrative Agent), executed copies of IRS Form W-9 certifying that such Lender is exempt from U.S. federal backup withholding tax;

(B)       any Foreign Lender shall, to the extent it is legally entitled to do so, deliver to the Borrower and the Administrative Agent (in such number of

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copies as shall be requested by the recipient) on or prior to the date on which such Foreign Lender becomes a Lender under this Agreement (and from time to time thereafter upon the reasonable request of the Borrower or the Administrative Agent), whichever of the following is applicable:

(1)            in the case of a Foreign Lender claiming the benefits of an income tax treaty to which the United States is a party (x) with respect to payments of interest under any Loan Document, executed copies of IRS Form W-8BEN (or W-8BEN-E, as applicable) establishing an exemption from, or reduction of, U.S. federal withholding Tax pursuant to the “interest” article of such tax treaty and (y) with respect to any other applicable payments under any Loan Document, IRS Form W-8BEN (or W-8BEN-E, as applicable) establishing an exemption from, or reduction of, U.S. federal withholding Tax pursuant to the “business profits” or “other income” article of such tax treaty;

(2)            executed copies of IRS Form W-8ECI;

(3)            in the case of a Foreign Lender claiming the benefits of the exemption for portfolio interest under Section 881(c) of the Code, (x) a certificate substantially in the form of Exhibit H-l to the effect that such Foreign Lender is not a “bank” within the meaning of Section 881(c)(3)(A) of the Code, a “10 percent shareholder” of the Borrower within the meaning of Section 881(c)(3)(B) of the Code, or a “controlled foreign corporation” described in Section 881(c)(3)(C) of the Code (a “U.S. Tax Compliance Certificate”) and (y) executed copies of IRS Form W-8BEN (or W-8BEN-E, as applicable); or

(4)            to the extent a Foreign Lender is not the beneficial owner, executed copies of IRS Form W-8IMY, accompanied by IRS Form W-8ECI, IRS Form W-8BEN (or W-8BEN-E, as applicable), a U.S. Tax Compliance Certificate substantially in the form of Exhibit H-2 or Exhibit H-3 , IRS Form W-9, and/or other certification documents from each beneficial owner, as applicable; provided that if the Foreign Lender is a partnership and one or more direct or indirect partners of such Foreign Lender are claiming the portfolio interest exemption, such Foreign Lender may provide a U.S. Tax Compliance Certificate substantially in the form of Exhibit H-4 on behalf of each such direct and indirect partner;

(C)           any Foreign Lender shall, to the extent it is legally entitled to do so, deliver to the Borrower and the Administrative Agent (in such number of copies as shall be requested by the recipient) on or prior to the date on which such Foreign Lender becomes a Lender under this Agreement (and from time to time thereafter upon the reasonable request of the Borrower or the Administrative Agent), executed copies of any other form prescribed by applicable law as a basis for claiming exemption from or a reduction in U.S. federal withholding Tax, duly completed, together with such supplementary documentation as may be

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prescribed by applicable law to permit the Borrower or the Administrative Agent to determine the withholding or deduction required to be made; and

(D)          if a payment made to a Lender under any Loan Document would be subject to U.S. federal withholding Tax imposed by FATCA if such Lender were to fail to comply with the applicable reporting requirements of FATCA (including those contained in Section 1471(b) or 1472(b) of the Code, as applicable), such Lender shall deliver to the Borrower and the Administrative Agent at the time or times prescribed by law and at such time or times reasonably requested by the Borrower or the Administrative Agent such documentation prescribed by applicable law (including as prescribed by Section 1471 (b)(3)(C)(i) of the Code) and such additional documentation reasonably requested by the Borrower or the Administrative Agent as may be necessary for the Borrower and the Administrative Agent to comply with their obligations under FATCA and to determine that such Lender has complied with such Lender’s obligations under FATCA or to determine the amount to deduct and withhold from such payment. Solely for purposes of this clause (D), “FATCA” shall include any amendments made to FATCA after the date of this Agreement.

Each Lender agrees that if any form or certification it previously delivered expires or becomes obsolete or inaccurate in any respect, it shall update such form or certification or promptly notify the Borrower and the Administrative Agent in writing of its legal inability to do so.

(h)          Treatment of Certain Refunds. If any party determines, in its sole discretion exercised in good faith, that it has received a refund of any Taxes as to which it has been indemnified pursuant to this Section 3.01 (including by the payment of additional amounts pursuant to this Section 3.01), it shall pay to the indemnifying party an amount equal to such refund (but only to the extent of indemnity payments made under this Section with respect to the Taxes giving rise to such refund), net of all out-of-pocket expenses (including Taxes) of such indemnified party and without interest (other than any interest paid by the relevant Governmental Authority with respect to such refund). Such indemnifying party, upon the request of such indemnified party, shall repay to such indemnified party the amount paid over pursuant to this paragraph (h) (plus any penalties, interest or other charges imposed by the relevant Governmental Authority) in the event that such indemnified party is required to repay such refund to such Governmental Authority. Notwithstanding anything to the contrary in this paragraph (h), in no event will the indemnified party be required to pay any amount to an indemnifying party pursuant to this paragraph (h) the payment of which would place the indemnified party in a less favorable net after-Tax position than the indemnified party would have been in if the Tax subject to indemnification and giving rise to such refund had not been deducted, withheld or otherwise imposed and the indemnification payments or additional amounts with respect to such Tax had never been paid. This paragraph shall not be construed to require any indemnified party to make available its Tax returns (or any other information relating to its Taxes that it deems confidential) to the indemnifying party or any other Person.

(i)           Survival. Each party’s obligations under this Section 3.01 shall survive the resignation or replacement of the Administrative Agent or any assignment of rights by, or the

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replacement of, a Lender, the termination of the Commitments and the repayment, satisfaction or discharge of all obligations under any Loan Document.

Section 3.02 Increased Cost and Reduced Return; Capital and Liquidity Requirements.

(a)          Increased Costs Generally. If any Change in Law shall:

(i)         impose, modify or deem applicable any reserve, special deposit, compulsory loan, insurance charge or similar requirement against assets of, deposits with or for the account of, or credit extended or participated in by, any Lender;

(ii)        subject any Recipient to any Taxes (other than (A) Indemnified Taxes, (B) Taxes described in clauses (b) through (d) of the definition of “Excluded Taxes” and (C) Connection Income Taxes) on its loans, loan principal, letters of credit, commitments, or other obligations, or its deposits, reserves, other liabilities or capital attributable thereto; or

(iii)        impose on any Lender any other condition, cost or expense (other than Taxes) affecting this Agreement or Loans made by such Lender;

and the result of any of the foregoing shall be to increase the cost to such Lender or such other Recipient of making, converting to, continuing or maintaining any Loan or of maintaining its obligation to make any such Loan, or to reduce the amount of any sum received or receivable by such Lender or other Recipient hereunder (whether of principal, interest or any other amount) then, upon request of such Lender or other Recipient, the Borrower will pay to such Lender or other Recipient, as the case may be, such additional amount or amounts as will compensate such Lender or other Recipient, as the case may be, for such additional costs incurred or reduction suffered.

(b)          Capital Requirements. If any Lender reasonably determines that any Change in Law affecting such Lender or any lending office of such Lender or such Lender’s holding company, if any, regarding capital or liquidity requirements, has or would have the effect of reducing the rate of return on such Lender’s capital or on the capital of such Lender’s holding company, if any, as a consequence of this Agreement, the Commitments of such Lender or the Loans made by such Lender to a level below that which such Lender or such Lender’s holding company could have achieved but for such Change in Law (taking into consideration such Lender’s policies and the policies of such Lender’s holding company with respect to capital adequacy), then from time to time the Borrower will pay to such Lender such additional amount or amounts as will compensate such Lender or such Lender’s holding company for any such reduction suffered.

(c)          Certificates for Reimbursement. A certificate of a Lender setting forth the amount or amounts necessary to compensate such Lender or its holding company, as the case may be, as specified in paragraph (a) or (b) of this Section and delivered to the Borrower, shall be conclusive absent manifest error. The Borrower shall pay such Lender the amount shown as due on any such certificate within 10 days after receipt thereof.

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(d)          Delay in Requests. Failure or delay on the part of any Lender to demand compensation pursuant to this Section 3.02 shall not constitute a waiver of such Lender’s right to demand such compensation; provided that the Borrower shall not be required to compensate a Lender pursuant to this Section for any increased costs incurred or reductions suffered more than nine months prior to the date that such Lender notifies the Borrower of the Change in Law giving rise to such increased costs or reductions, and of such Lender’s intention to claim compensation therefor (except that, if the Change in Law giving rise to such increased costs or reductions is retroactive, then the nine-month period referred to above shall be extended to include the period of retroactive effect thereof).

Section 3.03 Matters Applicable to All Requests for Compensation. The Administrative Agent or any Lender claiming compensation under this Article III shall deliver a certificate to the Borrower setting forth the additional amount or amounts to be paid to it hereunder, which shall be conclusive absent manifest error. In determining such amount, the Administrative Agent or such Lender may use any reasonable averaging and attribution methods.

Section 3.04 Mitigation Obligations; Replacement of Lenders under Certain Circumstances.

(a)          Designation of a Different Applicable Lending Office. If any Lender requests compensation under Section 3.02, or requires the Borrower to pay any Indemnified Taxes or additional amounts to any Lender or any Governmental Authority for the account of any Lender pursuant to Section 3.01, then such Lender shall (at the request of the Borrower) use reasonable efforts to designate a different Applicable Lending Office for funding or booking its Loans hereunder or to assign its rights and obligations hereunder to another of its offices, branches or affiliates, if, in the judgment of such Lender, such designation or assignment (i) would eliminate or reduce amounts payable pursuant to Section 3.02 or Section 3.01, as the case may be, in the future, and (ii) would not subject such Lender to any unreimbursed cost or expense and would not otherwise be disadvantageous to such Lender. The Borrower hereby agrees to pay all reasonable and documented costs and expenses incurred by any Lender in connection with any such designation or assignment.

(b)          Replacement of Lenders. If any Lender requests compensation under Section 3.02, or if the Borrower is required to pay any Indemnified Taxes or additional amounts to any Lender or any Governmental Authority for the account of any Lender pursuant to Section 3.01 and, in each case, such Lender has declined or is unable to designate a different Applicable Lending Office in accordance with Section 3.04(a), or if any Lender is a Non-Consenting Lender, then the Borrower may, at its sole expense and effort, upon notice to such Lender and the Administrative Agent, require such Lender to assign and delegate, without recourse (in accordance with and subject to the restrictions contained in, and consents required by, Section 10.07(b)), all of its interests, rights (other than its existing rights to payments pursuant to Section 3.02 or Section 3.01) and obligations under this Agreement and the related Loan Documents to an Eligible Assignee that shall assume such obligations (which assignee may be another Lender, if a Lender accepts such assignment); provided that:

(iv)          the Borrower shall have paid to the Administrative Agent the assignment fee (if any) specified in Section 10.07(b);

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(v)          such Lender shall have received payment of an amount equal to the outstanding principal of its Loans, accrued interest thereon, accrued fees and all other amounts payable to it hereunder and under the other Loan Documents from the assignee (to the extent of such outstanding principal and accrued interest and fees) or the Borrower (in the case of all other amounts);

(vi)          in the case of any such assignment resulting from a claim for compensation under Section 3.02 or payments required to be made pursuant to Section 3.01, such assignment will result in a reduction in such compensation or payments thereafter;

(vii)         such assignment does not conflict with applicable law; and

(viii)        in the case of any assignment resulting from a Lender becoming a Non-Consenting Lender, the applicable assignee shall have consented to the applicable amendment, waiver or consent.

A Lender shall not be required to make any such assignment or delegation if, prior thereto, as a result of a waiver by such Lender or otherwise, the circumstances entitling the Borrower to require such assignment and delegation cease to apply.

(c)          In the event that (i) the Borrower or the Administrative Agent has requested that the Lenders consent to a departure or waiver of any provisions of the Loan Documents or agree to any amendment thereto, (ii) the consent, waiver or amendment in question requires the agreement of all affected Lenders in accordance with the terms of Section 10.01 or all the Lenders and (iii) the Required Lenders have agreed to such consent, waiver or amendment, then any Lender who does not agree to such consent, waiver or amendment shall be deemed a “Non-Consenting Lender”.

Section 3.05 Survival. All of the Borrower’s obligations under this Article III shall survive termination of the Aggregate Commitments and repayment of all other Obligations hereunder.

ARTICLE IV

CONDITIONS PRECEDENT TO LOANS

Section 4.01 Conditions to Loans. The obligation of each Lender to make its Loans hereunder is subject to satisfaction of the following conditions precedent, except as otherwise agreed between the Borrower, the Administrative Agent and the Required Lenders:

(a)          The Administrative Agent’s or the Lenders’ (as applicable) receipt of the following, each properly executed by a Responsible Officer of the signing Loan Party, and each in form and substance reasonably satisfactory to the Required Lenders:

(i)          executed counterparts of this Agreement, the Guarantee and Collateral Agreement, the Pledge Agreement, the UK Debenture, the UK Share

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Charge 1, the Irish Security Documents and each other Loan Document to be entered into as of the Closing Date by each party thereto;

(ii)          an original Note executed by the Borrower in favor of each Lender that has requested a Note;

(iii)         a Committed Loan Notice relating to the Loans; and

(iv)          the certificates, documents, instruments, agreements, legal opinions and deliverables set forth on the Closing Checklist attached hereto as Schedule 1.

(b)          With respect to the Chapter 11 Case, there shall have been entered a final order confirming the Reorganization Plan in form and substance reasonably satisfactory to the Lenders, and the “Effective Date” as set forth in the Reorganization Plan shall have occurred prior to or simultaneously with the Closing Date and all conditions precedent to the occurrence of such Effective Date shall have been satisfied (or waived by the Lenders).

(c)          The Borrower shall have issued Convertible Notes in the amount of $125,000,000 in exchange for (a) the Specified Portion (as hereinafter defined) of the Existing Convertible Notes and all other unsecured; indebtedness and liabilities of the Borrower (other than Department of Justice and other government related payments and trade claims, which will remain unimpaired), which shall be allocated among the applicable creditors on a ratable basis based on the respective principal or face amount of their respective unsecured claims, (b) the “Roll Up Loans” under (and as defined in) the Existing Bridge Credit Agreement (plus accrued fees and interest thereon), which shall receive such Convertible Notes on a dollar for dollar basis in full satisfaction of the applicable creditors’ claims, and (c) the aggregate amount of obligations under the Existing DIP Credit Agreement on a dollar for dollar basis that are not repaid in cash on the effective date of the Reorganization Plan. As used herein, the term “Specified Portion” means an amount equal to (i) $102,000,000 minus (ii) the aggregate amount of obligations under the Existing DIP Credit Agreement that are not repaid in cash on the effective date of the Reorganization Plan.

(d)          The $42,000,000 rights offering to the creditors of the Borrower and the $18,000,000 equity offering to certain current or future shareholders of the Parent contemplated by the Backstop Subscription Agreement shall have been consummated or the Backstop Subscription Agreement shall remain in full force and effect.

(e)          The acquisition of the Borrower and its Subsidiaries by the Parent or one of its Subsidiaries (the “Acquisition”) shall have been completed on the terms set forth in the Plan Funding Agreement and all other documentation relating thereto, in each case in form and substance reasonably satisfactory to the Lenders. All conditions precedent to the Acquisition shall have been met (or waived with the consent of the Lenders) and the Acquisition shall have been consummated in accordance with the terms of the Plan Funding Agreement and such other documentation (without any amendment, modification or waiver of any of the provisions thereof that would be materially adverse to the Lenders without the consent of the Lenders) and all requirements of law.

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(f)           [Reserved].

(g)          The Lenders shall have received at least three (3) Business Days prior to the Closing Date all documentation and other information required by regulatory authorities under applicable “know your customer” and anti-money laundering rules and regulations, including without limitation the USA PATRIOT Act, in order to allow the Lenders to comply therewith, in each case, to the extent requested at least ten (10) Business Days prior to the Closing Date.

(h)          The Administrative Agent shall have received a certificate signed by a Responsible Officer of the Borrower in substantially the form of Exhibit F certifying that (i) the representations and warranties of the Borrower and each other Loan Party contained in Article V or any other Loan Document shall be true and correct in all material respects on and as of the Closing Date (before and after giving effect to the incurrence of the Loans); provided that to the extent that such representations and warranties specifically refer to an earlier date, they shall be true and correct in all material respects as of such earlier date; provided further that any representation and warranty that is qualified as to “materiality”, “Material Adverse Effect” or similar language shall be true and correct (after giving effect to any qualification therein) in all respects on such respective dates and (ii) no Default or Event of Default shall exist, or would result from the incurrence of the Loans or from the application of the proceeds therefrom.

(i)          There shall be no order, injunction or decree of any Governmental authority restraining or prohibiting the funding or conversion, as applicable, of the Loans on the Closing Date.

(j)          The Lenders shall have received a customary payoff letter and lien termination statements and lien release documents (or similar documents), in each case, reasonably satisfactory to the Lenders, with respect to the Amryt Finance Contract and the obligations thereunder, and upon funding of the New Money Loans and the application of such proceeds (in accordance with the steps plan disclosed the Lenders) to the obligations under the Amryt Finance Contract, such obligations shall be paid in full and all liens securing such obligations shall be terminated within one (1) Business Day of the Closing Date.

Without limiting the generality of the provisions of Section 9.03, for purposes of determining compliance with the conditions specified in this Section 4.01, each Lender that has signed this Agreement shall be deemed to have consented to, approved or accepted or to be satisfied with, each document or other matter required thereunder to be consented to or approved by or acceptable or satisfactory to a Lender unless the Administrative Agent shall have received notice from such Lender prior to the Closing Date specifying its objection thereto.

ARTICLE V

REPRESENTATIONS AND WARRANTIES

Each of the Parent and the Borrower represents and warrants to the Administrative Agent and the Lenders that:

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Section 5.01 Existence, Qualification and Power; Compliance with Laws. Except as set forth on Schedule 5.01 or, in the case of clause (d), Schedule 5.06, each Loan Party and each of its Subsidiaries (a) is duly incorporated, organized or formed, and validly existing and in good standing under the Laws of the jurisdiction of its incorporation or organization (to the extent such concept exists in such jurisdiction), (b) has all requisite power and authority to (i) own or lease its assets and carry on its business and (ii) execute, deliver and perform its obligations under the Loan Documents to which it is a party, (c) is duly qualified and in good standing (to the extent such concept exists) under the Laws of each jurisdiction where its ownership, lease or operation of properties or the conduct of its business requires such qualification, (d) is in compliance with all Laws (including, without limitation, Regulation X of the FRB), orders, writs, injunctions and orders and (e) has all requisite governmental licenses, authorizations, consents and approvals to operate its business as currently conducted, except, with respect to the foregoing clauses (c), (d) and (e), as would not, individually or in the aggregate, be reasonably likely to have a Material Adverse Effect.

Section 5.02 Authorization; No Contravention. The execution, delivery and performance by each Loan Party of each Loan Document to which such Person is a party, and the consummation of the Transaction, (a) are within such Loan Party’s corporate or other powers, (b) have been duly authorized by all necessary corporate or other organizational action, and (c) do not and will not (i) contravene the terms of any of such Person’s Organization Documents, (ii) except as set forth on Schedule 5.02, conflict with or result in any breach or contravention of, or the creation of any Lien under (other than as permitted by Section 7.01), or require any payment to be made under (x) any Material Contracts to which such Person is a party or affecting such Person or the properties of such Person or any of its Subsidiaries or (y) any material order, injunction, writ or decree of any Governmental Authority or any arbitral award to which such Person or its property is subject, or (iii) violate any material applicable Law.

Section 5.03 Governmental Authorization; Other Consents. No approval, consent, exemption, authorization, or other action by, or notice to, or filing with, any Governmental Authority or any other Person is necessary or required in connection with (a) the execution, delivery or performance by, or enforcement against, any Loan Party of this Agreement or any other Loan Document, (b) the grant by any Loan Party of the Liens granted by it pursuant to the Collateral Documents, (c) the perfection or maintenance of the Liens created under the Collateral Documents (including the priority thereof) or (d) the exercise by the Administrative Agent or any Lender of its rights under the Loan Documents or the remedies in respect of the Collateral pursuant to the Collateral Documents, in each case, other than (a) those that have been duly obtained or made and which are in full force and effect, (b) the filing of UCC financing statements and (c) the filings or other actions necessary to perfect Liens under the Loan Documents.

Section 5.04 Binding Effect. This Agreement and each other Loan Document has been duly executed and delivered by each Loan Party that is party thereto. This Agreement and each other Loan Document constitutes a legal, valid and binding obligation of such Loan Party, enforceable against each Loan Party that is party thereto in accordance with its terms, subject to the effects of bankruptcy, insolvency, fraudulent conveyance, moratorium, reorganization and other similar laws relating to or affecting creditors’ rights generally.

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Section 5.05 [Reserved].

Section 5.06 Litigation. Except for claims, actions, suits, investigations, litigation or proceeding and set forth on Schedule 5.06, there is no action, suit, investigation, litigation or proceeding affecting any Loan Party or its Subsidiaries, including any Environmental Action, pending or, to the knowledge of any Loan Party, threatened in writing before any Governmental Authority or arbitrator that (i) would be reasonably likely to result in liabilities in excess of the Threshold Amount or (ii) purports to affect the legality, validity or enforceability of any Loan Document.

Section 5.07 Ownership of Property; Liens. (a) Each Loan Party and its Subsidiaries is the legal and beneficial owner of the Collateral pledged by it free and clear of any Lien, except for Permitted Liens.

(b)          Each Loan Party and each of its Subsidiaries has good and marketable title in fee simple to, or valid leasehold interests in, or easements or other limited property interests in, all real property used in the ordinary conduct of its business, free and clear of all Liens except for defects in title that do not materially interfere with its ability to conduct its business or to utilize such assets for their intended purposes and Liens permitted by Section 7.01 and except where the failure to have such title or other interest would not reasonably be expected to have a Material Adverse Effect. Set forth as Schedule 5.07(b) hereto is a complete and accurate list of all real property owned by any Loan Party or any of its Subsidiaries, showing, as of the date hereof, the street address, state and any other relevant jurisdiction, record owner and fair market value. Set forth on Schedule 5.07(b) hereto is a complete and accurate list of all leases of real property under which any Loan Party or any Subsidiary is the tenant, showing as of the date hereof the street address, state and any other relevant jurisdiction, parties thereto, sublessee (if any), expiration date and annual base rental cost thereof.

Section 5.08 Enforceable Obligations. The provisions of the Collateral Documents are effective to create in favor of the Administrative Agent for the benefit of the Secured Parties, a legal, valid and enforceable fixed and floating charges and first priority Lien, as applicable in any relevant jurisdiction, or security interest in all right, title and interest of the Loan Parties in the Collateral and all proceeds thereof and in the case of any UK Collateral, upon the timely and proper filing of the related Collateral Documents, any relevant additional security documents and the security interests created by it or them with Companies House, the Administrative Agent, for the benefit of the Secured Parties, has a fully perfected security interest in all right, title and interest in all of the UK Collateral, subject to no other Liens other than Permitted Liens, in each case, to the extent perfection can be accomplished under applicable law through these actions.

Section 5.09 Environmental Compliance. Except as set forth on Schedule 5.09 or as would not individually be reasonably expected to result in a liability in excess of the Threshold Amount to the Loan Parties and their Subsidiaries (provided that the aggregate of all such events, circumstances, developments and liabilities could not reasonably be expected to result in a Material Adverse Effect):

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(a)          The operations and properties of each Loan Party and each of its Subsidiaries comply in all material respects with all applicable Environmental Laws and Environmental Permits, all past non-compliance with such Environmental Laws and Environmental Permits has been resolved without ongoing obligations or costs, and no circumstances exist that would be reasonably likely to (A) to the knowledge of the Loan Parties, form the basis of an Environmental Action against any Loan Party or any Subsidiary or any of their properties or (B) cause any such property to be subject to any restrictions on ownership, occupancy, use or transferability under any Environmental Law.

(b)          None of the properties currently or, to the knowledge of the Loan Parties, formerly, owned or operated by any Loan Party or any of its Subsidiaries is listed or, to such Loan Party’s or each of its Subsidiaries’ knowledge, proposed for listing on the NPL or on the CERCLIS or any analogous foreign, state or local list or is adjacent to any such property; there are no, and, to the knowledge of the Loan Parties, never have been, any underground or aboveground storage tanks other than in compliance with applicable Environmental Laws or any surface impoundments, septic tanks, pits, sumps or lagoons in which Hazardous Materials are being or have been treated, stored or disposed on any property currently owned or operated by any Loan Party or any of its Subsidiaries or, to the best of its knowledge, on any property formerly owned or operated by any Loan Party or any of its Subsidiaries other than in compliance with applicable Environmental Laws; and other than in compliance with applicable Environmental Laws, there is no asbestos or asbestos-containing material on any property currently owned or operated by any Loan Party or any of its Subsidiaries; and Hazardous Materials have not been released, discharged or disposed of by any Loan Party or any of its Subsidiaries on any property currently or formerly owned or operated by any Loan Party or any of its Subsidiaries other than in material compliance with applicable Environmental Laws.

(c)          Neither any Loan Party nor any of its Subsidiaries is undertaking, and has not completed, either individually or together with other potentially responsible parties, any investigation or assessment or remedial or response action relating to any actual or threatened release, discharge or disposal of Hazardous Materials at any site, location or operation, either voluntarily or pursuant to the order of any governmental or regulatory authority or the requirements of any Environmental Law; and all Hazardous Materials generated, used, treated, handled or stored at, or transported by or on behalf of any Loan Party or any of its Subsidiaries to or from, any property currently or formerly owned or operated by any Loan Party or any of its Subsidiaries have, to the knowledge of the Loan Parties, been disposed of in a manner not reasonably expected to result in material liability to any Loan Party or any of its Subsidiaries.

(d)          Each Loan Party and each of its Subsidiaries has obtained all material Environmental Permits required for ownership and operation of its property and business as presently conducted. No Loan Party nor any of its Subsidiaries has received any written notification pursuant to any applicable Environmental Law or otherwise has knowledge that (A) any work, repairs, construction or capital expenditures are required to be made in order to be in or continue to be in compliance with any applicable Environmental Laws or any material Environmental Permit or (B) any Environmental

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Permit is about to be reviewed, made subject to new limitations or conditions, revoked, withdrawn or terminated.

(e)          Except as would not reasonably be expected to result in a material liability, no Loan Party nor any of its Subsidiaries has contractually assumed any liability or obligation under or relating to any applicable Environmental Law.

(f)          Nothing contained in this Section 5.09 is intended to apply to any action, suit, investigation, litigation or proceeding (including any Environmental Action) relating to exposure to asbestos, in any form, or any asbestos containing materials.

Section 5.10 Taxes. (a) Each of the Loan Parties and each of their respective Subsidiaries has timely filed all income and all other material tax returns and reports required to be filed, and have timely paid all Taxes (whether or not shown on such tax returns or reports) and all other amounts of federal, provincial, state, municipal, foreign and other taxes, assessments, fees and other governmental charges levied or imposed upon them or their properties, income or assets otherwise due and payable, except those which are set forth on Schedule 5.10 or are being contested in good faith by appropriate proceedings and for which adequate reserves have been provided in accordance with IFRS.

(b)          Except as set forth on Schedule 5.10 or as would not, individually or in the aggregate, be reasonably likely to result in any material liability, (i) there are no claims being asserted in writing with respect to any amounts of taxes, (ii) there are no presently effective waivers or extensions of statutes in writing with respect to any amounts of taxes, and (iii) no tax returns are being examined by, and no written notification of intention to examine has been received from, the Internal Revenue Service or any other taxing authority, in each case, with respect to the Loan Parties or any of their respective Subsidiaries.

(c)          No Loan Party nor any of its Subsidiaries is party to any tax sharing agreement other than with an affiliate included in a consolidated or combined tax return.

Section 5.11 Compliance with ERISA. (a) Each Plan is in compliance with the applicable provisions of ERISA, the Code and other federal or state Laws, except as is not, either individually or in the aggregate, reasonably likely to have a Material Adverse Effect.

(b)          (i) No ERISA Event has occurred or is reasonably expected to occur; (ii) none of the Loan Parties or any of their Subsidiaries has incurred, or reasonably expects to incur, any liability (and no event has occurred which, with the giving of notice under Section 4219 of ERISA, would result in such liability) under Section 4201 et seq. or 4243 of ERISA with respect to a Multiemployer Plan; and (iii) none of the Loan Parties or any of their Subsidiaries or any ERISA Affiliate has engaged in a transaction that would be subject to Section 4069 or 4212(c) of ERISA.

Section 5.12 Labor Matters. There are no strikes pending or, to the knowledge of any Loan Party, threatened in writing against any Loan Party or any of its Subsidiaries that, individually or in the aggregate, would reasonably be expected to have a Material Adverse Effect. The (i) hours worked and payments made to employees of any Loan Party or any of its Subsidiaries have not been in violation in any material respect of the Fair Labor Standards Act or

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any other applicable law dealing with such matters and (ii) all material payments due from any Loan Party or any of its Subsidiaries or for which any claim may be made against any Loan Party or any of its Subsidiaries, on account of wages and employee health and welfare insurance and other benefits have been paid or accrued as a liability on the books of such Loan Party or such Subsidiary to the extent required by IFRS.

Section 5.13 Insurance. The properties of the Loan Parties and their Subsidiaries are insured in the manner contemplated by Section 6.07.

Section 5.14 Subsidiaries; Equity Interests. As of the date hereof, the Loan Parties do not have any Subsidiaries other than those specifically disclosed in Schedule 5.14, and all of the outstanding Equity Interests in each such Person and each such Subsidiary have been validly issued, are fully paid and non-assessable. As of the date hereof, Schedule 5.14 (a) sets forth the name and ownership interest of each Person that owns any Equity Interests in the direct and indirect Subsidiaries of the Parent, (b) sets forth the name and jurisdiction of organization of the Parent and each direct and indirect Subsidiary of the Parent and (c) sets forth the ownership interest of each direct and indirect Subsidiary of the Parent, including the percentage of such ownership.

Section 5.15 Margin Regulations; Investment Company Act; Anti-Terrorism Laws; Sanctions and Other Regulations. (a) None of the Loan Parties or any of their Subsidiaries is engaged nor will engage, principally or as one of its important activities, in the business of purchasing or carrying margin stock (within the meaning of Regulation U issued by the FRB), or extending credit for the purpose of purchasing or carrying margin stock, and no proceeds of any Loans will be used for any purpose that violates Regulation U issued by the FRB.

(b)          None of the Loan Parties or any of their Subsidiaries is required to be registered as an “investment company” under the Investment Company Act of 1940, as amended.

(c)          No Loan Party nor any of its Subsidiaries or to its knowledge any of the respective officers, directors, brokers or agents of such Loan Party or Subsidiary has violated any applicable Anti-Terrorism Law in any material respect.

(d)          No Loan Party, nor any of its Subsidiaries, any of their respective directors, officers or employees, or to the knowledge of the Loan Party, any agent of the Loan Party or any Subsidiary that act in any capacity in connection with the Loans, is (i) a Sanctioned Person, (ii) organized, resident or located in a Sanctioned Country, (iii) in violation of Sanctions, or (iv) engaged in any transactions or dealings with a Sanctioned Person or in a Sanctioned Country; and each Loan Party has instituted and maintains policies and procedures designed to ensure continued compliance by each Loan Party, its Subsidiaries, and their respective directors, officers, employees and agents with Sanctions.

(e)          No Loan Party or any of its Subsidiaries or to its knowledge any of the respective officers, directors, brokers or agents of such Loan Party or Subsidiary acting or benefiting in any capacity in connection with the Loans (i) deals in, or otherwise engages in any transaction related to, any property or interests in property blocked pursuant to any Anti-

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Terrorism Law or (ii) engages in or conspires to engage in any transaction that evades or avoids, or has the purpose of evading or avoiding, or attempts to violate, any of the prohibitions set forth in any Anti-Terrorism Law.

(f)          No Loan Party nor any of its Subsidiaries or any of the respective officers, directors, brokers or agents of such Loan Party or Subsidiary will directly or indirectly use the proceeds of the Loans or otherwise make available such proceeds to any individual or entity (i) for the purpose of funding, financing, or facilitating any activities, business or transaction of or with a Sanctioned Person, or in any Sanctioned Country, or (ii) in any manner that would result in a violation of Sanctions by any party to this agreement.

(g)          None of the Loan Parties or any of its Subsidiaries nor, to the knowledge of the Borrower, any director, officer, agent, employee or other person acting on behalf of the Borrower or any of its Subsidiaries has taken any action, directly or indirectly, that would result in a material violation by such persons of the Foreign Corrupt Practices Act of 1977, as amended, and the rules and regulations thereunder or any other applicable anti-corruption law (collectively, “Anti-Corruption Laws”); and the Loan Parties have instituted and maintain policies and procedures designed to ensure continued compliance therewith in all material respects.

(h)          None of the Loan Parties or any of its Subsidiaries is a “holding company” or an “affiliate” of a “holding company” or a “subsidiary company” of a “holding company” as each term is defined and used in the Public Utility Holding Company Act of 2005.

Section 5.16 Disclosure. No report, financial statement, certificate or other written information furnished by or on behalf of any Loan Party or any of its Subsidiaries to the Administrative Agent or any Lender in connection with the transactions contemplated hereby and the negotiation of this Agreement or delivered hereunder or any other Loan Document (as modified or supplemented by other information so furnished) when taken as a whole contains when furnished any material misstatement of fact or omits to state any material fact necessary to make the statements therein, in the light of the circumstances under which they were made, not materially misleading; provided to the extent any information constitutes projections or other forward-looking information, the Borrower represents only that such information was prepared in good faith based upon assumptions believed to be reasonable at the time of preparation; it being understood that such projections may vary from actual results and that such variances may be material.

Section 5.17 Intellectual Property. As of the date hereof, set forth on Schedule 5.17 and the schedules to the Collateral Documents is a complete and accurate list of all Registered patents, trademarks, service marks, domain names and copyrights, owned by any Loan Party or any of its Subsidiaries and all IP Agreements (as defined in the Collateral Documents) as of such date, showing as of such date the jurisdiction in which each such item of Registered Intellectual Property is registered or in which an application is pending and the registration or application number. Each Loan Party and each of its Subsidiaries owns or has the right to use, all of the trademarks, service marks, trade names, domain names, copyrights, patents, know-how, technology and other intellectual property recognized under applicable Law (collectively, “Intellectual Property”) that are material to the operation of their respective

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businesses as currently conducted and, to the knowledge of the Loan Parties, except as set forth in the “Disputes or Litigation” section of Schedule 5.17, the use of such Intellectual Property by such Person or the operation of their respective businesses is not infringing upon any Intellectual Property rights held by any other Person and there are no other disputes or litigation proceedings involving such Intellectual Property.

Section 5.18 Plan Funding Agreement. As of the Closing Date, the Borrower has delivered to the Administrative Agent and the Lenders a complete and correct copy of the Plan Funding Agreement (including all schedules, exhibits, amendments, supplements, modifications and other material documents delivered pursuant thereto or in connection therewith or with the Acquisition).

Section 5.19 EEA Financial Institution. Neither the Borrower nor any other Loan Party is an EEA Financial Institution.

Section 5.20 Contractual Obligations. Set forth on Schedule 5.20 hereto are all Material Contracts to which the Loan Parties and their Subsidiaries are party as of the Closing Date. As of the Closing Date, none of the Loan Parties or their Subsidiaries have knowledge of any events of default under any such Material Contracts.

ARTICLE VI

AFFIRMATIVE COVENANTS

So long as any Lender shall have any Commitment outstanding hereunder or any Loan or other Obligation hereunder which is accrued and payable shall remain unpaid or unsatisfied, each of the Parent and the Borrower shall, and shall (except in the case of the covenants set forth in Section 6.01, Section 6.02 and Section 6.03) cause the Loan Parties and their Subsidiaries to:

Section 6.01 Financial Statements. Deliver to the Administrative Agent who shall, upon the request of any Lender, make such information available to such Lender:

(a)          Quarterly and Annual Financial Statements. (i) As soon as available, but in any event, within fifty-five (55) days after the end of each of the first three (3) fiscal quarters of each Fiscal Year of the Parent (commencing with the first full fiscal quarter ended after the Closing Date), unaudited internally prepared balance sheet of the Parent and its Subsidiaries as at the end of such fiscal quarter, and the related unaudited internally prepared consolidated statements of income or operations and cash flows for such fiscal quarter, certified by a Responsible Officer of the Parent as fairly presenting in all material respects the financial condition, results of operations and cash flows of the Parent and its Subsidiaries in accordance with IFRS, subject to year-end adjustments, and (ii) as soon as available, but no later than one hundred twenty (120) days after the last day of the Parent’s Fiscal Year, audited consolidated financial statements of the Parent for the Fiscal Year then ended (to be comprised of a consolidated balance sheet and income statement and cash flows covering the Parent and its Subsidiaries’ operations for such

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Fiscal Year), which financial statements shall have been audited by independent public accountants of recognized national standing and accompanied by an opinion of such accountants (which shall not be qualified as to scope or contain any going concern or other qualification) to the effect that such consolidated financial statements fairly present, in all material respects, the financial position and results of operations of the Parent and its Subsidiaries on a consolidated basis in accordance with IFRS.

(b)          Management Discussion and Analysis Reports. Simultaneously with the delivery of each set of consolidated financial statements referred to in Section 6.01(a), a report setting forth management’s analysis and discussion of the condition (financial and otherwise) and operations, in respect of the business of the Parent and its Subsidiaries.

(c)          Annual Budget. As soon as available, but in any event, within sixty (60) days after the last day of the Parent’s Fiscal Year, the Borrower shall deliver to the Administrative Agent and the Lenders an annual budget (each, as amended pursuant to Section 6.01(e), a “Budget”) consisting of projections of the Loan Parties’ and their Subsidiaries’ consolidated and consolidating financial performance for the forthcoming Fiscal Year on a quarter by quarter basis (including assumptions made in the build-up of such budget).

(d)          Monthly Financial Statements. At the request of the Required Lenders during the continuation of an Event of Default, the Borrower shall provide to the Lenders a consolidated balance sheet of the Parent and its Subsidiaries as of the end of last fiscal month, and the related consolidated statements of income or operations for such fiscal month.

(e)          Budget Updates. At the request of the Required Lenders during the continuation of an Event of Default, the Borrower shall provide to the Lenders any updates to the projections set forth in the Budget.

(f)          Other Statements. Copies of all statements, reports, notices made available to the Parent’s security holders generally, to such lenders or to any other holders of Indebtedness for borrowed money, including, without limitation, (i) notice of the occurrence of any default, which notice shall specify the nature thereof, the period of existence thereof and what action the Borrower proposes to take with respect thereto and (ii) notice of the occurrence of any matter that has resulted or could reasonably be expected to result in a Material Adverse Effect.

(g)         Notices to Convertible Note Holders. Copies of all notices to or from the holders or the trustee of the Convertible Notes and agreements and documents (including any amendments or modifications thereto) entered into in connection with the Convertible Notes, in each case, within one (1) Business Day of delivery, receipt or execution as the case may be.

Section 6.02 Certificates; Reports; Other Information. Promptly deliver to the Administrative Agent who shall, make such information available on the Platform:

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(a)          upon delivery of the financial statements referred to in Section 6.01(a) a duly completed Compliance Certificate signed by a Responsible Officer of the Borrower; and

(b)          promptly after the same are publicly available, copies of all annual, regular, periodic and special reports and registration statements which the Parent files with the SEC or with any successor Governmental Authority (other than amendments to any registration statement (to the extent such registration statement, in the form it became effective, is delivered), exhibits to any registration statement and, if applicable, any registration statement on Form S-8) and in any case not otherwise required to be delivered to the Administrative Agent pursuant hereto.

Delivery of any reports, information and documents under Section 6.01 and Section 6.02 as well as any such reports, information and documents pursuant to this Agreement, to the Administrative Agent and the Lenders is for informational purposes only and the Administrative Agent’s and Lenders’ receipt of such shall not constitute constructive notice of any information contained therein or determinable from information contained therein, including the Borrower’s compliance with any of its covenants hereunder (as to which the Administrative Agent and the Lenders are entitled to rely exclusively on the Compliance Certificates). The Administrative Agent and the Lenders shall have no responsibility or liability for the filing, timeliness or content of any report required under Section 6.01 or Section 6.02 or any other reports, information and documents required under this Agreement (aside from any report that is expressly the responsibility of the Lenders subject to the terms hereof).

Section 6.03 Notice Requirements; Other Information. Promptly after a Responsible Officer obtains knowledge thereof, notify the Administrative Agent and each Lender of each of the following events or circumstances and provide to the Administrative Agent the following information and documents who shall make such information available on the Platform:

(a)          the occurrence of any Default, which notice shall specify the nature thereof, the period of existence thereof and what action the Borrower proposes to take with respect thereto;

(b)          the occurrence of any matter that has resulted or could reasonably be expected to result in a Material Adverse Effect;

(c)          the commencement of, or any material development in, any litigation or governmental proceeding (including without limitation pursuant to any applicable Environmental Laws) pending against any Loan Party or any of the Subsidiaries that could reasonably be expected to be determined adversely and, if so determined, to result in a Material Adverse Effect;

(d)          the occurrence of any ERISA Event above the Threshold Amount or the breach of any representation in Section 5.12;

(e)          any information with respect to environmental matters as required by Section 6.04(b);

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(f)          copies of all notices, requests and other documents received by any Loan Party or any of its Subsidiaries under or pursuant to any instrument, indenture, loan or credit or similar agreement relating to Indebtedness in excess of the Threshold Amount regarding or related to any breach or default by any party thereto or any other event that could materially impair the value of the interests or the rights of any Loan Party or otherwise have a Material Adverse Effect and copies of any amendment, modification or waiver of any provision of any such instrument, indenture, loan or credit or similar agreement relating to Indebtedness in excess of the Threshold Amount and, from time to time upon request by the Administrative Agent (at the direction of the Required Lenders), such information and reports regarding such instruments, indentures and loan and credit and similar agreements relating to Indebtedness in excess of the Threshold Amount as the Administrative Agent may reasonably request (at the direction of the Required Lenders);

(g)          a tax event or liability not previously disclosed in writing by the Borrower to the Administrative Agent which would reasonably be expected to result in a material liability, together with any other information as may be reasonably requested by the Required Lenders to enable the Required Lenders to evaluate such matters;

(h)          any occurrence of a Change of Control; and

(i)          any change (i) in any Loan Party’s corporate name, (ii) any Loan Party’s identity and corporate structure, (iii) any Loan Party’s taxpayer identification number or (iv) any Loan Party’s jurisdiction of incorporation.

Section 6.04 Environmental Matters. (a) Comply and cause each Loan Party and each of its Subsidiaries to comply, in all material respects, with all applicable Environmental Laws and Environmental Permits; obtain and renew, and cause each of its Subsidiaries to obtain and renew, all material Environmental Permits required under Environmental Laws for its operations and properties; and conduct, and cause each of its Subsidiaries to conduct, any investigation, study, sampling and testing, and undertake any cleanup, removal, remedial or other action required to remove and clean up all releases or threatened releases of Hazardous Materials from any of its properties, as required under, and in accordance with the requirements of all Environmental Laws; provided, however, that no Loan Party nor any of its Subsidiaries shall be required to undertake any such cleanup, removal, remedial or other action to the extent that its obligation to do so is being contested in good faith and by proper proceedings and, to the extent required by IFRS, appropriate reserves are being maintained with respect to such circumstances.

(b)          Promptly, and in any event within ten (10) Business Days, after a Responsible Officer obtains knowledge thereof, notify the Administrative Agent of or, deliver to the Administrative Agent, for further distribution to each Lender, copies of any and all material, non-privileged written communications and material, non-privileged documents concerning:

(i)          any Environmental Action against or of any non-compliance by any Loan Party or any of its Subsidiaries with any Environmental Law or Environmental Permit that would (1) reasonably be expected to result in a liability to any Loan Party in excess of the Threshold Amount or (2) cause any Mortgaged Properties to be subject to

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any restrictions on ownership, occupancy, use or transferability under any Environmental Law;

(ii)          to the extent any of the following is reasonably expected to result in a liability to any Loan Party in excess of the Threshold Amount: (1) any occurrence of any release or threatened release of Hazardous Materials required to be reported to any Governmental Authority under applicable Environmental Law, (2) any remedial actions taken by any Loan Party or its Subsidiaries in respect of any such release or threatened release that could reasonably be expected to result in an Environmental Action or (3) the Loan Parties’ discovery of any occurrence of or condition on any real property adjoining or in the vicinity of any site or facility that would be reasonably expected to cause such site or facility or any part thereof to be subject to any restrictions on the ownership, occupancy, transferability or use thereof under any Environmental Laws;

(iii)          to the extent any of the following is reasonably expected to result in a liability to any Loan Party in excess of the Threshold Amount: (1) any Permitted Acquisition that could reasonably be expected to (A) expose any Loan Party or any of its Subsidiaries to, or result in, Environmental Actions or (B) affect the ability of the Loan Parties and their Subsidiaries to maintain in full force and effect all Governmental Authorizations and Environmental Permits required for the continued operations of their respective businesses existing prior to such Permitted Acquisition and (2) any action proposed to be taken by any Loan Party or any of its Subsidiaries to modify current operations in a manner that would reasonably be expected to subject any Loan Party or any of its Subsidiaries to any material additional obligations or requirements under Environmental Laws;

(iv)          copies of all material environmental reports or audits (whether produced by any Loan Party or any of its Subsidiaries or any third party or Governmental Authority) and any Phase I or Phase II reports in respect of any sites or real property owned, leased or operated by any Loan Party or any of its Subsidiaries that are in possession or control of any Loan Party or any of its Subsidiaries;

(v)          to the extent any of the following is reasonably expected to result in a liability to any Loan Party in excess of the Threshold Amount: copies of any and all material, non-privileged written communications with respect to (A) any Environmental Action, (B) any release or threatened release or non-compliance with any Environmental Law required to be reported to any Governmental Authority and (C) any request for information from a Governmental Authority that suggests such Governmental Authority is investigating the potential responsibility of any Loan Party or any of its Subsidiaries as a potentially responsible party;

(vi)          the good faith belief that a release of Hazardous Materials, or a violation of Environmental Law reasonably likely to result in a fine or penalty in excess of the Threshold Amount, has occurred on or after the Closing Date, and within 60 days after such request and at the expense of the Borrower, any additional environmental site assessment reports for any Loan Party or any of its Subsidiaries’ properties described in such request prepared by an environmental consulting firm acceptable to the Required

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Lenders, indicating the presence or absence of such Hazardous Materials and the estimated cost of any compliance, removal or remedial action in connection with any such Hazardous Materials on such properties; without limiting the generality of the foregoing, if the Required Lenders reasonably determine at any time that a material risk exists that any such report will not be provided within the time referred to above, the Administrative Agent may retain an environmental consulting firm to prepare such report at the expense of the Borrower, and the Borrower hereby grants and agrees to cause any Loan Party and any Subsidiary that owns any property described in such request to grant at the time of such request to the Administrative Agent, the Lenders, such firm and any agents or representatives thereof, the right, subject to the rights of tenants, to enter onto their respective properties to undertake such an assessment; and

(vii)          any such other documents and information as the Administrative Agent (at the direction of the Required Lenders) may reasonably request from time to time.

Section 6.05 Maintenance of Existence. (a) Preserve, renew and maintain in full force and effect its legal existence, structure and name under the Laws of the jurisdiction of its organization and (b) take all commercially reasonable action to maintain all rights, privileges (including its good standing), permits, licenses and franchises necessary or desirable in the normal conduct of its business, except (i) other than with respect to any Loan Party, to the extent the Parent’s board of directors (or in the case of clause (b), a Responsible Officer) shall determine that the preservation thereof is no longer desirable in the conduct of the business of the Loan Parties and their Subsidiaries and to the extent that the loss thereof shall not be disadvantageous to the Loan Parties, their Subsidiaries or the Lenders in any material respect, (ii) pursuant to a transaction permitted by Section 7.04 or Section 7.05 or (iii) in the case of clause (b), failure to do so could not reasonably be expected to have a Material Adverse Effect.

Section 6.06 Maintenance of Properties. (a) Maintain, preserve and protect all of its material properties and equipment that are used or useful in the operation of its business in good working order, repair and condition, ordinary wear and tear excepted and casualty or condemnation excepted, and (make all commercially reasonable and appropriate repairs, renewals, replacements, modifications, improvements, upgrades, extensions and additions thereof except where failure to do so would not reasonably be expected to materially adversely affect the use of the related property.

Section 6.07 Maintenance of Insurance. Maintain with financially sound and reputable insurance companies (in the good faith judgment of management of the Parent), insurance with respect to its properties and business against loss or damage of the kinds customarily insured against by Persons engaged in the same or similar business, of such types and in such amounts (after giving effect to any self-insurance reasonable and customary for similarly situated Persons engaged in the same or similar businesses as the Loan Parties and their Subsidiaries) as are customarily carried by Person engaged in similar businesses and owning or leasing similar properties in the same general areas in which such Loan Party or such Subsidiary operates. The Parent and the Borrower shall cause all property policies to have a lender’s loss payable endorsement showing Administrative Agent as lender loss payee and use commercially reasonable efforts to cause such endorsement to provide that the insurer must give

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Administrative Agent at least twenty (20) days’ notice before canceling, amending, or declining to renew its policy. All liability policies shall show, or have endorsements showing, Administrative Agent as an additional insured, and all such policies (or the loss payable and additional insured endorsements) shall provide that the insurer shall give Administrative Agent at least twenty (20) days’ notice before canceling, amending, or declining to renew its policy. At any Lender’s request, each Loan Party shall deliver certified copies of policies and evidence of all premium payments. Proceeds payable under any casualty policy in connection with a Casualty Event shall be subject to Section 2.02(b)(ii).

Section 6.08 Compliance with Laws. Comply in all material respects with the requirements of all Laws and all orders, writs, injunctions, decrees and judgments applicable to it or to its business or property, except where such non-compliance is not, either individually or in the aggregate, reasonably likely to have a Material Adverse Effect.

Section 6.09 Books and Records. Maintain proper books of record and account, in which entries that are full, true and correct in all material respects and as are sufficient to permit the preparation of financial statements in conformity with IFRS, consistently applied, shall be made of all material financial transactions and matters involving the assets and business of any of the Loan Parties.

Section 6.10 Inspection Rights; Lender Calls. (a) Permit representatives and independent contractors of the Administrative Agent and each Lender (including, without limitation, financial advisors retained by or for the benefit of the Administrative Agent or the Lenders or their counsel, including the Financial Advisor) to visit and inspect any properties and books and records of the Loan Parties and their Subsidiaries (subject, in the case of third party customer sites, to customary access agreements) and to discuss its affairs, finances and accounts with its directors, officers, advisors and independent public accountants, all at the reasonable expense of the Borrower and at such reasonable times during normal business hours and as often as may be reasonably desired, upon reasonable advance notice to the Borrower; provided, however, that such visits and inspections shall be coordinated through the Required Lenders and any review of books and records shall be done no more frequently than quarterly absent the continuation of an Event of Default. The Administrative Agent and the Lenders shall give the Borrower the opportunity to participate in any discussions with the Borrower’s independent public accountants to the extent reasonably feasible. No Loan Party nor any Subsidiary shall be required to disclose to the Administrative Agent or any Lender any information that, in the opinion of counsel to such Loan Party or such Subsidiary, is prohibited by Law to be disclosed, is subject to attorney client privilege or constitutes attorney work product or the disclosure of which would cause a material breach of a binding non-disclosure agreement with a third party to the extent such agreement is not made in contemplation of the avoidance of this Section 6.10.

(b)          On a quarterly basis, upon the reasonable request of the Required Lenders, the Borrower’s chief financial officer, together with the Borrower’s financial advisor shall hold a conference call (at a mutually agreeable time, the cost of such call to be paid by the Borrower) with the Administrative Agent and the Lenders, on which conference calls shall be reviewed the Loan Parties’ financial performance, operations, current trends and variance reports

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Section 6.11 Additional Guarantors. To the extent any Person is required to become a Guarantor under the Loan Documents, (i) cause such Person to become a Guarantor by executing and delivering to the Administrative Agent a counterpart of the Guarantee and Collateral Agreement or a Guarantee and Collateral Agreement Supplement substantially in the form attached to the Guarantee and Collateral Agreement, and (ii) deliver to the Administrative Agent any applicable documents of the types referred to in Section 4.01(a) and, if reasonably requested by the Required Lenders, favorable opinions of counsel to such Person under New York Law, if applicable (which shall cover, among other things, the legality, validity, binding effect and enforceability of the Guarantee and Collateral Agreement), all in form, content and scope reasonably satisfactory to the Required Lenders.

Section 6.12 Use of Proceeds. Use the proceeds of any Loan, whether directly or indirectly, solely in a manner consistent with the Permitted Uses. Notwithstanding the foregoing, no part of the proceeds of any Loan shall be used directly or indirectly to finance in any way payment of the fees and expenses of any Person incurred in connection with (i) the investigation (including discovery proceedings), initiation or prosecution of any claims, causes of action, adversary proceedings, suits, arbitrations, proceedings, applications, motions or other litigation of any type adverse to any of the Secured Parties or any of their respective Affiliates, agents or representatives, or their respective rights and remedies under or in respect of the Loans provided pursuant to this Agreement; (ii) challenging the amount, validity, perfection, priority or enforceability of, or asserting any defense, counterclaim or offset to, the obligations and liens and security interests granted under the Loan Documents, applicable non-bankruptcy law or otherwise; or (iii) attempting to prevent, hinder or otherwise delay any of the Lenders’ or the Administrative Agent’s assertion, enforcement or realization upon any of the Collateral.

Section 6.13 Anti-Corruption and Sanctions Laws. To the extent existing on the Closing Date, the Loan Parties will maintain in effect such policies and procedures designed to promote compliance in all material respects by the Loan Parties, their Subsidiaries, and their respective directors, officers, employees, and agents with the FCPA and any other applicable anti-corruption laws as well as Sanctions.

Section 6.14 Taxes. Pay and discharge, and will cause each Loan Party and each of its Subsidiaries to pay and discharge, all material Taxes, assessments and governmental charges or levies arising after the Closing Date imposed upon it or upon its income or profits, or upon any properties belonging to it, in each case on a timely basis, which, if unpaid when due and payable, may reasonably be expected to become a tax Lien upon any properties of any Loan Party or any of its Subsidiaries thereof not otherwise permitted under this Agreement; provided that no Loan Party nor any of its Subsidiaries shall be required to pay any such Tax, assessment, charge, levy or claim which is being contested in good faith and by proper proceedings if it has maintained adequate reserves with respect thereto in accordance with IFRS unless and until any tax Lien resulting therefrom attaches to its property and becomes enforceable against its other creditors.

Section 6.15 End of Fiscal Years; Fiscal Quarters. Cause (i) its Fiscal Year to end on or about December 31 of each calendar year and (ii) its fiscal quarters to end on or about March 31, June 30, September 30 and December 31 of each calendar year, in each case unless otherwise approved by the Required Lenders.

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Section 6.16 ERISA. (a) ERISA Events and ERISA Reports. (i) Promptly and in any event within ten (10) days after any Loan Party, any Subsidiary or any ERISA Affiliate knows or has reason to know that any ERISA Event has occurred, provide to the Administrative Agent a statement of a Responsible Officer of the Borrower describing such ERISA Event and the action, if any, that such Loan Party, such Subsidiary or such ERISA Affiliate has taken and proposes to take with respect thereto and (ii) on the date any records, documents or other information must be furnished to the PBGC with respect to any Plan pursuant to Section 4010 of ERISA, provide to the Administrative Agent a copy of such records, documents and information.

(b)          Plan Terminations. Promptly and in any event within five (5) Business Days after receipt thereof by any Loan Party or any ERISA Affiliate, provide to the Administrative Agent copies of each notice from the PBGC stating its intention to terminate any Plan or to have a trustee appointed to administer any Plan.

(c)          Plan Annual Reports. Promptly and in any event within 30 days after the filing thereof with the Internal Revenue Service, provide to the Administrative Agent copies of each Schedule B (Actuarial Information) to the annual report (Form 5500 Series) with respect to each Plan.

(d)          Multiemployer Plan Notices. Promptly and in any event within five (5) Business Days after receipt thereof by any Loan Party, any Subsidiary or any ERISA Affiliate from the sponsor of a Multiemployer Plan, provide to the Administrative Agent copies of each notice concerning (i) the imposition of Withdrawal Liability by any such Multiemployer Plan, (ii) the reorganization or termination, or a determination that such Multiemployer Plan is in endangered or critical status, within the meaning of Title IV of ERISA, of any such Multiemployer Plan or (iii) the amount of liability incurred, or that may be incurred, by such Loan Party, such Subsidiary or such ERISA Affiliate in connection with any event described in clause (i) or (ii).

Section 6.17 Further Assurances.

(a)          Execute and deliver, or cause to be executed and delivered, to the Administrative Agent such reasonable documents and agreements, and shall take or cause to be taken such reasonable actions, as the Administrative Agent may, from time to time, reasonably request to carry out the terms and conditions of this Agreement and the other Loan Documents.

(b)          In the event any Loan Party or any of its Subsidiaries acquires fee title to any real property with a fair market value in excess of $1,000,000, within thirty (30) days (or such longer period as the Required Lenders may agree in their sole discretion) after such acquisition, such Loan Party or such Subsidiary shall deliver, or cause to be delivered, to the Administrative Agent a fully-executed Mortgage with respect to such real property, together with such title insurance policies, surveys, appraisals, evidence of insurance, legal opinions, environmental assessments and other documents and certificates reasonably requested by the Administrative Agent and the Required Lenders.

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(c)          Each of the Parent and the Borrower shall, and shall cause each of their direct and indirect Subsidiaries to, execute such guarantees, pledge agreements, control agreement and security documents as shall be customary in the applicable local jurisdictions to grant to Administrative Agent, for the benefit of the Secured Parties, a security interest and guaranty of the Obligations secured by a first priority Lien (in form and substance reasonably satisfactory to the Secured Parties) over substantially all assets of the Parent and its Subsidiaries (including all equity interests of each Subsidiary of Parent); provided, that (i) no Foreign Subsidiary of the Borrower or of Amryt Pharmaceuticals DAC shall be required to become a Guarantor or otherwise grant Liens on its assets to the extent it is an Immaterial Subsidiary or organized within France and (ii) such guaranty and security with respect to any Foreign Subsidiary of the Borrower or of Amryt Pharmaceuticals DAC (including over the equity of such Foreign Subsidiary) shall be limited as agreed by both the Parent and the Required Lenders to the extent the Parent and the Required Lenders both determine that such guaranty and granting of liens will result in material adverse tax consequences to the Loan Parties and their Subsidiaries or would violate applicable law in any material respect.

Section 6.18 Business. Each of the Parent and the Borrower will only, and will only permit their respective Subsidiaries to, engage directly or indirectly in the business engaged in by the Parent, the Borrower and their respective Subsidiaries as of the Closing Date and reasonable extensions thereof and businesses ancillary, corollary, synergistic or complimentary thereto.

Section 6.19 Landlord Agreements. To the extent requested by the Lenders, each Loan Party shall use commercially reasonable efforts to obtain a landlord agreement or bailee or mortgagee waivers, as applicable, from the lessor of each leased property, bailee in possession of any Collateral or mortgagee of any owned property with respect to (to the extent leased) the Borrower’s headquarters and each location where any Collateral having a fair market value in excess of $1,000,000 is stored or where material books and records are located, which agreement shall be in form and substance reasonably satisfactory to the Required Lenders.

Section 6.20 Post-Closing Matters. Comply with each of the requirements set forth on Schedule 6.20 no later than the applicable date specified thereon.

ARTICLE VII

NEGATIVE COVENANTS

So long as any Lender shall have any Commitment outstanding hereunder or any Loan or other Obligation hereunder which is accrued and payable shall remain unpaid or unsatisfied, each of the Parent and the Borrower shall not, and shall not permit any of the Loan Parties or their Subsidiaries to, directly or indirectly:

Section 7.01 Liens. Create, incur, assume or suffer to exist any Lien upon any of its property, assets or revenues (including accounts receivable), whether now owned or hereafter acquired, other than the following Liens (collectively, “Permitted Liens”):

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(a)          Liens pursuant to any Loan Document;

(b)          Liens existing on the Closing Date and listed on Schedule 7.01(b);

(c)          Liens for taxes, assessments or governmental charges which are not overdue for a period of more than thirty (30) days or which are being contested in good faith and by appropriate proceedings, if adequate reserves with respect thereto are maintained on the books of the applicable Person to the extent required in accordance with IFRS;

(d)          statutory or common law Liens of landlords, carriers, warehousemen, mechanics, materialmen, repairmen, suppliers, construction contractors or other like Liens arising in the ordinary course of business which secure amounts not to exceed $50,000 and not overdue for a period of more than thirty (30) days or if more than thirty (30) days overdue, are unfiled (or if filed have been discharged or stayed) and no other action has been taken to enforce such Lien or which are being contested in good faith, if adequate reserves with respect thereto are maintained on the books of the applicable Person to the extent required in accordance with IFRS;

(e)          (i) pledges and deposits in the ordinary course of business securing liability for reimbursement or indemnification obligations of (including obligations in respect of letters of credit or bank guarantees for the benefit of) insurance carriers providing property, casualty or liability insurance to the Borrower or any Subsidiary and (ii) Liens securing the financing of insurance premiums (to the extent such Liens extend to the unearned premiums for such insurance) in the ordinary course of business;

(f)          Liens consisting of deposits made in connection with Indebtedness of the types permitted under Sections 7.03(e) or 7.03(g) (in each case, other than for borrowed money) entered into in the ordinary course of business or to secure the obligations otherwise permitted;

(g)          easements, rights-of-way, covenants, conditions, restrictions, encroachments, and other survey defects protrusions and other similar encumbrances and minor title defects affecting real property which were not incurred in connection with Indebtedness and do not in any case materially and adversely interfere with the use of the property encumbered thereby for its intended purposes;

(h)          Liens securing Indebtedness permitted under Section 7.03(c); provided that (i) such Liens attach concurrently with or within 120 days after the acquisition, or the completion of the construction, repair, replacement or improvement (as applicable) of the property subject to such Liens, (ii) such Liens do not at any time encumber any property other than the property financed by such Indebtedness, replacements thereof and additions and accessions to such property and the proceeds and the products thereof and customary security deposits, and (iii) with respect to Capital Leases, such Liens do not at any time extend to or cover any assets (except for additions and accessions to such assets, replacements and products thereof and customary security deposits) other than the assets subject to such Capital Leases;

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(i)          Liens arising by virtue of any contractual, statutory or common law provision relating to banker’s Liens, rights of set-off or similar rights and remedies (i) relating to the establishment of depository relations with banks or other financial institutions not given in connection with the incurrence of Indebtedness, (ii) relating to pooled deposit or sweep accounts of the Borrower or any Subsidiary of the Borrower that is a Subsidiary Guarantor (so long as such Subsidiary remains a Subsidiary Guarantor) to permit satisfaction of overdraft or similar obligations incurred in the ordinary course of business of the Borrower or such Subsidiary Guarantor or (iii) relating to purchase orders and other agreements entered into with customers of the Borrower or any of its Subsidiaries in the ordinary course of business;

(j)          Liens arising from precautionary Uniform Commercial Code financing statement filings regarding leases entered into by the Borrower and its Subsidiaries in the ordinary course of business;

(k)          any zoning, land-use or similar law or right reserved to or vested in any Governmental Authority to control or regulate the use of any real property;

(1)          the modification, replacement, renewal or extension of any Lien permitted by clause (b) of this Section 7.01; provided that (i) the Lien does not extend to any additional property or additional Indebtedness (except with respect to paid-in-kind obligations pursuant to the terms of such Indebtedness as in effect on the Closing Date) other than (A) after-acquired property that is affixed or incorporated into the property covered by such Lien or financed by Indebtedness permitted under Section.7.03, and (B) proceeds and products thereof; and (ii) the renewal, extension or refinancing of the obligations secured or benefited by such Liens is permitted by Section 7.03;

(m)          nonconsensual statutory Liens arising after the Closing Date;

(n)          judgment Liens in existence for less than thirty (30) days after the entry thereof, or with respect to which execution has been stayed or the payment of which is covered in full by insurance maintained with responsible insurance companies, or which judgment Liens do not otherwise result in an Event of Default under Section 8.01(i);

(o)          any interest or title of a lessor, licensor or sublessor under any lease, license or sublease entered into by the Borrower or any of its Subsidiaries in the ordinary course of its business and covering only the assets so leased, or subleased;

(p)          other Liens not on borrowed money with respect to which the aggregate amount of the obligations secured thereby does not exceed $100,000 at any time outstanding; provided, that no such Liens shall be on Equity Interests of the Parent or any of its direct or indirect Subsidiaries;

(q)          to the extent constituting a Lien and permitted under Section 7.05, any non-exclusive licenses of Intellectual Property granted to third parties and set forth on Schedule 5.17 and other non-exclusive licenses after the Closing Date, in each case to the extent not resulting in a legal transfer of title of the licensed Intellectual Property and in the ordinary course of business, subject to exclusivity on territory aside from the United

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States or Europe;

(r)          to the extent constituting Liens and permitted under Section 7.05, any leases, subleases, licenses, or sublicenses (other than licenses of Intellectual Property) granted to third parties that do not materially interfere with the Loan Parties’ ordinary course of business;

(s)          Liens securing Indebtedness permitted under Section 7.03(1); provided that such Liens do not at any time extend to or cover any assets other than the equipment financed under such finance leases;

(t)          Liens consisting of cash deposits not to exceed $400,000 securing Indebtedness permitted under Section 7.03(i); and

(u)          Liens securing the Amryt Finance Contract from the period from the Closing Date up through one (1) Business Day following the Closing Date which Liens shall be terminated upon repayment of the obligations thereunder to occur no later than one (1) Business Day following the Closing Date.

provided that no Permitted Lien shall consist of a Lien granted by a Loan Party or a Subsidiary of a Loan Party which has not also granted Liens to secure the Obligations.

Section 7.02 Investments. Make any Investments, except:

(a)          Investments by the Loan Parties or their Subsidiaries in cash and Cash Equivalents;

(b)          loans and advances to officers, directors or employees in the ordinary course of the business of the Loan Parties and their Subsidiaries in an aggregate principal amount not to exceed $50,000 at any time outstanding;

(c)          Investments existing as of the Closing Date and disclosed on Schedule 7.02(c) and Investments consisting of any modification, replacement, renewal, reinvestment or extension of any such Investment; provided that the amount of any Investment permitted pursuant to this Section 7.02(c) is not increased from the amount of such Investment on the Closing Date;

(d)          so long as immediately before and after giving effect to any such Investment, no Default has occurred and is continuing, other Investments that do not exceed $250,000 in the aggregate (net of any return or distribution of capital or repayments of principal in respect thereof);

(e)          Investments received in connection with the bankruptcy or reorganization of, or settlement of delinquent accounts and disputes with, customers and suppliers, in each case in the ordinary course of business; and

(f)          other Investments made (i) by any Subsidiary that is not a Loan Party in any other Subsidiary that is not a Loan Party, (ii) by any Subsidiary of the Parent in any

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Loan Party organized within the United States, Republic of Ireland or United Kingdom, (iii) by any Subsidiary of the Parent in any Loan Party organized within Germany in an aggregate amount not to exceed $5,000,000 for all such Investments and (iv) by any Loan Party in any other Subsidiary that is not a Loan Party in an aggregate amount not to exceed $5,000,000 for all such Investments.

Section 7.03 Indebtedness. Create, incur, assume or suffer to exist any Indebtedness, except the following, without duplication:

(a)          Indebtedness of the Borrower and other Loan Parties under the Loan Documents;

(b)          Indebtedness outstanding on the Closing Date and listed on Schedule 7.03(b):

(c)          additional Capital Leases incurred after the Closing Date and purchase money Indebtedness in an aggregate amount not to exceed $750,000 in the aggregate at any time outstanding, and any Refinancing Indebtedness in respect of such Indebtedness; provided that any such Indebtedness (x) in the case of additional Capital Leases or purchase money Indebtedness, shall be secured only by the asset subject to such additional Capital Leases or acquired asset in connection with the incurrence of such Indebtedness, as the case may be, and (ii) in the case of purchase money Indebtedness, shall constitute not less than 75% of the aggregate consideration paid with respect to such asset;

(d)          other unsecured Indebtedness in an aggregate principal amount not to exceed $250,000 at any time outstanding;

(e)          Indebtedness in respect of performance of bids, trade contracts, governmental contracts and leases (other than Indebtedness for borrowed money), statutory obligations, surety, stay, indemnity, customs and appeal bonds, performance bonds and other obligations of a like nature (including those to secure health, safety and environmental obligations), and, in each case, letters of credit in respect thereof, incurred in the ordinary course of business;

(f)          non-recourse Indebtedness incurred by the Loan Parties or any of their Subsidiaries to finance the payment of insurance premiums of such Person;

(g)          Indebtedness owed to any Person providing worker’s compensation, unemployment insurance and other social security legislation, health, disability or other employee benefits or property, casualty or liability insurance to the Loan Parties or any of their Subsidiaries incurred in connection with such Person providing such benefits or insurance pursuant to customary reimbursement or indemnification obligations to such Person;

(h)          to the extent constituting Indebtedness, each of the Investments permitted pursuant to Section 7.02;

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(i)          reimbursement obligations owed to banks and financial institutions with respect to credit card services in an aggregate amount at any one time not exceeding $400,000;

(j)          Indebtedness consisting of accounts payable incurred in the ordinary course of business past due for more than 120 days after its stated due date (except for accounts payable contested in good faith) which do not in the aggregate exceed $750,000;

(k)          Indebtedness of the Borrower and the Loan Parties under the Deed Poll Constituting Loan Notes issued in connection with the CVR Instrument made by the Parent in an aggregate principal amount not to exceed $85,000,000 at any time outstanding;

(1)          finance leases with respect to AP101 and AP103 equipment in an amount not to exceed $5,000,000 in the aggregate at any time outstanding; provided that such Indebtedness shall be secured only by the equipment financed thereunder;

(m)          unsecured Indebtedness of the Borrower and the Loan Parties under the Convertible Notes; and

(n)          Indebtedness under the Amryt Finance Contract from the period from the Closing Date up through one (1) Business Day following the Closing Date which Indebtedness shall be paid in full no later than one (1) Business Day following the Closing Date;

provided that no Loan Party or Subsidiary of any Loan Party shall be obligated with respect to any other Indebtedness unless it is also obligated with respect to the Obligations.

Section 7.04 Fundamental Changes. Merge, dissolve, liquidate, consolidate with or into another Person, split or allow any change to the ownership of the Subsidiaries of the Parent or Dispose of (whether in one transaction or in a series of transactions) all or substantially all of its assets (whether now owned or hereafter acquired) to or in favor of any Person other than (i) with respect to any Subsidiary of Borrower or any Subsidiary of Amryt Pharmaceuticals DAC, (a) to a Loan Party organized in the same country as such Person or to a Loan Party organized within the United States, The Republic of Ireland or United Kingdom and solely to the extent that Agent and Lenders retain perfected Liens on all the assets of such Person to the same extent and with the same priority as existed prior to such transaction or (b) in the case of a Subsidiary that is not a Loan Party, to another Subsidiary of the Parent; and (ii) with respect to the Intermediate Parent, to the Parent and solely to the extent that Agent and Lenders retain perfected Liens on all the assets of the Intermediate Parent to the same extent and with the same priority as existed prior to such transaction.

Section 7.05 Dispositions. Make any Disposition or enter into any agreement to make any Disposition, except:

(a)          Dispositions of obsolete, worn out or surplus property, whether now owned or hereafter acquired, in the ordinary course of business and Dispositions of

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property no longer used or useful in the conduct of the business of the Parent and its Subsidiaries, in each case to the extent constituting immaterial property;

(b)          Dispositions in the ordinary course of business of Cash Equivalents;

(c)          sales of inventory in the ordinary course of business;

(d)          Dispositions (other than of material Intellectual Property or of assets relating to metreleptin) for fair market value; provided that (i) the amount of Dispositions does not exceed $250,000 individually or $2,500,000 in the aggregate for all Dispositions during the term of this Agreement, (ii) immediately prior to and immediately after giving effect to such Disposition, no Default or Event of Default shall have occurred and be continuing or would result therefrom and (iii) no less than one hundred percent (100%) of the consideration received for any such Disposition is received in cash;

(e)          the leasing, as lessor, of real or personal property not presently used or useful in such Person’s business and is otherwise in the ordinary course of business;

(f)          Dispositions of equipment or other assets, to the extent that such equipment is exchanged for credit against the purchase price of similar replacement equipment or assets or the proceeds of such Dispositions are reasonably promptly applied to the purchase price of similar replacement equipment, all in the ordinary course of business;

(g)          Dispositions constituting an Intellectual Property that is not material to the conduct of the business of the Loan Parties and their Subsidiaries;

(h)          Dispositions otherwise permitted by Sections 7.01, 7.02 or 7.03 and Dispositions from any Subsidiary that is not a Loan Party to any other Subsidiary that is not a Loan Party;

(i)          Dispositions consisting of a Permitted AP License; and

(j)          Dispositions consisting of licenses of AP 102 assets.

provided that the proceeds of any Dispositions permitted hereunder shall be applied in accordance with the requirements of Section 2.02(b)(i) to the extent required under such section; provided further that, in the case of Dispositions under Section 7.05(i), the Borrower shall provide prior written notice to the Administrative Agent and the Lenders of any such Disposition and, so long as no Default or Event of Default has occurred and is continuing, the Borrower may (x) reinvest up to (i) 50% of the proceeds of any Permitted AP License generating proceeds in an amount less than $30,000,000 and (ii) 75% of the proceeds of any Permitted AP License generating proceeds in an amount equal to or greater than $30,000,000, in each case with such reinvestment occurring within 12 months of receipt of such proceeds or (y) commit to using such 50% or 75% (as applicable) portion of the proceeds in a clinical development program approved by the Parent’s board of directors.

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Section 7.06 Restricted Payments. Declare or make, directly or indirectly, any Restricted Payment, except:

(a)          to the extent constituting a Restricted Payment, the payment of fees of non-insider directors not to exceed an annual amount of $500,000 and the reimbursement of reasonable expenses;

(b)          the Subsidiaries of the Borrower may make direct or indirect Restricted Payments to the Borrower and other Subsidiaries of the Borrower that are Loan Parties and in the case of Subsidiaries that are not Loan Parties to other Subsidiaries that are not Loan Parties;

(c)          (i) Restricted Payments to the Parent to be used for (A) customary director indemnification and compensation payments to the Parent’s director nominees serving on the board of directors of the Parent or serving on the board of directors of any of the Parent’s direct or indirect Subsidiaries and (B) payment of income Taxes to the extent such income Taxes are attributable to the income of its direct or indirect Subsidiaries, and (ii) so long as no Event of Default has occurred and is continuing, Restricted Payments to the Parent and to Amryt Pharmaceuticals DAC to be used for financial and other reporting and similar customary administrative posts and expenses attributable and fairly allocable to the Loan Parties (including audit and professional fees and other ordinary course operating and administrative expenses incurred by the Parent in its capacity as the ultimate holding company of the Loan Parties);

(d)          the Parent may declare and make dividend payments or other distributions payable solely in the common stock or other common Equity Interests of such Person (other than Disqualified Equity Interests); and

(e)          any transaction expressly permitted under Section 7.08(d) to the extent constituting a Restricted Payment.

Section 7.07 Change in Nature of Business. Engage in any line of business other than those lines of business conducted by the Loan Parties and their Subsidiaries on the date hereof or any business reasonably related or ancillary thereto; provided, that Aegerion Securities Corporation shall not engage in any business activities, maintain any assets or incur any Indebtedness.

Section 7.08 Transactions with Affiliates. Enter into any transaction of any kind with any Affiliate of the Loan Parties, whether or not in the ordinary course of business, other than:

(a)          any transactions expressly permitted under Section 7.02, Section 7.04 and Section 7.06; provided that all parties to such transactions are Loan Parties or their Wholly-owned Subsidiaries;

(b)          so long as it has been approved by the Parent’s board of directors or other governing body to the extent required in accordance with applicable law, (i) customary indemnifications of non-officer directors of the Loan Parties and their respective

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Subsidiaries and (ii) the payment of reasonable and customary compensation and indemnification arrangements and benefit plans for officers and employees of the Loan Parties and their respective Subsidiaries in the ordinary course of business, in each case to the extent approved by a majority of independent directors of the Parent’s board of directors;

(c)          transactions (i) under the agreements existing on the Closing Date and listed on Schedule 7.08(c) and (ii) transactions contemplated to be consummated on or in connection with the Closing Date as described in the “Phoenix/Atlas Transaction Structure” memo dated as of September 24, 2019 disclosed to the Lenders prior to the Closing Date; and

(d)          transactions consisting of intercompany services among the Parent and its wholly-owned Subsidiaries in the ordinary course of business on an arm’s length basis, charged on a cost plus a maximum of 10% basis.

Section 7.09 Prepayments and Modifications of Certain Agreements. (a) Amend or modify any of the terms of any Indebtedness in an outstanding amount exceeding the Threshold Amount of any of the Loan Parties or their Subsidiaries if such amendment or modification would add or change any terms in a manner adverse to the Loan Parties or the Lenders, or shorten the final maturity or average life to maturity of any such Indebtedness or require any payment to be made sooner than originally scheduled or increase the interest rate applicable thereto.

(b)          Make or permit any distribution or payment (whether in cash or otherwise) of principal, interest or any other amounts on any Indebtedness (other than the Obligations), or seek or obtain approval by the Borrower’s board of directors to permit the foregoing, other than scheduled payments of principal and interest under such Indebtedness as in effect on the Closing Date or on the date incurred if incurred after the Closing Date.

(c)          Amend or modify, or permit the amendment, modification or waiver of, any provision of any Material Contract to which any Loan Party or any Subsidiary thereof is a party or by which it or any of its property or assets is bound, in each case after the original execution and delivery thereof (or, if later, the date hereof) in any substantive manner that would be adverse to the Lenders’ interests hereunder, without the written consent of the Required Lenders.

(d)          Amend or modify, or permit the amendment, modification or waiver of, any provision of (i) the Deed Poll Constituting Contingent Value Rights issued in connection with the reorganization of the Borrower and the Acquisition and (ii) the Deed Poll Constituting Loan Notes issued in connection with the CVR Instrument made by the Parent, in each case after the original execution and delivery thereof (or, if later, the date hereof) in any manner, without the written consent of the Required Lenders.

Section 7.10 Negative Pledge. Enter into or suffer to exist, or permit any of its Subsidiaries to enter into or suffer to exist, (x) any agreement prohibiting or conditioning the creation or assumption of any Lien upon any of its property or assets except (a) agreements in

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favor of the Administrative Agent or (b) prohibitions or conditions under (i) any Capital Lease permitted by Section 7.03(c) solely to the extent that such Capital Lease prohibits a Lien on the property subject thereto, or (ii) by reason of customary provisions restricting pledges, assignments, subletting or other transfers contained in leases, licenses and similar agreements entered into in the ordinary course of business (provided that such restrictions are limited to the property or assets subject to such leases, licenses or similar agreements, as the case may be) or (iii) any Indebtedness outstanding on the Closing Date (including, for the avoidance of doubt, the Convertible Notes) or (y) any agreement or arrangement limiting the ability of any of its Subsidiaries to declare or pay dividends or other distributions in respect of its Equity Interests or repay or prepay any Indebtedness owed to, make loans or advances to, or otherwise transfer assets to or make Investments in, the Borrower or any of its Subsidiaries of the Borrower (whether through a covenant restricting dividends, loans, asset transfers or investments, a financial covenant or otherwise), except (a) the Loan Documents and (b) any Indebtedness outstanding on the Closing Date (including, for the avoidance of doubt, the Convertible Notes).

Section 7.11 Amendments to Organization Documents. Amend, or permit any of its Subsidiaries to amend, its certificate of incorporation, constitution, association or bylaws or other Organization Documents in a manner adverse to the interests of the Lenders hereunder, without the prior written consent of the Required Lenders.

Section 7.12 Use of Proceeds. (a) Use, directly or indirectly, the proceeds of the Loans, or lend, contribute or otherwise make available such proceeds to any subsidiary, joint venture partner or other Person, (x) to fund, finance, or facilitate any activities, business, or transaction of or with any Sanctioned Person, or in any Sanctioned Country, or (y) in any other manner that would result in a violation of Sanctions by any Person (including any Person participating in the Loans, whether as underwriter, advisor, investor, or otherwise).

(b)          Use any part of the proceeds of the Loans directly or indirectly, in furtherance of an offer, payment, promise to pay, or authorization of the payment or giving of money, or anything else of value, to any Person in violation of any Anti-Corruption Law.

Section 7.13 Accounting Changes. Make any change in (a) accounting policies or reporting practices, except as required by IFRS, or (b) Fiscal Year.

Section 7.14 OFAC. (a) Become a Sanctioned Person, (b) become organized, resident or located in a Sanctioned Country, or (c) engage in any transactions or dealings with a Sanctioned Person or in a Sanctioned Country in violation of Sanctions.

Section 7.15 Ownership of Subsidiaries. Notwithstanding any other provisions of this Agreement to the contrary, organize, create, acquire or permit to exist after the Closing Date any Subsidiaries of the Parent other than (a) those existing on the Closing Date and set forth on Schedule 5.14 and (b) Subsidiaries formed after the Closing Date subject to compliance with Section 6.17.

Section 7.16 Compliance With Certain Laws.

(a)          (i) Violate any Anti-Terrorism Laws, (ii) engage in any transaction, investment, undertaking or activity that conceals the identity, source or destination of the

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proceeds from any category of prohibited offenses designated by the Organization for Economic Co-operation and Development’s Financial Action Task Force on Money Laundering or (iii) permit any of their respective Affiliates to violate these laws or engage in these actions.

(b)          (i) Deal in, or otherwise engage in any transaction related to, any property or interests in property blocked pursuant to any Anti-Terrorism Law, (ii) engage in or conspire to engage in any transaction that evades or avoids, or has the purpose of evading or avoiding, or attempt to violate, any of the prohibitions set forth in any Anti-Terrorism Law.

(c)          Become an “investment company” or a company controlled by an “investment company” under the Investment Company Act of 1940, as amended.

Section 7.17 Minimum Liquidity. Permit the Liquidity Amount to be less than $10,000,000 at any time.

Section 7.18 Immaterial Subsidiaries. Any Immaterial Subsidiary existing on or after the Closing Date shall no longer constitute an Immaterial Subsidiary (and shall otherwise be subject to the requirements of Section 6.17) to the extent (a) in the case of any Immaterial Subsidiary formed or acquired after the Closing Date, such Immaterial Subsidiary shall at any time have (i) assets with a fair market value in excess of $250,000 or (ii) annual revenues (excluding intercompany revenues) in excess of $250,000 (excluding intercompany accounts) or (b) such Immaterial Subsidiary, together with all other Immaterial Subsidiaries, shall at any time have (i) assets with a fair market value in excess of $3,000,000 (excluding intercompany accounts) or (ii) annual revenues (excluding intercompany revenues) in excess of $3,000,000.

ARTICLE VIII

EVENTS OF DEFAULT AND REMEDIES

Section 8.01 Events of Default. Any of the following events referred to in this Section 8.01 shall constitute an “Event of Default”:

(a)          Non-Payment. Any Loan Party fails to pay (i) when and as required to be paid herein, any amount of principal of any Loan or (ii) within three (3) Business Days after the same becomes due in cash, any interest on any Loan or any other amount payable hereunder or with respect to any other Loan Document; or

(b)          Specific Covenants. The Borrower fails to perform or observe any term, covenant or agreement contained in any of Section 6.01(a), Section 6.01(d). Section 6.01(f), Section 6.03(a), Section 6.05, Section 6.07, Section 6.10(b), Section 6.12, Section 6.20 or Article VII; or

(c)          Other Defaults. Any Loan Party fails to perform or observe any other covenant or agreement (not specified in Section 8.01(a) or (b) above) contained in any Loan Document on its part to be performed or observed and such failure continues for fifteen (15) days after receipt by the Borrower of written notice thereof by the Administrative Agent or the Required Lender; or

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(d)          Representations and Warranties. Any representation, warranty, certification or statement of fact made or deemed made by or on behalf of any Loan Party herein, in any other Loan Document, or in any document required to be delivered in connection herewith or therewith shall be incorrect or misleading in any material respect when made or deemed made; or

(e)          Cross-Default. Any Loan Party or any Subsidiary (A) fails to make any payment beyond the applicable grace period with respect thereto, if any (whether by scheduled maturity, required prepayment, acceleration, demand, or otherwise) in respect of any Indebtedness (other than Indebtedness hereunder) having an aggregate principal amount of not less than the Threshold Amount, or (B) fails to observe or perform any other agreement or condition relating to any such Indebtedness, or any other event occurs, the effect of which default or other event is to cause, or to permit the holder or holders of such Indebtedness (or a trustee or agent on behalf of such holder or holders or beneficiary or beneficiaries) to cause, with the giving of notice if required, such Indebtedness to become due or to be repurchased, prepaid, defeased or redeemed (automatically or otherwise), or an offer to repurchase, prepay, defease or redeem such Indebtedness to be made, prior to its stated maturity; or

(f)          Insolvency Proceedings, Etc. Any Loan Party or any Subsidiary institutes or consents to the institution of any proceeding under any Debtor Relief Law, or makes a general assignment for the benefit of creditors; or applies for or consents to the appointment of any receiver, interim receiver, receiver and manager, trustee, custodian, conservator, liquidator, Irish law examiner, rehabilitator, administrator, administrative receiver or similar officer for it or for all or any material part of its property; or any receiver, interim receiver, receiver and manager, trustee, custodian, conservator, liquidator, Irish law examiner, rehabilitator, administrator, administrative receiver or similar officer is appointed, without the application or consent of such Person and the appointment continues undischarged or unstayed for sixty (60) calendar days; or any proceeding under any Debtor Relief Law relating to any such Person or to all or any material part of its property is instituted without the consent of such Person and continues undismissed or unstayed for sixty (60) calendar days; or an order for relief is entered in any such proceeding; provided, that a dissolution of any Subsidiary of Borrower or any Subsidiary of Amryt Pharmaceuticals DAC permitted under Section 7.04 shall not constitute an Event of Default under this clause (f); or

(g)          UK Insolvency Event. Any UK Insolvency Event occurs with respect to any UK Loan Party; or

(h)          Inability to Pay Debts; Attachment. (i) Any Loan Party or any Subsidiary becomes unable or admits in writing its inability or fails generally to pay its debts as they become due, or (ii) any writ or warrant of attachment or execution or similar process in respect of a claim in excess of the Threshold Amount is issued or levied against all or any material part of the property of the Loan Parties and their Subsidiaries, taken as a whole, and is not released, vacated, stayed or fully bonded within sixty (60) days after its issue or levy; or

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(i)          Judgments. There is entered against any Loan Party or any Subsidiary a final judgment or order for the payment of money in an aggregate amount exceeding the Threshold Amount (to the extent not covered by independent third-party insurance as to which the insurer has been notified of such judgment or order and does not deny or fail to confirm coverage) and such judgment or order shall not have been satisfied, vacated, discharged or stayed or bonded pending an appeal for a period of sixty (60) consecutive days; provided, that with respect to the Brazilian litigation matter disclosed to the Lenders prior to the Closing Date, the first $[***] of any such judgement arising from such matter shall not count towards the Threshold Amount calculation; or

(j)          ERISA. (i) An ERISA Event occurs with respect to a Pension Plan or Multiemployer Plan which has resulted or would reasonably be expected to result in liability of any Loan Party under Title IV of ERISA in an aggregate amount which would reasonably be expected to exceed the Threshold Amount, (ii) any Loan Party, any Subsidiary or any ERISA Affiliate fails to pay when due, after the expiration of any applicable grace period, any installment payment with respect to its Withdrawal Liability under Section 4201 of ERISA under a Multiemployer Plan in an aggregate amount which would reasonably be expected to exceed the Threshold Amount, or (iii) any Loan Party, any Subsidiary or any ERISA Affiliate shall have been notified by the sponsor of a Multiemployer Plan that such Multiemployer Plan is in reorganization or is being terminated, within the meaning of Title IV of ERISA, and as a result of such reorganization or termination the aggregate annual contributions of the Loan Parties, the Subsidiaries and the ERISA Affiliates to all Multiemployer Plans that are then in reorganization or being terminated have been or will be increased over the amounts contributed to such Multiemployer Plans for the plan years of such Multiemployer Plans immediately preceding the plan year in which such reorganization or termination occurs by an aggregate amount which would reasonably be expected to exceed the Threshold Amount; or

(k)          Invalidity of Loan Documents. Any material provision of any Loan Document, at any time after its execution and delivery and for any reason other than as expressly permitted hereunder or thereunder or the satisfaction in full of all the Obligations, ceases to be in full force and effect; or any Loan Party contests in any manner the validity or enforceability of any provision of any Loan Document; or any Loan Party denies that it has any or further liability or obligation under any Loan Document (other than as a result of repayment in full of the Obligations and termination of the Aggregate Commitments), purports to revoke or rescind any Loan Document or asserts that any Collateral Document is invalid or unenforceable; or

(1)          Change of Control; Structure. There occurs any Change of Control; or

(m)          Liens. Any Collateral Document shall for any reason cease to create a valid and perfected Lien (having the priorities specified therein) on and security interest in the Collateral; or

(n)          Dissolution or Liquidation. Any Loan Party voluntarily or involuntarily dissolves or is dissolved, liquidates or is liquidated or files a motion with a bankruptcy

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court seeking authorization to dissolve or liquidate other than as provided in Section 7.04; or

(o)          Failure to Conduct Business. If any Loan Party is enjoined, restrained or in any way prevented by court order from continuing to conduct all or any material part of its business affairs or any Loan Party or any of their respective Subsidiaries’ cessation of all or any material part of its business operations (other than in connection with a sale of assets permitted by the Loan Documents or otherwise consented to by the Required Lenders); or

(p)          Independent Directors. With respect to the board of directors of the Parent, the independent directors no longer constitute the number of independent directors required by the SEC, AIM or other applicable market.

Section 8.02 Remedies Upon Event of Default. (a) If any Event of Default occurs and is continuing, the Administrative Agent shall (subject to Article 9 of this Agreement), at the request of the Required Lenders, take any or all of the following actions:

(i)          declare the commitment of each Lender to make Loans to be terminated, whereupon such commitments shall be terminated;

(ii)          declare the unpaid principal amount of all outstanding Loans, all interest accrued and unpaid thereon, and all other amounts owing or payable hereunder or under any other Loan Document to be immediately due and payable, without presentment, demand, protest or other notice of any kind, all of which are hereby expressly waived by the Borrower;

(iii)          set-off against any outstanding Obligations amounts held for the account of the Loan Parties as cash collateral or in the accounts of any Loan Party maintained by or with the Administrative Agent, any Lender or their respective Affiliates; and

(iv)          take any action or exercise on behalf of itself and the Lenders all rights and remedies available to it and the Lenders under the Loan Documents or applicable Law; provided, that notwithstanding the foregoing, upon the occurrence of an Event of Default under Section 8.01(f), all Commitments shall be automatically terminated and all Obligations shall automatically become due and payable.

(b)          If an Event of Default has occurred and is continuing: (i) the Administrative Agent shall have for the benefit the Secured Parties, in addition to all other rights of the Administrative Agent and the Lenders, the rights and remedies of a secured party under the Uniform Commercial Code; (ii) the Administrative Agent may, at any time, take possession of the Collateral and keep it on any Loan Party’s premises, at no cost (including any charge pursuant to Section 506(c) of the Bankruptcy Code) to the Administrative Agent or any Lender, or remove any part of it to such other place or places as the Administrative Agent may desire, or the Borrower shall, upon the Administrative Agent’s demand, at the Borrower’s cost, assemble the Collateral and make it available to the Administrative Agent at a place or places reasonably convenient to the Administrative Agent; and (iii) the Administrative Agent may sell and deliver

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any Collateral at public or private sales, for cash, upon credit or otherwise, at such prices and upon such terms as the Administrative Agent deems advisable at the direction of the Required Lenders, and may, if the Administrative Agent at the direction of the Required Lenders deems it reasonable, postpone or adjourn any sale of the Collateral by an announcement at the time and place of sale or of such postponed or adjourned sale without giving a new notice of sale. Without in any way requiring notice to be given in the following manner, the Loan Parties agree that any notice by the Administrative Agent of sale, disposition or other intended action hereunder or in connection herewith, whether required by the Uniform Commercial Code or otherwise, shall constitute reasonable notice to the Loan Parties if such notice is mailed by registered or certified mail, return receipt requested, postage prepaid, or is delivered personally against receipt to the Borrower, at least ten (10) Business Days prior to such action to the Borrower’s address specified herein. If any Collateral is sold on terms other than payment in full at the time of sale, no credit shall be given against the Obligations until the Administrative Agent or the Lenders receive payment, and if the buyer defaults in payment, the Administrative Agent may resell the Collateral without further notice to the Loan Parties. In the event the Administrative Agent seeks to take possession of all or any portion of the Collateral by judicial process, the Loan Parties irrevocably waives: (A) the posting of any bond, surety or security with respect thereto which might otherwise be required; (B) any demand for possession prior to the commencement of any suit or action to recover the Collateral; and (C) any requirement that the Administrative Agent retain possession and not dispose of any Collateral until after trial or final judgment. The Loan Parties agree that the Administrative Agent has no obligation to preserve rights to the Collateral or marshal any Collateral for the benefit of any Person. The Administrative Agent is hereby granted a license or other right to use, without charge, but subject to the terms of the of licenses to the Loan Parties with respect to Intellectual Property licensed to the Loan Parties, the Loan Parties’ Intellectual Property and advertising matter, or any similar property, in completing production of, advertising or selling any Collateral, provided, that such licenses to be granted hereunder with respect to trademarks and service marks shall be subject to the maintenance of quality standards with respect to the goods and services on which such trademarks and service marks are used sufficient to preserve the validity and enforceability of such trademark and service marks and the applicable Loan Party’s rights under all licenses and all franchise agreements shall inure to the Administrative Agent’s benefit for such purpose. The proceeds of sale shall be applied first to all expenses of sale, including attorneys’ fees, and then to the Obligations in accordance with Section 8.03.

Section 8.03 Application of Funds. If the circumstances described in Section 2.08(f) have occurred, or after the exercise of remedies provided for in Section 8.02 any amounts received on account of the Obligations shall be applied by the Administrative Agent in the following order:

First, to payment of that portion of the Obligations constituting fees, indemnities, expenses and other amounts (other than principal and interest, but including Attorney Costs payable under Section 10.04 and amounts payable under Article III) payable to the Administrative Agent in its capacity as such;

Second, to payment of that portion of the Obligations constituting fees, indemnities and other amounts (other than principal and interest) payable to the Lenders (including Attorney Costs payable under Section 10.04 and amounts payable under

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Article III), ratably among them in proportion to the amounts described in this clause Second payable to them;

Third, to payment of that portion of the Obligations constituting accrued and unpaid interest and fees in respect of the Loans, ratably among the Lenders in proportion to the respective amounts described in this clause Third payable to them;

Fourth, to payment of that portion of the Obligations constituting unpaid principal or face amounts of the Loans, ratably among the Lenders in proportion to the respective amounts described in this clause Fourth held by them;

Fifth, to the payment of all other Obligations of the Loan Parties that are due and payable to the Administrative Agent and the other Secured Parties on such date, ratably based upon the respective aggregate amounts of all such Obligations owing to the Administrative Agent and the other Secured Parties on such date; and

Last, the balance, if any, after all of the Obligations have been indefeasibly paid in full, for the account of and paid to the Loan Parties or whoever may be lawfully entitled thereto.

The Loan Parties shall remain liable for any deficiency.

ARTICLE IX

ADMINISTRATIVE AGENT AND OTHER AGENTS

Section 9.01 Appointment and Authorization. (a) Each Lender hereby irrevocably appoints, designates and authorizes the Administrative Agent to take such action on its behalf under the provisions of this Agreement and each other Loan Document and to exercise such powers and perform such duties as are expressly delegated to it by the terms of this Agreement or any other Loan Document, together with such powers as are reasonably incidental thereto. Notwithstanding any provision to the contrary contained in this Agreement or in any other Loan Document, the Administrative Agent shall have no duties or responsibilities, except those expressly set forth herein, nor shall the Administrative Agent have or be deemed to have any fiduciary relationship with any Lender or Participant, and no implied covenants, functions, responsibilities, duties, obligations or liabilities shall be read into this Agreement or any other Loan Document or otherwise exist against the Administrative Agent. Without limiting the generality of the foregoing sentence, the use of the term “agent” herein and in the other Loan Documents with reference to the Administrative Agent is not intended to connote any fiduciary or other implied (or express) obligations arising under agency doctrine of any applicable Law. Instead, such term is used merely as a matter of market custom, and is intended to create or reflect only an administrative relationship between independent contracting parties.

Notwithstanding any provision contained in this Agreement providing for any action in the Administrative Agent’s reasonable discretion or approval of any action or matter in the Administrative Agent’s reasonable satisfaction, the Administrative Agent shall not have any duty to take any discretionary action or exercise any discretionary powers, except discretionary rights and powers expressly contemplated hereby or by the other Loan Documents that the

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Administrative Agent is required to exercise as directed in writing by the Required Lenders (or such other number or percentage of the Lenders as shall be expressly provided for herein or in the other Loan Documents) which may be delivered by electronic transmission (including e-mail by such Lenders or counsel to the Required Lenders (which on the date hereof is Latham & Watkins LLP); provided that the Administrative Agent shall not be required to take any action that, in its opinion or the opinion of its counsel, may expose the Administrative Agent to liability or that is contrary to any Loan Document or applicable Law and shall, in the Administrative Agent’s sole discretion, be accompanied by indemnity or security satisfactory to the Administrative Agent and subject to the indemnification set forth in Section 9.07. The Administrative Agent shall not, except as expressly set forth herein and in the other Loan Documents, have any duty to disclose, and shall not be liable for the failure to disclose, any information relating to the Borrower, any other Loan Party or any of their respective Affiliates that is communicated to or obtained by the Person serving as the Administrative Agent or any other Agent-Related Person in any capacity.

The Administrative Agent shall not be responsible for or have any duty to ascertain or inquire into (i) any statement, warranty or representation made in or in connection with this Agreement or any other Loan Document, (ii) the contents of any certificate, report or other document delivered hereunder or thereunder or in connection herewith or therewith, (iii) the performance or observance of any of the covenants, agreements or other terms or conditions set forth herein or therein or the occurrence of any Default, (iv) the validity, enforceability, effectiveness or genuineness of this Agreement, any other Loan Document or any other agreement, instrument or document, or the creation, perfection or priority of any Lien purported to be created by the Collateral Documents, (v) the value or the sufficiency of any Collateral, or (vi) the satisfaction of any condition set forth in Article IV or elsewhere herein, other than to confirm receipt of items expressly required to be delivered to the Administrative Agent.

(b)          The Administrative Agent shall also act as the “collateral agent” under the Loan Documents, and each of the Lenders (in its capacity as a Lender) hereby irrevocably appoints and authorizes the Administrative Agent to act as the agent of (and to hold any security interest, charge or other Lien created by the Collateral Documents for and on behalf of or on trust for) such Lender for purposes of acquiring, holding and enforcing any and all Liens on Collateral granted by any of the Loan Parties to secure any of the Obligations, together with such powers and discretion as are reasonably incidental thereto. In this connection, the Administrative Agent, as “collateral agent” (and any co-agents, sub-agents and attorneys-in-fact appointed by the Administrative Agent pursuant to Section 9.02 for purposes of holding or enforcing any Lien on the Collateral (or any portion thereof) granted under the Collateral Documents, or for exercising any rights and remedies thereunder at the direction of the Administrative Agent), shall be entitled to the benefits of all provisions of this Article IX (including Section 9.07, as though such co-agents, sub-agents and attorneys-in-fact were the “collateral agent” under the Loan Documents) as if set forth in full herein with respect thereto.

Section 9.02 Delegation of Duties. The Administrative Agent may execute any of its duties under this Agreement or any other Loan Document (including for purposes of holding or enforcing any Lien on the Collateral (or any portion thereof) granted under the Collateral Documents or of exercising any rights and remedies thereunder) by or through

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Affiliates, agents, employees or attorneys-in-fact, such sub-agents as shall be deemed necessary by the Administrative Agent, and shall be entitled to advice of counsel, both internal and external, and other consultants or experts concerning all matters pertaining to such duties. The Administrative Agent shall not be responsible for the negligence or misconduct of any agent or sub-agent or attorney-in-fact that it selects in the absence of gross negligence or willful misconduct as determined by a final nonappealable judgment of a court of competent jurisdiction.

Section 9.03 Liability of the Administrative Agent. No Agent-Related Person shall (a) be liable to any Lender for any action taken or omitted to be taken by any of them under or in connection with this Agreement or any other Loan Document or the transactions contemplated hereby (except for its own gross negligence or willful misconduct, as determined by the final nonappealable judgment of a court of competent jurisdiction, in connection with its duties expressly set forth herein), or (b) be responsible in any manner to any Lender or Participant for any recital, statement, representation or warranty made by any Loan Party or any officer thereof, contained herein or in any other Loan Document, or in any certificate, report, statement or other document referred to or provided for in, or received by the Administrative Agent under or in connection with, this Agreement or any other Loan Document, or the validity, effectiveness, genuineness, enforceability or sufficiency of this Agreement or any other Loan Document, or the perfection or priority of any Lien or security interest created or purported to be created under the Collateral Documents, or for any failure of any Loan Party or any other party to any Loan Document to perform its obligations hereunder or thereunder. No Agent-Related Person shall be under any obligation to any Lender or Participant to ascertain or to inquire as to the observance or performance of any of the agreements contained in, or conditions of, this Agreement or any other Loan Document, or to inspect the properties, books or records of any Loan Party or any Affiliate thereof. The Administrative Agent shall not be responsible or liable for special, indirect, punitive or consequential loss or damage of any kind whatsoever (including, but not limited to, loss of profit) irrespective of whether the Administrative Agent has been advised of the likelihood of such loss or damage and regardless of the form of action. In no event shall the Administrative Agent be responsible or liable for any failure or delay in the performance of its obligations hereunder arising out of or caused by, directly or indirectly, forces beyond its control, including without limitation, strikes, work stoppages, accidents, acts of war or terrorism, civil or military disturbances, nuclear or natural catastrophes or acts of God, future changes in applicable law or regulation, and interruptions, loss or malfunctions of utilities, communications or computer (software and hardware) services; it being understood that the Administrative Agent shall use reasonable efforts consistent with accepted practices in the banking industry to resume performance as soon as practicable under the circumstances.

Section 9.04 Reliance by the Administrative Agent. (a) The Administrative Agent shall be entitled to rely, and shall be fully protected in relying, upon any writing, communication, signature, resolution, representation, notice, consent, certificate, affidavit, letter, telegram, facsimile, telex or telephone message, electronic mail message, statement or other document or conversation believed by it in good faith to be genuine and correct and to have been signed, sent or made by the proper Person or Persons, and upon advice and statements of legal counsel (including counsel to any Loan Party), independent accountants and other experts selected by the Administrative Agent. The Administrative Agent shall be fully justified in failing or refusing to take any action under any Loan Document unless it shall first receive such

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advice or concurrence of the Required Lenders as it deems appropriate and, if it so requests, it shall first be indemnified to its satisfaction by the Lenders against any and all liability and expense which may be incurred by it by reason of taking or continuing to take any such action. The Administrative Agent shall in all cases be fully protected in acting, or in refraining from acting, under this Agreement or any other Loan Document in accordance with a request or consent of the Required Lenders (or such greater number of Lenders as may be expressly required hereby in any instance) and such request and any action taken or failure to act pursuant thereto shall be binding upon all the Lenders.

(b)          For purposes of determining compliance with the conditions specified in Section 4.01, each Lender that has signed this Agreement shall be deemed to have consented to, approved or accepted or to be satisfied with, each document or other matter required thereunder to be consented to or approved by or acceptable or satisfactory to a Lender unless the Administrative Agent shall have received notice from such Lender prior to the proposed Closing Date specifying its objection thereto.

Section 9,05 Notice of Default. The Administrative Agent shall not be deemed to have knowledge or notice of the occurrence of any Default, except with respect to defaults in the payment of principal, in interest and fees required tobe paid to the Administrative Agent for the  account of the Lenders, unless the Administrative Agent shall have received written notice from a Lender or the Borrower referring to this Agreement, describing such Default and stating that such notice is a “notice of default”. The Administrative Agent will promptly notify the Lenders of its receipt of any such notice. The Administrative Agent shall take such action with respect to any Event of Default as may be directed by the Required Lenders in accordance with Article VIII; provided that unless and until the Administrative Agent has received any such direction, the Administrative Agent may (but shall not be obligated to) take such action, or refrain from taking such action, with respect to such Event of Default as it shall deem advisable or in the best interest of the Lenders.

Section 9.06 Credit Decision; Disclosure of Information by the Administrative Agent. Each Lender acknowledges that no Agent-Related Person has made any representation or warranty to it, and that no act by the Administrative Agent hereafter taken, including any consent to and acceptance of any assignment or review of the affairs of any Loan Party or any Affiliate thereof, shall be deemed to constitute any representation or warranty by any Agent-Related Person to any Lender as to any matter, including whether Agent-Related Persons have disclosed material information in their possession. Each Lender represents to the Administrative Agent that it has, independently and without reliance upon any Agent-Related Person and based on such documents and information as it has deemed appropriate, made its own appraisal of and investigation into the business, prospects, operations, property, financial and other condition and creditworthiness of the Loan Parties and their respective Subsidiaries, and all applicable bank or other regulatory Laws relating to the transactions contemplated hereby, and made its own decision to enter into this Agreement and to extend credit to the Borrower and the other Loan Parties hereunder. Each Lender also represents that it will, independently and without reliance upon any Agent-Related Person and based on such documents and information as it shall deem appropriate at the time, continue to make its own credit analysis, appraisals and decisions in taking or not taking action under this Agreement and the other Loan Documents, and to make such investigations as it deems necessary to inform itself as to the business, prospects,

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operations, property, financial and other condition and creditworthiness of the Borrower and the other Loan Parties. Except for notices, reports and other documents expressly required to be furnished to the Lenders by the Administrative Agent herein, the Administrative Agent shall not have any duty or responsibility to provide any Lender with any credit or other information concerning the business, prospects, operations, property, financial and other condition or creditworthiness of any of the Loan Parties or any of their respective Affiliates which may come into the possession of any Agent-Related Person.

Section 9.07 Indemnification of the Administrative Agent. Whether or not the transactions contemplated hereby are consummated, the Lenders shall indemnify upon demand each Agent-Related Person (to the extent not reimbursed by or on behalf of any Loan Party and without limiting the obligation of any Loan Party to do so), pro rata, and hold harmless each Agent-Related Person from and against any and all Indemnified Liabilities to the extent incurred by it; provided that no Lender shall be liable for the payment to any Agent-Related Person of any portion of such Indemnified Liabilities to the extent resulting from such Agent-Related Person’s own gross negligence or willful misconduct, as determined by the final non-appealable judgment of a court of competent jurisdiction; provided that no action taken in accordance with the directions of the Required Lenders (or such other number or percentage of the Lenders as shall be required by the Loan Documents) shall be deemed to constitute gross negligence or willful misconduct for purposes of this Section 9.07. In the case of any investigation, litigation or proceeding giving rise to any Indemnified Liabilities, this Section 9.07 applies whether any such investigation, litigation or proceeding is brought by any Lender or any other Person. Without limitation of the foregoing, each Lender shall reimburse the Administrative Agent upon demand for its ratable share of any costs or out-of-pocket expenses (including Attorney Costs) incurred by the Administrative Agent in connection with the preparation, execution, delivery, administration, modification, amendment or enforcement (whether through negotiations, legal proceedings or otherwise) of, or legal advice in respect of rights or responsibilities under, this Agreement, any other Loan Document, or any document contemplated by or referred to herein, to the extent that the Administrative Agent is not reimbursed for such expenses by or on behalf of the Borrower; provided that such reimbursement by the Lenders shall not affect the Borrower’s continuing reimbursement obligations with respect thereto, if any. The undertaking in this Section 9.07 shall survive termination of the Aggregate Commitments, the payment of all other Obligations and the resignation or removal of the Administrative Agent.

Section 9.08 The Administrative Agent in its Individual Capacity. The Administrative Agent and its Affiliates may make loans to, issue letters of credit for the account of, accept deposits from, acquire Equity Interests in and generally engage in any kind of banking, trust, financial advisory, underwriting or other business with each of the Loan Parties and their respective Affiliates as though the Administrative Agent were not the Administrative Agent hereunder and without notice to or consent of the Lenders. The Lenders acknowledge that, pursuant to such activities, the Administrative Agent or its Affiliates may receive information regarding any Loan Party or any Affiliate of a Loan Party (including information that may be subject to confidentiality obligations in favor of such Loan Party or such Affiliate) and acknowledge that the Administrative Agent shall not be under any obligation to provide such information to them. With respect to its Loans, the Administrative Agent shall have the same rights and powers under this Agreement as any other Lender and may exercise such rights and

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powers as though it were not the Administrative Agent, and the terms “Lender” and “Lenders” include Cantor Fitzgerald Securities in its individual capacity.

Section 9.09 Successor Agents. The Administrative Agent may resign as the Administrative Agent upon thirty (30) days’ notice to the Lenders and the Borrower. If the Administrative Agent resigns under this Agreement, the Required Lenders shall appoint a successor agent for the Lenders. If no successor agent is appointed prior to the effective date of the resignation of the Administrative Agent, the retiring Administrative Agent may appoint, after consulting with the Lenders, a successor agent from among the Lenders. Upon the acceptance of its appointment as successor agent hereunder, the Person acting as such successor agent shall succeed to all the rights, powers and duties of the retiring Administrative Agent and the term “Administrative Agent”, shall mean such successor administrative agent and/or supplemental administrative agent, as the case may be, and the retiring Administrative Agent’s appointment, powers and duties as the Administrative Agent shall be terminated. After the retiring Administrative Agent’s resignation hereunder as the Administrative Agent, the provisions of this Article IX and Section 10.04 and Section 10.05 shall inure to its benefit as to any actions taken or omitted to be taken by it while it was the Administrative Agent under this Agreement. If no successor agent has accepted appointment as the Administrative Agent by the date which is thirty (30) days following the retiring Administrative Agent’s notice of resignation, the retiring Administrative Agent’s resignation shall nevertheless thereupon become effective and the Lenders shall perform all of the duties of the Administrative Agent hereunder until such time, if any, as the Required Lenders appoint a successor agent as provided for above. Lenders assuming the role of Administrative Agent as specified in the immediately preceding sentence shall assume the rights and obligations of the Administrative Agent (including the indemnification provisions set forth in Section 9.07) as if each such Lender were the Administrative Agent. Upon the acceptance of any appointment as the Administrative Agent hereunder by a successor and upon the execution and filing or recording of such financing statements, or amendments thereto, and such amendments or supplements to the Mortgages, and such other instruments or notices, as may be necessary or desirable, or as the Required Lenders may reasonably request, in order to continue the perfection of the Liens granted or purported to be granted by the Collateral Documents, the successor Administrative Agent shall thereupon succeed to and become vested with all the rights, powers, discretion, privileges, and duties of the retiring Administrative Agent, and the retiring Administrative Agent shall be discharged from its duties and obligations under the Loan Documents.

Section 9.10 Administrative Agent May File Proofs of Claim. The Administrative Agent (irrespective of whether the principal of any Loan shall then be due and payable as herein expressed or by declaration or otherwise and irrespective of whether the Administrative Agent shall have made any demand on the Borrower) shall be entitled and empowered, by intervention in such proceeding or otherwise:

(a)          to file and prove an administrative claim for the whole amount of the principal and interest owing and unpaid in respect of the Loans and all other Obligations that are owing and unpaid and to file such other documents as may be necessary or advisable in order to have the claims of the Lenders and the Administrative Agent (including any claim for the reasonable compensation, expenses, disbursements and advances of the Lenders and the Administrative Agent and their respective agents and

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counsel and all other amounts due the Lenders and the Administrative Agent under Section 2.05 and Section 10.04 or otherwise hereunder) allowed in an applicable proceeding; and

(b)          to collect and receive any monies or other property payable or deliverable on any such claims and to distribute the same; and

(c)          any custodian, receiver, assignee, trustee, liquidator, sequestrator or other similar official in any such judicial proceeding is hereby authorized by each Lender to make such payments to the Administrative Agent and, in the event that the Administrative Agent shall consent to the making of such payments directly to the Lenders, to pay to the Administrative Agent any amount due for the reasonable compensation, expenses, disbursements and advances of the Administrative Agent and its agents and counsel, and any other amounts due to the Administrative Agent under Section 2.05 and Section 10.04 or otherwise hereunder.

Nothing contained herein shall be deemed to authorize the Administrative Agent to authorize or consent to or accept or adopt on behalf of any Lender any plan of reorganization, arrangement, adjustment or composition affecting the Obligations or the rights of any Lender or to authorize the Administrative Agent to vote in respect of the claim of any Lender in any such proceeding.

Section 9.11 Release of Collateral and Guarantee. The Lenders irrevocably agree and authorize the Administrative Agent:

(a)          to release any Lien on any property granted to or held by the Administrative Agent under any Loan Document (i) upon termination of the Aggregate Commitments and payment in full in cash of all Obligations (other than (A) contingent indemnification obligations not yet accrued and payable and (B) any other obligation (including a guarantee) that is contingent in nature) (the date upon which the conditions in this Section 9.1 l(a)(i) shall have been satisfied, the “Termination Date”), (ii) upon any permitted sale, lease, transfer or other disposition of any item of Collateral of any Loan Party (including, without limitation, as a result of the sale, in accordance with the terms of the Loan Documents, of the Loan Party that owns such Collateral) in accordance with the terms of the Loan Documents, (iii) subject to Section 10.01, if the release of such Lien is approved, authorized or ratified in writing by the Required Lenders, or (iv) if the property subject to such Lien is owned by a Guarantor, upon release of such Guarantor from its obligations under the Guarantee and Collateral Agreement pursuant to clause (b) below; and

(b)          in the case of any Subsidiary, such Person ceasing to be subject to Section 6.11 as a result of a transaction permitted hereunder (as certified by a Responsible Officer) and the Borrower notifying the Administrative Agent in writing that it wishes such Guarantor to be released from its obligations under the Guarantee and Collateral Agreement.

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The Administrative Agent will, at the Borrower’s expense, execute and deliver to such Loan Party such documents as such Loan Party may reasonably request to evidence the release of Collateral pursuant to this Section 9.11 from the assignment and security interest granted under the Collateral Documents (or the release of the Guarantor from its Guarantee Obligations in respect of the Obligations) in accordance with the terms of the Loan Documents (provided that the Borrower shall have delivered to the Administrative Agent a certificate of a Responsible Officer certifying that such transaction has been consummated in compliance with the Loan Documents and the execution and delivery of such documents are authorized and permitted under the Loan Documents, and the Administrative Agent may conclusively rely on such certification without further inquiry). Upon request by the Administrative Agent at any time, the Required Lenders will confirm in writing the Administrative Agent’s authority to release its interest in particular types or items of property in accordance with this Section 9.11.

Section 9.12 Other Agents; Arrangers and Managers. None of the Lenders shall have any right, power, obligation, liability, responsibility or duty under this Agreement other than those applicable to all Lenders as such. Without limiting the foregoing, none of the Lenders shall have or be deemed to have any fiduciary relationship with any other Lender. Each Lender acknowledges that it has not relied, and will not rely, on any of the other lenders in deciding to enter into this Agreement or in taking or not taking action hereunder.

Section 9,13 Appointment of Supplemental Administrative Agent. (a) It is the purpose of this Agreement and the other Loan Documents that there shall be no violation of any Law of any jurisdiction denying or restricting the right of banking corporations or associations to transact business as agent or trustee in such jurisdiction. It is recognized that in case of litigation under this Agreement or any of the other Loan Documents, and in particular in case of the enforcement of any of the Loan Documents, or in case the Administrative Agent deems in its reasonable discretion that by reason of any present or future Law of any jurisdiction it may not exercise any of the rights, powers or remedies granted herein or in any of the other Loan Documents or take any other action which may be desirable or necessary in connection therewith, the Administrative Agent is hereby authorized to appoint an additional individual or institution selected by the Administrative Agent in its sole discretion as a separate trustee, co-trustee, administrative agent, collateral agent, administrative sub-agent or administrative co-agent (any such additional individual or institution being referred to herein individually as a “Supplemental Administrative Agent” and collectively as “Supplemental Administrative Agents”).

(b)          In the event that the Administrative Agent appoints a Supplemental Administrative Agent with respect to any Collateral, (i) each and every right, power, privilege or duty expressed or intended by this Agreement or any of the other Loan Documents to be exercised by or vested in or conveyed to the Administrative Agent with respect to such Collateral shall be exercisable by and vest in such Supplemental Administrative Agent to the extent, and only to the extent, necessary to enable such Supplemental Administrative Agent to exercise such rights, powers and privileges with respect to such Collateral and to perform such duties with respect to such Collateral, and every covenant and obligation contained in the Loan Documents and necessary to the exercise or performance thereof by such Supplemental Administrative Agent shall run to and be enforceable by either the Administrative Agent or such Supplemental Administrative Agent, and (ii) the provisions of this Article IX and of Section 10.04 and

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Section 10.05 that refer to the Administrative Agent shall inure to the benefit of such Supplemental Administrative Agent and all references therein to the Administrative Agent shall be deemed to be references to the Administrative Agent and/or such Supplemental Administrative Agent, as the context may require.

(c)          Should any instrument in writing from any Loan Party be required by any Supplemental Administrative Agent so appointed by the Administrative Agent for more fully and certainly vesting in and confirming to him or it such rights, powers, privileges and duties, the Borrower shall, or shall cause such Loan Party to, execute, acknowledge and deliver any and all such instruments promptly upon request by the Administrative Agent. In case any Supplemental Administrative Agent, or a successor thereto, shall die, become incapable of acting, resign or be removed, all the rights, powers, privileges and duties of such Supplemental Administrative Agent, to the extent permitted by Law, shall vest in and be exercised by the Administrative Agent until the appointment of a new Supplemental Administrative Agent.

ARTICLE X

MISCELLANEOUS

Section 10.01 Amendments, Etc. No amendment or waiver of any provision of this Agreement or any Loan Document, nor consent to any departure by any Loan Party therefrom, shall in any event be effective unless the same shall be in writing and signed by the Required Lenders, the Borrower and the Loan Parties party to such Loan Document, and then such waiver or consent shall be effective only in the specific instance and for the specific purpose for which given; provided, however, that:

(a)          no amendment, waiver or consent shall, unless in writing and signed by all of the Lenders, do any of the following at any time:

(i)          change the number of Lenders or the percentage of (x) the Commitments or (y) the aggregate unpaid principal amount of Loans that, in each case, shall be required for the Lenders or any of them to take any action hereunder (including pursuant to any change to the definition of “Required Lenders”),

(ii)          release one or more Guarantors (or otherwise limit such Guarantors’ liability with respect to the Obligations owing to the Administrative Agent and the Lenders under the Guarantee and Collateral Agreement), if such release or limitation is in respect of all or substantially all of the value represented by the Guarantee and Collateral Agreement to the Lenders,

(iii)          release, or subordinate the Administrative Agent’s Liens in, all or substantially all of the Collateral in any transaction or series of related transactions (other than as expressly permitted herein),

(iv)          amend any provision of this Section 10.01;

(b)          no amendment, waiver or consent shall, unless in writing and signed by each Lender specified below for such amendment, waiver or consent:

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(i)        increase the Commitments of a Lender without the consent of such Lender;

(ii)      reduce the principal of, or stated rate of interest on, the Loans owed to a Lender or any fees or other amounts stated to be payable hereunder or under the other Loan Documents to such Lender without the consent of such Lender; provided if the Required Lenders agree to waive, or forbear from exercising remedies with respect to, any Event of Default and such waiver or forbearance is effective in accordance with this Section 10.01 or if the Required Lenders agree to change any financial definitions that would reduce the stated rate of interest or any fees or other non-principal amounts stated to be payable hereunder or under the other Loan Documents pursuant to any amendment, waiver or consent not being effected in order to reduce the stated rate of interest or such fees or other amounts, then only the consent of the Required Lenders shall be necessary to waive any obligation of the Borrower to pay interest at the Default Rate in connection with such Event of Default or reduce the stated rate of interest or such fees in connection with such amendment, waiver or consent described in this proviso to clause (b)(ii), as applicable; or

(iii)     postpone any date scheduled for any payment of principal of, or interest on, the Loans pursuant to Section 2.03 or Section 2.04, any date scheduled for payment or for any date fixed for any payment of fees hereunder in each case payable to a Lender without the consent of such Lender; or

(iv)      modify Section 8.03 in any manner that adversely affects the Lenders without the consent of each Lender directly and adversely affected thereby; or

(v)       modify Section 2.09 without the consent of each Lender directly and adversely affected thereby;

provided further that no amendment, waiver or consent shall, unless in writing and signed by the Administrative Agent in addition to the Lenders required above to take such action, affect the rights or duties of the Administrative Agent under this Agreement or the other Loan Documents.

Section 10.02 Notices and Other Communications; Facsimile and Electronic Copies. (a) General. Unless otherwise expressly provided herein, all notices and other communications provided for hereunder or under any other Loan Document shall be in writing (and, as to service of process, only in writing and in accordance with applicable law) and, to the extent set forth in Section 10.02(e), in an electronic medium and delivered as set forth in Section 10.02(e). All such written notices shall be mailed, faxed or delivered to the applicable address, facsimile number or electronic mail address, and all notices and other communications expressly permitted hereunder to be given by telephone shall be made to the applicable telephone number, as follows:

(i)        if to any Loan Party:

c/o Amryt Pharma plc

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90 Harcourt Street

Dublin 2, Ireland

Attention:   Rory Nealon

                   John McEvoy

Telephone: [***]

Email: [***]

            [***]

With a copy (which shall not constitute notice) to:

Gibson, Dunn & Crutcher LLP

200 Park Avenue

New York, NY 10166

Attention:   William Sorabella, Esq.

                   J. Eric Wise, Esq

(ii)      if to the Administrative Agent, to the address, facsimile number, electronic mail address or telephone number specified for such Person on Schedule 10.02 or to such other address, facsimile number, electronic mail address or telephone number as shall be designated by such party in a notice to the other parties from time to time; and

(iii)      if to any other Lender, to the address, facsimile number or electronic mail address specified in its Administrative Questionnaire or to such other address, facsimile number, electronic mail address or telephone number as shall be designated by such party in a written notice to the Borrower and the Administrative Agent.

All such notices and other communications shall be deemed to be given or made upon the earlier to occur of (i) actual receipt by the relevant party hereto and (ii) (A) if delivered by hand or by courier, when signed for by or on behalf of the relevant party hereto; (B) if delivered by mail, four (4) Business Days after deposit in the mails, postage prepaid; (C) if delivered by facsimile, when sent and receipt has been confirmed by telephone; and (D) if delivered by electronic mail (which form of delivery is subject to the provisions of Section 10.02(b)), when delivered; provided that notices and other communications to the Borrower and the Administrative Agent pursuant to Article II shall not be effective until actually received by such Person during the Person’s normal business hours. In no event shall a voice mail message be effective as a notice, communication or confirmation hereunder.

(b)       Effectiveness of Facsimile Documents and Signatures. Loan Documents may be transmitted and/or signed by facsimile or other electronic transmission (including a .pdf or .tif copy); provided that original copies are delivered promptly thereafter (it being understood that the failure to request or deliver the same shall not limit the effectiveness of any document or signature delivered by facsimile or electronic transmission).

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(c)       Reliance by the Administrative Agent and Lenders. The Administrative Agent and the Lenders shall be entitled to rely and act upon any notices (including telephonic Committed Loan Notices) in good faith given by or on behalf of the Borrower even if (i) such notices were not made in a manner specified herein, were incomplete or were not preceded or followed by any other form of notice specified herein, or (ii) the terms thereof, varied from any confirmation thereof. The Borrower shall indemnify each Agent-Related Person and each Lender from all losses, costs, expenses and liabilities resulting from the reliance by such Person on each notice given by or on behalf of the Borrower in the absence of gross negligence or willful misconduct by such Agent-Related Person or such Lender as determined by a final nonappealable judgment.

(d)       Notice to other Loan Parties. The Borrower agrees that notices to be given to any other Loan Party under this Agreement or any other Loan Document may be given to the Borrower in accordance with the provisions of this Section 10.02 with the same effect as if given to such other Loan Party in accordance with the terms hereunder or thereunder.

(e)       The Borrower hereby agrees that it will provide to the Administrative Agent all information, documents and other materials that it is obligated to furnish to the Administrative Agent pursuant to the Loan Documents, including, without limitation, all notices, requests, financial statements, financial and other reports, certificates and other information materials, but excluding any such communication that (i) relates to a request for a new Loan, (ii) relates to the payment of any principal or other amount due under this Agreement prior to the scheduled date therefor, (iii) provides notice of any Default or Event of Default under this Agreement or (iv) is required to be delivered to satisfy any condition precedent to the effectiveness of this Agreement and/or any Loan hereunder (all such non-excluded communications being referred to herein collectively as “Communications”), by transmitting the Communications in an electronic/soft medium in a format acceptable to the Administrative Agent to an electronic mail address specified by the Administrative Agent to the Borrower. In addition, the Borrower agrees to continue to provide the Communications to the Administrative Agent in the manner specified in the Loan Documents but only to the extent requested by the Administrative Agent. The Borrower further agrees that the Administrative Agent may make the Communications available to the Lenders by posting the Communications on IntraLinks or a substantially similar electronic transmission system (the “Platform”).

(f)       THE PLATFORM IS PROVIDED “AS IS” AND “AS AVAILABLE.” THE AGENT PARTIES (AS DEFINED BELOW) DO NOT WARRANT THE ACCURACY OR COMPLETENESS OF THE COMMUNICATIONS OR THE ADEQUACY OF THE PLATFORM AND EXPRESSLY DISCLAIM LIABILITY FOR ERRORS OR OMISSIONS IN THE COMMUNICATIONS. NO WARRANTY OF ANY KIND, EXPRESS, IMPLIED OR STATUTORY, INCLUDING, WITHOUT LIMITATION, ANY WARRANTY OF MERCHANTABILITY, FITNESS FOR A PARTICULAR PURPOSE, NON-INFRINGEMENT OF THIRD PARTY RIGHTS OR FREEDOM FROM VIRUSES OR OTHER CODE DEFECTS, IS MADE BY THE AGENT PARTIES IN CONNECTION WITH THE COMMUNICATIONS OR THE PLATFORM. IN NO EVENT SHALL THE ADMINISTRATIVE AGENT OR ANY OF ITS AFFILIATES OR ANY OF THEIR RESPECTIVE OFFICERS, DIRECTORS, EMPLOYEES, AGENTS, ADVISORS OR REPRESENTATIVES (COLLECTIVELY, “AGENT PARTIES”) HAVE ANY LIABILITY TO

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THE BORROWER, ANY LENDER OR ANY OTHER PERSON OR ENTITY FOR DAMAGES OF ANY KIND, INCLUDING, WITHOUT LIMITATION, DIRECT OR INDIRECT, SPECIAL, INCIDENTAL OR CONSEQUENTIAL DAMAGES, LOSSES OR EXPENSES (WHETHER IN TORT, CONTRACT OR OTHERWISE) ARISING OUT OF THE BORROWER’S OR THE ADMINISTRATIVE AGENT’S TRANSMISSION OF COMMUNICATIONS THROUGH THE PLATFORM, EXCEPT TO THE EXTENT THE LIABILITY OF ANY AGENT PARTY IS FOUND IN A FINAL NON-APPEALABLE JUDGMENT BY A COURT OF COMPETENT JURISDICTION TO HAVE RESULTED PRIMARILY FROM SUCH AGENT PARTY’S GROSS NEGLIGENCE OR WILLFUL MISCONDUCT.

(g)       The Administrative Agent agrees that the receipt in accordance with Section 10.02 of the Communications by the Administrative Agent at its e-mail address set forth on Schedule 10.02 shall constitute effective delivery of the Communications to the Administrative Agent for purposes of the Loan Documents. Each Lender agrees that notice to it (as provided in the next sentence) specifying that the Communications have been posted to the Platform shall constitute effective delivery of the Communications to such Lender for purposes of the Loan Documents. Each Lender agrees (i) to notify the Administrative Agent in writing (including by electronic communication) from time to time of such Lender’s e-mail address to which the foregoing notice may be sent by electronic transmission and (ii) that the foregoing notice may be sent to such e-mail address. Nothing herein shall prejudice the right of the Administrative Agent or any Lender to give any notice or other communication pursuant to any Loan Document in any other manner specified in such Loan Document.

(h)       Each Loan Party hereby acknowledges that certain of the Lenders may be “public-side” Lenders (i.e., Lenders that do not wish to receive material non-public information with respect to any Loan Party or its securities) (each, a “Public Lender”). Each Loan Party hereby agrees that (i) Communications that are to be made available on the Platform to Public Lenders who notify the Borrower and the Administrative Agent of such Lender’s status as a Public Lender shall be clearly and conspicuously marked by such Loan Party as “PUBLIC,” which, at a minimum, shall mean that the word “PUBLIC” shall appear prominently on the first page thereof, (ii) by marking Communications “PUBLIC,” each Loan Party shall be deemed to have authorized the Administrative Agent and the Lenders to treat such Communications as either publicly available information or not material information (although it may contain sensitive business information and remains subject to the confidentiality undertakings of Section 10.08) with respect to such Loan Party or its securities for purposes of United States Federal and state securities laws, (iii) all Communications marked “PUBLIC” are permitted to be made available through a portion of the Platform designated “Public Side Information,” and (iv) the Administrative Agent shall be entitled to treat any Communications that are not marked “PUBLIC” as being suitable only for posting on a portion of the Platform not designated “Public Side Information.”

(i)        EACH LENDER ACKNOWLEDGES THAT UNITED STATES FEDERAL AND STATE SECURITIES LAWS PROHIBIT ANY PERSON WITH MATERIAL, NON-PUBLIC INFORMATION ABOUT AN ISSUER FROM PURCHASING OR SELLING SECURITIES OF SUCH ISSUER OR, SUBJECT TO CERTAIN LIMITED EXCEPTIONS, FROM COMMUNICATING SUCH INFORMATION TO ANY OTHER

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PERSON. EACH LENDER AGREES TO COMPLY WITH APPLICABLE LAW AND ITS RESPECTIVE CONTRACTUAL OBLIGATIONS WITH RESPECT TO CONFIDENTIAL AND MATERIAL NON-PUBLIC INFORMATION. Each Lender that is not a Public Lender confirms to the Administrative Agent that such Lender has adopted and will maintain internal policies and procedures reasonably designed to permit such Lender to take delivery of Restricting Information (as defined below) and maintain its compliance with applicable law and its respective contractual obligations with respect to confidential and material non-public information. A Public Lender may elect not to receive Communications and Information that contains material non-public information with respect to the Loan Parties or their securities (such Communications and Information, collectively, “Restricting Information”), in which case it will identify itself to the Administrative Agent as a Public Lender. Such Public Lender shall not take delivery of Restricting Information and shall not participate in conversations or other interactions with the Agent Parties, any Lender or any Loan Party in which Restricting Information may be discussed. No Agent Party, however, shall by making any Communications and Information (including Restricting Information) available to a Lender (including any Public Lender), by participating in any conversations or other interactions with a Lender (including any Public Lender) or otherwise, be responsible or liable in any way for any decision a Lender (including any Public Lender) may make to limit or to not limit its access to the Communications and Information. In particular, no Agent Party shall have, and the Administrative Agent, on behalf of all Agent Parties, hereby disclaims, any duty to ascertain or inquire as to whether or not a Lender (including any Public Lender) has elected to receive Restricting Information, such Lender’s policies or procedures regarding the safeguarding of material nonpublic information or such Lender’s compliance with applicable laws related thereto. Each Public Lender acknowledges that circumstances may arise that require it to refer to Communications and Information that might contain Restricting Information. Accordingly, each Public Lender agrees that it will nominate at least one designee to receive Communications and Information (including Restricting Information) on its behalf and identify such designee (including such designee’s contact information) on such Public Lender’s Administrative Questionnaire. Each Public Lender agrees to notify the Administrative Agent in writing from time to time of such Public Lender’s designee’s address to which notice of the availability of Restricting Information may be sent. Each Public Lender confirms to the Administrative Agent and the Lenders that are not Public Lenders that such Public Lender Understands and agrees that the Administrative Agent and such other Lenders may have access to Restricting Information that is not available to such Public Lender and that such Public Lender has elected to make its decision to enter into this Agreement and to take or not take action under or based upon this Agreement, any other Loan Document or related agreement knowing that, so long as such Person remains a Public Lender, it does not and will not be provided access to such Restricting Information. Nothing in this Section 10.02(i) shall modify or limit a Lender’s (including any Public Lender) obligations under Section 10.08 with regard to Communications and Information and the maintenance of the confidentiality of or other treatment of Communications or Information.

Section 10.03 No Waiver; Cumulative Remedies. No failure by any Lender or the Administrative Agent to exercise, and no delay by any such Person in exercising, any right, remedy, power or privilege hereunder or under any other Loan Document shall operate as a waiver thereof; nor shall any single or partial exercise of any right, remedy, power or privilege hereunder preclude any other or further exercise thereof or the exercise of any other right, remedy, power or privilege. The rights, remedies, powers and privileges herein provided, and

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provided under each other Loan Document, are cumulative and not exclusive of any rights, remedies, powers and privileges provided by Law.

Section 10.04 Costs and Expenses. The Borrower agrees (a) to pay or reimburse the Administrative Agent and Lenders for all reasonable and documented out-of-pocket costs and expenses incurred after the Closing Date (but not before) in connection with the administration of this Agreement and the other Loan Documents, and any amendment, waiver, consent or other modification of the provisions hereof and thereof requested by the Borrower or negotiated in consultation with Borrower (in each case, whether or not the transactions contemplated thereby are consummated), including all Attorney Costs, (b) to pay or reimburse the Administrative Agent and each Lender for all reasonable and documented out-of-pocket costs and expenses incurred in connection with the enforcement of any rights or remedies under this Agreement or the other Loan Documents (including all Attorney Costs and other costs and expenses incurred in connection with any workout or restructuring in respect of the Loans and all such costs and expenses incurred during any legal proceeding and (c) without limiting the generality of the foregoing, to pay all reasonable and documented out-of-pocket fees and expenses of any financial advisory, appraisers or accounting firm retained by or for the benefit of the Administrative Agent or Lenders or by Latham & Watkins LLP, as counsel to the Lenders. The foregoing costs and expenses shall include all reasonable search., filing, recording and title insurance charges and fees related thereto, and other reasonable and documented out-of-pocket expenses incurred by the Administrative Agent. The agreements in this Section 10.04 shall survive the termination of the Aggregate Commitments and repayment of all other Obligations. All amounts due under this Section 10.04 shall be paid within ten (10) Business Days of receipt by the Borrower of an invoice relating thereto setting forth such expenses in reasonable detail. If any Loan Party fails to pay when due any costs, expenses or other amounts payable by it hereunder or under any Loan Document, such amount may be paid on behalf of such Loan Party by the Administrative Agent in its sole discretion.

Section 10.05 Indemnification by the Borrower. (a) Whether or not the transactions contemplated hereby are consummated, the Borrower shall indemnify and hold harmless the Administrative Agent, each Agent-Related Person (including, without limitation, Shipman & Goodwin LLP), each Lender, any financial advisors and/or consultants retained by the Administrative Agent or any Lender and their respective Affiliates, directors, officers, employees, counsel, agents, trustees, management companies (including employees of such management companies), advisors and attorneys-in-fact (including without limitation, Latham & Watkins LLP) (collectively the “Indemnitees”) from and against any and all liabilities, obligations, losses, taxes, damages, penalties, claims, demands, actions, judgments, suits, costs, expenses and disbursements (including one counsel to the Administrative Agent and a separate counsel to the Lenders, taken as a whole) (and, in the event of any actual conflict of interest, additional counsel to the affected parties) of any kind or nature whatsoever which may at any time be imposed on, incurred by or asserted against any such Indemnitee in any way relating to or arising out of or in connection with (i) the execution, delivery, enforcement, performance or administration of any Loan Document or any other agreement, letter or instrument delivered in connection with the transactions contemplated thereby or the consummation of the transactions contemplated thereby, (ii) any Commitment or Loan or the use or proposed use of the proceeds therefrom, (iii) any actual or alleged presence or release of Hazardous Materials on, at, under or from any property currently or formerly owned or operated by the Borrower, any Subsidiary or

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any other Loan Party, or any Environmental Liability related to the Borrower, any Subsidiary or any other Loan Party, or (iv) any actual or prospective claim, litigation, investigation or proceeding relating to any of the foregoing, whether based on contract, tort or any other theory (including any investigation of, preparation for, or defense of any pending or threatened claim, investigation, litigation or proceeding) (any of the foregoing described in this clause (iv), a “Proceeding”) (all the foregoing described in clauses (i) to (iv), collectively, the “Indemnified Liabilities”), in all cases, whether or not caused by or arising, in whole or in part, out of the negligence of the Indemnitee and whether brought by an Indemnitee, a third party or by the Borrower or any other Loan Party or any of the Borrower’s or such Loan Party’s directors, shareholders or creditors, and regardless of whether any Indemnitee is a party thereto and whether or not any of the transactions contemplated hereby are consummated; provided that such indemnity shall not, as to any Indemnitees, be available to the extent that such liabilities, obligations, losses, damages, penalties, claims, demands, actions, judgments, suits, costs, expenses or disbursements resulted from the gross negligence or willful misconduct of such Indemnitee or of any affiliate, director, officer, employee, counsel, agent or attorney-in-fact of such Indemnitee as determined by a final non-appealable judgment of a court of competent jurisdiction. No Indemnitee shall be liable for any damages arising from the use by others of any information or other materials obtained through the Platform, nor shall any Indemnitee or any Loan Party have any liability for any special, punitive, indirect or consequential damages relating to this Agreement or any other Loan Document. All amounts due in respect of costs, expenses and disbursements under this Section 10.05 shall be paid within ten (10) Business Days after demand therefor; provided, that each Indemnitee receiving any such reimbursement shall repay such amounts to the relevant Loan Party in the event that such Indemnitee shall not be entitled thereto pursuant to the provisions hereof. The agreements in this Section 10.05 shall survive the resignation or removal of the Administrative Agent, the replacement of any Lender, the termination of the Aggregate Commitments and the repayment, satisfaction or discharge of all the other Obligations. This Section 10.5(a) shall not apply with respect to Taxes other than any Taxes that represent losses, claims, damages, etc. arising from any non-Tax claim. For the avoidance of doubt, in no event shall the Borrower or any Loan Party pay the Lenders or the Administrative Agent for any out of pocket costs or expenses incurred prior to the Closing Date.

(b)       The Borrower shall not be liable for any settlement of any Proceedings effected without its consent (which consent shall not be unreasonably withheld or delayed), but if settled with the Borrower’s consent or if there is a final judgment for the plaintiff in such Proceedings, the Borrower shall indemnify and hold harmless each Indemnitee from and against any Indemnified Liabilities in accordance with the foregoing clause (a). The Borrower shall not, without the prior written consent of an Indemnitee (which consent shall not be unreasonably withheld or delayed), effect any settlement or consent to the entry of any judgment of any pending or threatened Proceedings in respect of which indemnity could have been sought hereunder by such Indemnitee unless (i) such settlement includes an unconditional release of such Indemnitee in form and substance satisfactory to such Indemnitee from all liability on claims that are the subject matter of such Proceedings, (ii) does not include any statement as to or any admission of fault, culpability or a failure to act by or on behalf of any Indemnitee and (iii) contains customary confidentiality and non-disparagement provisions.

(c)       In the event that an Indemnitee is requested or required to appear as a witness in any action brought by or on behalf of or against the Borrower or any of its

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Subsidiaries or Affiliates in which such Indemnitee is not named as a defendant, the Borrower shall reimburse such Indemnitee for all reasonable and documented expenses incurred by it in connection with such Indemnitee’s appearing and preparing to appear as such a witness, including without limitation, the reasonable and documented fees and expenses of its legal counsel.

Section 10.06 Payments Set Aside. To the extent that any payment by or on behalf of the Borrower is made to the Administrative Agent or any Lender, or the Administrative Agent or any Lender exercises its right of setoff, and such payment or the proceeds of such setoff or any part thereof is subsequently invalidated, declared to be fraudulent or preferential, set aside or required (including pursuant to any settlement entered into by the Administrative Agent or such Lender in its discretion) to be repaid to a trustee, receiver or any other party, in connection with any proceeding, then (a) to the extent of such recovery, the obligation or part thereof originally intended to be satisfied shall be revived and continued in full force and effect as if such payment had not been made or such setoff had not occurred, and (b) each Lender severally agrees to pay to the Administrative Agent upon demand its applicable share of any amount so recovered from or repaid by the Administrative Agent, plus interest thereon from the date of such demand to the date such payment is made at a rate per annum equal to the Federal Funds Rate.

Section 10.07 Successors and Assigns.

(a)          Successors and Assigns Generally. The provisions of this Agreement shall be binding upon and inure to the benefit of the parties hereto and their respective successors and assigns permitted hereby, except that neither the Borrower nor any other Loan Party may assign or otherwise transfer any of its rights or obligations hereunder without the prior written consent of the Administrative Agent and each Lender, and no Lender may assign or otherwise transfer any of its rights or obligations hereunder except (i) to an assignee in accordance with the provisions of Section 10.07(b), (ii) by way of participation in accordance with the provisions of Section 10.07(d), (iii) by way of pledge or assignment of a security interest subject to the restrictions of Section 10.07(e) or (iv) to an SPC in accordance with the provisions of Section 10.07(f) (and any other attempted assignment or transfer by any party hereto shall be null and void). Nothing in this Agreement, expressed or implied, shall be construed to confer upon any Person (other than the parties hereto, their respective successors and assigns permitted hereby, Participants to the extent provided in Section 10.07(d) and, to the extent expressly contemplated hereby, the Related Parties of each of the Administrative Agent and the Lenders) any legal or equitable right, remedy or claim under or by reason of this Agreement.

(b)          Assignments by Lenders. Any Lender may at any time assign to one or more assignees all or a portion of its Commitment and/or the Loans at the time owing to it (and its rights and obligations under this Agreement relating thereto); provided that any such assignment shall be subject to the following conditions:

(i)           Minimum Amounts.

(A)       in the case of an assignment of the entire remaining amount of the assigning Lender’s Commitment and/or the Loans at the time owing to it or contemporaneous assignments to related Approved Funds (determined after

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giving effect to such assignments) that equal at least the amount specified in paragraph (b)(i)(B) of this Section in the aggregate or in the case of an assignment to a Lender, an Affiliate of a Lender or an Approved Fund, no minimum amount need be assigned; and

(B)      in any case not described in paragraph (b)(i)(A) of this Section, the aggregate amount of the Commitment (which for this purpose includes Loans outstanding thereunder) or, if the applicable Commitment is not then in effect, the principal outstanding balance of the Loans of the assigning Lender subject to each such assignment (determined as of the date the Assignment and Assumption with respect to such assignment is delivered to the Administrative Agent or, if “Trade Date” is specified in the Assignment and Assumption, as of the Trade Date) shall not be less than $1,000,000 unless the Borrower consents (such consent not to be unreasonably withheld or delayed and shall not be required if an Event of Default exists).

(ii)       Proportionate Amounts. Each partial assignment shall be made as an assignment of a proportionate part of all the assigning Lender’s rights and obligations under this Agreement with respect to the Loan or the Commitment assigned.

(iii)      Required Consents. Any such assignment shall require the prior written consent of the Borrower, which consent shall not be unreasonably withheld, conditioned, delayed or burdened (provided, that it shall be deemed to be reasonable for the Borrower not to consent to any assignment to any Disqualified Person); provided, however, that (A) no consent of the Borrower shall be required for an assignment to a Lender, to an Affiliate of a Lender, to an Approved Fund or, if an Event of Default has occurred and is continuing, to any other assignee other than to any Disqualified Person, and (B) the Borrower shall be deemed to have consented to any such assignment unless it objects thereto by written notice delivered to the Administrative Agent within ten (10) Business Days after having received notice thereof; and

(iv)       Assignment and Assumption. The parties to each assignment shall execute and deliver to the Administrative Agent an Assignment and Assumption, together with a processing and recordation fee of $3,500; provided that the Administrative Agent may, in its sole discretion, elect to waive such processing and recordation fee in the case of any assignment. The assignee, if it is not a Lender, shall deliver to the Administrative Agent an Administrative Questionnaire and the tax documentation required pursuant to Section 3.01.

(v)        No Assignment to Certain Persons. No such assignment shall be made to the Borrower or any of the Borrower’s Affiliates or Subsidiaries or any Disqualified Person.

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(vi)      No Assignment to Natural Persons. No such assignment shall be made to a natural Person (or a holding company, investment vehicle or trust for, or owned and operated for the primary benefit of, a natural Person).

Subject to acceptance and recording thereof by the Administrative Agent pursuant to Section 10.07(c), from and after the effective date specified in each Assignment and Assumption, the assignee thereunder shall be a party to this Agreement and, to the extent of the interest assigned by such Assignment and Assumption, have the rights and obligations of a Lender under this Agreement, and the assigning Lender thereunder shall, to the extent of the interest assigned by such Assignment and Assumption, be released from its obligations under this Agreement (and, in the case of an Assignment and Assumption covering all of the assigning Lender’s rights and obligations under this Agreement, such Lender shall cease to be a party hereto) but shall continue to be entitled to the benefits of Sections 3.01, 3.02, 10.04 and 10.05 with respect to facts and circumstances occurring prior to the effective date of such assignment. Any assignment or transfer by a Lender of rights or obligations under this Agreement that does not comply with this paragraph shall be treated for purposes of this Agreement as a sale by such Lender of a participation in such rights and obligations in accordance with paragraph (d) of this Section.

(c)       Register. The Administrative Agent, acting solely for this purpose as an agent of the Borrower, shall maintain at the Administrative Agent’s Office a copy of each Assignment and Assumption delivered to it and a register for the recordation of the names and addresses of the Lenders, and the Commitments of, and principal amounts (and stated interest) of the Loans owing to, each Lender pursuant to the terms hereof from time to time (the “Register”). The entries in the Register shall be conclusive absent manifest error, and the Borrower, the Administrative Agent and the Lenders shall treat each Person whose name is recorded in the Register pursuant to the terms hereof as a Lender hereunder for all purposes of this Agreement. The Register shall be available for inspection by the Borrower and any Lender, at any reasonable time and from time to time upon reasonable prior notice.

(d)       Participations. Any Lender may at any time, without the consent of, or notice to, the Borrower or the Administrative Agent, sell participations to any Person (other than a natural Person, or a holding company, investment vehicle or trust for, or owned and operated for the primary benefit of, a natural Person, or the Borrower or any of the Borrower’s Affiliates or Subsidiaries or any Disqualified Person) (each, a “Participant”) in all or a portion of its Commitment and/or the Loans at the time owing to it (and its rights and obligations under this Agreement relating thereto); provided that (i) such Lender’s obligations under this Agreement shall remain unchanged, (ii) such Lender shall remain solely responsible to the other parties hereto for the performance of such obligations, and (iii) the Borrower, the Administrative Agent and Lenders shall continue to deal solely and directly with such Lender in connection with such Lender’s rights and obligations under this Agreement. For the avoidance of doubt, each Lender shall be responsible for the indemnity under Section 9.07 with respect to any payments made by such Lender to its Participant(s).

Any agreement or instrument pursuant to which a Lender sells such a participation shall provide that such Lender shall retain the sole right to enforce this Agreement and the other Loan Documents and to approve any amendment, modification or waiver of any

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provision of this Agreement or the other Loan Documents; provided that such agreement or instrument may provide that such Lender will not, without the consent of the Participant, agree to any amendment, waiver or other modification described in Section 10.01(a) or Section 10.01(b) that directly and adversely affects such Participant. The Borrower agrees that each Participant shall be entitled to the benefits of Sections 3.01 and 3.02 (subject to the requirements and limitations therein, including the requirements under Section 3.01(g) (it being understood that the documentation required under Section 3.01(g) shall be delivered to the participating Lender)) to the same extent as if it were a Lender and had acquired its interest by assignment pursuant to paragraph (b) of this Section; provided that such Participant shall not be entitled to receive any greater payment under Sections 3.01 or 3.02, with respect to any participation, than its participating Lender would have been entitled to receive, except to the extent such entitlement to receive a greater payment results from a Change in Law that occurs after the Participant acquired the applicable participation. Each Lender that sells a participation agrees, at the Borrower’s request and expense, to use reasonable efforts to cooperate with the Borrower to effectuate the provisions of Section 3.04(b) with respect to any Participant. To the extent permitted by applicable Law, each Participant also shall be entitled to the benefits of Section 10.09 as though it were a Lender; provided that such Participant agrees to be subject to Section 2.09 as though it were a Lender. Each Lender that sells a participation shall, acting solely for this purpose as a non-fiduciary agent of the Borrower, maintain a register on which it enters the name and address of each Participant and the principal amounts (and stated interest) of each Participant’s interest in the Loans or other obligations under the Loan Documents (the “Participant Register”); provided that no Lender shall have any obligation to disclose all or any portion of the Participant Register (including the identity of any Participant or any information relating to a Participant’s interest in any commitments, loans, letters of credit or its other obligations under any Loan Document) to any Person except to the extent that such disclosure is necessary to establish that such commitment, loan, letter of credit or other obligation is in registered form under Section 5f. 103-1(c) of the United States Treasury Regulations. The entries in the Participant Register shall be conclusive absent manifest error, and such Lender shall treat each Person whose name is recorded in the Participant Register as the owner of such participation for all purposes of this Agreement notwithstanding any notice to the contrary. For the avoidance of doubt, the Administrative Agent (in its capacity as Administrative Agent) shall have no responsibility for maintaining a Participant Register.

(e)       Certain Pledges. Any Lender may at any time pledge or assign a security interest in all or any portion of its rights under this Agreement (including under its Note, if any) to secure obligations of such Lender, including any pledge or assignment to secure obligations to a Federal Reserve Bank or central bank having jurisdiction over such Lender; provided that no such pledge or assignment shall release such Lender from any of its obligations hereunder or substitute any such pledgee or assignee for such Lender as a party hereto.

(f)        SPCs. Notwithstanding anything to the contrary contained herein, any Lender (a “Granting Lender”) may grant to a special purpose funding vehicle identified as such in writing from time to time by the Granting Lender to the Administrative Agent and the Borrower (an “SPC”) the option to provide all or any part of any Loan that such Granting Lender would otherwise be obligated to make pursuant to this Agreement; provided that (i) nothing herein shall constitute a commitment by any SPC to fund any Loan and (ii) if an SPC elects not to exercise such option or otherwise fails to make all or any part of such Loan, the Granting

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Lender shall be obligated to make such Loan pursuant to the terms hereof. Each party hereto hereby agrees that (i) neither the grant to any SPC nor the exercise by any SPC of such option shall increase the costs or expenses or otherwise increase or change the obligations of the Borrower under this Agreement (including its obligations under Section 3.01 or 3.02), (ii) no SPC shall be liable for any indemnity or similar payment obligation under this Agreement for which a Lender would be liable and such liability shall remain with the Granting Lender, and (iii) the Granting Lender shall for all purposes, including the approval of any amendment, waiver or other modification of any provision of any Loan Document, remain the lender of record hereunder. The making of a Loan by an SPC hereunder shall utilize the Commitment of the Granting Lender to the same extent, and as if, such Loan were made by such Granting Lender. Notwithstanding anything to the contrary contained herein, any SPC may (i) with notice to, but without prior consent of the Borrower and the Administrative Agent, assign all or any portion of its right to receive payment with respect to any Loan to the Granting Lender and (ii) disclose on a confidential basis any non-public information relating to its funding of Loans to any rating agency, commercial paper dealer or provider of any surety or Guarantee Obligation or credit or liquidity enhancement to such SPC.

(g)       Notwithstanding anything to the contrary contained herein, (1) any Lender may in accordance with applicable Law create a security interest in all or any portion of the Loans owing to it and the Note, if any, held by it and (2) any Lender that is a Fund may create a security interest in all or any portion of the Loans owing to it and the Note, if any, held by it to the trustee for holders of obligations owed, or securities issued, by such Fund as security for such obligations or securities; provided that unless and until such trustee actually becomes a Lender in compliance with the other provisions of this Section 10.07, (i) no such pledge shall release the pledging Lender from any of its obligations under the Loan Documents and (ii) such trustee shall not be entitled to exercise any of the rights of a Lender under the Loan Documents even though such trustee may have acquired ownership rights with respect to the pledged interest through foreclosure or otherwise.

Section 10.08 Confidentiality. Each of the Administrative Agent and the Lenders agrees to maintain the confidentiality of the Information and to not use or disclose such information, except that Information may be disclosed (a) to its Affiliates and its and its Affiliates’ directors, officers, employees, trustees, investment advisors and agents, including accountants, legal counsel and other advisors (it being understood that the Persons to whom such disclosure is made will be informed of the confidential nature of such Information and instructed to keep such Information confidential); (b) to the extent requested by any Governmental Authority or examiner regulating any Lender or the Administrative Agent; (c) to the extent required by applicable Laws or regulations or by any subpoena or similar legal process; (d) to any other party to this Agreement; (e) to any pledgee referred to in Section 10.07(e) or Section 10.07(g), Eligible Assignee of or Participant in, or any prospective Eligible Assignee of or Participant in, any of its rights or obligations under this Agreement (it being understood that the Persons to whom such disclosure is made will be informed of the confidential nature of such Information and instructed to keep such Information confidential); (f) with the written consent of the Borrower; (g) to the extent such Information becomes publicly available other than as a result of a breach of this Section 10.08 by the disclosing party; (h) to any rating agency when required by it (it being understood that, prior to any such disclosure, such rating agency shall undertake to preserve the confidentiality of any Information relating to the Loan Parties received by it from

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such Lender); or (i) in connection with the exercise of any remedies hereunder or under any other Loan Document or any action or proceeding relating to this Agreement or any other Loan Document or the enforcement of rights hereunder or thereunder. In addition, the Administrative Agent and the Lenders may disclose the existence of this Agreement and information about this Agreement to market data collectors, similar service providers to the lending industry, and service providers to the Administrative Agent and the Lenders in connection with the administration and management of this Agreement, the other Loan Documents, the Commitments and the Loans. For the purposes of this Section 10.08, “Information” means all information received from any Loan Party or its Affiliates or its Affiliates’ directors, officers, employees, trustees, investment advisors or agents, relating to the Borrower or any of their Subsidiaries or their business, other than any such information that is publicly available to the Administrative Agent or any Lender prior to disclosure by any Loan Party other than as a result of a breach of this Section 10.08, including, without limitation, information delivered pursuant to Section 6.01, 6.02 or 6.03 hereof.

Section 10.09 Setoff.   In addition to any rights and remedies of the Administrative Agent and the Lenders provided by Law, upon the occurrence and during the continuance of any Event of Default, each Lender and its Affiliates and the Administrative Agent and its Affiliates is authorized at any time and from time to time, without prior notice to the Borrower or any other Loan Party, any such notice being waived by the Borrower (on its own behalf and on behalf of each Loan Party and its Subsidiaries) to the fullest extent permitted by applicable Law, to set off and apply any and all deposits (general or special, time or demand, provisional or final) at any time held by, and. other Indebtedness at any time owing by, such Lender and its Affiliates or the Administrative Agent and its Affiliates, as the case may be, to or for the credit or the account of the respective Loan Parties and their Subsidiaries against any and all Obligations owing to such Lender and its Affiliates or the Administrative Agent and its Affiliates hereunder or under any other Loan Document, now or hereafter existing, irrespective of whether or not the Administrative Agent or such Lender or Affiliate shall have made demand under this Agreement or any other Loan Document and although such Obligations may be contingent or unmatured or denominated in a currency different from that of the applicable deposit or Indebtedness. Each Lender and the Administrative Agent agrees promptly to notify the Borrower and the Administrative Agent after any such set off and application made by such Lender or the Administrative Agent, as the case may be; provided that the failure to give such notice shall not affect the validity of such setoff and application. The rights of the Administrative Agent and each Lender under this Section 10.09 are in addition to other rights and remedies (including other rights of setoff) that the Administrative Agent and such Lender may have.

Section 10.10 Counterparts. This Agreement and each other Loan Document may be executed in one or more counterparts, each of which shall be deemed an original, but all of which together shall constitute one and the same instrument. Delivery by facsimile transmission or other electronic transmission (including a .pdf or .tif copy) of an executed counterpart of a signature page to this Agreement and each other Loan Document shall be effective as delivery of an original executed counterpart of this Agreement and such other Loan Document; provided that original signatures shall be promptly delivered thereafter, it being understood that that the failure to request or deliver the same shall not limit the effectiveness of any document or signature delivered by facsimile or electronic transmission.

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Section 10.11 Integration. The Loan Documents comprise the complete and integrated agreement of the parties on the subject matter hereof and thereof and supersedes all prior agreements, written or oral, on such subject matter. In the event of any conflict or inconsistency between the provisions of this Agreement and those of any other Loan Document, the provisions of this Agreement shall control; provided that the inclusion of supplemental rights or remedies in favor of the Administrative Agent or the Lenders in any other Loan Document shall not be deemed a conflict or inconsistency with this Agreement. Each Loan Document was drafted with the joint participation of the respective parties thereto and shall be construed neither against nor in favor of any party, but rather in accordance with the fair meaning thereof.

Section 10.12 Survival of Representations and Warranties. All representations and warranties made hereunder and in any other Loan Document or other document delivered pursuant hereto or thereto or in connection herewith or therewith shall survive the execution and delivery hereof and thereof. Such representations and warranties have been or will be relied upon by the Administrative Agent and each Lender, regardless of any investigation made by the Administrative Agent or any Lender or on their behalf and notwithstanding that the Administrative Agent or any Lender may have had notice or knowledge of any Default at the time of any Loan, and shall continue in full force and effect as long as any Loan or any other Obligation hereunder shall remain unpaid or unsatisfied.

Section 10.13 Severability. If any provision of this Agreement or the other Loan Documents is held to be illegal, invalid or unenforceable, the legality, validity and enforceability of the remaining provisions of this Agreement and the other Loan Documents shall not be affected or impaired thereby. The invalidity of a provision in a particular jurisdiction shall not invalidate or render unenforceable such provision in any other jurisdiction.

Section 10.14 GOVERNING LAW. (a) THIS AGREEMENT AND EACH OTHER LOAN DOCUMENT SHALL BE GOVERNED BY, AND CONSTRUED IN ACCORDANCE WITH, THE LAW OF THE STATE OF NEW YORK (EXCEPT, WITH RESPECT TO ANY OTHER LOAN DOCUMENT, AS OTHERWISE EXPRESSLY PROVIDED THEREIN); PROVIDED THAT THE ADMINISTRATIVE AGENT AND THE LENDERS SHALL RETAIN ALL RIGHTS ARISING UNDER FEDERAL LAW.

(b)       ANY LEGAL ACTION OR PROCEEDING ARISING UNDER ANY LOAN DOCUMENT OR IN ANY WAY CONNECTED WITH OR RELATED OR INCIDENTAL TO THE DEALINGS OF THE PARTIES HERETO OR ANY OF THEM WITH RESPECT TO ANY LOAN DOCUMENT, OR THE TRANSACTIONS RELATED HERETO OR THERETO, IN EACH CASE WHETHER NOW EXISTING OR HEREAFTER ARISING, MAY BE BROUGHT IN THE COURTS OF THE STATE OF NEW YORK LOCATED IN NEW YORK COUNTY OR OF THE UNITED STATES FOR THE SOUTHERN DISTRICT OF SUCH STATE, AND BY EXECUTION AND DELIVERY OF THIS AGREEMENT, THE BORROWER, THE ADMINISTRATIVE AGENT AND EACH LENDER CONSENTS, FOR ITSELF AND IN RESPECT OF ITS PROPERTY, TO THE NON-EXCLUSIVE JURISDICTION OF THOSE COURTS. THE BORROWER, THE ADMINISTRATIVE AGENT AND EACH LENDER IRREVOCABLY WAIVES ANY OBJECTION, INCLUDING ANY OBJECTION TO THE LAYING OF VENUE OR BASED ON THE GROUNDS OF FORUM NON CONVENIENS, WHICH IT MAY NOW OR HEREAFTER HAVE TO THE

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BRINGING OF ANY ACTION OR PROCEEDING IN SUCH JURISDICTION IN RESPECT OF ANY LOAN DOCUMENT OR OTHER DOCUMENT RELATED THERETO.

Section 10.15 WAIVER OF RIGHT TO TRIAL BY JURY. EACH PARTY TO THIS AGREEMENT HEREBY EXPRESSLY WAIVES ANY RIGHT TO TRIAL BY JURY OF ANY CLAIM, DEMAND, ACTION OR CAUSE OF ACTION ARISING UNDER ANY LOAN DOCUMENT OR IN ANY WAY CONNECTED WITH OR RELATED OR INCIDENTAL TO THE DEALINGS OF THE PARTIES HERETO OR ANY OF THEM WITH RESPECT TO ANY LOAN DOCUMENT, OR THE TRANSACTIONS RELATED HERETO OR THERETO, IN EACH CASE WHETHER NOW EXISTING OR HEREAFTER ARISING, AND WHETHER FOUNDED IN CONTRACT OR TORT OR OTHERWISE; AND EACH PARTY HEREBY AGREES AND CONSENTS THAT ANY SUCH CLAIM, DEMAND, ACTION OR CAUSE OF ACTION SHALL BE DECIDED BY COURT TRIAL WITHOUT A JURY, AND THAT ANY PARTY TO THIS AGREEMENT MAY FILE AN ORIGINAL COUNTERPART OR A COPY OF THIS SECTION 10.15 WITH ANY COURT AS WRITTEN EVIDENCE OF THE CONSENT OF THE SIGNATORIES HERETO TO THE WAIVER OF THEIR RIGHT TO TRIAL BY JURY.

Section 10.16 Binding Effect. This Agreement shall become effective when it shall have been executed by the Borrower and the Administrative Agent, and the Administrative Agent shall have been notified by each Lender that each such Lender has executed it and thereafter shall be binding upon and inure to the benefit of the Borrower, the Administrative Agent and each Lender and their respective successors and assigns, except that the Borrower shall not have the right to assign its rights hereunder or any interest herein without the prior written consent of the Required Lenders.

Section 10.17 Lender Action. Each Lender agrees that it shall not take or institute any actions or proceedings, judicial or otherwise, for any right or remedy against any Loan Party or any other obligor under any of the Loan Documents (including the exercise of any right of setoff, rights on account of any banker’s lien or similar claim or other rights of self-help), or institute any actions or proceedings, or otherwise commence any remedial procedures, with respect to any Collateral or any other property of any such Loan Party, without the prior written consent of the Administrative Agent. The provision of this Section 10.17 are for the sole benefit of the Lenders and shall not afford any right to, or constitute a defense available to, any Loan Party.

Section 10.18 USA PATRIOT Act. Each Lender hereby notifies the Borrower that pursuant to the requirements of the USA PATRIOT Act, it is required to obtain, verify and record information that identifies the Borrower, which information includes the name and address of the Borrower and other information that will allow such Lender to identify the Borrower in accordance with the USA PATRIOT Act. The Borrower agrees to provide, and to cause each other Loan Party to provide, such information promptly upon request.

Section 10.19 No Advisory or Fiduciary Responsibility. In connection with all aspects of each transaction contemplated hereby (including in connection with any amendment, waiver or other modification hereof or of any other Loan Document), the Borrower acknowledges and agrees, and acknowledges and agrees that it has informed its other Affiliates,

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that: (i) (A) no fiduciary, advisory or agency relationship between any of the Borrower and its Subsidiaries and the Administrative Agent or any Lender is intended to be or has been created in respect of any of the transactions contemplated hereby and by the other Loan Documents, irrespective of whether the Administrative Agent or any Lender has advised or is advising any of the Borrower and its Subsidiaries on other matters, (B) the arranging and other services regarding this Agreement provided by the Administrative Agent and the Lenders are arm’s-length commercial transactions between the Loan Parties, on the one hand, and the Administrative Agent and the Lenders, on the other hand, (C) the Borrower has consulted its own legal, accounting, regulatory and tax advisors to the extent it has deemed appropriate, and (D) the Borrower is capable of evaluating, and understands and accepts, the terms, risks and conditions of the transactions contemplated hereby and by the other Loan Documents; (ii) (A) the Administrative Agent and each of the Lenders is and has been acting solely as a principal and, except as may otherwise be expressly agreed in writing by the relevant parties, has not been, is not, and will not be acting as an advisor, agent or fiduciary for the Borrower or any of its Affiliates, or any other Person and (B) none of the Administrative Agent or any Lender has any obligation to the Borrower or any of its Affiliates with respect to the transactions contemplated hereby except those obligations expressly set forth herein and in the other Loan Documents; and (iii) the Administrative Agent and the Lenders and their respective Affiliates may be engaged in a broad range of transactions that involve interests that differ from those of the Borrower and its Affiliates, and none of the Administrative Agent or any Lender has any obligation to disclose any of such interests and transactions to the Borrower or any of its Affiliates. To the fullest extent permitted by law, the Borrower hereby waives and releases any claims that it may have against the Administrative Agent and the Lenders with respect to any breach or alleged breach of agency or fiduciary duty in connection with any aspect of any transaction contemplated hereby.

Section 10.20 Acknowledgement and Consent to Bail-In of EEA Financial Institutions. Notwithstanding anything to the contrary in any Loan Document or in any other agreement, arrangement or understanding among any such parties, each party hereto acknowledges that any liability of any Lender that is an EEA Financial Institution arising under any Loan Document, to the extent such liability is unsecured, may be subject to the Write-Down and Conversion Powers of an EEA Resolution Authority and agrees and consents to, and acknowledges and agrees to be bound by:

(a)          the application of any Write-Down and Conversion Powers by an EEA Resolution Authority to any such liabilities arising hereunder which may be payable to it by any Lender that is an EEA Financial Institution; and

(b)          the effects of any Bail-In Action on any such liability, including, if applicable:

(i)        a reduction in full or in part or cancellation of any such liability;

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(ii)       a conversion of all, or a portion of, such liability into shares or other instruments of ownership in such EEA Financial Institution, its parent undertaking, or a bridge institution that may be issued to it or otherwise conferred on it, and that such shares or other instruments of ownership will be accepted by it in lieu of any rights with respect to any such liability under this Agreement or any other Loan Document; or

(iii)      the variation of the terms of such liability in connection with the exercise of the Write-Down and Conversion Powers of any EEA Resolution Authority.

[Remainder of Page Intentionally Blank]

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IN WITNESS WHEREOF, the parties hereto have caused this Agreement to be duly executed as of the date first above written.

AEGERION PHARMACEUTICALS, INC., as Borrower
By:	/s/ Joe Wiley
	Name: Joe Wiley
	Title: Director and President

[Signature Page to Credit Agreement]

AMRYT PHARMA HOLDINGS PLC, to be renamed on or around the date hereof as AMRYT PHARMA PLC (company number: 12107859), as Parent
By:	/s/ Joe Wiley
	Name: Joe Wiley
	Title: Director

[Signature Page to Credit Agreement]

CANTOR FITZGERALD SECURITIES, as Administrative Agent
By:	/s/ James Buccola
Name: James Buccola
Title: Head of Fixed Income

[Signature Page to Credit Agreement]

ATHYRIUM OPPORTUNITIES II ACQUISITION LP, as a Lender
By: Athyrium Opportunities Associates II LP, its general partner
By: Athyrium GP Holdings LLC, its general partner
By:	/s/ Andrew C. Hyman
	Name:      Andrew C. Hyman
	Title:        Authorized Signatory

ATHYRIUM OPPORTUNITIES III ACQUISITION LP, as a Lender
By: Athyrium Opportunities Associates III LP, its general partner
By: Athyrium Opportunities Associates III GP LLC, its general partner
By:	/s/ Andrew C. Hyman
	Name:      Andrew C. Hyman
	Title:        Authorized Signatory

[Signature Page to Credit Agreement]

HIGHBRIDGE SCF LOAN SPV, L.P., as a Lender
By: Highbridge Capital Management, LLC, as Trading Manager
By:	/s/ Jonathan Segal
Name: Jonathan Segal
Title: Managing Director

[Signature Page to Credit Agreement]

EXHIBIT A-l

FORM OF
COMMITTED LOAN NOTICE

To: Cantor Fitzgerald Securities

as Administrative Agent for the Lenders

1801 N. Military Trail, Suite 202

Boca Raton, FL 33431

Telecopier: (646) 219-1180

Attention: N. Horning (Aegerion Pharmaceuticals)

E-mail: [***]

and

Cantor Fitzgerald Securities

900 West Trade Street, Suite 725

Charlotte, North Carolina 28202

Phone: (747) 374-0574

Telecopier: (646) 390-1764

Attention: B.Young (Aegerion Pharmaceuticals)

E-mail: [***]

[Insert Date]

Ladies and Gentlemen:

Reference is made to the Credit Agreement dated as of September 24, 2019 (as amended, restated, supplemented or otherwise modified from time to time, the “Credit Agreement”) among Aegerion Pharmaceuticals, Inc., a Delaware corporation (the “Borrower”), Amryt Pharma Holdings plc, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around September 24, 2019 as Amryt Pharma plc), Cantor Fitzgerald Securities, as Administrative Agent, and each Lender from time to time party thereto. All capitalized terms used and not defined herein have the meanings ascribed thereto in the Credit Agreement.

Pursuant to Section 4.01 of the Credit Agreement, the Borrower hereby gives you notice that it requests the New Money Loans (and the deemed advance of the Rollover Loans) under the Credit Agreement, and in connection therewith set forth below are the terms on which the New Money Loans and the Rollover Loans are requested to be made or deemed to be advanced, as applicable

(A)      Date of New Money Loans and Rollover Loans (which is a Business Day)

(B)      Principal amount of New Money Loans

(C)      Funds in respect of New Money Loans are requested to be disbursed to the following account(s) of the Borrower¹

¹ Specify the location and number of the Borrower’s account to which funds are to be disbursed, which shall comply with the requirements of the Credit Agreement.

(D)       Principal amount of Rollover Loans deemed advanced

[signature page follows]

AEGERION PHARMACEUTICALS, INC., as Borrower
By:
	Name:
	Title:

EXHIBIT A-2

FORM OF
PREPAYMENT NOTICE

[Insert Date]

Cantor Fitzgerald Securities
as Administrative Agent for the Lenders
1801 N. Military Trail, Suite 202
Boca Raton, FL 33431
Telecopier: (646) 219-1180
Attention: N. Horning (Aegerion Pharmaceuticals)
E-mail: [***]

and

Cantor Fitzgerald Securities
900 West Trade Street, Suite 725
Charlotte, North Carolina 28202
Phone: (747) 374-0574

Telecopier: (646) 390-1764
Attention: B. Young (Aegerion Pharmaceuticals)
E-mail: [***]

Ladies and Gentlemen:

Reference is made to the Credit Agreement dated as of September 24, 2019 (as amended, restated, supplemented or otherwise modified from time to time, the “Credit Agreement”) among Aegerion Pharmaceuticals, Inc., a Delaware corporation (the “Borrower”), Amryt Pharma Holdings plc, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around September 24, 2019 as Amryt Pharma plc), Cantor Fitzgerald Securities, as Administrative Agent, and each Lender from time to time party thereto. All capitalized terms used and not defined herein have the meanings ascribed thereto in the Credit Agreement.

Pursuant to Section 2.02[(a)]/[(b)] of the Credit Agreement, we hereby give you notice that we shall prepay certain of the Loans under the Credit Agreement, and such notice may only be rescinded in accordance with Section 2.02 of the Credit Agreement.

1. Date of prepayment:______/______/______

2. Aggregate principal amount¹ of prepayment is equal to $__________________ (with reasonably detailed calculation).

3. Accrued and unpaid interest on amount of prepayment is equal to $__________________.

4. [Make-Whole Premium on amount of prepayment is equal to $__________________.]²

1	Each prepayment shall be in a principal amount of $5,000,000 or a whole multiple of $1,000,000 in excess thereof or, in each case, if less, the entire principal amount thereof then outstanding.
2	If applicable.

[signature page follows]

AEGERION PHARMACEUTICALS, INC., as Borrower
By:
	Name:
	Title:

EXHIBIT A-3

FORM OF
PIK ELECTION REQUEST

[Insert Date]

Cantor Fitzgerald Securities
as Administrative Agent for the Lenders
1801 N. Military Trail, Suite 202
Boca Raton, FL 33431
Telecopier: (646) 219-1180

Attention: N. Horning (Aegerion Pharmaceuticals)
E-mail: [***]

and

Cantor Fitzgerald Securities
900 West Trade Street, Suite 725
Charlotte, North Carolina 28202

Phone: (747) 374-0574

Telecopier: (646) 390-1764
Attention: B. Young (Aegerion Pharmaceuticals)
E-mail: [***]

Ladies and Gentlemen:

Reference is made to the Credit Agreement dated as of September 24, 2019 (as amended, restated, supplemented or otherwise modified from time to time, the “Credit Agreement”) among Aegerion Pharmaceuticals, Inc., a Delaware corporation (the “Borrower”), Amryt Pharma Holdings plc, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around September 24, 2019 as Amryt Pharma plc), Cantor Fitzgerald Securities, as Administrative Agent, and each Lender from time to time party thereto. All capitalized terms used and not defined herein have the meanings ascribed thereto in the Credit Agreement.

Pursuant to Section 2.04(a) of the Credit Agreement, we hereby give you notice of our election for PIK Interest.

1.
Amount of interest to be paid in kind in respect of the Loans on the Interest Payment Date of [________]: $ __________.

2. Amount of interest to be paid in cash in respect of the Loans on the Interest Payment Date of [________]: $ __________.

[signature page follows]

AEGERION PHARMACEUTICALS, INC., as Borrower
By:
	Name:
	Title:

EXHIBIT B

FORM OF
NOTE

$__________ [Insert date]

FOR VALUE RECEIVED, the undersigned hereby unconditionally promises to pay to [Lender] (the “Lender”) on each date set forth under that certain Credit Agreement (as defined below) and on the Maturity Date (terms used without definition shall have the meanings assigned to such terms in that certain Credit Agreement dated as of September 24, 2019 (as amended, restated, amended and restated, supplemented or otherwise modified from time to time, the “Credit Agreement”), among Aegerion Pharmaceuticals, Inc., a Delaware corporation (the “Borrower”), Amryt Pharma Holdings plc, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around September 24, 2019 as Amryt Pharma plc), Cantor Fitzgerald Securities, as Administrative Agent, and each Lender from time to time party thereto), the aggregate unpaid principal amount of the Loans made by the Lender to the Borrower pursuant to Section 2.01 of the Credit Agreement, such payment or payments to be in immediately available funds. The Borrower further agrees to pay interest on such principal amount from time to time outstanding, at said office, at a rate or rates per annum and payable on such dates as are determined pursuant to the Credit Agreement.

The Borrower promises to pay interest on any overdue principal of and, to the extent permitted by law, overdue interest on the Loans in accordance with Section 2.04 of the Credit Agreement from their due dates.

The Borrower hereby waives diligence, presentment, demand, protest and notice of any kind whatsoever. The nonexercise by the holder of any of its rights hereunder in any particular instance shall not constitute a waiver thereof in that or any subsequent instance.

The Loans evidenced by this Note (this “Note”) and all payments and prepayments of the principal hereof and interest hereon and the respective dates thereof shall be endorsed by the holder hereof on the schedule attached hereto and made a part hereof, or on a continuation thereof which shall be attached hereto and made a part hereof, or otherwise recorded by such holder in its internal records; provided, however, that any failure of the holder hereof to make such a notation or any error in such notation shall not in any manner affect the obligation of the Borrower to make payments of principal and interest in accordance with the terms of this Note and the Credit Agreement.

This Note evidences the Loans referred to in the Credit Agreement which, among other things, contains provisions for the acceleration of the maturity hereof upon the happening of certain events, for optional and mandatory prepayment of the principal hereof prior to the maturity hereof and for the amendment or waiver of certain provisions of the Credit Agreement, all upon the terms and conditions therein specified. This Note is entitled to the benefit of the Credit Agreement and the Collateral Documents.

THIS NOTE SHALL BE GOVERNED BY, AND CONSTRUED IN ACCORDANCE WITH, THE LAW OF THE STATE OF NEW YORK.

In the event of a conflict between this Note and the Credit Agreement, the provisions of the Credit Agreement will govern.

[signature page follows]

Form of Term Note

C-2

AEGERION PHARMACEUTICALS, INC., as Borrower
By:
	Name:
	Title:

LOANS AND REPAYMENTS OF LOANS

Date Amount of Loans Amount of
Principal of
Loans
Repaid Unpaid Principal
Balance of
Loans Notation Made By

EXHIBIT C

FORM OF
COMPLIANCE CERTIFICATE

To: Cantor Fitzgerald Securities

as Administrative Agent for the Lenders

1801 N. Military Trail, Suite 202

Boca Raton, FL 33431

Telecopier: (646) 219-1180

Attention: N. Horning (Aegerion Pharmaceuticals)

E-mail: [***]

and

Cantor Fitzgerald Securities

900 West Trade Street, Suite 725

Charlotte, North Carolina 28202

Phone:(747) 374-0574

Telecopier: (646) 390-1764

Attention: B. Young (Aegerion Pharmaceuticals)

E-mail: [***]

Ladies and Gentlemen:

This Compliance Certificate (this “Certificate”) is being delivered pursuant to Section 6.02(a) of the Credit Agreement dated as of September 24, 2019 (as amended, restated, supplemented or otherwise modified from time to time, the ‘‘Credit Agreement”) among Aegerion Pharmaceuticals, Inc. (the “Borrower”), Amryt Pharma Holdings plc, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around September 24, 2019 as Amryt Pharma plc) (the “Parent”), Cantor Fitzgerald Securities, as Administrative Agent, and each Lender from time to time party thereto. All capitalized terms used and not defined herein have the meanings ascribed thereto in the Credit Agreement.

(a)       This Certificate is delivered in conjunction with [the unaudited internally prepared balance sheet and the related unaudited internally prepared consolidated statements of income or operations and cash flows of the Parent and its Subsidiaries for the fiscal quarter of the Parent ended as of [______] (the “Financials Date”). Such financial statements fairly present, in all material respects, the financial condition, results of operations and cash flows of the Parent and its Subsidiaries in accordance with IFRS, subject to year-end adjustments.] [the audited consolidated financial statements consisting of a consolidated balance sheet and income statement and cash flows covering the operations of the Parent and its Subsidiaries for the fiscal year of the Parent ended as of [______] (the “Financials Date”). Such financial statements have been audited by independent public accountants of recognized national standing and accompanied by an opinion of such accountants (which is not qualified as to scope and does not contain any going concern or other qualification) to the effect that such consolidated financial statements fairly present, in all material respects, the financial position and results of operations of the Parent and its Subsidiaries on a consolidated basis in accordance with IFRS.]

(b)       The Borrower hereby certifies, represents and warrants that, as of the Financials Date and the date hereof, no Event of Default has occurred and is continuing [other than as follows:].

(c)       [Attached hereto as Annex I is a supplement to Schedules 5.07(b), 5.14, 5.17 and 5.20 of the Credit Agreement, updating the information set forth in Schedules 5.07(b), 5.14, 5.17 and 5.20 of the Credit Agreement as of the date hereof.] [The Borrower hereby certifies, represents and warrants that there has been no material change in the information set forth in Schedules 5.07(b), 5.14, 5.17 and 5.20 of the Credit Agreement since the Closing Date or the latest supplement to Schedules 5.07(b), 5.14, 5.17 and 5.20 of the Credit Agreement delivered to the Administrative Agent.]

(d)       [Attached hereto as Annex II is a supplement to Schedule II to the Security Agreement, updating the information set forth therein as of the date hereof.] [The Borrower hereby certifies, represents and warrants that there has been no material change in the information set forth in Schedule II to the Security Agreement since the date of the Security Agreement or the latest supplement to the Schedules thereto delivered to the Administrative Agent.]

IN WITNESS WHEREOF, the undersigned has caused this Certificate to be executed and delivered by a duly authorized Responsible Officer on this [____] day of [______], 20[___].

AEGERION PHARMACEUTICALS, INC.
By:
	Name:
	Title:

EXHIBIT D

FORM OF
ASSIGNMENT AND ASSUMPTION

Reference is made to the Credit Agreement dated as of September 24, 2019 (as amended, restated, supplemented or otherwise modified from time to time, the “Credit Agreement”) by and among AEGERION PHARMACEUTICALS, INC., a Delaware corporation (the “Borrower”), AMRYT PHARMA HOLDINGS PLC, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around September 24, 2019 as AMRYT PHARMA PLC), CANTOR FITZGERALD SECURITIES, as Administrative Agent, and each Lender from time to time party thereto. Capitalized terms used herein without definition shall have the meanings assigned to such terms in the Credit Agreement.

The “Assignor” referred to on Schedule 1 hereto (the “Assignor”) and the “Assignee” referred to on Schedule 1 hereto (the “Assignee”)¹ agrees severally with respect to all information relating to it and its assignment hereunder and on Schedule 1 hereto as follows:

1.    The Assignor hereby sells and assigns, without recourse except as to the representations and warranties made by it herein, to the Assignee, and the Assignee hereby purchases and assumes from the Assignor, an interest in and to the Assignor’s Commitments and/or Loans (and its rights and obligations relating thereto) under the Credit Agreement as of the Effective Date (as defined herein) equal to the percentage interest specified on Schedule 1 hereto of all outstanding rights and obligations identified on Schedule 1. After giving effect to such sale and assignment, the Assignee’s Commitments and the amount of the Loan owing to the Assignee will be as set forth on Schedule 1 hereto.

2.    The Assignor (i) represents and warrants that its name set forth on Schedule 1 hereto is its legal name, that it is the legal and beneficial owner of the interest or interests being assigned by it hereunder and that such interest or interests are free and clear of any lien, encumbrance or other adverse claim; (ii) makes no representation or warranty and assumes no responsibility with respect to any statements, warranties or representations made in or in connection with any Loan Document or the execution, legality, validity, enforceability, genuineness, sufficiency or value of, or the perfection or priority of any lien or security interest created or purported to be created under or in connection with, any Loan Document or any other instrument or document furnished pursuant thereto; and (iii) makes no representation or warranty and assumes no responsibility with respect to the financial condition of any Loan Party or the performance or observance by any Loan Party of any of its obligations under any Loan Document or any other instrument or document furnished pursuant thereto.

3.    The Assignee (i) confirms that, to the extent it has so requested, it has received a copy of the Credit Agreement, together with copies of such other documents and information as it has deemed appropriate to make its own credit analysis and decision to enter into this Assignment and Assumption; (ii) agrees that it will, independently and without reliance upon the Administrative Agent, any Assignor or any other Lender and based on such documents and information as it shall deem appropriate at the time, continue to make its own credit decisions in taking or not taking action under the Credit Agreement; (iii) represents and warrants that its name set forth on Schedule 1 hereto is its legal name; (iv) confirms that it is an Eligible Assignee and is not (x) a natural person, (y) the Borrower or an Affiliate of the Borrower or (z) a Disqualified Person; (v) appoints and authorizes the Administrative Agent to take such

¹ The form is to be adjusted in connection with any multiple party assignments.

action as agent on its behalf and to exercise such powers and discretion under the Loan Documents as are delegated to the Administrative Agent by the terms thereof, together with such powers and discretion as are reasonably incidental thereto; (vi) agrees that it will perform in accordance with their terms all of the obligations that by the terms of the Credit Agreement are required to be performed by it as a Lender; and (vii) attaches any other forms required under Section 3.01 of the Credit Agreement (and undertakes to deliver to the Administrative Agent originals of any such U.S. Internal Revenue Service form) and a completed Administrative Questionnaire required to be provided pursuant to Section 10.07(b) of the Credit Agreement.

4.    Following the execution of this Assignment and Assumption, it will be delivered to the Administrative Agent for acceptance and recording by the Administrative Agent, together with the processing fee referenced in Section 10.07(b)(iv). The effective date for this Assignment and Assumption (the “Effective Date”) shall be the date that such assignment is recorded in the Register pursuant to Section 10.07 of the Credit Agreement.

5.    Upon such acceptance and recording by the Administrative Agent, as of the Effective Date, (i)the Assignee shall be a party to the Credit Agreement and, to the extent provided in this Assignment and Assumption, have the rights and obligations of a Lender thereunder and (ii) the Assignor shall, to the extent provided in this Assignment and Assumption, relinquish its rights and be released from its obligations under the Credit Agreement in its capacity as a Lender (other than its rights and obligations under the Loan Documents that are specified under the terms of such Loan Documents to survive the payment in full of the Obligations of the Loan Parties under the Loan Documents to the extent any claim thereunder relates to an event arising prior to the Effective Date of this Assignment and Assumption) and, if this Assignment and Assumption covers all of the remaining portion of the rights and obligations of the Assignor in its capacity as a Lender under the Credit Agreement, the Assignor shall cease to be a party thereto in its capacity as a Lender.

6.    Upon such acceptance and recording by the Administrative Agent, from and after the Effective Date, the Administrative Agent shall make all payments under the Credit Agreement and the other Loan Documents in respect of the interest assigned hereby (including, without limitation, all payments of principal, interest and commitment fees with respect thereto) to the Assignee for amounts which have accrued from and after the Effective Date and to the Assignor for amounts which have accrued to but excluding the Effective Date.

7.    THIS ASSIGNMENT AND ASSUMPTION SHALL BE GOVERNED BY, AND CONSTRUED IN ACCORDANCE WITH, THE LAW OF THE STATE OF NEW YORK.

8.    This Assignment and Assumption may be executed in one or more counterparts, each of which shall be deemed an original, but all of which together shall constitute one and the same instrument. Delivery by facsimile transmission or other electronic transmission (including a .pdf or .tif copy) of an executed counterpart of a signature page to this Assignment and Assumption shall be effective as delivery of an original executed counterpart of this Assignment and Assumption; provided that original signatures shall be promptly delivered thereafter, it being understood that the failure to request or deliver the same shall not limit the effectiveness of this Assignment and Assumption or any signature delivered by facsimile or electronic transmission.

[signature page follows]

IN WITNESS WHEREOF, the Assignor and the Assignee have caused this Assignment and Assumption to be executed by their officers thereunto duly authorized as of the date specified thereon.

__________, as Assignor
[Type or print legal name of Assignor]
By:
	Title:
Dated: __________, 20__

__________, as Assignee
[Type or print legal name of Assignee]
By:
	Title:
Dated: __________, 20__
Lending Office:

[Accepted and Approved this ___ day of__________, 20__]
2 [CANTOR FITZGERALD SECURITIES, as Administrative Agent
By
	Name:
	Title:]

² If required by the Credit Agreement.

[Approved this___ day of__________, 20__]

³[AEGERION PHARMACEUTICALS, INC., as Borrower

By:

Name:  

Title:]

³ If required by the Credit Agreement.

SCHEDULE 1
TO
ASSIGNMENT AND ASSUMPTION

Loans/Commitment	Aggregate Amount of Loans/Commitment of all Lenders	Amount of Loans/Commitment Assigned	Percentage Assigned of Loans/Commitment of all Lenders
Loans	$	$	%

EXHIBIT E-l

FORM OF
GUARANTEE AND COLLATERAL AGREEMENT

EXHIBIT E-2

FORM OF
PLEDGE AGREEMENT

 

EXHIBIT F

FORM OF
OFFICER’S CERTIFICATE

This Officer’s Certificate (this “Certificate”) is delivered on September 24, 2019 pursuant to Section 4.01 of the Credit Agreement, dated as of the date hereof (as amended, restated, supplemented or otherwise modified from time to time, the “Credit Agreement”) among AEGERION PHARMACEUTICALS, INC., a Delaware corporation (the “Borrower”), AMRYT PHARMA HOLDINGS PLC, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around September 24, 2019 as AMRYT PHARMA PLC), CANTOR FITZGERALD SECURITIES, as Administrative Agent, and each Lender from time to time party thereto. Capitalized terms used herein which are not otherwise defined herein shall have the meanings assigned to such terms in the Credit Agreement.

I, [Name], [Title] of the Borrower DO HEREBY CERTIFY to the Administrative Agent and the Lenders, on behalf of the Borrower in my capacity as a Responsible Officer and not individually, that:

1.       No Default or Event of Default exists, or will result from, the incurrence of the Loans or from the application of proceeds therefrom.

2.       The representations and warranties of the Borrower and each other Loan Party contained in Article V of the Credit Agreement or any other Loan Document are true and correct in all material respects on and as of the Closing Date (before and after giving effect to the incurrence of the Loans); provided that to the extent that such representations and warranties specifically refer to an earlier date, they were true and correct in all material respects as of such earlier date; provided further that any representation and warranty that is qualified as to “materiality”, “Material Adverse Effect” or similar language is true and correct (after giving effect to any qualification therein) in all respects on such respective dates.

[Signature page follows.]

IN WITNESS WHEREOF, the undersigned has duly executed and delivered this Certificate as of the date first set forth above.

AEGERION PHARMACEUTICALS, INC.

Name:

Title:

EXHIBIT G

FORM OF
ADMINISTRATIVE AGENT QUESTIONNAIRE

[Provided under separate cover]

EXHIBIT H-1

FORM OF
U.S. TAX COMPLIANCE CERTIFICATE

(For Foreign Lenders That Are Not Partnerships For U.S. Federal Income Tax Purposes)

Reference is made to the Credit Agreement dated as of September 24, 2019 (as amended, restated, supplemented or otherwise modified from time to time, the “Credit Agreement”) among Aegerion Pharmaceuticals, Inc., a Delaware corporation (the “Borrower”), Amryt Pharma Holdings plc, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around September 24, 2019 as Amryt Pharma plc) (the “Parent”), Cantor Fitzgerald Securities, as Administrative Agent, and each Lender from time to time party thereto. All capitalized terms used and not defined herein have the meanings ascribed thereto in the Credit Agreement.

Pursuant to the provisions of Section 3.01 of the Credit Agreement, the undersigned hereby certifies that (i) it is the sole record and beneficial owner of the Loans (as well as any Notes evidencing such Loans) in respect of which it is providing this certificate, (ii) it is not a bank within the meaning of Section 881(c)(3)(A) of the Code, (iii) it is not a ten percent shareholder of the Borrower within the meaning of Section 871(h)(3)(B) of the Code and (iv) it is not a controlled foreign corporation related to the Borrower as described in Section 881(c)(3)(C) of the Code.

The undersigned has furnished the Administrative Agent and the Borrower with a certificate of its non-U.S. Person status on IRS Form W-8BEN or IRS Form W-8BEN-E, as applicable. By executing this certificate, the undersigned agrees that (1) if the information provided on this certificate changes, the undersigned shall promptly so inform the Borrower and the Administrative Agent, and (2) the undersigned shall have at all times furnished the Borrower and the Administrative Agent with a properly completed and currently effective certificate in either the calendar year in which each payment is to be made to the undersigned, or in either of the two calendar years preceding such payments.

Unless otherwise defined herein, terms defined in the Credit Agreement and used herein shall have the meanings given to them in the Credit Agreement.

[NAME OF LENDER]
By:
	Name:
	Title:
Date:______ __, 20[  ]

EXHIBIT H-2

FORM OF
U.S. TAX COMPLIANCE CERTIFICATE

(For Foreign Participants That Are Not Partnerships For U.S. Federal Income Tax Purposes)

Reference is made to the Credit Agreement dated as of September 24, 2019 (as amended, restated, supplemented or otherwise modified from time to time, the “Credit Agreement”) among Aegerion Phannaceuticals, Inc., a Delaware corporation (the “Borrower”), Amryt Pharma Holdings plc, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around September 24, 2019 as Amryt Pharma plc) (the “Parent”), Cantor Fitzgerald Securities, as Administrative Agent, and each Lender from time to time party thereto. All capitalized terms used and not defined herein have the meanings ascribed thereto in the Credit Agreement.

Pursuant to the provisions of Sections 3.01 and 10.07(d) of the Credit Agreement, the undersigned hereby certifies that (i) it is the sole record and beneficial owner of the participation in respect of which it is providing this certificate, (ii) it is not a bank within the meaning of Section 881(c)(3)(A) of the Code, (iii) it is not a ten percent shareholder of the Borrower within the meaning of Section 871(h)(3)(B) of the Code, and (iv) it is not a controlled foreign corporation related to the Borrower as described in Section 881(c)(3)(C) of the Code.

The undersigned has furnished its participating Lender with a certificate of its non-U.S. Person status on IRS Form W-8BEN or IRS Form W-8BEN-E, as applicable. By executing this certificate, the undersigned agrees that (1) if the information provided on this certificate changes, the undersigned shall promptly so inform such Lender in writing, and (2) the undersigned shall have at all times furnished such Lender with a properly completed and currently effective certificate in either the calendar year in which each payment is to be made to the undersigned, or in either of the two calendar years preceding such payments.

Unless otherwise defined herein, terms defined in the Credit Agreement and used herein shall have the meanings given to them in the Credit Agreement.

[NAME OF PARTICIPANT]
By:
	Name:
	Title:
Date:______ __, 20[  ]

EXHIBIT H-3

FORM OF

U.S. TAX COMPLIANCE CERTIFICATE

(For Foreign Participants That Are Partnerships For U.S. Federal Income Tax Purposes)

Reference is made to the Credit Agreement dated as of September 24, 2019 (as amended, restated, supplemented or otherwise modified from time to time, the “Credit Agreement”) among Aegerion Pharmaceuticals, Inc., a Delaware corporation (the “Borrower”), Amryt Pharma Holdings plc, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around September 24, 2019 as Amryt Pharma plc) (the “Parent”), Cantor Fitzgerald Securities, as Administrative Agent, and each Lender from time to time party thereto. All capitalized terms used and not defined herein have the meanings ascribed thereto in the Credit Agreement.

Pursuant to the provisions of Sections 3.01 and 10.07(d) of the Credit Agreement, the undersigned hereby certifies that (i) it is the sole record owner of the participation in respect of which it is providing this certificate, (ii) its direct or indirect partners/members are the sole beneficial owners of such participation, (iii) with respect such participation, neither the undersigned nor any of its direct or indirect partners/members is a bank extending credit pursuant to a loan agreement entered into in the ordinary course of its trade or business within the meaning of Section 881(c)(3)(A) of the Code, (iv) none of its direct or indirect partners/members is a ten percent shareholder of the Borrower within the meaning of Section 871(h)(3)(B) of the Code and (v) none of its direct or indirect partners/members is a controlled foreign corporation related to the Borrower as described in Section 881(c)(3)(C) of the Code.

The undersigned has furnished its participating Lender with IRS Form W-8IMY accompanied by one of the following forms from each of its partners/members that is claiming the portfolio interest exemption: (i) an IRS Form W-8BEN or IRS Form W-8BEN-E, as applicable, or (ii) an IRS Form W-8IMY accompanied by an IRS Form W-8BEN or IRS Form W-8BEN-E, as applicable, from each of such partner’s/member’s beneficial owners that is claiming the portfolio interest exemption. By executing this certificate, the undersigned agrees that (1) if the information provided on this certificate changes, the undersigned shall promptly so inform such Lender and (2) the undersigned shall have at all times furnished such Lender with a properly completed and currently effective certificate in either the calendar year in which each payment is to be made to the undersigned, or in either of the two calendar years preceding such payments.

Unless otherwise defined herein, terms defined in the Credit Agreement and used herein shall have the meanings given to them in the Credit Agreement.

[NAME OF PARTICIPANT]
By:
	Name:
	Title:
Date:______ __, 20[  ]

EXHIBIT H-4

FORM OF

U.S. TAX COMPLIANCE CERTIFICATE

(For Foreign Lenders That Are Partnerships For U.S. Federal Income Tax Purposes)

Reference is made to the Credit Agreement dated as of September 24, 2019 (as amended, restated, supplemented or otherwise modified from time to time, the “Credit Agreement”) among Aegerion Pharmaceuticals, Inc., a Delaware corporation (the “Borrower”), Amryt Pharma Holdings plc, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around September 24, 2019 as Amryt Pharma plc) (the “Parent”), Cantor Fitzgerald Securities, as Administrative Agent, and each Lender from time to time party thereto. All capitalized terms used and not defined herein have the meanings ascribed thereto in the Credit Agreement.

Pursuant to the provisions of Section 3.01 of the Credit Agreement, the undersigned hereby certifies that (i) it is the sole record owner of the Loans (as well as any Notes evidencing such Loans) in respect of which it is providing this certificate, (ii) its direct or indirect partners/members are the sole beneficial owners of such Loans (as well as any Notes evidencing such Loans), (iii) with respect to the extension of credit pursuant to this Credit Agreement or any other Loan Document, neither the undersigned nor any of its direct or indirect partners/members is a bank extending credit pursuant to a loan agreement entered into in the ordinary course of its trade or business within the meaning of Section 881(c)(3)(A) of the Code, (iv) none of its direct or indirect partners/members is a ten percent shareholder of the Borrower within the meaning of Section 871(h)(3)(B) of the Code and (v) none of its direct or indirect partners/members is a controlled foreign corporation related to the Borrower as described in Section 881(c)(3)(C) of the Code.

The undersigned has furnished the Administrative Agent and the Borrower with IRS Form W-8IMY accompanied by one of the following forms from each of its partners/members that is claiming the portfolio interest exemption: (i) an IRS Form W-8BEN or IRS Form W-8BEN-E, as applicable, or (ii) an IRS Form W-8IMY accompanied by an IRS Form W-8BEN or IRS Form W-8BEN-E, as applicable, from each of such partner’s/member’s beneficial owners that is claiming the portfolio interest exemption. By executing this certificate, the undersigned agrees that (1) if the information provided on this certificate changes, the undersigned shall promptly so inform the Borrower and the Administrative Agent, and (2) the undersigned shall have at all times furnished the Borrower and the Administrative Agent with a properly completed and currently effective certificate in either the calendar year in which each payment is to be made to the undersigned, or in either of the two calendar years preceding such payments.

Unless otherwise defined herein, terms defined in the Credit Agreement and used herein shall have the meanings given to them in the Credit Agreement.

[NAME OF LENDER]
By:
	Name:
	Title:
Date:______ __, 20[  ]

EX-10.6

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

 Exhibit 10.6

Execution Version

AEGERION PHARMACEUTICALS. INC.,

THE GUARANTOR PARTIES HERETO

and

GLAS TRUST COMPANY LLC

as Trustee

INDENTURE

Dated as of September 24, 2019

5.00% Convertible Senior Notes due 2025

CROSS REFERENCE TABLE*

Trust  Indenture  Act  Section		Indenture  Section
310	(a)(1)	11.09
	(a)(2)	11.09
	(a)(3)	N/A
	(a)(4)	N/A
	(a)(5)	N/A
	(b)	11.09, 11.03
	(c)	N/A
311	(a)	11.03
	(b)	11.03
	(c)	N/A
312	(a)	2.08
	(b)	N/A
	(c)	N/A
313	(a)	N/A
	(b)(1)	N/A
	(b)(2)	N/A
	(c)	N/A
	(d)	N/A
314	(a)	3.02(A)
	(b)	N/A
	(c)(1)	12.02(A)
	(c)(2)	12.02(B)
	(c)(3)	N/A
	(d)	N/A
	(e)	12.02(A), 12.02(B)
	(f)	N/A
315	(a)	11.01(B)
	(b)	11.05
	(c)	11.01(A)
	(d)	11.01(C)
	(e)	7.12
316	(a) (last sentence)	2.15
	(a)(1)(A)	7.06
	(a)(1)(B)	7.05
	(a)(2)	N/A
	(b)	7.08
	(c)	N/A
317	(a)(1)	7.09
	(a)(2)	7.10
	(b)	2.07

Trust  Indenture  Act  Section		Indenture  Section
318	(a)	N/A
	(b)	12.17
	(c)	N/A

N/A means not applicable. 

* This Cross Reference Table is not part of the Indenture.

TABLE OF CONTENTS

			Page
Article 1.    Definitions; Rules of Construction			1
	Section 1.01.	Definitions	1
	Section 1.02.	Other Definitions	17
	Section 1.03.	Rules of Construction	18
Article 2.   The Notes			19
	Section 2.01.	Form, Dating and Denominations	19
	Section 2.02.	Execution, Authentication and Delivery	20
	Section 2.03.	Initial Notes and Additional Notes	21
	Section 2.04.	Method of Payment	21
	Section 2.05.	Accrual of Interest; Defaulted Amounts; When Payment Date is Not a Business Day	22
	Section 2.06.	Registrar, Paying Agent and Conversion Agent	22
	Section 2.07.	Paying Agent and Conversion Agent to Hold Property in Trust	23
	Section 2.08.	Holder Lists	24
	Section 2.09.	Legends	24
	Section 2.10.	Transfers and Exchanges; Certain Transfer Restrictions	24
	Section 2.11.	Exchange and Cancellation of Notes to Be Converted, Redeemed or Repurchased	30
	Section 2.12.	Replacement Notes	31
	Section 2.13.	Registered Holders; Certain Rights with Respect to Global Notes	31
	Section 2.14.	Cancellation	32
	Section 2.15.	Notes Held by the Company	32
	Section 2.16.	Temporary Notes	32
	Section 2.17.	Outstanding Notes	32
	Section 2.18.	Repurchases by the Company	33
	Section 2.19.	CUSIP and ISIN Numbers	33
Article 3.   Covenants			34
	Section 3.01.	Payment on Notes	34
	Section 3.02.	Reports	34
	Section 3.03.	Additional Interest	34
	Section 3.04.	Additional Amounts	35
	Section 3.05.	Compliance and Default Certificates	38
	Section 3.06.	Stay, Extension and Usury Laws	38
	Section 3.07.	Corporate Existence	38
	Section 3.08.	Restriction on Acquisition of Notes by the Company	38
	Section 3.09.	Further Instruments and Acts	39
	Section 3.10.	Listing	39
	Section 3.11.	Restricted Payments	39
	Section 3.12.	Dispositions	40
	Section 3.13.	Indebtedness	41

- i -

Article 4.   Repurchase and Redemption			43
	Section 4.01.	No Sinking Fund	43
	Section 4.02.	Right of Holders to Require the Company to Repurchase Notes upon a Fundamental Change	43
	Section 4.03.	Right of the Company to Redeem the Notes	47
Article 5.   Conversion			51
	Section 5.01.	Right to Convert	51
	Section 5.02.	Conversion Procedures	52
	Section 5.03.	Settlement upon Conversion	53
	Section 5.04.	Reserve and Status of Ordinary Shares Issued upon Conversion	56
	Section 5.05.	Adjustments to the Conversion Rate	56
	Section 5.06.	Voluntary Adjustments	66
	Section 5.07.	Adjustments to the Conversion Rate in Connection with a Make-Whole Fundamental Change	66
	Section 5.08.	Restriction on Conversions	68
	Section 5.09.	Effect of Ordinary Share Change Event	69
	Section 5.10.	Responsibility of Trustee	70
Article 6.   Successors			71
	Section 6.01.	When the Company May Merge, Etc.	71
	Section 6.02.	Successor Corporation Substituted	71
Article 7.   Defaults and Remedies			72
	Section 7.01.	Events of Default	72
	Section 7.02.	Acceleration	74
	Section 7.03.	Sole Remedy for a Failure to Report	74
	Section 7.04.	Other Remedies	75
	Section 7.05.	Waiver of Past Defaults	76
	Section 7.06.	Control by Majority	76
	Section 7.07.	Limitation on Suits	76
	Section 7.08.	Absolute Right of Holders to Receive Payment and Conversion Consideration	77
	Section 7.09.	Collection Suit by Trustee	77
	Section 7.10.	Trustee May File Proofs of Claim	77
	Section 7.11.	Priorities	78
	Section 7.12.	Undertaking for Costs	78
Article 8.   Amendments, Supplements and Waivers			78
	Section 8.01.	Without the Consent of Holders	78
	Section 8.02.	With the Consent of Holders	79
	Section 8.03.	Notice of Amendments, Supplements and Waivers	81
	Section 8.04.	Revocation, Effect and Solicitation of Consents; Special Record Dates; Etc.	81
	Section 8.05.	Notations and Exchanges	81
	Section 8.06.	Trustee to Execute Supplemental Indentures	82

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Article 9.   Guarantees			82
	Section 9.01.	Guarantees	82
	Section 9.02.	Limitation on Guarantor Liability	83
	Section 9.03.	Execution and Delivery of Guarantee	84
	Section 9.04.	When Guarantors May Merge, Etc.	84
	Section 9.05.	Future Guarantors	85
	Section 9.06.	Application of Certain Provisions to the Guarantors	86
Article 10.   Satisfaction and Discharge			86
	Section 10.01.	Termination of Company’s Obligations	86
	Section 10.02.	Repayment to Company	87
	Section 10.03.	Reinstatement	87
Article 11.   Trustee			87
	Section 11.01.	Duties of the Trustee	87
	Section 11.02.	Rights of the Trustee	89
	Section 11.03.	Individual Rights of the Trustee	90
	Section 11.04.	Trustee’s Disclaimer	90
	Section 11.05.	Notice of Defaults	91
	Section 11.06.	Compensation and Indemnity	91
	Section 11.07.	Replacement of the Trustee	92
	Section 11.08.	Successor Trustee by Merger, Etc.	93
	Section 11.09.	Eligibility; Disqualification	93
Article 12.   Miscellaneous			94
	Section 12.01.	Notices	94
	Section 12.02.	Delivery of Officer’s Certificate and Opinion of Counsel as to Conditions Precedent	96
	Section 12.03.	Statements Required in Officer’s Certificate and Opinion of Counsel	96
	Section 12.04.	Rules by the Trustee, the Registrar and the Paying Agent	96
	Section 12.05.	No Personal Liability of Directors, Officers, Employees and Shareholders	96
	Section 12.06.	Governing Law; Waiver of Jury Trial	97
	Section 12.07.	Submission to Jurisdiction	97
	Section 12.08.	No Adverse Interpretation of Other Agreements	97
	Section 12.09.	Successors	97
	Section 12.10.	Force Majeure	97
	Section 12.11.	U.S.A. PATRIOT Act	98
	Section 12.12.	Calculations; Determinations	98
	Section 12.13.	Severability	98
	Section 12.14.	Counterparts	98
	Section 12.15.	Table of Contents, Headings, Etc.	98
	Section 12.16.	Withholding Taxes	99
	Section 12.17.	Trust Indenture Act Controls	99
Exhibits
Exhibit A: Form of Note			A-l

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Exhibit B: Form of Global Note Legend B-1

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INDENTURE, dated as of September 24, 2019, among Aegerion Pharmaceuticals, Inc., a Delaware corporation, as issuer (the “Company”), Amryt Pharma Holdings plc, a company incorporated in England and Wales with company number 12107859 (to be renamed on or around the date hereof as Amryt Pharma plc) (the “Parent”), Amryt Pharma plc, a company incorporated in England and Wales with company number 05316808 (to be renamed and re-registered on or around the date hereof as Amryt Pharma Holdings Limited) (“Old Parent”), the additional guarantors listed on the signature pages hereto, as guarantors (together with the Parent and Old Parent, the “Guarantors”), and GLAS Trust Company LLC, a limited liability company organized and existing under the laws of the State of New Hampshire, as trustee (the “Trustee”).

Each party to this Indenture (as defined below) agrees as follows for the benefit of the other party and for the equal and ratable benefit of the Holders (as defined below) of the Company’s 5.00% Convertible Senior Notes due 2025 (the “Notes”).

Article 1.      DEFINITIONS; RULES OF CONSTRUCTION

Section 1.01. Definitions.

“144A Global Note” means a Global Note bearing the Private Placement Legend and deposited with or on behalf of, and registered in the name of, the Depositary or its nominee that will be issued in a denomination equal to the outstanding principal amount of the Notes and initially issued in reliance on Rule 144A.

“Additional Interest” means any interest that accrues on any Note pursuant to Section 3.03.

“Affiliate” has the meaning set forth in Rule 144 as in effect on the Issue Date.

“Applicable Procedures” means, with respect to any transfer or exchange of or for beneficial interests in any Global Note, the rules and procedures of the Depositary, Euroclear and Clearstream that apply to such transfer or exchange.

“Attributable Indebtedness” means, at any date, (a) in respect of any Capital Lease Obligation (other than a lease resulting from a Sale Leaseback) of any Person, the capitalized amount thereof that would appear on a balance sheet of such Person prepared as of such date in accordance with IFRS, and (b) in respect of any Sale Leaseback, the present value, discounted in accordance with IFRS, at the interest rate implicit in the related lease, of the obligations of the lessee for net rental payments over the remaining term of such lease (including any period for which such lease has been extended or may, at the option of the lessor be extended).

“Authorized Denomination” means, with respect to a Note, a principal amount thereof equal to $1,000 or any integral multiple of $1 in excess thereof.

“Bankruptcy Law” means Title 11, United States Code, or any similar U.S. federal or state or non-U.S. law for the relief of debtors.

“Board of Directors” means the board of directors of the Company or a committee of

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such board duly authorized to act on behalf of such board.

“Business Day” means any day other than (i) a Saturday, (ii) a Sunday or (iii) any day on which banking institutions in New York City or London are authorized or required by law or executive order to close or be closed.

“Capital Lease” means, with respect to any Person, any leasing or similar arrangement conveying the right to use any property, whether real or personal property, or a combination thereof, by that Person as lessee that, in conformity with IFRS, is required to be accounted for as a capital lease on the balance sheet of such Person.

“Capital Lease Obligation” means, with respect to any Person, all monetary or financial obligations of such Person and its Subsidiaries under any Capital Leases, and the amount of such obligations shall be the capitalized amount thereof determined in accordance with IFRS, and the stated maturity thereof shall be the date of the last payment of rent or any other amount due under such lease prior to the first date on which such lease may be terminated by the lessee without payment of a penalty; provided that any obligations that were not required to be included on the balance sheet of such Person as capital lease obligations when incurred but are subsequently re-characterized as capital lease obligations due to a change in accounting rules after the Issue Date shall for all purposes hereunder not be treated as a Capital Lease Obligation.

“Capital Stock” of any Person means any and all shares of, interests in, rights to purchase, warrants or options for, participations in, or other equivalents of, in each case however designated, the equity of such Person, but excluding any debt securities convertible into such equity.

“Cash Equivalents” means any of the following: (A) readily marketable direct obligations of the Government of the United States or any agency or instrumentality thereof or obligations unconditionally guaranteed by the full faith and credit of the Government of the United States, (B) securities with maturities of 365 days or less from the date of acquisition that are issued or fully guaranteed by any state, district or territory of the United States, by any political subdivision or taxing authority of any such state, district or territory or by any foreign government, the securities of which state, district or territory, taxing authority or foreign government (as the case may be) are rated at least A by S&P or A by Moody’s, (C) commercial paper maturing not more than two hundred and seventy (270) days from the date of issue and issued by a corporation (other than an Affiliate of the Company or any Guarantor) organized under the laws of any state of the United States of America or of the District of Columbia and, at the time of acquisition thereof, rated A 2 or higher by S&P, P 2 or higher by Moody’s or F2 or higher by Fitch, (D) money market mutual or similar funds that invest substantially all of their assets in one or more type of securities satisfying the requirements of clauses (A) through (C) of this definition, (E) Investments, classified in accordance with IFRS as current assets of the Parent or any of its Subsidiaries, in money market investment programs registered under the Investment Company Act of 1940, as amended, which are administered by financial institutions having capital of at least $500,000,000, and the portfolios of which are limited solely to Investments of the character, quality and maturity described in clause (A) of this definition, (F) agencies (LSE’s), State (municipal bonds), or corporate bonds having a long term rating of at least A- or

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A3 from S&P, Moody’s or Fitch, having maturities of not more than fifteen (15) months from the date of acquisition and (G) money market funds having a rating of AAAm/Aaa or better from S&P, Moody’s or Fitch.

“Change in Tax Law” means any change or amendment in the laws, rules or regulations of a Relevant Taxing Jurisdiction, or any change in an official written interpretation, administration or application of such laws, rules or regulations by any legislative body, court, governmental taxing authority or regulatory or administrative authority of such Relevant Taxing Jurisdiction (including the enactment of any legislation and the publication of any judicial decision or regulatory or administrative interpretation or determination) affecting taxation, which change or amendment (A) had not been theretofore publicly announced; and (B) becomes effective on or after September 24, 2019 (or, if the Relevant Taxing Jurisdiction was not a Relevant Taxing Jurisdiction on such date, the date on which such Relevant Taxing Jurisdiction became a Relevant Taxing Jurisdiction).

“Close of Business” means 5:00 p.m., New York City time.

“Company” means the Person named as such in the first paragraph of this Indenture and, subject to Article 6, its successors and assigns.

“Company Order” means a written request or order signed on behalf of the Company by one (1) of its Officers and delivered to the Trustee.

“Conversion Date” means, with respect to a Note, the first Business Day on which the requirements set forth in Section 5.02(A) to convert such Note are satisfied.

“Conversion Price” means, as of any time, an amount equal to (A) one thousand dollars ($1,000) divided by (B) the Conversion Rate in effect at such time.

“Conversion Rate” initially means 386.75 Ordinary Shares per $1,000 principal amount of Notes; provided, however, that the Conversion Rate is subject to adjustment pursuant to Article 5; provided, further, that whenever this Indenture refers to the Conversion Rate as of a particular date without setting forth a particular time on such date, such reference will be deemed to be to the Conversion Rate immediately after the Close of Business on such date.

“Conversion Share” means any Ordinary Share issued or issuable upon conversion of any Note.

“Cumulative Credit” means the sum of (without duplication) (A) five million dollars ($5,000,000), (B) fifty percent (50%) of the consolidated net income of the Parent for the period from the Issue Date to the end of the Company’s most recently ended fiscal quarter for which financials statements have been delivered in accordance with Section 3.02(A) at the time of such Restricted Payment and (C) one hundred percent (100%) of the aggregate net proceeds, of property received by the Company after the Issue Date from the issue or sale of Equity Interests (other than any Equity Interests under the CVR Instrument, excluding any such net proceeds used to pay amounts under the CVR Instrument.

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“CVR Instrument” means the deed poll entered into by the Parent on or around the date of this Indenture constituting certain contingent value rights.

“Daily Cash Amount” means, with respect to any VWAP Trading Day, the lesser of (A) the applicable Daily Maximum Cash Amount; and (B) the Daily Conversion Value for such VWAP Trading Day.

“Daily Conversion Value” means, with respect to any VWAP Trading Day, one-twentieth (l/20th) of the product of (A) the Conversion Rate on such VWAP Trading Day; and (B) the Daily VWAP per Ordinary Share on such VWAP Trading Day.

“Daily Maximum Cash Amount” means, with respect to the conversion of any Note, the quotient obtained by dividing (A) the Specified Dollar Amount applicable to such conversion by (B) twenty (20).

“Daily Share Amount” means, with respect to any VWAP Trading Day, the quotient obtained by dividing (A) the excess, if any, of the Daily Conversion Value for such VWAP Trading Day over the applicable Daily Maximum Cash Amount by (B) the Daily VWAP for such VWAP Trading Day. For the avoidance of doubt, the Daily Share Amount will be zero for such VWAP Trading Day if such Daily Conversion Value does not exceed such Daily Maximum Cash Amount.

“Daily VWAP” means, for any VWAP Trading Day, the per share volume-weighted average price of the Ordinary Shares on the Relevant Stock Exchange in respect of the period from the scheduled open of trading until the scheduled close of trading of the primary trading session on such VWAP Trading Day (or, if such volume-weighted average price is unavailable, the market value of one Ordinary Share on such VWAP Trading Day, determined, using a volume-weighted average price method, by a nationally recognized independent investment banking firm selected by the Company, which may include any of the Investors). The Daily VWAP will be determined without regard to after-hours trading or any other trading outside of the regular trading session. The Daily VWAP for any VWAP Trading Day will be expressed in U.S. dollars and, if expressed in a different currency for such VWAP Trading Day as determined above (which, for the avoidance of doubt, will be the case at the time the Notes are initially issued), will be translated to U.S. dollars at the Prevailing Exchange Rate on such VWAP Trading Day.

“Deed Poll Constituting Loan Notes” means the deed poll to be entered into by the Parent in the form set out in schedule 1 of the CVR Instrument if it elects to satisfy its payment obligations under the CVR Instrument by the issue of loan notes.

“Default” means any event that is (or, after notice, passage of time or both, would be) an Event of Default.

“Default Settlement Method” means Combination Settlement with a Specified Dollar Amount of $1,000 per $1,000 principal amount of Notes; provided, however, that the Company

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may, from time to time, change the Default Settlement Method by sending notice of the new Default Settlement Method to the Holders, the Trustee and the Conversion Agent.

“Depositary” means The Depository Trust Company or its successor.

“Depositary Participant” means any member of, or participant in, the Depositary.

“Depositary Procedures” means, with respect to any conversion, transfer, exchange or transaction involving a Global Note or any beneficial interest therein, the rules and procedures of the Depositary applicable to such conversion, transfer, exchange or transaction.

“Disposition” or “Dispose” means the sale, transfer, license, lease or other disposition of any asset or property by the Parent or any of its Subsidiaries (including any Spin-Off, Sale Leaseback and any sale of Equity Interests, but excluding any issuance by the Company or a Guarantor of its own Equity Interests).

“Disqualified Equity Interests” has the meaning set forth in the Senior Secured Credit Facility.

“Equity Interests” means, with respect to any Person, all of the shares of Capital Stock of (or other ownership or profit interests in) such Person, all of the warrants, options or other rights for the purchase or acquisition from such Person of shares of Capital Stock of (or other ownership or profit interests in) such Person, all of the securities convertible into or exchangeable for shares of Capital Stock of (or other ownership or profit interests in) such Person or warrants, rights or options for the purchase or acquisition from such Person of such shares (or such other interests), and all of the other ownership or profit interests in such Person (including partnership, member or trust interests therein), whether voting or nonvoting, in each case, excluding the Notes, and any refinancings thereof.

“Ex-Dividend Date” means, with respect to an issuance, dividend or distribution on the Ordinary Shares, the first date on which the Ordinary Shares trade on the Relevant Stock Exchange (or, if there is no Relevant Stock Exchange, on the other applicable exchange or in the applicable market), regular way, without the right to receive such issuance, dividend or distribution (including pursuant to due bills or similar arrangements required by the relevant stock exchange). For the avoidance of doubt, any alternative trading convention on the applicable exchange or market in respect of the Ordinary Shares under a separate ticker symbol or CUSIP number will not be considered “regular way” for this purpose.

“ExchangeAct” means the U.S. Securities Exchange Act of 1934, as amended.

“Freely Tradable” means, with respect to any Note, that such Note would be eligible to be offered, sold or otherwise transferred pursuant to Rule 144 or otherwise if held by a Person that is not an Affiliate of the Company, and that has not been an Affiliate of the Company during the immediately preceding three (3) months, without any requirements as to volume, manner of sale, availability of current public information or notice under the Securities Act (except that, during the six (6) month period beginning on, and including, the date that is six (6) months after

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the Last Original Issue Date of such Note, any such requirement as to the availability of current public information will be disregarded if the same is satisfied at that time), and such Note (x) is not identified by a “restricted” CUSIP or ISIN number; and (y) is not represented by any certificate that bears a restricted note legend.

“Fundamental Change” means any of the following events:

(A)          a “person” or “group” (within the meaning of Section 13(d)(3) of the Exchange Act), other than the Parent or its Wholly Owned Subsidiaries, or their respective employee benefit plans, files any report with the SEC indicating that such person or group has become the direct or indirect “beneficial owner” (as defined below) of shares of the Parent’s common equity representing more than fifty percent (50%) of the voting power of all of the Parent’s then-outstanding common equity; provided, however, that, for these purposes, no “person” or “group” will be deemed to be the beneficial owner of any securities tendered pursuant to a tender or exchange offer made by or on behalf of such “person” or “group” until such tendered securities are accepted for purchase or exchange under such offer:

(B)           the consummation of (i) any sale, lease or other transfer, in one transaction or a series of transactions, of all or substantially all of the assets of the Parent and its Subsidiaries, taken as a whole, to any Person, other than solely to one or more of the Parent’s Wholly Owned Subsidiaries; or (ii) any transaction or series of related transactions in connection with which (whether by means of merger, consolidation,: share exchange, combination, reclassification, recapitalization, acquisition, liquidation or otherwise) all of the Ordinary Shares are exchanged for, converted into, acquired for, or constitutes solely the right to receive, other securities, cash or other property (other than changes resulting solely from a subdivision or combination, or a change in par value, of the Ordinary Shares); provided, however, that any merger, consolidation, share exchange or combination of the Parent pursuant to which the Persons that directly or indirectly “beneficially owned” (as defined below) all classes of the Parent’s common equity immediately before such transaction directly or indirectly “beneficially own,” immediately after such transaction, more than fifty percent (50%) of all classes of common equity of the surviving, continuing or acquiring company or other transferee, as applicable, or the parent thereof, in substantially the same proportions vis-a-vis each other as immediately before such transaction will be deemed not to be a Fundamental Change pursuant to this clause (B);

(C)           the Parent’s shareholders approve any plan or proposal for the liquidation or dissolution of the Parent; or

(D)           the Ordinary Shares cease to be listed on any Permitted Exchange;

provided, however, that a transaction or event described in clause (A) or (B) above will not constitute a Fundamental Change if at least ninety percent (90%) of the consideration received or to be received by the holders of Ordinary Shares (excluding cash payments for fractional shares or pursuant to dissenters rights), in connection with such transaction or event, consists of shares of common stock or ordinary shares listed (or depositary receipts representing shares of common stock or ordinary shares, which depositary receipts are listed) on any Permitted Exchange, or that will be so listed when issued or exchanged in connection with such transaction or event, and such transaction or event constitutes an Ordinary Share Change Event whose Reference Property

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consists of such consideration.

Notwithstanding the foregoing, (i) the transactions contemplated by the Plan (including the establishment of a holding company above the Parent) shall not be deemed, individually or in the aggregate, to constitute a Fundamental Change and (ii) a Fundamental Change shall not be deemed to occur pursuant to clause (A) or (B) above if (x) Parent becomes a direct or indirect wholly-owned subsidiary of a holding company or a holding company becomes the successor to Parent under Section 6.02 pursuant to a transaction that is permitted under Section 6.01 of the Indenture and (y) the direct or indirect holders of the Voting Stock of such holding company immediately following that transaction (or a series of related transactions) are substantially the same (and hold in the same proportions) as the holders of Parent’s Voting Stock immediately prior to that transaction. “Voting Stock” means, with respect to any specified Person as of any date, the capital stock of such Person that is at the time entitled to vote generally in the election of the board of directors of such Person.

For purposes of clause (A) of the definition of Fundamental Change, no Fundamental Change shall be deemed to occur as a result of any of Athyrium Capital Management, LP or its affiliates or Highbridge MSF International Ltd., 1992 Tactical Credit Master Fund, L.P. or Highbridge SCF Special Situations SPV, L.P., or their respective affiliates, or any combination of the foregoing persons, becoming the “beneficial owner” of more than 50% of the voting power of all of the Parent’s then-outstanding common equity.

For the purposes of this definition, (x) any transaction or event described in both clause (A) and in clause (B)(i) or (ii) above (without regard to the proviso in clause (B)) will be deemed to occur solely pursuant to clause (B) above (subject to such proviso); and (y) whether a Person is a “beneficial owner” and whether shares are “beneficially owned” will be determined in accordance with Rule 13d-3 under the Exchange Act.

“Fundamental Change Repurchase Date” means the date fixed for the repurchase of any Notes by the Company pursuant to Section 4.02(C).

“Fundamental Change Repurchase Notice” means a notice (including a notice substantially in the form of the “Fundamental Change Repurchase Notice” set forth in Exhibit A) containing the information, or otherwise complying with the requirements, set forth in Section 4.02(F)(i) and Section 4.02(F)(ii).

“Fundamental Change Repurchase Price” means the cash price payable by the Company to repurchase any Note upon its Repurchase Upon Fundamental Change, calculated pursuant to Section 4.02(D).

“Global Note” means a Note that is represented by a certificate substantially in the form set forth in Exhibit A, registered in the name of the Depositary or its nominee and bearing the Global Note Legend, duly executed by the Company and authenticated by the Trustee, and deposited with the Trustee, as custodian for the Depositary.

“Global Note Legend” means a legend substantially in the form set forth in Exhibit B.

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“Guarantee” means the guarantee by each Guarantor of the Company’s obligations under this Indenture and the Notes pursuant to Article 9.

“Guarantor” means the Persons named as such in the first paragraph of this Indenture, each other Person that becomes a Guarantor by executing an amended or supplemental indenture pursuant to Sections 8.01(B) and 9.03 and, subject to Section 9.04, the successors and assigns of the foregoing.

“Holder” means a person in whose name a Note is registered on the Registrar’s books.

“IAI Global Note” means a Global Note bearing the Private Placement Legend, duly executed by the Company and authenticated by the Trustee, and deposited with the Trustee, as custodian for the Depositary, and initially issued to Institutional Accredited Investors.

“IFRS” means International Financial Reporting Standards, as in effect from time to time.

“Immaterial Subsidiaries” has the meaning set forth in the Senior Secured Credit Facility.

“Indebtedness” of any Person means, without duplication, (a) all obligations of such Person for borrowed money, (b) all obligations of such Person evidenced by bonds, debentures, notes or similar instruments, (c) all obligations of such Person under conditional sale or other title retention agreements relating to property acquired by such Person, (d) all obligations of such Person in respect of the deferred purchase price of property or services (excluding (i) accounts payable and other accrued liabilities incurred in the ordinary course of business not past due for more than one hundred twenty (120) days after its stated due date (except for accounts payable contested in good faith), (ii) any earn out obligation until such obligation is both required to be reflected as a liability on the balance sheet of such Person in accordance with IFRS and not paid after becoming due and payable, (iii) deferred or equity compensation arrangements entered into in the ordinary course of business and payable to directors, officers or employees) and (iv) milestone payments due to Software AG Stiftung in connection with Birkin AG in an aggregate amount not to exceed 38,000,000 Euros which shall be payable solely on the basis of the criteria disclosed pursuant to the Senior Secured Credit Facility prior to the Issue Date), (e) all Indebtedness (excluding prepaid interest thereon) of others secured by (or for which the holder of such Indebtedness has an existing right, contingent or otherwise, to be secured by) any lien on property owned or acquired by such Person, whether or not the Indebtedness secured thereby has been assumed but, in the case of Indebtedness which is not assumed by such Person, limited to the lesser of (x) the amount of such Indebtedness and (y) the fair market value of such property, (f) all guarantees by such Person of Indebtedness of others, (g) all Attributable Indebtedness of such Person, (h) all obligations, contingent or otherwise, of such Person as an account party in respect of letters of credit and letters of guaranty (excluding the portion thereof that has been fully cash collateralized in a manner permitted by the Senior Secured Credit Facility), (i) all obligations, contingent or otherwise, of such Person in respect of bankers’ acceptances, surety bonds and performance bonds, whether or not matured and (j) all obligations of such Person in

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respect of Disqualified Equity Interests. The Indebtedness of any Person shall include the Indebtedness of any other entity (including any partnership in which such Person is a general partner) to the extent such Person is directly liable therefor as a result of such Person’s ownership interest in or other relationship with such entity, except to the extent the terms of such Indebtedness provide that such Person is not liable therefor. Anything herein to the contrary notwithstanding, obligations in respect of any Indebtedness that has been irrevocably defeased (either covenant or legal) or satisfied and discharged pursuant to the terms of the instrument creating or governing such Indebtedness shall not constitute Indebtedness.

“Indenture” means this Indenture, as amended or supplemented from time to time.

“Indirect Participant” means a Person who holds a beneficial interest in a Global Note through a Participant.

“Institutional Accredited Investor” means an institution that is an “accredited investor” as defined in Rule 501(a)(1), (2), (3) or (7) under the Securities Act.

“Intellectual Property” has the meaning set forth in the Senior Secured Credit Facility.

“Internal Revenue Code” means the United States Internal Revenue Code of 1986, as amended.

“Investment” in any Person, means any loan or advance to such Person, any purchase or other acquisition of any voting Equity Interests or other Equity Interests or Indebtedness or the assets comprising a division or business unit or a substantial part or all of the business of such Person, any capital contribution to such Person or any other direct or indirect investment in such Person.

“Investors” means existing unsecured creditors of the Company, other than the Department of Justice and other governmental entities and trade creditors, allocated (a) on a pro rata basis based on the respective principal or face amount of the respective unsecured claims and (b) dollar for dollar in the case of the existing $22.5 million roll up loans under the Company’s secured bridge financing that was funded in November 2018 (plus accrued fees and interest thereon, estimated to be approximately $500,000) and any amounts funded under the debtor-in-possession financing upon the emergence of the Company’s bankruptcy case that are not paid in full in cash on the Plan effective date.

“Interest Payment Date” means, with respect to a Note, each April 1 and October 1 of each year, commencing on April 1, 2020 (or commencing on such other date specified in the certificate representing such Note). For the avoidance of doubt the Maturity Date is an Interest Payment Date.

“Issue Date” means September 24, 2019.

“Last Original Issue Date” means (A) with respect to any Notes issued pursuant to the Plan, and any Notes issued in exchange therefor or in substitution thereof, the Issue Date and (B)

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with respect to any Notes issued pursuant to Section 2.03(B), and any Notes issued in exchange therefor or in substitution thereof, either (i) the date such Notes are originally issued or (ii) such other date as is specified in an Officer’s Certificate delivered to the Trustee before the original issuance of such Notes.

“Last Reported Sale Price” of the Ordinary Shares for any Trading Day means the closing sale price per share (or, if no closing sale price is reported, the average of the last bid price and the last ask price per share or, if more than one in either case, the average of the average last bid prices and the average last ask prices per share) of the Ordinary Shares on such Trading Day as reported in composite transactions for the Relevant Stock Exchange. If the Last Reported Sale Price cannot be determined pursuant to the previous sentence, then the Last Reported Sale Price will be the average of the mid-point of the last bid price and the last ask price per Ordinary Share on such Trading Day from each of at least three (3) nationally recognized independent investment banking firms selected by the Company, which may include any of the Investors. Neither the Trustee nor the Conversion Agent will have any duty to determine the Last Reported Sale Price. The Last Reported Sale Price will be determined without regard to after-hours trading or any other trading outside of the regular trading session. The Last Reported Sale Price for any Trading Day will be expressed in U.S. dollars and, if expressed in a different currency for such Trading Day as determined above (which, for the avoidance of doubt, will be the case at the time the Notes are initially issued), will be translated to U.S. dollars at the Prevailing Exchange Rate on such Trading Day.

“Make-Whole Fundamental Change” means (A) a Fundamental Change (determined after giving effect to the proviso immediately after clause (D) of the definition thereof, but without regard to the proviso to clause (B)(ii) of the definition thereof); or (B) the sending of a Redemption Notice pursuant to Section 4.03(G); provided, however, that the sending of a Redemption Notice will constitute a Make-Whole Fundamental Change only with respect to the Notes called for Redemption pursuant to such Redemption Notice and not with respect to any other Notes.

“Make-Whole Fundamental Change Conversion Period” has the following meaning:

(A)          in the case of a Make-Whole Fundamental Change pursuant to clause (A) of the definition thereof, the period from, and including, the Make-Whole Fundamental Change Effective Date of such Make-Whole Fundamental Change to, and including, the thirty fifth (35th) Trading Day after such Make-Whole Fundamental Change Effective Date (or, if such Make-Whole Fundamental Change also constitutes a Fundamental Change, to, but excluding, the related Fundamental Change Repurchase Date); and

(B)           in the case of a Make-Whole Fundamental Change pursuant to clause (B) of the definition thereof, the period from, and including, the Redemption Notice Date for the related Redemption, to, and including, the Business Day immediately before the related Redemption Date;

provided, however, that if the Conversion Date for the conversion of a Note occurs during the Make-Whole Fundamental Change Conversion Period for both a Make-Whole Fundamental Change occurring pursuant to clause (A) of the definition of “Make-Whole Fundamental

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Change” and a Make-Whole Fundamental Change occurring pursuant to clause (B) of such definition, then, notwithstanding anything to the contrary in Section 5.07, solely for purposes of such conversion, (x) such Conversion Date will be deemed to occur solely during the Make-Whole Fundamental Change Conversion Period for the Make-Whole Fundamental Change with the earlier Make-Whole Fundamental Change Effective Date; and (y) the Make-Whole Fundamental Change with the later Make-Whole Fundamental Change Effective Date will be deemed not to have occurred (unless the converting Holder notifies the Company to the contrary concurrently on the Conversion Date); provided, further, that if a Holder provides a notice of waiver of the provisions set forth in Section 5.08(A) during any Make-Whole Fundamental Change Conversion Period, such notice shall constitute a Conversion Date occurring during such Make-Whole Fundamental Change Conversion Period and the Company will deliver the relevant Conversion Consideration following the completion of the 61 calendar day notice period.

“Make-Whole Fundamental Change Effective Date” means (A) with respect to a Make-Whole Fundamental Change pursuant to clause (A) of the definition thereof, the date on which such Make-Whole Fundamental Change occurs or becomes effective; and (B) with respect to a Make-Whole Fundamental Change pursuant to clause (B) of the definition thereof, the applicable Redemption Notice Date.

“Market Disruption Event” means, with respect to any date, the occurrence or existence, during the one-half hour period ending at the scheduled close of trading on such date on the Relevant Stock Exchange, of any material suspension or limitation imposed on trading (by reason of movements in price exceeding limits permitted by the relevant exchange or otherwise) in the Ordinary Shares or in any options contracts or futures contracts relating to the Ordinary Shares.

“Maturity Date” means April 1, 2025.

“Nasdaq”means the NASDAQ Global Market or The NASDAQ Global Select Market (or any of their respective successors).

“Net Cash Proceeds” means:

(A)          with respect to the Disposition of any asset by the Company or any Guarantor or any of their Subsidiaries the excess, if any, of (i) the sum of cash and Cash Equivalents received in connection with such Disposition (including any cash or Cash Equivalents received by way of deferred payment pursuant to, or by monetization of, a note receivable or otherwise, but only as and when so received) over (ii) the sum of (w) the principal amount of any Indebtedness permitted by this Indenture that is secured by a lien on the asset subject to such Disposition and that is repaid (and is timely repaid) in connection therewith (other than the Notes), (x) the reasonable out of pocket expenses actually incurred and paid by the Parent or any of its Subsidiaries in connection with such Disposition (including, reasonable attorney’s, accountant’s and other similar professional advisor’s fees, investment banking fees, survey costs, title insurance premiums, and related search and recording charges, transfer taxes, deed or mortgage recording taxes, other customary expenses and brokerage, consultant or other customary fees) to third parties (other than the Company, the Guarantors or any of their respective Affiliates), (y)

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taxes paid or reasonably estimated to be actually payable or that are actually accrued in connection therewith with respect to the current tax year as a result of any gain recognized in connection therewith by such Person or any of the direct or indirect stockholders thereof and attributable to such Disposition; provided that, if the amount of any estimated taxes pursuant to this subclause (y) exceeds the amount of taxes actually required to be paid in cash, the aggregate amount of such excess shall constitute Net Cash Proceeds and (z) any reasonable reserve actually maintained in respect of (1) the sale price of such asset or assets established in accordance with IFRS, and (2) any liabilities associated with such asset or assets and retained by the Parent or any of its Subsidiaries after such sale or other Disposition thereof, including pension and other post employment benefit liabilities and liabilities related against any indemnification obligations associated with such transaction and it being understood that “Net Cash Proceeds” shall include any cash or Cash Equivalents (a) received upon the Disposition of any non cash consideration received by such Person in any such Disposition, and (b) received upon the reversal (without the satisfaction of any applicable liabilities in cash in a corresponding amount) of any reserve described in subclause (z) above or, if such liabilities have not been satisfied in cash and such reserve not reversed within two (2) years after such Disposition, the amount of such reserve; and

(B)           with respect to the incurrence or issuance of any Indebtedness by the Company, the Guarantors or any of their respective Subsidiaries not permitted under Section 3.13, the excess, if any, of (i) the sum of the cash received in connection with such incurrence or issuance over (ii) the investment banking fees, underwriting discounts, commissions, costs and other out-of-pocket expenses and other customary expenses (including reasonable attorney’s, accountant’s and other similar professional advisor’s fees), incurred by such Person in connection with such incurrence or issuance to third parties (other than the Company, the Guarantors or any of their respective Affiliates).

“Note Agent” means any Registrar, Paying Agent or Conversion Agent.

“Notes” means the 5.00% Convertible Senior Notes due 2025 issued by the Company pursuant to this Indenture.

“Observation Period” means, with respect to any Note to be converted, (A) subject to clause (B) below, if the Conversion Date for such Note occurs on or before the twenty-fifth (25th) Scheduled Trading Day immediately before the Maturity Date, the twenty (20) consecutive VWAP Trading Days beginning on, and including, the third (3rd) VWAP Trading Day immediately after such Conversion Date; (B) if such Conversion Date occurs on or after the date the Company has sent a Redemption Notice calling such Note for Redemption pursuant to Section 4.03(G) and before the related Redemption Date, the twenty (20) consecutive VWAP Trading Days beginning on, and including, the twenty-first (21st) Scheduled Trading Day immediately before such Redemption Date; and (C) subject to clause (B) above, if such Conversion Date occurs after the twenty-fifth (25th) Scheduled Trading Day immediately before the Maturity Date, the twenty (20) consecutive VWAP Trading Days beginning on, and including, the twenty-first (21st) Scheduled Trading Day immediately before the Maturity Date.

“Officer” means the Chairman of the Board of Directors, the Chief Executive Officer, the President, the Chief Operating Officer, the Chief Financial Officer, the Chief Accounting

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Officer, the Treasurer, any Assistant Treasurer, the Controller, the Secretary or any Vice-President of the Company; provided, that in the case of an Officer’s Certificate delivered pursuant to Section 3.05, Officer shall mean the Chief Executive Officer, Chief Financial Officer or Chief Accounting Officer.

“Officer’s Certificate” means a certificate that is signed on behalf of the Company by one (1) of its Officers and that meets the requirements of Section 12.03.

“Open of Business” means 9:00 a.m., New York City time.

“Opinion of Counsel” means an opinion, from legal counsel (including an employee of, or counsel to, the Company or any of its Subsidiaries) reasonably acceptable to the Trustee, that meets the requirements of Section 12.03, subject to customary qualifications and exclusions.

“Ordinary Shares” means the ordinary shares of the Parent, subject to Section 5.09.

“Parent” means the Person named as such in the first paragraph of this Indenture and, subject to Article 6, its successors and assigns.

“Parent Board” means the board of directors of Parent or a committee of such board duly authorized to act on behalf of such board.

“Participant” means, with respect to the Depositary, Euroclear or Clearstream, a Person who has an account with the Depositary, Euroclear or Clearstream, respectively (and, with respect to DTC, shall include Euroclear and Clearstream).

“Permitted Exchange” means the London Stock Exchange’s AIM Market, the Irish Stock Exchange’s Euronext Growth Market, The New York Stock Exchange or Nasdaq (or any of their respective successors).

“Person” or “person” means any individual, corporation, partnership, limited liability company, joint venture, association, joint-stock company, trust, unincorporated organization or government or other agency or political subdivision thereof. Any division or series of a limited liability company, limited partnership or trust will constitute a separate “person” under this Indenture.

“Physical Note” means a Note (other than a Global Note) that is represented by a certificate substantially in the form set forth in Exhibit A, registered in the name of the Holder of such Note and duly executed by the Company and authenticated by the Trustee.

“Plan” means the plan of reorganization filed with respect to the Company’s Chapter 11 bankruptcy case and confirmed by a final order.

“Prevailing Exchange Rate” means, for purposes of translating, as of any date, any amount in non-U.S. currency to U.S. dollars, the spot mid rate of exchange between such currencies prevailing as of 9am, New York City time, on such date, as displayed on, or derived

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from, Bloomberg page “BFIX” (or, if such page is not available, its equivalent successor page) in respect of such currencies. If such rate cannot be determined as provided in the immediately preceding sentence on such date (which, for the purpose of this definition, will be deemed to be the “Affected Day”), then the Prevailing Exchange Rate for such date will be determined mutatis mutandis but with respect to the immediately preceding day on which such rate can be so determined; provided, however, that, if such immediately preceding day is before the fifth day before such Affected Day, or, if such rate cannot be so determined, then the Prevailing Exchange Rate will be determined in such other manner as prescribed in good faith by an independent advisor.

“Private Placement Legend” means a legend substantially in the form set forth in Exhibit C.

“Redemption” means the repurchase of any Note by the Company pursuant to Section 4.03.

“Redemption Date” means the date fixed for the repurchase of any Notes by the Company pursuant to a Redemption.

“Redemption Notice Date” means, with respect to a Redemption, the date on which the Company sends the Redemption Notice for such Redemption pursuant to Section 4.03.

“Redemption Price” means the cash price payable by the Company to redeem any Note upon its Redemption, calculated pursuant to Section 4.03(F).

“Refinancing Indebtedness” has the meaning set forth in the Senior Secured Credit Facility.

“Regular Record Date” has the following meaning with respect to an Interest Payment Date: (A) if such Interest Payment Date occurs on April 1, the immediately preceding March 16; and (B) if such Interest Payment Date occurs on October 1, the immediately preceding September 16.

“Relevant Stock Exchange” means (i) the London Stock Exchange’s AIM Market, or (ii) if the Ordinary Shares are not then listed on the London Stock Exchange’s AIM Market, the principal exchange or other market on which the Ordinary Shares are then listed or admitted for trading (and, in the case of this clause (ii), the identity of the Relevant Stock Exchange will be determined in good faith by the Board of Directors); provided that if the Ordinary Shares or American Depositary Receipts representing Ordinary Shares (“ADRs”) are at any time listed on Nasdaq, references to the London Stock Exchange’s AIM Market in this definition shall be deemed to be replaced with Nasdaq unless, as of any relevant date of determination, the aggregate share trading volume of the Ordinary Shares for the preceding twenty (20) Trading Days on the London Stock Exchange’s AIM Market exceeds that on Nasdaq. For the avoidance of doubt, in any case where this definition would result in a reference to or computation based on ADRs, the relevant reference or computation will be appropriately adjusted to reflect the relevant ADR exchange ratio relative to the Ordinary Shares.

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“Repurchase Upon Fundamental Change” means the repurchase of any Note by the Company pursuant to Section 4.02.

“Responsible Officer” means (A) any vice president, assistant vice president, any trust officer or assistant trust officer, or any other officer of the Trustee customarily performing functions similar to those performed by any of the above designated officers and (B) with respect to a particular corporate trust matter, any other officer of the Trustee to whom such matter is referred because of his or her knowledge of and familiarity with the particular subject and who shall have direct responsibility for the administration of this Indenture.

“Restricted Global Note” means a Global Note bearing the Private Placement Legend.

“Restricted Payment” means any:

(A)          dividend or other distribution (whether in cash, securities or other property) or any payment (whether in cash, securities or other property), in each case, with respect to any capital stock or other Equity Interest of any Person or any of its Subsidiaries, including any sinking fund or similar deposit, on account of the purchase, retraction, redemption, retirement, defeasance, acquisition, cancellation or termination of any such capital stock or other Equity Interest, or on account of any return of capital to any Person’s stockholders, partners or members (or the equivalent of any thereof and including any thereof acquired through the exercise of warrants or rights of conversion, exchange or purchase); and

(B)           payment of any management or similar type fees by the Company or any Guarantors to any parent Affiliate thereof that is not a Guarantor.

“Rule 144” means Rule 144 under the Securities Act (or any successor rule thereto), as the same may be amended from time to time.

“Sale Leaseback” means any transaction or series of related transactions pursuant to which the Parent or any of its Subsidiaries (a) sells, transfers or otherwise disposes of any property, real or personal, whether now owned or hereafter acquired, and (b) as part of such transaction, thereafter rents or leases such property or other property that it intends to use for substantially the same purpose or purposes as the property being sold, transferred or disposed.

“Scheduled Trading Day” means any day that is scheduled to be a Trading Day on the Relevant Stock Exchange. If the Ordinary Shares are not so listed or traded, then “Scheduled Trading Day” means a Business Day.

“SEC” means the U.S. Securities and Exchange Commission.

“Securities Act” means the U.S. Securities Act of 1933, as amended.

“Security” means any Note or Conversion Share.

“Senior Secured Credit Facility” means the Credit Agreement, dated as of September

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24, 2019, among the Company, Old Parent, the lenders party thereto and Cantor Fitzgerald Securities, as administrative agent.

“Settlement Method” means Cash Settlement, Physical Settlement or Combination Settlement.

“Significant Subsidiary” means, with respect to any Person, any Subsidiary of such Person that constitutes a “significant subsidiary” (as defined in Rule l-02(w) of Regulation S-X under the Exchange Act) of such Person.

“Special Interest” means any interest that accrues on any Note pursuant to Section 7.03.

“Specified Dollar Amount” means, with respect to the conversion of a Note to which Combination Settlement applies, the maximum cash amount per $1,000 principal amount of such Note deliverable upon such conversion (excluding cash in lieu of any fractional Ordinary Share), as determined by the Company in U.S. dollars.

“Share Price” has the following meaning for any Make-Whole Fundamental Change: (A) if the holders of Ordinary Shares receive only cash in consideration for their Ordinary Shares in such Make-Whole Fundamental Change and such Make-Whole Fundamental Change is pursuant to clause (B) of the definition of “Fundamental Change,” then the Share Price is the amount of cash paid per Ordinary Share in such Make-Whole Fundamental Change; and (B) in all other cases, the Share Price is the average of the Last Reported Sale Prices per Ordinary Share for the five (5) consecutive Trading Days ending on, and including, the Trading Day immediately before the Make-Whole Fundamental Change Effective Date of such Make-Whole Fundamental Change.

“Subsidiary” means, with respect to any Person, (A) any corporation, association or other business entity (other than a partnership or limited liability company) of which more than fifty percent (50%) of the total voting power of the Capital Stock entitled (without regard to the occurrence of any contingency, but after giving effect to any voting agreement or shareholders’ agreement that effectively transfers voting power) to vote in the election of directors, managers or trustees, as applicable, of such corporation, association or other business entity is owned or controlled, directly or indirectly, by such Person or one or more of the other Subsidiaries of such Person; and (B) any partnership or limited liability company where (i) more than fifty percent (50%) of the capital accounts, distribution rights, equity and voting interests, or of the general and limited partnership interests, as applicable, of such partnership or limited liability company are owned or controlled, directly or indirectly, by such Person or one or more of the other Subsidiaries of such Person, whether in the form of membership, general, special or limited partnership or limited liability company interests or otherwise; and (ii) such Person or any one or more of the other Subsidiaries of such Person is a controlling general partner of, or otherwise controls, such partnership or limited liability company.

“Tax” means any tax, duty, levy, impost, assessment or other governmental charge (including penalties and interest and other similar liabilities related thereto).

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“Tax Redemption” means the Redemption of any Note pursuant to Section 4.03(C).

“Trading Day” means any day on which (A) there is no Market Disruption Event; and (B) trading in the Ordinary Shares generally occurs on the Relevant Stock Exchange. If the Ordinary Shares are not so listed or traded, then “Trading Day” means a Business Day.

“Trust Indenture Act” means the U.S. Trust Indenture Act of 1939, as amended.

“Trustee” means the Person named as such in the first paragraph of this Indenture until a successor replaces it in accordance with the provisions of this Indenture and, thereafter, means such successor.

“Unrestricted Global Note” means a Global Note that does not bear and is not required to bear the Private Placement Legend.

“VWAP Market Disruption Event” means, with respect to any date, (A) the failure by the Relevant Stock Exchange to open for trading during its regular trading session on such date; or (B) the occurrence or existence, for more than one half hour period in the aggregate, of any suspension or limitation imposed on trading (by reason of movements in price exceeding limits permitted by the Relevant Stock Exchange or otherwise) in the Ordinary Shares or in any options contracts or futures contracts relating to the Ordinary Shares, and such suspension or limitation occurs or exists at any time before 1:00 p.m., New York City time, on such date.

“VWAP Trading Day” means a day on which (A) there is no VWAP Market Disruption Event; and (B) trading in the Ordinary Shares generally occurs on the Relevant Stock Exchange. If the Ordinary Shares are not so listed or traded, then “VWAP Trading Day” means a Business Day.

“Wholly Owned Subsidiary” of a Person means any Subsidiary of such Person all of the outstanding Capital Stock or other ownership interests of which (other than directors’ qualifying shares) are owned by such Person or one or more Wholly Owned Subsidiaries of such Person.

Section 1.02. Other Definitions.

Term	Defined in Section
“Additional Amounts”	3.04(A)
“Additional Shares”	5.07(A)
“Business Combination Event”	6.01(A)
“Cash Settlement”	5.03(A)
“Combination Settlement”	5.03(A)
“Conversion Agent”	2.06(A)
“Conversion Consideration”	5.03(B)
“Default Interest”	2.05(B)
“Defaulted Amount”	2.05(B)
“Event of Default”	7.01(A)

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“Expiration Date”	5.05(A)(v)
“Expiration Time”	5.05(A)(v)
“FATCA”	3.04(A)(iv)
“Fundamental Change Notice”	4.02(E)
“Fundamental Change Repurchase Right”	4.02(A)
“Guaranteed Obligations”	9.01(A)
“Guarantor Business Combination Event”	9.04(A)
“Initial Notes”	2.03(A)
“Junior Indebtedness”	3.13(C)
“Ordinary Share Change Event”	5.09(A)
“Paying Agent”	2.06(A)
“Physical Settlement”	5.03(A)
“Redemption Notice”	4.03(G)
“Reference Property”	5.09(A)
“Reference Property Unit”	5.09(A)
“Register	2.06(B)
“Registrar”	2.06(A)
“Relevant Taxing Jurisdiction”	3.04(A)
“Reporting Event of Default”	7.03(A)
“Specified Courts”	12.07
“Spin-Off	5.05(A)(iii)(2)
“Spin-Off Valuation Period”	5.05(A)(iii)(2)
“Stated Interest”	2.05(A)
“Successor Corporation”	6.01(A)
“Successor Person”	5.09(A)
“Tax Redemption Opt-Out Election”	4.03(C)(ii)
“Tax Redemption Opt-Out Election Notice”	4.03(C)(ii)(l)
“Tender/Exchange Offer Valuation Period”	5.05(A)(v)
“Transfer Taxes”	3.04(B)

Section 1.03. Rules of Construction.

For purposes of this Indenture:

(A)          “or” is not exclusive;

(B)           “including” means “including without limitation”;

(C)           “will” expresses a command;

(D)           the “average” of a set of numerical values refers to the arithmetic average of such numerical values;

(E)           a merger involving, or a transfer of assets by, a limited liability company, limited partnership or trust will be deemed to include any division of or by, or an allocation of assets to a series of, such limited liability company, limited partnership or trust, or any unwinding of any such division or allocation;

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(F)           words in the singular include the plural and in the plural include the singular, unless the context requires otherwise;

(G)           “herein,” “hereof’’ and other words of similar import refer to this Indenture as a whole and not to any particular Article, Section or other subdivision of this Indenture, unless the context requires otherwise;

(H)           references to currency mean the lawful currency of the United States of America, unless the context requires otherwise;

(I)            the exhibits, schedules and other attachments to this Indenture are deemed to form part of this Indenture; and

(J)            the term “interest,” when used with respect to a Note, includes any Additional Interest and Special Interest, unless the context requires otherwise.

For purposes of this Indenture, the following terms of the Trust Indenture Act have the following meanings:

(i)            “Commission” means the SEC;

(ii)           “indenture securities” means the Notes;

(iii)           “indenture security holder” means a Holder;

(iv)          “indenture to be qualified” means this Indenture;

(v)           “indenture trustee” or “institutional trustee” means the Trustee; and

(vi)           “obligor” on the indenture securities means the Company.

All other terms used in this Indenture that are defined by the Trust Indenture Act (including by reference to another statute) or the related rules of the SEC, and not defined in this Indenture, have the respective meanings so defined by the Trust Indenture Act or such rules.

Article 2. THE NOTES

Section 2.01. Form, Dating and Denominations.

The Notes and the Trustee’s certificate of authentication will be substantially in the form set forth in Exhibit A. The Notes will bear the legends required by Section 2.09 and may bear notations, legends or endorsements required by law, stock exchange rule or usage or the Depositary. Each Note will be dated as of the date of its authentication.

Except to the extent otherwise provided in a Company Order delivered to the Trustee in connection with the issuance and authentication thereof, the Notes will be issued initially in the form of one or more Global Notes. Global Notes may be exchanged for Physical Notes, and

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Physical Notes may be exchanged for Global Notes, only as provided in Section 2.10.

The Notes will be issuable only in registered form without interest coupons and only in Authorized Denominations.

Each certificate representing a Note will bear a unique registration number that is not affixed to any other certificate representing another outstanding Note.

The terms contained in the Notes constitute part of this Indenture, and, to the extent applicable, the Company and the Trustee, by their execution and delivery of this Indenture, agree to such terms and to be bound thereby; provided, however, that, to the extent that any provision of any Note conflicts with the provisions of this Indenture, the provisions of this Indenture will control for purposes of this Indenture and such Note.

Section 2.02. Execution, Authentication and Delivery.

(A)          Due Execution by the Company. At least one (1) duly authorized Officer will sign the Notes on behalf of the Company by manual or facsimile signature. A Note’s validity will not be affected by the failure of any Officer whose signature is on any Note to hold, at the time such Note is authenticated, the same or any other office at the Company.

(B)           Authentication by the Trustee and Delivery.

(i)      No Note will be valid until it is authenticated by the Trustee. A Note will be deemed to be duly authenticated only when an authorized signatory of the Trustee (or a duly appointed authenticating agent) manually signs the certificate of authentication of such Note.

(ii)      The Trustee will cause an authorized signatory of the Trustee (or a duly appointed authenticating agent) to manually sign the certificate of authentication of a Note only if (1) the Company delivers such Note to the Trustee; (2) such Note is executed by the Company in accordance with Section 2.02(A); and (3) the Company delivers a Company Order to the Trustee that (a) requests the Trustee to authenticate such Note; and (b) sets forth the name of the Holder of such Note and the date as of which such Note is to be authenticated. If such Company Order also requests the Trustee to deliver such Note to any Holder or to the Depositary, then the Trustee will promptly deliver such Note in accordance with such Company Order.

(iii)     The Trustee may appoint an authenticating agent acceptable to the Company to authenticate Notes. A duly appointed authenticating agent may authenticate Notes whenever the Trustee may do so under this Indenture, and a Note authenticated as provided in this Indenture by such an agent will be deemed, for purposes of this Indenture, to be authenticated by the Trustee. Each duly appointed authenticating agent will have the same rights to deal with the Company as the Trustee would have if it were performing the duties that the authentication agent was validly appointed to undertake.

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Section 2.03. Initial Notes and Additional Notes.

(A)          Initial Notes. On the Issue Date, there will be originally issued one hundred twenty four million nine hundred ninety-nine thousand nine hundred ninety-seven dollars ($124,999,999) aggregate principal amount of Notes, subject to the provisions of this Indenture (including Section 2.02). Notes issued pursuant to this Section 2.03(A), and any Notes issued in exchange therefor or in substitution thereof, are referred to in this Indenture as the “Initial Notes.”

(B)           Additional Notes. The Company may, subject to the provisions of this Indenture (including Section 2.02), originally issue additional Notes with the same terms as the initial Notes (except, to the extent applicable, with respect to the date as of which interest begins to accrue on such additional Notes and the first Interest Payment Date and the Last Original Issue Date of such additional Notes), which additional Notes will, subject to the foregoing, be considered to be part of the same series of, and rank equally and ratably with all other, Notes issued under this Indenture; provided, however, that if any such additional Notes are not fungible with other Notes issued under this Indenture for federal income tax or federal securities laws purposes, then such additional Notes will be identified by a separate CUSIP number or by no CUSIP number.

Section 2.04. Method of Payment.

(A)          Global Notes. The Company will pay, or cause the Paying Agent to pay, the principal (whether due upon maturity on the Maturity Date, Redemption on a Redemption Date or repurchase on a Fundamental Change Repurchase Date or otherwise) of, interest on, and any cash Conversion Consideration for, any Global Note to the Depositary by wire transfer of immediately available funds no later than the time the same is due as provided in this Indenture.

(B)           Physical Notes. The Company will pay, or cause the Paying Agent to pay, the principal (whether due upon maturity on the Maturity Date, Redemption on a Redemption Date or repurchase on a Fundamental Change Repurchase Date or otherwise) of, interest on, and any cash Conversion Consideration for, any Physical Note no later than the time the same is due as provided in this Indenture as follows: (i) if the principal amount of such Physical Note is at least five million dollars ($5,000,000) (or such lower amount as the Company may choose in its sole and absolute discretion) and the Holder of such Physical Note entitled to such payment has delivered to the Paying Agent or the Trustee, no later than the time set forth in the immediately following sentence, a written request that the Company make such payment by wire transfer to an account of such Holder within the United States, by wire transfer of immediately available funds to such account; and (ii) in all other cases, by check mailed to the address of the Holder of such Physical Note entitled to such payment as set forth in the Register. To be timely, such written request must be so delivered no later than the Close of Business on the following date: (x) with respect to the payment of any interest due on an Interest Payment Date, the immediately preceding Regular Record Date; (y) with respect to any cash Conversion Consideration, the relevant Conversion Date; and (z) with respect to any other payment, the date that is fifteen (15) calendar days immediately before the date such payment is due.

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Section 2.05. Accrual of Interest; Defaulted Amounts; When Payment Date is Not a Business Day.

(A)          Accrual of Interest. Each Note will accrue interest at a rate per annum equal to 5.00% (the “Stated Interest”), plus any Additional Interest and Special Interest that may accrue pursuant to Sections 3.03 and 7.03, respectively. Stated Interest on each Note will (i) accrue from, and including, the most recent date to which Stated Interest has been paid or duly provided for (or, if no Stated Interest has theretofore been paid or duly provided for, the date set forth in the certificate representing such Note as the date from, and including, which Stated Interest will begin to accrue in such circumstance) to, but excluding, the date of payment of such Stated Interest; and (ii) be, subject to Sections 4.02(D), 4.03(F) and 5.02(D) (but without duplication of any payment of interest), payable semi-annually in arrears on each Interest Payment Date, beginning on the first Interest Payment Date set forth in the certificate representing such Note, to the Holder of such Note as of the Close of Business on the immediately preceding Regular Record Date. Stated Interest, and, if applicable, Additional Interest and Special Interest, on the Notes will be computed on the basis of a 360-day year comprised of twelve 30-day months.

(B)           Defaulted Amounts. If the Company fails to pay any amount (a “Defaulted Amount”) payable on a Note on or before the due date therefor as provided in this Indenture, then, regardless of whether such failure constitutes an Event of Default, (i) such Defaulted Amount will forthwith cease to be payable to the Holder of such Note otherwise entitled to such payment; (ii) to the extent lawful, interest (“Default Interest”) will accrue on such Defaulted Amount at a rate per annum equal to the rate per annum at which Stated Interest accrues, from, and including, such due date to, but excluding, the date of payment of such Defaulted Amount and Default Interest; (iii) such Defaulted Amount and Default Interest will be paid on a payment date selected by the Company to the Holder of such Note as of the Close of Business on a special record date selected by the Company, provided that such special record date must be no more than fifteen (15), nor less than ten (10), calendar days before such payment date; and (iv) at least fifteen (15) calendar days before such special record date, the Company will send notice to the Trustee and the Holders that states such special record date, such payment date and the amount of such Defaulted Amount and Default Interest to be paid on such payment date.

(C)           Delay of Payment when Payment Date is Not a Business Day. If the due date for a payment on a Note as provided in this Indenture is not a Business Day, then, notwithstanding anything to the contrary in this Indenture or the Notes, such payment may be made on the immediately following Business Day and no interest will accrue on such payment as a result of the related delay. Solely for purposes of the immediately preceding sentence, a day on which the applicable place of payment is authorized or required by law or executive order to close or be closed will be deemed not to be a “Business Day.”

Section 2.06. Registrar, Paying Agent and Conversion Agent.

(A)          Generally. The Company will maintain (i) an office or agency in the continental United States where Notes may be presented for registration of transfer or for exchange (the “Registrar”); (ii) an office or agency in the continental United States where Notes may be presented for payment (the “Paying Agent”); and (iii) an office or agency in the continental United States where Notes may be presented for conversion (the “Conversion Agent”). If the

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Company fails to maintain a Registrar, Paying Agent or Conversion Agent, then the Trustee will act as such. For the avoidance of doubt, the Company or any of its Subsidiaries may act as Registrar, Paying Agent or Conversion Agent.

(B)           Duties of the Registrar. The Registrar will keep a record (the “Register”) of the names and addresses of the Holders, the Notes held by each Holder and the transfer, exchange, repurchase, Redemption and conversion of Notes. Absent manifest error, the entries in the Register will be conclusive and the Company and the Trustee may treat each Person whose name is recorded as a Holder in the Register as a Holder for all purposes. The Register will be in written form or in any form capable of being converted into written form reasonably promptly.

(C)           Co-Agents; Company’s Right to Appoint Successor Registrars, Paying Agents and Conversion Agents. The Company may appoint one or more co-Registrars, co-Paying Agents and co-Conversion Agents, each of whom will be deemed to be a Registrar, Paying Agent or Conversion Agent, as applicable, under this Indenture. Subject to Section 2.06(A), the Company may change any Registrar, Paying Agent or Conversion Agent (including appointing itself or any of its Subsidiaries to act in such capacity) without notice to any Holder. The Company will notify the Trustee (and, upon request, any Holder) of the name and address of each Note Agent, if any, not a party to this Indenture and will enter into an appropriate agency agreement with each such Note Agent, which agreement will implement the provisions of this Indenture that relate to such Note Agent.

(D)           Initial Appointments. The Company appoints the Trustee as the initial Paying Agent, the initial Registrar and the initial Conversion Agent.

Section 2.07. Paying Agent and Conversion Agent to Hold Property in Trust.

The Company will require each Paying Agent or Conversion Agent that is not the Trustee to agree in writing that such Note Agent will (A) hold in trust for the benefit of Holders or the Trustee all money and other property held by such Note Agent for payment or delivery due on the Notes; and (B) notify the Trustee of any default by the Company in making any such payment or delivery. The Company, at any time, may, and the Trustee, while any Default continues, may, require a Paying Agent or Conversion Agent to pay or deliver, as applicable, all money and other property held by it to the Trustee, after which payment or delivery, as applicable, such Note Agent (if not the Company or any of its Subsidiaries) will have no further liability for such money or property. If the Company or any of its Subsidiaries acts as Paying Agent or Conversion Agent, then (A) it will segregate and hold in a separate trust fund for the benefit of the Holders or the Trustee all money and other property held by it as Paying Agent or Conversion Agent; and (B) references in this Indenture or the Notes to the Paying Agent or Conversion Agent holding cash, or other property, or to the delivery of cash or other property to the Paying Agent or Conversion Agent, in each case for payment or delivery to any Holders or the Trustee or with respect to the Notes, will be deemed to refer to cash or other property so segregated and held separately, or to the segregation and separate holding of such cash or other property, respectively. Upon the occurrence of any event pursuant to in clause (ix) or (x) of Section 7.01(A) with respect to the Company (or with respect to any Subsidiary of the Company acting as Paying Agent or Conversion Agent), the Trustee will serve as the Paying Agent or

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Conversion Agent, as applicable, for the Notes.

Section 2.08. Holder Lists.

If the Trustee is not the Registrar, the Company will furnish to the Trustee, no later than seven (7) Business Days before each Interest Payment Date, and at such other times as the Trustee may request, a list, in such form and as of such date or time as the Trustee may reasonably require, of the names and addresses of the Holders.

Section 2.09. Legends.

(A)          Global Note Legend. Each Global Note will bear the Global Note Legend (or any similar legend required by the Depositary for such Global Note).

(B)           Private Placement Legend. Any Restricted Global Note will bear the Private Placement Legend.

(C)           Other Legends. A Note may bear any other legend or text, not inconsistent with this Indenture, as may be required by applicable law or by any securities exchange or automated quotation system on which such Note is traded or quoted.

(D)           Acknowledgement and Agreement by the Holders. A Holder’s acceptance of any Note bearing any legend required by this Section 2.09 will constitute such Holder’s acknowledgement of, and agreement to comply with, the restrictions set forth in such legend.

Section 2.10. Transfers and Exchanges; Certain Transfer Restrictions.

(A)          Provisions Applicable to All Transfers and Exchanges.

(i) Subject to this Section 2.10, Physical Notes and beneficial interests in Global Notes may be transferred or exchanged from time to time and the Registrar will record each such transfer or exchange in the Register.

(ii) Each Note issued upon transfer or exchange of any other Note (such other Note being referred to as the “old Note” for purposes of this Section 2.10(A)(ii)) or portion thereof in accordance with this Indenture will be the valid obligation of the Company, evidencing the same indebtedness, and entitled to the same benefits under this Indenture, as such old Note or portion thereof, as applicable.

(iii) The Company, the Guarantors, the Trustee and the Note Agents will not impose any service charge on any Holder for any transfer, exchange or conversion of Notes, but the Company, the Guarantors, the Trustee, the Registrar and the Conversion Agent may require payment of a sum sufficient to cover any transfer tax or similar governmental charge that may be imposed in connection with any transfer, exchange or conversion of Notes, other than exchanges pursuant to Sections 2.11, 2.16 or 8.05 not involving any transfer.

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(iv) Notwithstanding anything to the contrary in this Indenture or the Notes, a Note may not be transferred or exchanged in part unless the portion to be so transferred or exchanged is in an Authorized Denomination.

(v) The Trustee will have no obligation or duty to monitor, determine or inquire as to compliance with any transfer restrictions imposed under this Indenture or applicable law with respect to any Security, other than to require the delivery of such certificates or other documentation or evidence as expressly required by this Indenture and to examine the same to determine substantial compliance as to form with the requirements of this Indenture.

(vi) Each Note issued upon transfer of, or in exchange for, another Note will bear each legend, if any, required by Section 2.09.

(vii) Upon satisfaction of the requirements of this Indenture to effect a transfer or exchange of any Note, the Company will cause such transfer or exchange to be effected as soon as reasonably practicable after the date of such satisfaction.

(B)          Transfers and Exchanges of Global Notes.

(i) Subject to the other clauses of this paragraph (B), no Global Note may be transferred or exchanged in whole except (x) by the Depositary to a nominee of the Depositary; (y) by a nominee of the Depositary to the Depositary or to another nominee of the Depositary; or (z) by the Depositary or any such nominee to a successor Depositary or a nominee of such successor Depositary.

(ii) Transfer and Exchange of Beneficial Interests in the Global Notes. The transfer and exchange of beneficial interests in the Global Notes will be effected through the Depositary, in accordance with the provisions of this Indenture and the Applicable Procedures. Beneficial interests in the Restricted Global Notes will be subject to restrictions on transfer comparable to those set forth herein to the extent required by the Securities Act. Transfers of beneficial interests in the Global Notes also will require compliance with either subparagraph (1) or (2) below, as applicable, and if applicable either subparagraph (3) or (4) below:

(1)          Transfer of Beneficial Interests in the Same Global Note. Beneficial interests in any Restricted Global Note may be transferred to Persons who take delivery thereof in the form of a beneficial interest in the same Restricted Global Note in accordance with the transfer restrictions set forth in the Private Placement Legend. Beneficial interests in any Unrestricted Global Note may be transferred to Persons who take delivery thereof in the form of a beneficial interest in an Unrestricted Global Note. No written orders or instructions shall be required to be delivered to the Registrar to effect the transfers described in this subsection (1).

(2)          All Other Transfers and Exchanges of Beneficial Interests in

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Global Notes. In connection with all transfers and exchanges of beneficial interests that are not subject to subsection (1) above, the transferor of such beneficial interest must deliver to the Registrar a written order, through a Participant or an Indirect Participant in the applicable Global Note given to the Depositary in accordance with the Applicable Procedures, instructing the Depositary to credit or cause to be credited a beneficial interest in the applicable Global Note in an amount equal to the beneficial interest to be transferred or exchanged and containing information regarding the Participant account to be credited with such increase.

(3)           Transfer of Beneficial Interests from one Restricted Global Note to Another Restricted Global Note. A beneficial interest in any Restricted Global Note may be transferred to a Person who takes delivery thereof in the form of a beneficial interest in another Restricted Global Note if the transfer complies with the requirements of subsection (2) above and the Registrar receives the following:

(A)  if the transferee will take delivery in the form of a beneficial interest in the 144A Global Note, a certificate from the holder certifying that the exchange or transfer is being effected pursuant to and in accordance with Rule 144A under the Securities Act, and that the beneficial interest is being transferred to a Person that the Transferor reasonably believes is purchasing the beneficial interest for its own account, or for one or more accounts with respect to which such Person exercises sole investment discretion, and such Person and each such account is a “qualified institutional buyer” within the meaning of Rule 144A in a transaction meeting the requirements of Rule 144A, and such Transfer is in compliance with any applicable blue sky securities laws of any state of the United States; and

(B) if the transferee will take delivery in the form of a beneficial interest in the IAI Global Note, a certificate from the holder certifying that the exchange or transfer is being effected in compliance with the transfer restrictions applicable to beneficial interests in the Restricted Global Note and pursuant to and in accordance with the Securities Act and any applicable blue sky securities laws of any state of the United States, and such exchange or transfer is being effected to an Institutional Accredited Investor and pursuant to an exemption from the registration requirements of the Securities Act, and the Transferor hereby further certifies that it has not engaged in any general solicitation within the meaning of Regulation D under the Securities Act and the Transfer complies with the transfer restrictions applicable to beneficial interests in a Restricted Global Note.

(4)           Transfer and Exchange of Beneficial Interests in a Restricted Global Note for Beneficial Interests in an Unrestricted Global Note. A beneficial interest in any Restricted Global Note may be exchanged by any holder thereof for a beneficial interest in an Unrestricted Global Note or transferred to a Person who takes delivery thereof in the form of a beneficial interest in an Unrestricted Global Note if the exchange or transfer complies with the requirements of subsection (2) above and: (x) such exchange or transfer is effected pursuant to an effective Registration Statement under the Securities Act, or (y) the Registrar receives (A) a certificate from such holder certifying that the exchange or transfer has been effected in compliance with any transfer

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restrictions applicable under the Securities Act, that the restrictions on transfer contained in the Private Placement Legend are not required in order to maintain compliance with the Securities Act and that the beneficial interest in the Unrestricted Global Note is being acquired in compliance with any applicable blue sky securities laws of any state of the United States, and (B) if the Registrar so requests, an Opinion of Counsel in form reasonably acceptable to the Registrar to the effect that such exchange or transfer is in compliance with the Securities Act.

(iii) No Global Note (or any portion thereof) may be transferred to, or exchanged for, a Physical Note; provided, however, that a Global Note will be exchanged, pursuant to customary procedures, for one or more Physical Notes if:

(1)            (x) the Depositary notifies the Company or the Trustee that the Depositary is unwilling or unable to continue as depositary for such Global Note or (y) the Depositary ceases to be a “clearing agency” registered under Section 17A of the Exchange Act and, in each case, the Company fails to appoint a successor Depositary within ninety (90) days of such notice or cessation;

(2)            an Event of Default has occurred and is continuing and the Company, the Trustee or the Registrar has received a written request from the Depositary, or from a holder of a beneficial interest in such Global Note, to exchange such Global Note or beneficial interest, as applicable, for one or more Physical Notes; or

(3)           the Company, in its sole discretion, permits the exchange of any beneficial interest in such Global Note for one or more Physical Notes at the request of the owner of such beneficial interest.

(iv) Upon satisfaction of the requirements of this Indenture to effect a transfer or exchange of any Global Note (or any portion thereof):

(1)           the Trustee will reflect any resulting decrease of the principal amount of such Global Note by notation on the “Schedule of Exchanges of Interests in the Global Note” forming part of such Global Note (and, if such notation results in such Global Note having a principal amount of zero, the Company may (but is not required to) instruct the Trustee to cancel such Global Note pursuant to Section 2.14);

(2)           if required to effect such transfer or exchange, then the Trustee will reflect any resulting increase of the principal amount of any other Global Note by notation on the “Schedule of Exchanges of Interests in the Global Note” forming part of such other Global Note;

(3)           if required to effect such transfer or exchange, then the Company will issue, execute and deliver, and the Trustee will authenticate, in each case in accordance with Section 2.02, a new Global Note bearing each legend, if any, required by Section 2.09; and

(4)           if such Global Note (or such portion thereof), or any beneficial

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interest therein, is to be exchanged for one or more Physical Notes, then the Company will issue, execute and deliver, and the Trustee will authenticate, in each case in accordance with Section 2.02, one or more Physical Notes that are in Authorized Denominations (not to exceed, in the aggregate, the principal amount of such Global Note to be so exchanged), are registered in such name(s) as the Depositary specifies (or as otherwise determined pursuant to customary procedures) and bear each legend, if any, required by Section 2.09.

(v) Each transfer or exchange of a beneficial interest in any Global Note will be made in accordance with the Depositary Procedures.

(C)          Transfers and Exchanges of Physical Notes.

(i) Subject to this Section 2.10, a Holder of a Physical Note may (x) transfer such Physical Note (or any portion thereof in an Authorized Denomination) to one or more other Person(s); (y) exchange such Physical Note (or any portion thereof in an Authorized Denomination) for one or more other Physical Notes in Authorized Denominations having an aggregate. principal amount equal to the aggregate principal amount of the Physical Note (or portion thereof) to be so exchanged: and (z) if then permitted by the Depositary Procedures, transfer such Physical Note (or any portion thereof in an Authorized Denomination) in exchange for a beneficial interest in one or more Global Notes; provided, however, that, to effect any such transfer or exchange, such Holder must:

(1)           surrender such Physical Note to be transferred or exchanged to the office of the Registrar, together with any endorsements or transfer instruments reasonably required by the Company, the Trustee or the Registrar; and

(2)           deliver such certificates, documentation or evidence as may be required pursuant to Section 2.10(D).

(ii) Upon the satisfaction of the requirements of this Indenture to effect a transfer or exchange of any Physical Note (such Physical Note being referred to as the “old Physical Note” for purposes of this Section 2.10(C)(ii)) of a Holder (or any portion of such old Physical Note in an Authorized Denomination):

(1)            such old Physical Note will be promptly cancelled pursuant to Section 2.14;

(2)            if such old Physical Note is to be transferred or exchanged only in part, then the Company will issue, execute and deliver, and the Trustee will authenticate, in each case in accordance with Section 2.02, one or more Physical Notes that (x) are in Authorized Denominations and have an aggregate principal amount equal to the principal amount of such old Physical Note not to be transferred or exchanged; (y) are registered in the name of such Holder; and (z) bear each legend, if any, required by Section 2.09;

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(3)      in the case of a transfer:

(a)           to the Depositary or a nominee thereof that will hold its interest in such old Physical Note (or such portion thereof) to be so transferred in the form of one or more Global Notes, the Trustee will reflect an increase of the principal amount of one or more existing Global Notes by notation on the “Schedule of Exchanges of Interests in the Global Note” forming part of such Global Note(s), which increase(s) are in Authorized Denominations and aggregate to the principal amount to be so transferred, and which Global Note(s) bear each legend, if any, required by Section 2.09; provided, however, that if such transfer cannot be so effected by notation on one or more existing Global Notes (whether because no Global Notes bearing each legend, if any, required by Section 2.09 then exist, because any such increase will result in any Global Note having an aggregate principal amount exceeding the maximum aggregate principal amount permitted by the Depositary or otherwise), then the Company will issue, execute and deliver, and the Trustee will authenticate, in each case in accordance with Section 2.02, one or more Global Notes that (x) are in Authorized Denominations and have an aggregate principal amount equal to the principal amount to be so transferred; and (y) bear each legend, if any, required by Section 2.09; and

(b)           to a transferee that will hold its interest in such old Physical Note (or such portion thereof) to be so transferred in the form of one or more Physical Notes, the Company will issue, execute and deliver, and the Trustee will authenticate, in each case in accordance with Section 2.02, one or more Physical Notes that (x) are in Authorized Denominations and have an aggregate principal amount equal to the principal amount to be so transferred; (y) are registered in the name of such transferee; and (z) bear each legend, if any, required by Section 2.09; and

(4)       in the case of an exchange, the Company will issue, execute and deliver, and the Trustee will authenticate, in each case in accordance with Section 2.02, one or more Physical Notes that (x) are in Authorized Denominations and have an aggregate principal amount equal to the principal amount to be so exchanged; (y) are registered in the name of the Person to whom such old Physical Note was registered; and (z) bear each legend, if any, required by Section 2.09.

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(D)           Requirement of Affiliates to Deliver Documentation and Other Evidence. If a Holder of any Note that is an Affiliate requests to register the transfer of such Note to the name of another Person, then the Company, the Guarantors, the Trustee and the Registrar may refuse to effect such identification, removal or transfer, as applicable, unless there is delivered to the Company, the Guarantors, the Trustee and the Registrar such certificates or other documentation or evidence as the Company, the Guarantors, the Trustee and the Registrar may reasonably require to determine that such transfer complies with the Securities Act and other applicable securities laws.

(E)           Transfers of Notes Subject to Redemption, Repurchase or Conversion. Notwithstanding anything to the contrary in this Indenture or the Notes, the Company, the Guarantors, the Trustee and the Registrar will not be required to register the transfer of or exchange any Note that (i) has been surrendered for conversion, except to the extent that any portion of such Note is not subject to conversion; (ii) is subject to a Fundamental Change Repurchase Notice validly delivered, and not withdrawn, pursuant to Section 4.02(F), except to the extent that any portion of such Note is not subject to such notice or the Company fails to pay the applicable Fundamental Change Repurchase Price when due or (iii) has been selected for Redemption pursuant to a Redemption Notice, except to the extent that any portion of such Note is not subject to Redemption or the Company fails to pay the applicable Redemption Price when due.

(F)           Neither the Trustee nor any Agent shall have any responsibility for any actions taken or not taken by the Depositary.

 Section 2.11. Exchange and Cancellation of Notes to Be Converted, Redeemed or Repurchased.

(A)          Partial Conversions, Redemptions and Repurchases of Physical Notes. If only a portion of a Physical Note of a Holder is to be converted pursuant to Article 5 or repurchased pursuant to a Repurchase Upon Fundamental Change or Redemption, then, as soon as reasonably practicable after such Physical Note is surrendered for such conversion, Redemption or repurchase, the Company will cause such Physical Note to be exchanged, pursuant and subject to Section 2.10(C), for (i) one or more Physical Notes that are in Authorized Denominations and have an aggregate principal amount equal to the principal amount of such Physical Note that is not to be so converted, redeemed or repurchased, as applicable, and deliver such Physical Note(s) to such Holder; and (ii) a Physical Note having a principal amount equal to the principal amount to be so converted, redeemed or repurchased, as applicable, which Physical Note will be converted, redeemed or repurchased, as applicable, pursuant to the terms of this Indenture; provided, however, that the Physical Note referred to in this clause (ii) need not be issued at any time after which such principal amount subject to such conversion, Redemption or repurchase, as applicable, is deemed to cease to be outstanding pursuant to Section 2.17.

(B)           Cancellation of Converted, Redeemed and Repurchased Notes.

(i) Physical Notes. If a Physical Note (or any portion thereof that has not theretofore been exchanged pursuant to Section 2.11(A)) of a Holder is to be converted pursuant to Article 5 or repurchased pursuant to a Repurchase

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Upon Fundamental Change or Redemption, then, promptly after the later of the time such Physical Note (or such portion) is deemed to cease to be outstanding pursuant to Section 2.17 and the time such Physical Note is surrendered for such conversion or repurchase, as applicable, (1) such Physical Note will be cancelled pursuant to Section 2.14; and (2) in the case of a partial conversion, Redemption or repurchase, the Company will issue, execute and deliver to such Holder, and the Trustee will authenticate, in each case in accordance with Section 2.02, one or more Physical Notes that (x) are in Authorized Denominations and have an aggregate principal amount equal to the principal amount of such Physical Note that is not to be so converted, redeemed or repurchased; (y) are registered in the name of such Holder; and (z) bear each legend, if any, required by Section 2.09.

(ii) Global Notes. If a Global Note (or any portion thereof) is to be converted pursuant to Article 5 or repurchased pursuant to a Repurchase Upon Fundamental Change or Redemption, then, promptly after the time such Note (or such portion) is deemed to cease to be outstanding pursuant to Section 2.17, the Trustee will reflect a decrease of the principal amount of such Global Note in an amount equal to the principal amount of such Global Note to be so converted, redeemed or repurchased, as applicable, by notation on the “Schedule of Exchanges of Interests in the Global Note” forming part of such Global Note (and, if the principal amount of such Global Note is zero following such notation, cancel such Global Note pursuant to Section 2.14).

Section 2.12. Replacement Notes.

If a Holder of any Note claims that such Note has been mutilated, lost, destroyed or wrongfully taken, then the Company will issue, execute and deliver, and the Trustee will authenticate, in each case in accordance with Section 2.02, a replacement Note upon surrender to the Trustee of such mutilated Note, or upon delivery to the Trustee of evidence of such loss, destruction or wrongful taking reasonably satisfactory to the Trustee and the Company. In the case of a lost, destroyed or wrongfully taken Note, the Company and the Trustee may require the Holder thereof to provide such security or indemnity that is reasonably satisfactory to the Company and the Trustee to protect the Company and the Trustee from any loss that any of them may suffer if such Note is replaced.

Every replacement Note issued pursuant to this Section 2.12 will be an additional obligation of the Company and will be entitled to all of the benefits of this Indenture equally and ratably with all other Notes issued under this Indenture.

Section 2.13. Registered Holders; Certain Rights with Respect to Global Notes.

Only the Holder of a Note will have rights under this Indenture as the owner of such Note. Without limiting the generality of the foregoing, Depositary Participants will have no rights as such under this Indenture with respect to any Global Note held on their behalf by the Depositary or its nominee, or by the Trustee as its custodian, and the Company, the Guarantors,

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the Trustee and the Note Agents, and their respective agents, may treat the Depositary as the absolute owner of such Global Note for all purposes whatsoever; provided, however, that (A) the Holder of any Global Note may grant proxies and otherwise authorize any Person, including Depositary Participants and Persons that hold interests in Notes through Depositary Participants, to take any action that such Holder is entitled to take with respect to such Global Note under this Indenture or the Notes; and (B) the Company and the Trustee, and their respective agents, may give effect to any written certification, proxy or other authorization furnished by the Depositary.

Section 2.14. Cancellation.

Without limiting the generality of Section 3.08, the Company may at any time deliver Notes to the Trustee for cancellation. The Registrar, the Paying Agent and the Conversion Agent will forward to the Trustee each Note duly surrendered to them for transfer, exchange, payment or conversion. The Trustee will promptly cancel all Notes so surrendered to it in accordance with its customary procedures. Without limiting the generality of Section 2.03(B), the Company may not originally issue new Notes to replace Notes that it has paid or that have been cancelled upon transfer, exchange, payment or conversion.

Section 2.15. Notes Held by the Company.

Without limiting the generality of Section 3.08, in determining whether the Holders of the required aggregate principal amount of Notes have concurred in any direction, waiver or consent, Notes owned by the Company will be deemed not to be outstanding; provided, however, that, for purposes of determining whether the Trustee is protected in relying on any such direction, waiver or consent, only Notes that the Trustee knows are so owned will be so disregarded.

Section 2.16. TEMPORARY NOTES.

Until definitive Notes are ready for delivery, the Company may issue, execute and deliver, and the Trustee will authenticate, in each case in accordance with Section 2.02, temporary Notes. Temporary Notes will be substantially in the form of definitive Notes but may have variations that the Company considers appropriate for temporary Notes. The Company will promptly prepare, issue, execute and deliver, and the Trustee will authenticate, in each case in accordance with Section 2.02, definitive Notes in exchange for temporary Notes. Until so exchanged, each temporary Note will in all respects be entitled to the same benefits under this Indenture as definitive Notes.

Section 2.17. Outstanding Notes.

(A)          Generally. The Notes that are outstanding at any time will be deemed to be those Notes that, at such time, have been duly executed and authenticated, excluding those Notes (or portions thereof) that have theretofore been (i) cancelled by the Trustee or delivered to the Trustee for cancellation in accordance with Section 2.14; (ii) assigned a principal amount of zero by notation on the “Schedule of Exchanges of Interests in the Global Note” forming part of any a Global Note representing such Note; (iii) paid in full in accordance with this Indenture; or (iv) deemed to cease to be outstanding to the extent provided in, and subject to, clause (B), (C) or

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(D) of this Section 2.17.

(B)           Replaced Notes. If a Note is replaced pursuant to Section 2.12, then such Note will cease to be outstanding at the time of its replacement, unless the Trustee and the Company receive proof reasonably satisfactory to them that such Note is held by a “bona fide purchaser” under applicable law.

(C)           Maturing Notes and Notes Called for Redemption or Subject to Repurchase. If, on a Redemption Date, a Fundamental Change Repurchase Date or the Maturity Date, the Paying Agent holds money sufficient to pay the aggregate Redemption Price, Fundamental Change Repurchase Price or principal amount, respectively, together, in each case, with the aggregate interest, in each case due on such date, then (unless there occurs a Default in the payment of any such amount) (i) the Notes (or portions thereof) to be redeemed or repurchased, or that mature, on such date will be deemed, as of such date, to cease to be outstanding, except to the extent provided in Sections 4.02(D), 4.03(F) or 5.02(D); and (ii) the rights of the Holders of such Notes (or such portions thereof), as such, will terminate with respect to such Notes (or such portions thereof), other than the right to receive the Redemption Price, Fundamental Change Repurchase Price or principal amount, as applicable, of, and accrued and unpaid interest on, such Notes (or such portions thereof), in each case as provided in this Indenture.

(D)           Notes to Be Converted. At the Close of Business on the Conversion Date for any Note (or any portion thereof) to be converted, such Note (or such portion) will (unless there occurs a Default in the delivery of the Conversion Consideration or interest due, pursuant to Section 5.03(B) or Section 5.02(D), upon such conversion) be deemed to cease to be outstanding, except to the extent provided in Section 5.02(D).

(E)           Cessation of Accrual of Interest. Except as provided in Sections 4.02(D), 4.03(F) or 5.02(D), interest will cease to accrue on each Note from, and including, the date that such Note is deemed, pursuant to this Section 2.17, to cease to be outstanding, unless there occurs a default in the payment or delivery of any cash or other property due on such Note.

Section 2.18. Repurchases by the Company.

Without limiting the generality of Section 2.14, the Company may, from time to time, repurchase Notes in open market purchases, in negotiated transactions or otherwise without delivering prior notice to Holders; provided that no Default or Event of Default will have occurred and be continuing at such time.

Section 2.19. CUSIP and ISIN Numbers.

The Company may use one or more CUSIP or ISIN numbers to identify any of the Notes, and, if so, the Company and the Trustee will use such CUSIP or ISIN number(s) in notices to Holders; provided, however, that (i) the Trustee makes no representation as to the correctness or accuracy of any such CUSIP or ISIN number; and (ii) the effectiveness of any such notice will not be affected by any defect in, or omission of, any such CUSIP or ISIN number. The Company will promptly notify the Trustee of any change in the CUSIP or ISIN number(s) identifying any Notes.

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Article 3. COVENANTS

Section 3.01. Payment on Notes.

(A)          Generally. The Company will pay or cause to be paid all the principal of, the Fundamental Change Repurchase Price and Redemption Price for, interest on, and other amounts due with respect to, the Notes on the dates and in the manner set forth in this Indenture.

(B)          Deposit of Funds. Before 10:00 A.M., New York City time, on each Redemption Date, Fundamental Change Repurchase Date or Interest Payment Date, and on the Maturity Date or any other date on which any cash amount is due on the Notes, the Company will deposit, or will cause there to be deposited, with the Paying Agent cash, in funds immediately available on such date, sufficient to pay the cash amount due on the applicable Notes on such date. The Paying Agent will return to the Company, as soon as practicable, any money not required for such purpose.

Section 3.02. Reports.

(A)          Generally. The Company will send to the Trustee (i) copies of all reports that the Company is required to file with the SEC pursuant to Section 13(a) or 15(d) of the Exchange Act within fifteen (15) calendar days after the date that the Company is required to file the same (after giving effect to all applicable grace periods under the Exchange Act); provided, however, that the Company need not send to the Trustee any material for which the Company has received, or is seeking in good faith and has not been denied, confidential treatment by the SEC and (ii) customary quarterly financial reports within forty-five (45) calendar days of the end of each fiscal quarter. Any report that the Company files with the SEC through the EDGAR system or the RNS system in the United Kingdom (or any successor thereto) or posts on its website will be deemed to be sent to the Trustee at the time such report is so filed via the EDGAR system or RNS system (or such successor) or posted on its website. Upon the request of any Holder, the Trustee will provide to such Holder a copy of any report that the Company has sent the Trustee pursuant to this Section 3.02(A), other than a report that is deemed to be sent to the Trustee pursuant to the preceding sentence.

(B)           Trustee’s Disclaimer. The Trustee need not determine whether the Company has filed any material via the EDGAR system (or such successor). The sending or filing of reports to the Trustee pursuant to Section 3.02(A) is for informational purposes only and will not be deemed to constitute actual or constructive notice to the Trustee of any information contained, or determinable from information contained, therein, including the Company’s compliance with any of its covenants under this Indenture (as to which the Trustee is entitled to conclusively rely on an Officer’s Certificate).

Section 3.03. Additional Interest.

(A)          Accrual of Additional Interest. If, at any time on or after the Last Original Issue Date of any Note, such Note is not Freely Tradable, then Additional Interest will accrue on such Note for each day during such period on which such failure is continuing or such Note is not Freely Tradable. For the avoidance of doubt, any Note bearing the Private Placement Legend is

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deemed held by an Affiliate of the Company and no Additional Interest will accrue on the such Note.

(B)           Amount and Payment of Additional Interest. Any Additional Interest that accrues on a Note pursuant to Section 3.03(A) will be payable on the same dates and in the same manner as the Stated Interest on such Note and will accrue at a rate per annum equal to one half of one percent (0.50%) of the principal amount thereof. For the avoidance of doubt, any Additional Interest that accrues on a Note will be in addition to the Stated Interest that accrues on such Note and in addition to any Special Interest that accrues on such Note.

(C)           Notice of Accrual of Additional Interest; Trustee’s Disclaimer. The Company will send notice to the Holder of each Note, and to the Trustee, of the commencement and termination of any period in which Additional Interest accrues on such Note. In addition, if Additional Interest accrues on any Note, then, no later than five (5) Business Days before each date on which such Additional Interest is to be paid, the Company will deliver an Officer’s Certificate to the Trustee and the Paying Agent stating (i) that the Company is obligated to pay Additional Interest on such Note on such date of payment; and (ii) the amount of such Additional Interest that is payable on such date of payment. The Trustee will have no duty to determine whether any Additional Interest is payable or the amount thereof.

Section 3.04. Additional Amounts.

(A)          Requirement to Pay Additional Amounts. All payments and deliveries made by, or on behalf of, the Company under or with respect to the Notes (including payment of the principal of, or the Redemption Price or Fundamental Change Repurchase Price for, or any interest on, or the delivery of any consideration due upon conversion of, any Note) will be made without withholding or deduction for, or on account of, any present or future Taxes, unless such withholding or deduction is required by law or regulation or by governmental policy having the force of law. If any Taxes levied by or on behalf of any jurisdiction (or any political subdivision or taxing authority thereof or therein) in which the Company or any Successor Corporation is, for tax purposes, organized or resident or doing business or through which payment is made or deemed to be made (each such jurisdiction, subdivision or authority, as applicable, a “Relevant Taxing Jurisdiction”) are required to be withheld or deducted from any payments or deliveries made under or with respect to the Notes, then, subject to Section 4.03(C)(ii), the Company or such Successor Corporation, as applicable, will pay to the holder of each note such additional amounts (the “Additional Amounts”) as may be necessary to ensure that the net amount received by the beneficial owner of such Note after such withholding or deduction (and after withholding or deducting any Taxes on the Additional Amounts) will equal the amounts that would have been received by such beneficial owner had no such withholding or deduction been required; provided, however, that such obligation to pay Additional Amounts will not apply to:

(i) any Tax that would not have been imposed but for:

(1)          the existence of any present or former connection between the Holder or beneficial owner of such Note and the Relevant Taxing Jurisdiction (other than merely holding or being a beneficial owner of such Note or the receipt or enforcement of payments thereunder), including such Holder or beneficial owner being or having been a

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national, domiciliary or resident, or treated as a resident, of, or being or having been physically present or engaged in a trade or business, or having had a permanent establishment, in, such Relevant Taxing Jurisdiction;

(2)          in cases where presentation of such Note is required to receive such payment or delivery, the presentation of such Note after a period of thirty (30) days after the later of (x) the date on which such payment or delivery became due and payable or deliverable, as applicable, pursuant to the terms of this Indenture and (y) the date such payment or delivery was made or duly provided for, except, in each case, to the extent that such Holder or beneficial owner would have been entitled to Additional Amounts if it presented such Note for payment or delivery, as applicable, at the end of such thirty (30) day period; or

(3)          the failure of such Holder or beneficial owner to comply with a timely request from the Company or the Successor Corporation, addressed to such Holder or beneficial owner, to (x) provide certification, information, documentation or other evidence concerning such Holder’s or beneficial owner’s nationality, residence, identity or connection with such Relevant Taxing Jurisdiction; or (y) make any declaration or satisfy any other reporting requirement prescribed by applicable law as a basis to reduce or eliminate the amount of Tax required to be deducted or withheld with respect to payments or deliveries made under or with respect to the Notes, in each case if and to the extent that such Holder or beneficial owner is legally entitled to comply with such request; provided, however, that compliance with any of the foregoing (other than the provision of customary certification forms issued by the US Internal Revenue Service or with respect to U.S. withholding taxes) shall not be required if in the Holder’s or beneficial owner’s reasonable judgment such compliance would subject such Holder or beneficial owner to any material unreimbursed cost or expense or would materially prejudice its legal or commercial position;

(ii) any estate, inheritance, gift, sale, transfer, personal property or similar Tax;

(iii) any tax that is payable other than by withholding or deduction from payments under or with respect to the Notes;

(iv) any withholding or deduction required by (x) sections 1471 through 1474 of the Internal Revenue Code and any current or future U.S. Treasury Regulations or rulings promulgated thereunder (“FATCA”); (y) any inter-governmental agreement between the United States and any other non-U.S. jurisdiction to implement FATCA or any law enacted by such other jurisdiction to give effect to such agreement; or (z) any agreement with the U.S. Internal Revenue Service pursuant to Section 1471(b)(1) of the Internal Revenue Code;

(v) any taxes imposed on or with respect to any payment by the Company to such Holder if such Holder is a fiduciary, partnership or person other than the sole beneficial owner of such payment, to the extent that such payment would be required, under the laws of such Relevant Taxing Jurisdiction, to be included for tax purposes in the income of a beneficiary or settlor with respect to such fiduciary, a partner or member of such partnership, or a beneficial owner, who would not have been entitled to such additional amounts had such beneficiary, settlor, partner, member or beneficial owner been the Holder thereof;

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(vi) withholding taxes imposed on amounts payable with respect to the Notes pursuant to a law or regulation in effect on the date on which a beneficial owner acquires any Notes; or

(vii) any combination of items referred to in the preceding clauses (i) through (vi), inclusive, above.

(B)           Indemnification for Other Taxes. The Company will pay and indemnify each Holder for any present or future stamp, issue, registration, transfer; court, documentary, excise or property Taxes (“Other Taxes”) levied by any Relevant Taxing Jurisdiction in connection with the execution, delivery, registration, issuance or enforcement of any of the Notes (other than on or in connection with a transfer of a note that occurs after the initial sales thereof) or the receipt of any payments or deliveries with respect to the Notes; provided, however, that, with respect to any such taxes attributable to the receipt of any payments or deliveries with respect to the Notes, Other Taxes will not include those excluded under any combination of clauses (i), (ii), (iv), (v) and (vi) of Section 3.04(A).

(C)           For the avoidance of doubt, if any Note is called for a Tax Redemption and the Redemption Date is after a Regular Record Date and on or before the next Interest Payment Date, then the Company’s obligation to pay Additional Amounts will apply to the interest payment due on such Note on such Interest Payment Date unless such Note is the subject to a Tax Redemption Opt-Out Election Notice.

(D)           Tax Receipts. If the Company is required to make any deduction or withholding from any payments with respect to the Notes, then the (i) Company will deliver to the Trustee official tax receipts (or, if, after expending reasonable efforts, the Company is unable to obtain such receipts, other evidence of payments) evidencing the remittance to the relevant tax authorities of the amounts so withheld or deducted; and (ii) the Trustee or the Company will provide a copy of such receipts or evidence, as applicable, upon request from any Holder or beneficial owner of any Notes for whom such deduction or withholding is required to be made.

(E)           Interpretation of Indenture and Notes. All references in this Indenture or the Notes to any payment on, or delivery with respect to, the Notes (including payment of the principal of, or the Redemption Price or Fundamental Change Repurchase Price for, or any interest on, or the delivery of any Conversion Consideration due upon conversion of, any Note) will, to the extent that Additional Amounts are payable in respect thereof, be deemed to include the payment of such Additional Amounts.

(F)           Survival of Obligations. The obligations set forth in this Section 3.04 will survive any transfer of Notes by a Holder (or, in the case of a Global Note, a holder of a beneficial

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interest therein).

Section 3.05. Compliance and Default Certificates.

(A)          Annual Compliance Certificate. Within ninety (90) days after December 31, 2019 and each fiscal year of the Company ending thereafter, the Company and the Guarantors will deliver an Officer’s Certificate to the Trustee stating (i) that the signatory thereto has supervised a review of the activities of the Company and the Guarantors during such fiscal year with a view towards determining whether any Default or Event of Default has occurred; and (ii) whether, to such signatory’s knowledge, a Default or Event of Default has occurred or is continuing (and, if so, describing all such Defaults or Events of Default and what action the Company or Guarantors is taking or proposes to take with respect thereto).

(B)           Default Certificate. If a Default or Event of Default occurs, then the Company will promptly deliver an Officer’s Certificate to the Trustee describing the same and what action the Company is taking or proposes to take with respect thereto.

Section 3.06. Stay, Extension and Usury Laws.

To the extent that it may lawfully do so, the Company (A) agrees that it will not at any time insist upon, plead, or in any manner whatsoever claim or take the benefit or advantage of, any stay, extension or usury law (wherever or whenever enacted or in force) that may affect the covenants or the performance of this Indenture; and (B) expressly waives all benefits or advantages of any such law and agrees that it will not, by resort to any such law, hinder, delay or impede the execution of any power granted to the Trustee by this Indenture, but will suffer and permit the execution of every such power as though no such law has been enacted.

Section 3.07. Corporate Existence.

Subject to Article 6, the Company will cause to preserve and keep in full force and effect:

(A)          its corporate existence in accordance with the organizational documents of the Company; and

(B)           the material rights (charter and statutory), licenses and franchises of the Company and its Subsidiaries;

provided, however, that the Company need not preserve or keep in full force and effect any such license or franchise if the Board of Directors determines that (x) the preservation thereof is no longer desirable in the conduct of the business of the Company and its Subsidiaries, taken as a whole; and (y) the loss thereof is not, individually or in the aggregate, materially adverse to the Holders.

Section 3.08. Restriction on Acquisition of Notes by the Company.

The Company will promptly deliver to the Trustee for cancellation all Notes that the Company or any of its Subsidiaries have purchased or otherwise acquired.

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Section 3.09. Further Instruments and Acts.

At the Trustee’s request, the Company will execute and deliver such further instruments and do such further acts as may be reasonably necessary or proper to more effectively carry out the purposes of this Indenture.

Section 3.10. Listing.

The Parent shall use commercially reasonable efforts to list the Ordinary Shares or ADRs representing Ordinary Shares on Nasdaq and maintain such listing for so long as any Notes are outstanding.

If the Company lists ADRs on Nasdaq, then the Company may by notice to the Trustee and the holders elect, subject to the terms of the ADR facility, to have future conversion of Notes settled in ADRs. For so long as such election is in effect, references to Ordinary Shares will be deemed to refer to ADRs and appropriate adjustment will be made to the settlement and trading provisions of the Notes to account for any deposit ratio between ADRs and Ordinary Shares; provided, that if ADRs cannot be issued to a converting holder under the terms of the ADR facility as then in place, then the settlement and the trading provisions will be deemed to refer to Ordinary Shares with respect to such conversion.

Section 3.11. Restricted Payments.

So long as any Notes remain outstanding, the Company and the Guarantors shall not, and shall not permit any of their Subsidiaries to declare or make, directly or indirectly, any Restricted Payment:

(A)          unless, at the time of such Restricted Payment:

(i) no Default shall have occurred and be continuing or would occur as a consequence thereof;

(ii) immediately after giving effect to such transaction on a pro forma basis, the Parent or such Subsidiary, as applicable, could incur $1.00 of additional Indebtedness under Section 3.13(F); and

(iii) such Restricted Payment, together with the aggregate amount of all other Restricted Payments made by the Parent or such Subsidiary after the Issue Date, excluding Restricted Payments permitted by Section 3.11(B), is less than the amount equal to the Cumulative Credit.

(B)           The provisions of Section 3.11(A) will not prohibit:

(i) Customary tax distributions and overhead payments;

(ii) Cashless exchange of Indebtedness;

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(iii) Restricted Payments in the amount of five million dollars ($5,000,000) for repurchases of Ordinary Shares; provided no Default or Event of Default will have occurred and be continuing at the time of such Restricted Payment;

(iv) to the extent constituting a Restricted Payment, the payment of fees of non-insider directors not to exceed an annual amount of [***] and the reimbursement of reasonable expenses;

(v) (i) direct or indirect Restricted Payments to the Company and other Subsidiaries of the Company that are Guarantors from other Subsidiaries of the Borrower and (ii) in the case of Subsidiaries that are not Guarantors, direct or indirect Restricted Payments to other Subsidiaries that are not Guarantors;

(vi) (i) Restricted Payments to the Parent to be used for (A) customary director indemnification and compensation payments to the Parent’s director nominees serving on the board of directors of Parent, Company or on the board of directors of any Subsidiary and (B) payment of income Taxes to the extent such income Taxes are attributable to the income of its direct or indirect Subsidiaries, and (ii) so long as no Event of Default has occurred and is continuing, Restricted Payments to the Parent and to Amryt Pharmaceuticals DAC to be used for financial and other reporting and similar customary administrative costs and expenses attributable and fairly allocable to the Company and the Guarantors (including audit and professional fees and other ordinary course operating and administrative expenses incurred by the Parent in its capacity as the ultimate holding company of the Company and the other Guarantors);

(vii)  the Parent may declare and make dividend payments or other distributions payable solely in the common stock or other common Equity Interests of such Person (other than Disqualified Equity Interests); and

(viii) transactions consisting of intercompany services among the Parent and its wholly-owned Subsidiaries in the ordinary course of business on an arm’s length basis, charged on a cost plus a maximum of 10% basis.

Section 3.12. Dispositions.

So long as any Notes remain outstanding, the Company and the Guarantors shall not, and shall not permit any of their Subsidiaries to declare or make, directly or indirectly, any Disposition or enter into any agreement to make any Disposition, except:

(A)          Dispositions of obsolete, worn out or surplus property, whether now owned or hereafter acquired, in the ordinary course of business and Dispositions of property no longer used or useful in the conduct of the business of the Parent and its Subsidiaries, in each case to the extent constituting immaterial property;

(B)           Dispositions in the ordinary course of business of Cash Equivalents;

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(C)           sales of inventory in the ordinary course of business;

(D)           Dispositions (other than of material Intellectual Property or of assets relating to metreleptin) for fair market value (as determined by the Company in good faith); provided that (i) the amount of Dispositions does not exceed $250,000 individually or $2,500,000 in the aggregate for all Dispositions while any Notes are outstanding, (ii) immediately prior to and immediately after giving effect to such Disposition, no Default or Event of Default shall have occurred and be continuing or would result therefrom and (iii) no less than one hundred percent (100%) of the consideration received for any such Disposition is received in cash;

(E)           the leasing, as lessor, of real or personal property not presently used or useful in such Person’s business and is otherwise in the ordinary course of business;

(F)           Dispositions of equipment or other assets, to the extent that such equipment is exchanged for credit against the purchase price of similar replacement equipment or assets or the proceeds of such Dispositions are reasonably promptly applied to the purchase price of similar replacement equipment, all in the ordinary course of business;

(G)           Dispositions constituting an Intellectual Property that is not material to the conduct of the business of the Parent, the Guarantors and their Subsidiaries;

(H)           Dispositions otherwise permitted by Section 3.13 and Dispositions from any Subsidiary that is not a Loan Party to any other Subsidiary that is not a Loan Party;

(I)            the licensing of AP101 and AP103 assets outside of the United States and European territories;

(J)            Dispositions consisting of licenses of AP102 assets; and

(K)           non-ordinary course Disposition of assets subject only to the receipt of fair market value (as determined by the Company in good faith), at least seventy five percent (75%) of the proceeds consisting of cash or Cash Equivalents, and Net Cash Proceeds being applied to repay secured Indebtedness, reinvested within twelve (12) months (or committed to be reinvested into a clinical development program approved by the Parent Board within twelve (12) months) or offered to repurchase the Notes at a purchase price (without premium or penalty) equal to 100% of the principal amount of such Notes plus accrued and unpaid interest on such Notes, if any, to, but excluding, such date of repurchase, which will be a Business Day of the Company’s choosing no later than the end of such twelve (12) month period.

Section 3.13. Indebtedness.

So long as any Notes remain outstanding, the Company and the Guarantors shall not, and shall not permit any of their Subsidiaries to, directly or indirectly, create, incur, assume or suffer to exist any Indebtedness, except the following, without duplication:

(A)          Indebtedness outstanding on the Issue Date (including the Notes);

(B)          Indebtedness incurred in connection with the Plan (including the Senior Secured

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Credit Facility, and which shall further permit the principal amount of indebtedness under the Senior Secured Credit Facility to be increased to one hundred million dollars ($100,000,000) prior to any refinancing of the Senior Secured Credit Facility);

(C)        additional Capital Leases incurred after the Issue Date and purchase money Indebtedness in an aggregate amount not to exceed $750,000 in the aggregate at any time outstanding, and any Refinancing Indebtedness in respect of such Indebtedness; provided that any such Indebtedness (i) in the case of additional Capital Leases or purchase money Indebtedness, shall be secured only by the asset subject to such additional Capital Leases or acquired asset in connection with the incurrence of such Indebtedness, as the case may be, and (ii) in the case of purchase money Indebtedness, shall constitute not less than 75% of the aggregate consideration paid with respect to such asset;

(D)          other unsecured Indebtedness in an aggregate principal amount not to exceed $250,000 at any time outstanding;

(E)Indebtedness subordinated to the Notes so long as such Indebtedness has a maturity date one year past the Maturity Date and an interest rate lower than the Notes (the “Junior Indebtedness”);

(F)       Indebtedness that is pari passu in right of payment to the Notes (including secured Indebtedness) if net pharmaceutical product revenue for the twelve (12) months prior to the incurrence of such Indebtedness, on a pro forma basis, exceeds 1:00 to 1:00 of all funded Indebtedness (excluding the Junior Indebtedness);

(G)Indebtedness in respect of performance of bids, trade contracts, governmental contracts and leases (other than Indebtedness for borrowed money), statutory obligations, surety, stay, indemnity, customs and appeal bonds, performance bonds and other obligations of a like nature (including those to secure health, safety and environmental obligations), and, in each case, letters of credit in respect thereof, incurred in the ordinary course of business;

(H)non-recourse Indebtedness incurred by the Company or the Guarantors or any of their Subsidiaries to finance the payment of insurance premiums of such Person;

(I)         Indebtedness owed to any Person providing worker’s compensation, unemployment insurance and other social security legislation, health, disability or other employee benefits or property, casualty or liability insurance to the Company or the Guarantors or any of their Subsidiaries incurred in connection with such Person providing such benefits or insurance pursuant to customary reimbursement or indemnification obligations to such Person;

(J)     reimbursement obligations owed to banks and financial institutions with respect to credit card services in an aggregate amount at any one time not exceeding $400,000;

(K)Indebtedness consisting of accounts payable incurred in the ordinary course of business past due for more than 120 days after its stated due date (except for accounts payable contested in good faith) which do not in the aggregate exceed $750,000;

(L)           Indebtedness of the Company and the Guarantors under the Deed Poll

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Constituting Loan Notes in an aggregate principal amount not to exceed $85,000,000 at any time outstanding;

(M)          finance leases with respect to AP101 and AP103 equipment in an amount not to exceed $5,000,000 in the aggregate at any time outstanding; provided that such Indebtedness shall be secured only by the equipment financed thereunder;

(N)           Indebtedness under that certain Finance Contract dated as of December 1, 2016 between Amryt Pharmaceuticals DAC and European Investment Bank, as the same may be amended, restated, supplemented or otherwise modified from time to time, from the period from the Issue Date up through one (1) Business Day following the Issue Date which Indebtedness shall be paid in full no later than one (1) Business Day following the Issue Date; and

(O)           The refinancing of any Indebtedness that was permitted under this Indenture when it was incurred, only to the extent such principal amount of such refinancing is not more than the principal amount of such Indebtedness being refinanced, plus any customary fees and reasonable expenses.

Article 4. REPURCHASE AND REDEMPTION

Section 4.01. No Sinking Fund.

No sinking fund is required to be provided for the Notes.

Section 4.02. Right of Holders to Require the Company to Repurchase Notes upon a Fundamental Change.

(A)          Right of Holders to Require the Company to Repurchase Notes Upon a Fundamental Change. Subject to the other terms of this Section 4.02, if a Fundamental Change occurs, then each Holder will have the right (the “Fundamental Change Repurchase Right”) to require the Company to repurchase such Holder’s Notes (or any portion thereof in an Authorized Denomination) on the Fundamental Change Repurchase Date for such Fundamental Change for a cash purchase price equal to the Fundamental Change Repurchase Price.

(B)           Repurchase Prohibited in Certain Circumstances. If the principal amount of the Notes has been accelerated and such acceleration has not been rescinded on or before the Fundamental Change Repurchase Date for a Repurchase Upon Fundamental Change (including as a result of the payment of the related Fundamental Change Repurchase Price, and any related interest pursuant to the proviso to Section 4.02(D), on such Fundamental Change Repurchase Date), then (i) the Company shall not repurchase any Notes pursuant to this Section 4.02; and (ii) the Company will cause any Notes theretofore surrendered for such Repurchase upon Fundamental Change to be returned to the Holders thereof (or, if applicable with respect to Global Notes, cancel any instructions for book-entry transfer to the Company, the Trustee or the Paying Agent of the applicable beneficial interest in such Notes in accordance with the Depositary Procedures).

(C)           Fundamental Change Repurchase Date. The Fundamental Change Repurchase Date for any Fundamental Change will be a Business Day of the Company’s choosing that is no

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more than thirty five (35), nor less than twenty (20), Business Days after the date the Company sends the related Fundamental Change Notice pursuant to Section 4.02(E).

(D)           Fundamental Change Repurchase Price. The Fundamental Change Repurchase Price for any Note to be repurchased upon a Repurchase Upon Fundamental Change following a Fundamental Change is an amount in cash equal to 100% of the principal amount of such Note plus accrued and unpaid interest on such Note, if any, to, but excluding, the Fundamental Change Repurchase Date for such Fundamental Change; provided, however, that if such Fundamental Change Repurchase Date is after a Regular Record Date and on or before the next Interest Payment Date, then (i) the Holder of such Note at the Close of Business on such Regular Record Date will be entitled, notwithstanding such Repurchase Upon Fundamental Change, to receive, on or, at the Company’s election, before such Interest Payment Date, the unpaid interest that would have accrued on such Note to, but excluding, such Interest Payment Date (assuming, solely for these purposes, that such Note remained outstanding through such Interest Payment Date, if such Fundamental Change Repurchase Date is before such Interest Payment Date); and (ii) the Fundamental Change Repurchase Price will not include accrued and unpaid interest on such Note to, but excluding, such Fundamental Change Repurchase Date. For the avoidance of doubt, if an Interest Payment Date is not a Business Day within the meaning of Section 2.05(C) and such Fundamental Change Repurchase Date occurs on the Business Day immediately after such Interest Payment Date, then (x) accrued and unpaid interest on Notes to, but excluding, such Interest Payment Date will be paid, in accordance with Section 2.05(C), on the next Business Day to Holders at of the Close of Business on the immediately preceding Regular Record Date; and (y) the Fundamental Change Repurchase Price will include interest on Notes to be repurchased from, and including, such Interest Payment Date.

(E)           Fundamental Change Notice. On or before the twentieth (20th) calendar day after the occurrence of a Fundamental Change, the Company will send to each Holder, the Trustee and the Paying Agent a notice of such Fundamental Change (a “Fundamental Change Notice”).

Such Fundamental Change Notice must state:

(i) briefly, the events causing such Fundamental Change;

(ii) the effective date of such Fundamental Change;

(iii) the procedures that a Holder must follow to require the Company to repurchase its Notes pursuant to this Section 4.02, including the deadline for exercising the Fundamental Change Repurchase Right and the procedures for submitting and withdrawing a Fundamental Change Repurchase Notice;

(iv) the Fundamental Change Repurchase Date for such Fundamental Change;

(v) the Fundamental Change Repurchase Price per $1,000 principal amount of Notes for such Fundamental Change (and, if such Fundamental Change Repurchase Date is after a Regular Record Date and on or before the next Interest Payment Date, the amount, manner and timing of the interest payment payable pursuant to the proviso to Section 4.02(D));

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(vi) the name and address of the Paying Agent and the Conversion Agent;

(vii) the Conversion Rate in effect on the date of such Fundamental Change Notice and a description and quantification of any adjustments to the Conversion Rate that may result from such Fundamental Change (including pursuant to Section 5.07);

(viii) that Notes for which a Fundamental Change Repurchase Notice has been duly tendered and not duly withdrawn must be delivered to the Paying Agent for the Holder thereof to be entitled to receive the Fundamental Change Repurchase Price;

(ix) that Notes (or any portion thereof) that are subject to a Fundamental Change Repurchase Notice that has been duly tendered may be converted only if such Fundamental Change Repurchase Notice is withdrawn in accordance with this Indenture; and

(x) the CUSIP and IS1N numbers, if any, of the Notes.

Neither the failure to deliver a Fundamental Change Notice nor any defect in a Fundamental Change Notice will limit the Fundamental Change Repurchase Right of any Holder or otherwise affect the validity of any proceedings relating to any Repurchase Upon Fundamental Change.

(F)           Procedures to Exercise the Fundamental Change Repurchase Right.

(i) Delivery of Fundamental Change Repurchase Notice and Notes to Be Repurchased. To exercise its Fundamental Change Repurchase Right for a Note following a Fundamental Change, the Holder thereof must deliver to the Paying Agent:

(1)           before the Close of Business on the second Business Day immediately before the related Fundamental Change Repurchase Date (or such later time as may be required by law), a duly completed, written Fundamental Change Repurchase Notice with respect to such Note; and

(2)           such Note, duly endorsed for transfer (if such Note is a Physical Note) or by book-entry transfer (if such Note is a Global Note).

The Paying Agent will promptly deliver to the Company a copy of each Fundamental Change Repurchase Notice that it receives.

(ii) Contents of Fundamental Change Repurchase Notices. Each Fundamental Change Repurchase Notice with respect to a Note must state:

(1)           if such Note is a Physical Note, the certificate number of such Note;

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(2)          the principal amount of such Note to be repurchased, which must be an Authorized Denomination; and

(3)          that such Holder is exercising its Fundamental Change Repurchase Right with respect to such principal amount of such Note;

provided, however, that if such Note is a Global Note, then such Fundamental Change Repurchase Notice must comply with the Depositary Procedures (and any such Fundamental Change Repurchase Notice delivered in compliance with the Depositary Procedures will be deemed to satisfy the requirements of this Section 4.02(F)).

(iii) Withdrawal of Fundamental Change Repurchase Notice. A Holder that has delivered a Fundamental Change Repurchase Notice with respect to a Note may withdraw such Fundamental Change Repurchase Notice by delivering a written notice of withdrawal to the Paying Agent at any time before the Close of Business on the second Business Day immediately before the related Fundamental Change Repurchase Date. Such withdrawal notice must state:

(1)          if such Note is a Physical Note, the certificate number of such Note;

(2)          the principal amount of such Note to be withdrawn, which must be an Authorized Denomination; and

(3)          the principal amount of such Note, if any, that remains subject to such Fundamental Change Repurchase Notice, which must be an Authorized Denomination;

provided, however, that if such Note is a Global Note, then such withdrawal notice must comply with the Depositary Procedures (and any such withdrawal notice delivered in compliance with the Depositary Procedures will be deemed to satisfy the requirements of this Section 4.02(F)).

Upon receipt of any such withdrawal notice with respect to a Note (or any portion thereof), the Paying Agent will (x) promptly deliver a copy of such withdrawal notice to the Company; and (y) if such Note is surrendered to the Paying Agent, cause such Note (or such portion thereof in accordance with Section 2.11, treating such Note as having been then surrendered for partial repurchase in the amount set forth in such withdrawal notice as remaining subject to repurchase) to be returned to the Holder thereof (or, if applicable with respect to any Global Note, cancel any instructions for book-entry transfer to the Company, the Trustee or the Paying Agent of the applicable beneficial interest in such Note in accordance with the Depositary Procedures).

(G)           Payment of the Fundamental Change Repurchase Price. Without limiting the Company’s obligation to deposit the Fundamental Change Repurchase Price within the time proscribed by Section 3.01(B), the Company will cause the Fundamental Change Repurchase

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Price for a Note (or portion thereof) to be repurchased pursuant to a Repurchase Upon Fundamental Change to be paid to the Holder thereof on or before the later of (i) the applicable Fundamental Change Repurchase Date; and (ii) the date (x) such Note is delivered to the Paying Agent (in the case of a Physical Note) or (y) the Depositary Procedures relating to the repurchase, and the delivery to the Paying Agent, of such Holder’s beneficial interest in such Note to be repurchased are complied with (in the case of a Global Note). For the avoidance of doubt, interest payable pursuant to the proviso to Section 4.02(D) on any Note to be repurchased pursuant to a Repurchase Upon Fundamental Change must be paid pursuant to such proviso regardless of whether such Note is delivered or such Depositary Procedures are complied with pursuant to the first sentence of this Section 4.02(G).

(H)          Compliance with Applicable Securities Laws. To the extent applicable, the Company will comply with all federal and state securities laws in connection with a Repurchase Upon Fundamental Change (including complying with Rules 13e-4 and 14e-l under the Exchange Act and filing any required Schedule TO, to the extent applicable) so as to permit effecting such Repurchase Upon Fundamental Change in the manner set forth in this Indenture.

(I)            Repurchase in Part, Subject to the terms of this Section 4.02, Notes may be repurchased pursuant to a Repurchase Upon Fundamental Change in part, but only in Authorized Denominations. Provisions of this Section 4.02 applying to the repurchase of a Note in whole will equally apply to the repurchase of a permitted portion of a Note.

Section 4.03. Right of the Company to Redeem the Notes.

(A)          No Right to Redeem Before October 1, 2022. The Company may not redeem the Notes at its option at any time before October 1, 2022, except pursuant to a Tax Redemption.

(B)           Right to Redeem the Notes on or After October 1, 2022. Subject to the terms of this Section 4.03, the Company has the right, at its election, to redeem all, or any portion in an Authorized Denomination, of the Notes, at any time and from time to time, on a Redemption Date on or after October 1, 2022 and on or before the twentieth (20th) Scheduled Trading Day immediately before the Maturity Date, for a cash purchase price equal to the Redemption Price, but only if the Daily VWAP per Ordinary Share exceeds one hundred and fifty percent (150%) of the Conversion Price on (i) each of at least twenty (20) VWAP Trading Days (whether or not consecutive) during the thirty (30) consecutive VWAP Trading Days ending on, and including, the VWAP Trading Day immediately before the Redemption Notice Date for such Redemption; and (ii) the VWAP Trading Day immediately before such Redemption Notice Date.

(C)           Right to Redeem the Notes After a Change in Tax Law.

(i) Generally. Subject to the terms of this Section 4.03, and without limiting the Company’s right to redeem any Notes pursuant to Section 4.03(B), the Company has the right, at its election, to redeem all, but not less than all, of the Notes, at any time, on a Redemption Date before the Maturity Date, for a cash purchase price equal to the Redemption Price, but only if (i) the Company has (or, on the next Interest Payment Date, would) become obligated to pay any Additional Amounts to Holders as a result of any Change

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in Tax Law; (ii) the Company cannot avoid such obligation by taking reasonable measures available to the Company; and (iii) the Company delivers to the Trustee (1) an Opinion of Counsel from outside legal counsel of recognized standing in the Relevant Taxing Jurisdiction attesting to clause (i) above; and (2) an Officer’s Certificate attesting to clauses (i) and (ii) above.

(ii) Tax Redemption Opt-Out Election. If the Company calls the Notes for a Tax Redemption, then, notwithstanding anything to the contrary in this Section 4.03 or in Section 3.04, each Holder will have the right to elect (a “Tax Redemption Opt-Out Election”) not to have such Holder’s Notes (or any portion thereof in an Authorized Denomination) redeemed pursuant to such Tax Redemption, in which case, from and after the Redemption Date for such Tax Redemption (or, if the Company fails to pay the Redemption Price due on such Redemption Date in full, from and after such time as the Company pays such Redemption Price in full), the Company will no longer have any obligation to pay any Additional Amounts with respect to such Notes solely as a result of such Change in Tax Law, and all future payments with respect to such Notes (other than any Conversion Consideration prior to the corresponding Redemption Date) will be subject to the deduction or withholding of such Relevant Taxing Jurisdiction’s taxes required by law to be deducted or withheld as a result of such Change in Tax Law (it being understood, for the avoidance of doubt, that if such Holder converts such Notes prior to the corresponding Redemption Date, then the Company will be obligated to pay Additional Amounts, if any, and subject to the limitations on the payment of such Additional Amounts set forth in Section 3.04, with respect to such conversion).

(1)          Tax Redemption Opt-Out Election Notice. To make a Tax Redemption Opt-Out Election with respect to any Note (or any portion thereof in an Authorized Denomination), the Holder of such Note must deliver a notice (a “Tax Redemption Opt-Out Election Notice”) to the Paying Agent before the Close of Business on the second Business Day immediately before the related Redemption Date, which notice must state: (x) if such Note is a Physical Note, the certificate number of such Note; (y) the principal amount of such Note as to which the Tax Redemption Opt-Out Election will apply, which must be an Authorized Denomination; and (z) that such Holder is making a Tax Redemption Opt-Out Election with respect to such Note (or such portion thereof); provided, however, that if such Note is a Global Note, then such notice must comply with the Depositary Procedures (and any such notice delivered in compliance with the Depositary Procedures will be deemed to satisfy the requirements of this Section 4.03(C)(ii)(1)).

(2)          Withdrawal of Tax Redemption Opt-Out Election Notice. A Holder that has delivered a Tax Redemption Opt-Out Election Notice with respect to any Note (or any portion thereof in an Authorized Denomination) may withdraw such Tax Redemption Opt-Out Election Notice by delivering a withdrawal notice to the Paying Agent at any time before the Close of Business on the second Business Day immediately before the related Redemption Date, which withdrawal notice must state: (x) if such Note

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is a Physical Note, the certificate number of such Note; (y) the principal amount of such Note as to which the Tax Redemption Opt-Out Election is being withdrawn, which must be an Authorized Denomination; and (z) that such Holder is withdrawing the Tax Redemption Opt-Out Election with respect to such Note (or such portion thereof); provided, however, that if such Note is a Global Note, then such withdrawal notice must comply with the Depositary Procedures (and any such withdrawal notice delivered in compliance with the Depositary Procedures will be deemed to satisfy the requirements of this Section 4.03(C)(ii)(2)).

(D)          Redemption Prohibited in Certain Circumstances. If the principal amount of the Notes has been accelerated and such acceleration has not been rescinded on or before the Redemption Date (including as a result of the payment of the related Redemption Price, and any related interest pursuant to the proviso to Section 4.03(F), on such Redemption Date), then (i) the Company may not call for Redemption or otherwise redeem any Notes pursuant to this Section 4.03; and (ii) the Company will cause any Notes theretofore surrendered for such Redemption to be returned to the Holders thereof (or, if applicable with respect to Global Notes, cancel any instructions for book-entry transfer to the Company, the Trustee or the Paying Agent of the applicable beneficial interests in such Notes in accordance with the Depositary Procedures).

(E)          Redemption Date. The Redemption Date for any Redemption will be a Business Day of the Company’s choosing that is no more than forty-five (45), nor less than twenty-five (25), Scheduled Trading Days after the Redemption Notice Date for such Redemption.

(F)          Redemption Price. The Redemption Price for any Note called for Redemption is an amount in cash equal to the principal amount of such Note plus accrued and unpaid interest on such Note to, but excluding, the Redemption Date for such Redemption; provided, however, that if such Redemption Date is after a Regular Record Date and on or before the next Interest Payment Date, then (i) the Holder of such Note at the Close of Business on such Regular Record Date will be entitled, notwithstanding such Redemption, to receive, on or, at the Company’s election, before such Interest Payment Date, the unpaid interest that would have accrued on such Note to, but excluding, such Interest Payment Date (assuming, solely for these purposes, that such Note remained outstanding through such Interest Payment Date, if such Redemption Date is before such Interest Payment Date); and (ii) the Redemption Price will not include accrued and unpaid interest on such Note to, but excluding, such Redemption Date. For the avoidance of doubt, if an Interest Payment Date is not a Business Day within the meaning of Section 2.05(C) and such Redemption Date occurs on the Business Day immediately after such Interest Payment Date, then (x) accrued and unpaid interest on Notes to, but excluding, such Interest Payment Date will be paid, in accordance with Section 2.05(C), on the next Business Day to Holders at of the Close of Business on the immediately preceding Regular Record Date; and (y) the Redemption Price will include interest on Notes to be redeemed from, and including, such Interest Payment Date.

(G)          Redemption Notice. To call any Notes for Redemption, the Company must (x) send to each Holder of such Notes, the Trustee and the Paying Agent a written notice of such Redemption (a “Redemption Notice”) and (y) substantially contemporaneously therewith, issue a press release through such national newswire service as the Company then uses (or publish the

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same through such other widely disseminated public medium as the Company then uses, including its website) containing the information set forth in the Redemption Notice.

Such Redemption Notice must state:

(i) that the Notes have been called for Redemption, briefly describing the Company’s Redemption right under this Indenture;

(ii) the Redemption Date for such Redemption;

(iii) the Redemption Price per $1,000 principal amount of Notes for such Redemption (and, if the Redemption Date is after a Regular Record Date and on or before the next Interest Payment Date, the amount, manner and timing of the interest payment payable pursuant to the proviso to Section 4.03(F));

(iv) the name and address of the Paying Agent and the Conversion Agent;

(v) that Notes called for Redemption may be converted at any time before the Close of Business on the second Business Day immediately before the Redemption Date (or, if the Company fails to pay the Redemption Price due on such Redemption Date in full, at any time until such time as the Company pays such Redemption Price in full);

(vi) the Conversion Rate in effect on the Redemption Notice Date for such Redemption and a description and quantification of any adjustments to the Conversion Rate that may result from such Redemption (including pursuant to Section 5.07);

(vii) the Settlement Method that will apply to all conversions of Notes with a Conversion Date that occurs on or after such Redemption Notice Date and before such Redemption Date; and

(viii) the CUSIP and ISIN numbers, if any, of the Notes.

On or before the Redemption Notice Date, the Company will send a copy of such Redemption Notice to the Trustee and the Paying Agent.

(H) Special Requirement for Notice of Tax Redemption. A Redemption Notice relating to a Tax Redemption must be sent pursuant to Section 4.03(G) no earlier than one hundred and eighty (180) calendar days before the earliest date on which the Company would have been required to make the related payment or withholding (assuming a payment in respect of the Notes were then due), and the obligation to pay Additional Amounts must be in effect as of the date the Company sends such Redemption Notice and must be expected to remain in effect at the time of the next payment or delivery in respect of the Notes.

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(I)            If less than all Notes then outstanding are called for Redemption, then:

(i) the Notes to be redeemed will be selected by the Company as follows: (1) in the case of Global Notes, in accordance with the Depositary Procedures; and (2) in the case of Physical Notes, pro rata, by lot or by such other method the Company considers fair and appropriate; and

(ii) if only a portion of a Note is subject to Redemption and such Note is converted in part, then the converted portion of such Note will be deemed to be from the portion of such Note that was subject to Redemption.

(J)           Payment of the Redemption Price. Without limiting the Company’s obligation to deposit the Redemption Price by the time proscribed by Section 3.01(B), the Company will cause the Redemption Price for a Note (or portion thereof) subject to Redemption to be paid to the Holder thereof on or before the later of (i) the applicable Redemption Date; and (ii) the date (x) such Note is delivered to the Paying Agent (in the case of a Physical Note) or (y) the Depositary Procedures relating to the repurchase, and the delivery to the Paying Agent, of such Holder’s beneficial interest in such Note to be repurchased are complied with (in the case of a Global Note). For the avoidance of doubt, interest payable pursuant to the proviso to Section 4.03(F) on any Note (or portion thereof) subject to Redemption must be paid pursuant to such proviso.

Article 5. CONVERSION

Section 5.01. Right to Convert.

(A)          Generally. Subject to the provisions of this Article 5, each Holder may, at its option, convert such Holder’s Notes into Conversion Consideration.

(B)          Conversions in Part. Subject to the terms of this Indenture, Notes may be converted in part, but only in Authorized Denominations. Provisions of this Article 5 applying to the conversion of a Note in whole will equally apply to conversions of a permitted portion of a Note.

(C)          When Notes May Be Converted.

(i) Generally. A Holder may convert its Notes at any time until the Close of Business on the second (2nd) Scheduled Trading Day immediately before the Maturity Date, unless such Notes have been previously redeemed or repurchased by the Company in accordance with this Indenture.

(ii) Limitations and Closed Periods. Notwithstanding anything to the contrary in this Indenture or the Notes:

(1)          Notes may be surrendered for conversion only after the Open of Business and before the Close of Business on a day that is a Business Day;

(2)          in no event may any Note be converted after the Close of Business

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on the second (2nd) Scheduled Trading Day immediately before the Maturity Date;

(3)          if the Company calls any Note for Redemption pursuant to Section 4.03, then the Holder of such Note may not convert such Note after the Close of Business on the second Business Day immediately before the applicable Redemption Date, except to the extent the Company fails to pay the Redemption Price for such Note in accordance with this Indenture; and

(4)          if a Fundamental Change Repurchase Notice is validly delivered pursuant to Section 4.02(F) with respect to any Note, then such Note may not be converted, except to the extent (a) such Note is not subject to such notice; (b) such notice is validly withdrawn in accordance with Section 4.02(F); or (c) the Company fails to pay the Fundamental Change Repurchase Price for such Note in accordance with this Indenture.

Section 5.02. Conversion Procedures.

(A) Generally.

(i) Global Notes. To convert a beneficial interest in a Global Note, the owner of such beneficial interest must (1) comply with the Depositary Procedures for converting such beneficial interest (at which time such conversion will become irrevocable); and (2) pay any amounts due pursuant to Section 5.02(D) or Section 5.02(E).

(ii) Physical Notes. To convert all or a portion of a Physical Note, the Holder of such Note must (1) complete, manually sign and deliver to the Conversion Agent the conversion notice attached to such Physical Note or a facsimile of such conversion notice; (2) deliver such Physical Note to the Conversion Agent (at which time such conversion will become irrevocable); (3) furnish any endorsements and transfer documents that the Company or the Conversion Agent may require; and (4) pay any amounts due pursuant to Section 5.02(D) or Section 5.02(E).

(B)          Effect of Converting a Note. At the Close of Business on the Conversion Date for a Note (or any portion thereof), such Note (or such portion thereof) will be deemed to cease to be outstanding (and, for the avoidance of doubt, no Person will be deemed to be a Holder of such Note (or such portion thereof) as of the Close of Business on such Conversion Date), except to the extent provided in Section 5.02(D).

(C)          Holder of Record of Conversion Shares. The Person in whose name any Ordinary Share is issuable upon conversion of any Note will be deemed to become the holder of record of such share as of the Close of Business on (i) the Conversion Date for such conversion, in the case of Physical Settlement; or (ii) the last VWAP Trading Day of the Observation Period for such conversion, in the case of Combination Settlement.

(D)          Interest Payable upon Conversion. If the Conversion Date of a Note is after a Regular Record Date and before the next Interest Payment Date, then (i) the Holder of such Note

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at the Close of Business on such Regular Record Date will be entitled, notwithstanding such conversion, to receive, on or, at the Company’s election, before such Interest Payment Date, the unpaid interest that would have accrued on such Note to, but excluding, such Interest Payment Date (assuming, solely for these purposes, that such Note remained outstanding through such Interest Payment Date).

(E)          Taxes and Duties. If a Holder converts a Note, the Company will pay any documentary, stamp or similar issue or transfer tax or duty due on the issue of any Ordinary Shares upon such conversion; provided, however, that if any tax or duty is due because such Holder requested such shares to be registered in a name other than such Holder’s name, then such Holder will pay such tax or duty and, until having received a sum sufficient to pay such tax or duty, the Conversion Agent may refuse to deliver any such shares to be issued in a name other than that of such Holder.

(F)          Conversion Agent to Notify Company of Conversions. If any Note is submitted for conversion to the Conversion Agent or the Conversion Agent receives any notice of conversion with respect to a Note, then the Conversion Agent will promptly notify the Company and the Trustee of such occurrence, together with any other information reasonably requested by the Company, and will cooperate with the Company to determine the Conversion Date for such Note.

Section 5.03. Settlement upon Conversion.

(A)          Upon the conversion of any Note, the Company will settle such conversion by paying or delivering, as applicable and as provided in this Article 5, either (x) Ordinary Shares, together, if applicable, with cash in lieu of fractional shares as provided in Section 5.03(B)(i)(1) (a “Physical Settlement”); (y) solely cash as provided in Section 5.03(B)(i)(2) (a “Cash Settlement”); or (z) a combination of cash and Ordinary Shares, together, if applicable, with cash in lieu of fractional shares as provided in Section 5.03(B)(i)(3) (a “Combination Settlement”).

The Company will have the right to elect the Settlement Method applicable to any conversion of a Note; provided, however, that:

(i) subject to clause (iii) below, all conversions of Notes with a Conversion Date that occurs on or after October 1, 2024 will be settled using the same Settlement Method, and the Company will send notice of such Settlement Method to Holders and the Conversion Agent no later than the Open of Business on October 1, 2024;

(ii) subject to clause (iii) below, if the Company elects a Settlement Method with respect to the conversion of any Note whose Conversion Date occurs before October 1, 2024, then the Company will send notice of such Settlement Method to the Holder of such Note and the Conversion Agent no later than the Close of Business on the Business Day immediately after such Conversion Date;

(iii) if any Notes are called for Redemption, then (1) the Company will

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specify, in the related Redemption Notice sent pursuant to Section 4.03(G), the Settlement Method that will apply to all conversions of Notes with a Conversion Date that occurs on or after the related Redemption Notice Date and before the related Redemption Date; and (2) if such Redemption Date occurs on or after October 1, 2024, then such Settlement Method must be the same Settlement Method that, pursuant to clause (i) above, applies to all conversions of Notes with a Conversion Date that occurs on or after October 1, 2024;

(iv) the Company will use the same Settlement Method for all conversions of Notes with a Conversion Date that occurs on the same day (and, for the avoidance of doubt, the Company will not be obligated to use the same Settlement Method with respect to conversions of Notes whose Conversion Dates occur on different days, except as provided in clause (i) or (iii) above);

(v) if the Company does not timely elect a Settlement Method with respect to the conversion of a Note, then the Company will be deemed to have elected the Default Settlement Method (and, for the avoidance of doubt, the failure to timely make such election will not constitute a Default or Event of Default);

(vi) if the Company timely elects Combination Settlement with respect to the conversion of a Note but does not timely notify the Holder of such Note of the applicable Specified Dollar Amount, then the Specified Dollar Amount for such conversion will be deemed to be $1,000 per $1,000 principal amount of Notes (and, for the avoidance of doubt, the failure to timely send such notification will not constitute a Default or Event of Default); and

(vii) the Settlement Method will be subject to Sections 5.09(A)(2).

(B) Conversion Consideration.

(i) Generally. Subject to Section 5.03(B)(ii) and Section 5.03(B)(iii), the type and amount of consideration (the “Conversion Consideration”) due in respect of each $1,000 principal amount of a Note to be converted will be as follows:

(1)          if Physical Settlement applies to such conversion, a number of Ordinary Shares equal to the Conversion Rate in effect on the Conversion Date for such conversion;

(2)          if Cash Settlement applies to such conversion, cash in an amount equal to the sum of the Daily Conversion Values for each VWAP Trading Day in the Observation Period for such conversion; or

(3)          if Combination Settlement applies to such conversion,

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consideration consisting of (a) a number of Ordinary Shares equal to the sum of the Daily Share Amounts for each VWAP Trading Day in the Observation Period for such conversion; and (b) an amount of cash equal to the sum of the Daily Cash Amounts for each VWAP Trading Day in such Observation Period.

(ii) Cash in Lieu of Fractional Shares. If Physical Settlement or Combination Settlement applies to the conversion of any Note and the number of Ordinary Shares deliverable pursuant to Section 5.03(B)(i) upon such conversion is not a whole number, then such number will be rounded down to the nearest whole number and the Company will deliver, in addition to the other consideration due upon such conversion, cash in lieu of the related fractional share in an amount equal to the product of (1) such fraction and (2) (x) the Daily VWAP on the Conversion Date for such conversion (or, if such Conversion Date is not a VWAP Trading Day, the immediately preceding VWAP Trading Day), in the case of Physical Settlement; or (y) the Daily VWAP on the last VWAP Trading Day of the Observation Period for such conversion, in the case of Combination Settlement.

(iii) Conversion of Multiple Notes by a Single Holder. If a Holder converts more than one (1) Note on a single Conversion Date, then the Conversion Consideration due in respect of such conversion will (in the case of any Global Note, to the extent permitted by, and practicable under, the Depositary Procedures) be computed based on the total principal amount of Notes converted on such Conversion Date by such Holder.

(iv) Notice of Calculation of Conversion Consideration. If Cash Settlement or Combination Settlement applies to the conversion of any Note, then the Company will determine the Conversion Consideration due thereupon promptly following the last VWAP Trading Day of the applicable Observation Period and will promptly thereafter send notice to the Trustee and the Conversion Agent of the same and the calculation thereof in reasonable detail. Neither the Trustee nor the Conversion Agent will have any duty to make any such determination.

(C)          Delivery of the Conversion Consideration. Except as set forth in Sections 5.05(A), 5.05(C) and 5.09, the Company will pay or deliver, as applicable, the Conversion Consideration due upon the conversion of any Note to the Holder as follows: (i) if Cash Settlement or Combination Settlement applies to such conversion, on or before the second (2nd) Business Day immediately after the last VWAP Trading Day of the Observation Period for such conversion; and (ii) if Physical Settlement applies to such conversion, on or before the second (2nd) Business Day immediately after the Conversion Date for such conversion.

(D)          Settlement of Accrued Interest Notwithstanding Conversion. If a Holder converts a Note, then the Company will pay accrued and unpaid interest, if any, to, but excluding, the applicable Conversion Date to such converting Holder, except as set forth in Section 5.02(D).

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Section 5.04. Reserve and Status of Ordinary Shares Issued upon Conversion.

(A)          Share Reserve. At all times when any Notes are outstanding, Parent will reserve, out of its authorized, unreserved and not outstanding Ordinary Shares, a number of Ordinary Shares sufficient to permit the conversion of all then-outstanding Notes, assuming (x) Physical Settlement will apply to such conversion; and (y) the Conversion Rate is increased by the maximum amount pursuant to which the Conversion Rate may be increased pursuant to Section 5.07.

(B)          Status of Conversion Shares; Listing. Each Conversion Share, if any, delivered upon conversion of any Note will be a newly issued or treasury share and will be duly and validly issued, fully paid, non-assessable, free from preemptive rights and free of any lien or adverse claim (except to the extent of any lien or adverse claim created by the action or inaction of the Holder of such Note or the Person to whom such Conversion Share will be delivered). If the Ordinary Shares are then listed on any securities exchange, or quoted on any inter-dealer quotation system, then the Company will use commercially reasonable efforts to cause each Conversion Share, when delivered upon conversion of any Note, to be admitted for listing on such exchange or quotation on such system.

Section 5.05. Adjustments to the Conversion Rate.

(A)          Events Requiring an Adjustment to the Conversion Rate. The Conversion Rate will be adjusted from time to time as follows:

(i) Share Dividends, Splits and Combinations. If Parent issues solely Ordinary Shares as a dividend or distribution on all or substantially all Ordinary Shares, or if Parent effects a share split or a share combination of the Ordinary Shares (in each case excluding an issuance solely pursuant to an Ordinary Share Change Event, as to which Section 5.09 will apply), then the Conversion Rate will be adjusted based on the following formula:

where:

CR₀= the Conversion Rate in effect immediately before the Open of Business on the Ex-Dividend Date for such dividend or distribution, or immediately before the Open of Business on the effective date of such share split or share combination, as applicable;

CR₁     = the Conversion Rate in effect immediately after the Open of Business on such Ex-Dividend Date or the Open of Business on such effective date, as applicable;

OS₀      = the number of Ordinary Shares outstanding immediately before the Open

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of Business on such Ex-Dividend Date or effective date, as applicable, without giving effect to such dividend, distribution, share split or share combination; and

OS₁     = the number of Ordinary Shares outstanding immediately after giving effect to such dividend, distribution, share split or share combination.

If any dividend, distribution, share split or share combination of the type described in this Section 5.05(A)(i) is declared or announced, but not so paid or made, then the Conversion Rate will be readjusted, effective as of the date the Parent Board determines not to pay such dividend or distribution or to effect such share split or share combination, to the Conversion Rate that would then be in effect had such dividend, distribution, share split or share combination not been declared or announced.

(ii) Rights, Options and Warrants. If Parent distributes, to all or substantially all holders of Ordinary Shares, rights, options or warrants (other than rights issued or otherwise distributed pursuant to a shareholder rights plan, as to which the provisions set forth in Sections 5.05(A)(iii)(1) and 5.05(E) will apply) entitling such holders, for a period of not more than sixty (60) calendar days after the record date of such distribution, to subscribe for or purchase Ordinary Shares at a price per share (translated, if necessary, to U.S. dollars at the Prevailing Exchange Rate on the Trading Day immediately before the date such distribution is announced) that is less than the average of the Last Reported Sale Prices per Ordinary Share for the ten (10) consecutive Trading Days ending on, and including, the Trading Day immediately before the date such distribution is announced, then the Conversion Rate will be increased based on the following formula:

where:

CR₀= the Conversion Rate in effect immediately before the Open of Business on the Ex-Dividend Date for such distribution;

CR₁= the Conversion Rate in effect immediately after the Open of Business on such Ex-Dividend Date;

OS     = the number of Ordinary Shares outstanding immediately before the Open of Business on such Ex-Dividend Date;

X       = the total number of Ordinary Shares issuable pursuant to such rights, options or warrants; and

Y       = a number of Ordinary Shares obtained by dividing (x) the aggregate price

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payable to exercise such rights, options or warrants by (y) the average of the Last Reported Sale Prices per Ordinary Share for the ten (10) consecutive Trading Days ending on, and including, the Trading Day immediately before the date such distribution is announced.

To the extent that Ordinary Shares are not delivered after the expiration of such rights, options or warrants (including as a result of such rights, options or warrants not being exercised), the Conversion Rate will be readjusted to the Conversion Rate that would then be in effect had the increase to the Conversion Rate for such distribution been made on the basis of delivery of only the number of Ordinary Shares actually delivered upon exercise of such rights, option or warrants. To the extent such rights, options or warrants are not so distributed, the Conversion Rate will be readjusted to the Conversion Rate that would then be in effect had the Ex-Dividend Date for the distribution of such rights, options or warrants not occurred.

For purposes of this Section 5.05(A)(ii), in determining whether any rights, options or warrants entitle holders of Ordinary Shares to subscribe for or purchase Ordinary Shares at a price per share that is less than the average of the Last Reported Sale Prices per Ordinary Share for the ten (10) consecutive Trading Days ending on, and including, the Trading Day immediately before the date the distribution of such rights, options or warrants is announced, and in determining the aggregate price payable to exercise such rights, options or warrants, there will be taken into account any consideration the Parent receives for such rights, options or warrants and any amount payable on exercise thereof, with the value of such consideration, if not cash, to be determined by the Parent Board.

(iii) Spin-Offs and Other Distributed Property.

(1)           Distributions Other than Spin-Offs. If Parent distributes shares of its Capital Stock, evidences of its indebtedness or other assets or property of the Company, or rights, options or warrants to acquire Capital Stock of Parent or other securities, to all or substantially all holders of the Ordinary Shares, excluding:

(v)          dividends, distributions, rights, options or warrants for which an adjustment to the Conversion Rate is required pursuant to Section 5.05(A)(i) or 5.05(A)(ii);

(w)         dividends or distributions paid exclusively in cash for which an adjustment to the Conversion Rate is required pursuant to Section 5.05(A)(iv);

(x)           rights issued or otherwise distributed pursuant to a shareholder rights plan, except to the extent provided in Section 5.05(E);

(y)          Spin-Offs for which an adjustment to the Conversion Rate is required pursuant to Section 5.05(A)(iii)(2); and

(z)           a distribution solely pursuant to an Ordinary Share Change

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Event, as to which Section 5.09 will apply,

then the Conversion Rate will be increased based on the following formula:

where:

CR₀= the Conversion Rate in effect immediately before the Open of Business on the Ex-Dividend Date for such distribution;

CR₁= the Conversion Rate in effect immediately after the Open of Business on such Ex-Dividend Date;

SP      = the average of the Last Reported Sale Prices per Ordinary Share for the ten (10) consecutive; Trading Days ending on, and including, the Trading Day immediately before such Ex-Dividend Date; and

FMV  = the fair market value (as determined by the Parent Board and expressed in U.S. dollars, translated, if necessary, at the Prevailing Exchange Rate on such Ex-Dividend Date), as of such Ex-Dividend Date, of the shares of Capital Stock, evidences of indebtedness, assets, property, rights, options or warrants distributed per Ordinary Share pursuant to such distribution;

provided, however, that if FMV is equal to or greater than SP, then, in lieu of the foregoing adjustment to the Conversion Rate, each Holder will receive, for each $1,000 principal amount of Notes held by such Holder on the record date for such distribution, at the same time and on the same terms as holders of Ordinary Shares, the amount and kind of shares of Capital Stock, evidences of indebtedness, assets, property, rights, options or warrants that such Holder would have received if such Holder had owned, on such record date, a number of Ordinary Shares equal to the Conversion Rate in effect on such record date.

To the extent such distribution is not so paid or made, the Conversion Rate will be readjusted to the Conversion Rate that would then be in effect had the adjustment been made on the basis of only the distribution, if any, actually made or paid.

(2)            Spin-Offs. If Parent distributes or dividends shares of Capital Stock of any class or series, or similar equity interest, of or relating to an Affiliate, a Subsidiary or other business unit of Parent to all or substantially all holders of the Ordinary Shares (other than solely pursuant to an Ordinary Share Change Event, as to which Section 5.09 will apply), and such Capital Stock or equity interest is listed or quoted (or will be listed or quoted upon the consummation of the transaction) on a U.S.

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national securities exchange or a reasonably comparable non-U.S. equivalent (as determined by the Parent Board) (a “Spin-Off), then the Conversion Rate will be increased based on the following formula:

where:

CR₀= the Conversion Rate in effect immediately before the Open of Business on the Ex-Dividend Date for such Spin-Off;

CR₁    = the Conversion Rate in effect immediately after the Open of Business on such Ex-Dividend Date;

FMV  = the product of (x) the average of the Last Reported Sale Prices per-share or unit of the Capital Stock or equity interests distributed in such Spin-Off over the ten (10) consecutive Trading Day period (the “Spin-Off Valuation Period”) beginning on, and including, such Ex-Dividend Date (such average to be determined as if references to Ordinary Shares in the definitions of Last Reported Sale Price, Trading Day and Market Disruption Event were instead references to such Capital Stock or equity interests); and (y) the number of shares or units of such Capital Stock or equity interests distributed per Ordinary Share in such Spin-Off; and

SP      = the average of the Last Reported Sale Prices per Ordinary Share for each Trading Day in the Spin-Off Valuation Period.

The adjustment to the Conversion Rate pursuant to this Section 5.05(A)(iii)(2) will be calculated as of the Close of Business on the last Trading Day of the Spin-Off Valuation Period but will be given effect immediately after the Open of Business on the Ex-Dividend Date for the Spin-Off, with retroactive effect. If a Note is converted and the Conversion Date (in the case of Physical Settlement) or any VWAP Trading Day of the applicable Observation Period (in the case of Cash Settlement or Combination Settlement) occurs during the Spin-Off Valuation Period, then, notwithstanding anything to the contrary in this Indenture or the Notes, the Company will, if necessary, delay the settlement of such conversion until the second (2nd) Business Day after the last day of the Spin-Off Valuation Period.

To the extent any dividend or distribution of the type set forth in this Section 5.05(A)(iii)(2) is declared but not made or paid, the Conversion Rate will be readjusted, effective as of the date the Parent Board determines not to make such distribution, to the Conversion Rate that would then be in effect had the adjustment been made on the basis of only the dividend or distribution, if any,

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actually made or paid.

(iv) Cash Dividends or Distributions. If any cash dividend or distribution is made to all or substantially all holders of Ordinary Shares, then the Conversion Rate will be increased based on the following formula:

where:

CR₀= the Conversion Rate in effect immediately before the Open of Business on the Ex-Dividend Date for such dividend or distribution;

CR₁   = the Conversion Rate in effect immediately after the Open of Business on such Ex-Dividend Date;

SP     = the Last Reported Sale Price per Ordinary Share on the Trading Day immediately before such Ex-Dividend Date; and

D       = the cash amount distributed per Ordinary Share in such dividend or distribution (translated, if necessary, to U.S. dollars at the Prevailing Exchange Rate on the Trading Day immediately before such Ex-Dividend Date);

provided, however, that if D is equal to or greater than SP, then, in lieu of the foregoing adjustment to the Conversion Rate, each Holder will receive, for each $1,000 principal amount of Notes held by such Holder on the record date for such dividend or distribution, at the same time and on the same terms as holders of Ordinary Shares, the amount of cash that such Holder would have received if such Holder had owned, on such record date, a number of Ordinary Shares equal to the Conversion Rate in effect on such record date.

To the extent such dividend or distribution is declared but not made or paid, the Conversion Rate will be readjusted to the Conversion Rate that would then be in effect had the adjustment been made on the basis of only the dividend or distribution, if any, actually made or paid.

(v) Tender Offers or Exchange Offers. If Parent or any of its Subsidiaries makes a payment in respect of a tender offer or exchange offer for Ordinary Shares (other than solely pursuant to an odd-lot tender offer pursuant to Rule 13e-4(h)(5) under the Exchange Act), and the value (expressed in U.S. dollars, translated, if necessary, at the Prevailing Exchange Rate on the Expiration Date, and determined as of the Expiration Time by the Parent Board) of the cash and other consideration paid per Ordinary Share in such tender or exchange offer exceeds the Last Reported Sale Price per

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Ordinary Share on the Trading Day immediately after the last date (the “Expiration Date”) on which tenders or exchanges may be made pursuant to such tender or exchange offer (as it may be amended), then the Conversion Rate will be increased based on the following formula:

where:

CR₀= the Conversion Rate in effect immediately before the time (the “Expiration Time”) such tender or exchange offer expires;

CR₁= the Conversion Rate in effect immediately after the Expiration Time;

AC     = the aggregate value (determined as of the Expiration Time by the Parent Board) of all cash and other consideration paid for Ordinary Shares purchased in such tender or exchange offer;

OS₀    = the number of Ordinary Shares outstanding immediately before the Expiration Time (before giving effect to the purchase of all Ordinary Shares accepted for purchase or exchange in such tender or exchange offer);

OS₁    = the number of Ordinary Shares outstanding immediately after the Expiration Time (after giving effect to the purchase or exchange of all Ordinary Shares accepted for purchase or exchange in such tender or exchange offer); and

SP     = the average of the Last Reported Sale Prices per Ordinary Share over the ten (10) consecutive Trading Day period (the “Tender/Exchange Offer Valuation; Period”) beginning on, and including, the Trading Day immediately after the Expiration Date;

provided, however, that the Conversion Rate will in no event be adjusted down pursuant to this Section 5.05(A)(v), except to the extent provided in the immediately following paragraph. The adjustment to the Conversion Rate pursuant to this Section 5.05(A)(v) will be calculated as of the Close of Business on the last Trading Day of the Tender/Exchange Offer Valuation Period but will be given effect immediately after the Expiration Time, with retroactive effect. If a Note is converted and the Conversion Date (in the case of Physical Settlement) or any VWAP Trading Day of the applicable Observation Period (in the case of Cash Settlement or Combination Settlement) occurs on the Expiration Date or during the Tender/Exchange Offer Valuation Period, then, notwithstanding anything to the contrary in this Indenture or the Notes, the Company will, if necessary, delay the settlement of such conversion until the second (2nd) Business Day after the last day of the Tender/Exchange Offer Valuation Period.

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To the extent such tender or exchange offer is announced but not consummated (including as a result of Parent being precluded from consummating such tender or exchange offer under applicable law), or any purchases or exchanges of Ordinary Shares in such tender or exchange offer are rescinded, the Conversion Rate will be readjusted to the Conversion Rate that would then be in effect had the adjustment been made on the basis of only the purchases or exchanges of Ordinary Shares, if any, actually made, and not rescinded, in such tender or exchange offer.

(B)          No Adjustments in Certain Cases.

(i) Where Holders Participate in the Transaction or Event Without Conversion. Notwithstanding anything to the contrary in Section 5.05(A), the Company will not be obligated to adjust the Conversion Rate on account of a transaction or other event otherwise requiring an adjustment pursuant to Section 5.05(A) (other than a share split or combination of the type set forth in Section 5.05(A)(i) or a tender or exchange Offer of the type set forth in Section 5.05(A)(v)) if each Holder participates, at the same time and on the same terms as holders of Ordinary Shares, and solely by virtue of being a Holder of Notes, in such transaction or event without having to convert such Holder’s Notes and as if such Holder held a number of Ordinary Shares equal to the product of (i) the Conversion Rate in effect on the related record date; and (ii) the aggregate principal amount (expressed in thousands) of Notes held by such Holder on such date.

(ii) Certain Events. The Company will not be required to adjust the Conversion Rate except as provided in Section 5.05 or Section 5.07. Without limiting the foregoing, the Company will not be obligated to adjust the Conversion Rate on account of:

(1)          except as otherwise provided in Section 5.05, the sale of Ordinary Shares by Parent, the Company or otherwise for a purchase price that is less than the market price per Ordinary Share or less than the Conversion Price;

(2)          the issuance of any Ordinary Shares by Parent, the Company or otherwise pursuant to any present or future plan providing for the reinvestment of dividends or interest payable on Parent’s securities and the investment of additional optional amounts in Ordinary Shares under any such plan;

(3)          the issuance of any Ordinary Shares or options or rights to purchase Ordinary Shares by Parent, the Company or otherwise pursuant to any present or future employee, director or consultant benefit plan or program of, or assumed by, Parent or any of its Subsidiaries;

(4)          the issuance of any Ordinary Shares by Parent, the Company or otherwise pursuant to any option, warrant, right, contingent value right or exercisable, convertible or exchangeable security of Parent or its Subsidiaries outstanding as of the

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Issue Date (other than an adjustment pursuant to Section 5.05(A)(iii)(1) in connection with the separation of rights under Parent’s shareholder rights plan existing as of the Issue Date);

(5)          solely a change in the par value of the Ordinary Shares;

(6)          accrued and unpaid interest on the Notes; or

(7)          the transactions contemplated by the Plan (including the establishment of a holding company above the Parent).

(C)          Adjustments Not Yet Effective. Notwithstanding anything to the contrary in this Indenture or the Notes, if:

(i) a Note is to be converted pursuant to Physical Settlement or Combination Settlement;

(ii) the record date, effective date or Expiration Time for any event that requires an adjustment to the Conversion Rate pursuant to Section 5.05(A) has occurred on or before the Conversion Date for such conversion (in the case of Physical Settlement) or on or before any VWAP Trading Day in the Observation Period for such conversion (in the case of Combination Settlement), but an adjustment to the Conversion Rate for such event has not yet become effective as of such Conversion Date or VWAP Trading Day, as applicable;

(iii) the Conversion Consideration due upon such conversion (in the case of Physical Settlement) or due in respect of such VWAP Trading Day (in the case of Combination Settlement) includes any whole Ordinary Shares; and

(iv) such shares are not entitled to participate in such event (because they were not held on the related record date or otherwise),

then, solely for purposes of such conversion, the Company will, without duplication, give effect to such adjustment on such Conversion Date (in the case of Physical Settlement) or such VWAP Trading Day (in the case of Combination Settlement). In such case, if the date on which the Company is otherwise required to deliver the consideration due upon such conversion is before the first date on which the amount of such adjustment can be determined, then the Company will delay the settlement of such conversion until the second (2nd) Business Day after such first date.

(D)          Conversion Rate Adjustments where Converting Holders Participate in the Relevant Transaction or Event. Notwithstanding anything to the contrary in this Indenture or the Notes, if:

(i) a Conversion Rate adjustment for any dividend or distribution becomes effective on any Ex-Dividend Date pursuant to Section 5.05(A);

(ii) a Note is to be converted pursuant to Physical Settlement or

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Combination Settlement;

(iii) the Conversion Date for such conversion (in the case of Physical Settlement) or any VWAP Trading Day in the Observation Period for such conversion (in the case of Combination Settlement) occurs on or after such Ex-Dividend Date and on or before the related record date;

(iv) the Conversion Consideration due upon such conversion (in the case of Physical Settlement) or due with respect to such VWAP Trading Day (in the case of Combination Settlement) includes any whole Ordinary Shares based on a Conversion Rate that is adjusted for such dividend or distribution; and

(v) such shares would be entitled to participate in such dividend or distribution (including pursuant to Section 5.02(C)),

then (x) in the case of Physical Settlement, such Conversion Rate adjustment will not be given effect for such conversion, and the Ordinary Shares issuable upon such conversion based on such unadjusted Conversion Rate will be entitled to participate in such dividend or distribution; and (y) in the case of Combination Settlement, the Conversion Rate adjustment relating to such Ex-Dividend Date will be made for such conversion in respect of such VWAP Trading Day, but the Ordinary Shares issuable with respect to such VWAP Trading Day based on such adjusted Conversion Rate will not be entitled to participate in such dividend or distribution.

(E)          Shareholder Rights Plans. If any Ordinary Shares are to be issued upon conversion of any Note and, at the time of such conversion, the Company has in effect any shareholder rights plan, then the Holder of such Note will be entitled to receive, in addition to, and concurrently with the delivery of, the Conversion Consideration otherwise payable under this Indenture upon such conversion, the rights set forth in such shareholder rights plan, unless such rights have separated from the Ordinary Shares at such time, in which case, and only in such case, the Conversion Rate will be adjusted pursuant to Section 5.05(A)(iii)(1) on account of such separation as if, at the time of such separation, the Company had made a distribution of the type referred to in such Section to all holders of the Ordinary Shares, subject to readjustment in accordance with such Section if such rights expire, terminate or are redeemed.

(F)          Limitation on Effecting Transactions Resulting in Certain Adjustments. The Company will not engage in or be a party to any transaction or event that would require the Conversion Rate to be adjusted pursuant to Section 5.05(A) or Section 5.07 to an amount that would result in the Conversion Price per Ordinary Share being less than the par value per Ordinary Share.

(G)          Equitable Adjustments to Prices. Except as otherwise set forth in Section 5.05(C) or Section 5.05(D), whenever any provision of this Indenture requires the Company to calculate the average of the Last Reported Sale Prices, or any function thereof, over a period of multiple days (including to calculate the Share Price or an adjustment to the Conversion Rate), or to calculate Daily VWAPs over an Observation Period, the Company will make proportionate adjustments, if any, to such calculations to account for any adjustment to the Conversion Rate

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pursuant to Section 5.05(A)(i) that becomes effective, or any event requiring such an adjustment to the Conversion Rate where the Ex-Dividend Date or effective date, as applicable, of such event occurs, at any time during such period or Observation Period, as applicable.

(H)          Calculation of Number of Outstanding Ordinary Shares. For purposes of Section 5.05(A), the number of Ordinary Shares outstanding at any time will (i) include shares issuable in respect of scrip certificates issued in lieu of fractions of Ordinary Shares; (ii) include shares issuable pursuant to the zero cost warrants issuable pursuant to the Plan and (iii) exclude Ordinary Shares held in the Company’s treasury (unless the Company pays any dividend or makes any distribution on Ordinary Shares held in its treasury).

(I)            Calculations. All calculations with respect to the Conversion Rate and adjustments thereto will be made to the nearest 1/10,000th of an Ordinary Share (with 5/100,000ths rounded upward).

(J)           Notice of Conversion Rate Adjustments. Upon the effectiveness of any adjustment to the Conversion Rate pursuant to Section 5.05(A), the Company will promptly send notice to the Holders, the Trustee and the Conversion Agent containing (i) a brief description of the transaction or other event on account of which such adjustment was made; (ii) the Conversion Rate in effect immediately after such adjustment; and (iii) the effective time of such adjustment.

Section 5.06. Voluntary Adjustments.

(A)          Generally. To the extent permitted by law and applicable stock exchange rules, the Company, from time to time, may (but is not required to) increase the Conversion Rate by any amount if (i) the Board of Directors determines that such increase is either (x) in the best interest of the Company; or (y) advisable to avoid or diminish any income tax imposed on holders of Ordinary Shares or rights to purchase Ordinary Shares as a result of any dividend or distribution of Ordinary Shares (or rights to acquire Ordinary Shares) or any similar event; (ii) such increase is in effect for a period of at least twenty (20) Business Days; and (iii) such increase is irrevocable during such period.

(B)          Notice of Voluntary Increases. If the Board of Directors determines to increase the Conversion Rate pursuant to Section 5.06(A), then, no later than the first Business Day of the related twenty (20) Business Day period referred to Section 5.06(A), the Company will send notice to each Holder of such increase, the amount thereof and the period during which such increase will be in effect.

Section 5.07. Adjustments to the Conversion Rate in Connection with a Make-Whole Fundamental Change.

(A)          Generally. If a Make-Whole Fundamental Change occurs and the Conversion Date for the conversion of a Note occurs during the related Make-Whole Fundamental Change Conversion Period, then, subject to this Section 5.07, the Conversion Rate applicable to such conversion will be increased by a number of shares (the “Additional Shares”) set forth in the table below corresponding (after interpolation as provided in, and subject to, the provisions below) to the Make-Whole Fundamental Change Effective Date and the Share Price of such

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Make-Whole Fundamental Change:

	Share Price
Make-Whole  Fundamental Change  Effective Date	$2.15	$2.25	$2.59	$3.00	$3.50	$3.88	$4.50	$6.00	$7.50	$10.00
10/1/19	121.4018	112.2628	86.4189	64.0178	45.5830	35.6131	23.9672	8.9946	2.6633	0.0000
4/1/20	122.8119	113.4598	87.0650	64.2588	45.5751	35.5048	23.7883	8.8226	2.5589	0.0000
4/1/21	124.4477	114.6413	87.1003	63.5532	44.4663	34.3045	22.6059	7.9912	2.0999	0.0000
4/1/22	123.9865	113.6540	84.9064	60.6895	41.4452	31.3841	20.0418	6.4074	1.3159	0.0000
4/1/23	119.4853	108.5144	78.4475	53.8290	34.9588	25.4501	15.1618	3.7706	0.3000	0.0000
4/1/24	106.7106	94.7416	62.8992	38.5379	21.5864	13.9042	6.5287	0.3851	0.0000	0.0000
4/1/25	77.3517	57.6944	0.0000	0.0000	0.0000	0.0000	0.0000	0.0000	0.0000	0.0000

If such Make-Whole Fundamental Change Effective Date or Share Price is not set forth in the table above, then:

(i) if such Share Price is between two Share Prices in the table above or the Make-Whole Fundamental Change Effective Date is between two dates in the table above, then the number of Additional Shares will be determined by a straight-line interpolation between the numbers of Additional Shares set forth for the higher and lower Share Prices in the table and the earlier and later dates in the table above, as applicable, based on a 365- or 366-day year, as applicable; and

(ii) if the Share Price is greater than [***] (subject to adjustment in the same manner as the Share Prices set forth in the column headings of the table above are adjusted pursuant to Section 5.07(B)), or less than [***] (subject to adjustment, in the same manner), per share, then no Additional Shares will be added to the Conversion Rate.

Notwithstanding anything to the contrary in this Indenture or the Notes, in no event will the Conversion Rate be increased to an amount that exceeds [***] Ordinary Shares per $1,000 principal amount of Notes, which amount is subject to adjustment in the same manner as, and at the same time and for the same events for which, the Conversion Rate is required to be adjusted pursuant to Section 5.05(A).

For the avoidance of doubt, (x) the sending of a Redemption Notice will constitute a Make-Whole Fundamental Change only with respect to the Notes called for Redemption pursuant to such Redemption Notice, and not with respect to any other Notes; and (y) the Conversion Rate applicable to the Notes not so called for Redemption will not be subject to increase pursuant to this Section 5.07 on account of such Redemption Notice.

The Share Prices in the table are expressed in U.S. dollars and, if expressed in a different currency as of any relevant Make-Whole Fundamental Change Effective Date, will be translated to U.S. dollars at the Prevailing Exchange Rate on such Make-Whole Fundamental Change Effective Date.

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(B)          Adjustment of Share Prices and Additional Shares. The Share Prices in the first row (i.e., the column headers) of the table set forth in Section 5.07(A) will be adjusted in the same manner as, and at the same time and for the same events for which, the Conversion Price is adjusted as a result of the operation of Section 5.05(A). The numbers of Additional Shares in the table set forth in Section 5.07(A) will be adjusted in the same manner as, and at the same time and for the same events for which, the Conversion Rate is adjusted pursuant to Section 5.07(A).

(C)          Notice of the Occurrence of a Make-Whole Fundamental Change. If a Make-Whole Fundamental Change occurs, then, promptly and in no event later than the Business Day immediately after the Make-Whole Fundamental Change Effective Date of such Make-Whole Fundamental Change, the Company will notify the Holders of the occurrence of such Make-Whole Fundamental Change and of such Make-Whole Fundamental Change Effective Date, briefly stating the circumstances under which the Conversion Rate will be increased pursuant to this Section 5.07 in connection with such Make-Whole Fundamental Change.

(D)          Settlement of Cash Make-Whole Fundamental Changes. For the avoidance of doubt, if holders of Ordinary Shares receive solely cash in a Make-Whole Fundamental Change, then, pursuant to Section 5.09, conversions of Notes will thereafter be settled no later than the second (2nd) Business Day after the relevant Conversion Date.

Section 5.08. Restriction on Conversions.

(A)          Notwithstanding anything to the contrary in this Indenture or the Notes, no Ordinary Shares will be issued upon conversion of any Note, no Note will be convertible by the Holder thereof, and the Company will not effect any conversion of any Note, in each case to the extent, and only to the extent, that such issuance, convertibility or conversion would result in such Holder or a “person” or “group” (within the meaning of Section 13(d)(3) of the Exchange Act) beneficially owning in excess of 9.9% of the then-outstanding Ordinary Shares. For these purposes, beneficial ownership and calculations of percentage ownership will be determined in accordance with Rule 13d-3 under the Exchange Act. For the avoidance of doubt, the limitations on the convertibility of any Note pursuant to this Section 5.08(A) will not, in themselves, cause such Note to cease to be outstanding (and interest will continue to accrue on any portion of a Note that has been tendered for conversion and whose convertibility is suspended pursuant to this Section 5.08(A)), and such limitations will cease to apply if and when such Note’s convertibility and conversion will not violate this Section 5.08(A). Each Holder, by notice to the Company, may elect in connection with any conversion of Notes that the provisions of this Section 5.08(A) not apply to such Holder; provided, however, upon delivery of such a notice to the Company, the provisions of this Section 5.08(A) will continue to apply to such conversion until the sixty-first (61st) calendar day following such delivery. For the avoidance of doubt, nothing in this Section 5.08(A) will affect the Company’s ability to elect any Settlement Method in accordance with this Indenture. Except to the extent the Company has received a notice as set forth in the preceding sentence, the Company and Trustee may conclusively rely on a Holder’s completion of the applicable procedures set forth in Section 5.02(A) as evidence that such conversion will not violate this Section 5.08(A).

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Section 5.09. Effect of Ordinary Share Change Event.

(A) Generally. If there occurs any:

(i) recapitalization, reclassification or change of the Ordinary Shares (other than (x) changes solely resulting from a subdivision or combination of the Ordinary Shares, (y) a change only in par value or from par value to no par value or no par value to par value and (z) share splits and share combinations that do not involve the issuance of any other series or class of securities);

(ii) consolidation, merger, combination or binding or statutory share exchange involving the Company or the Parent; or

(iii) sale, lease or other transfer of all or substantially all of the assets of the Parent and its Subsidiaries, taken as a whole, to any Person,

and, as a result of which, the Ordinary Shares are converted into, or are exchanged for, or represent solely the right to receive, other securities, cash or other property, or any combination of the foregoing (such an event, an “Ordinary Share Change Event,” and such other securities, cash or property, the “Reference Property,” and the amount and kind of Reference Property that a holder of one (1) Ordinary Share would be entitled to receive on account of such Ordinary Share Change Event (without giving effect to any arrangement not to issue or deliver a fractional portion of any security or property), a “Reference Property Unit”), then, notwithstanding anything to the contrary in this Indenture or the Notes,

(1)          from and after the effective time of such Ordinary Share Change Event, (I) the Conversion Consideration due upon conversion of any Note will be determined in the same manner as if each reference to any number of Ordinary Shares in this Article 5 (or in any related definitions) were instead a reference to the same number of Reference Property Units; (II) for purposes of Section 4.03, each reference to any number of Ordinary Shares in such Section (or in any related definitions) will instead be deemed to be a reference to the same number of Reference Property Units; and (III) for purposes of the definition of “Fundamental Change” and “Make-Whole Fundamental Change,” the terms “Ordinary Shares” and “common equity” will be deemed to mean the common equity (including depositary receipts representing common equity), if any, forming part of such Reference Property;

(2)          if such Reference Property Unit consists entirely of cash, then the Company will be deemed to elect Physical Settlement in respect of all conversions whose Conversion Date occurs on or after the effective date of such Ordinary Share Change Event and will pay the cash due upon such conversions no later than the second (2nd) Business Day after the relevant Conversion Date; and

(3)          for these purposes, the Daily VWAP or Last Reported Sale Price of any Reference Property Unit or portion thereof that does not consist of a class of securities will be the fair value of such Reference Property Unit or portion thereof, as applicable, determined in good faith by the Company (or, in the case of cash denominated in U.S.

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dollars, the face amount thereof).

If the Reference Property consists of more than a single type of consideration to be determined based in part upon any form of shareholder election, then the composition of the Reference Property Unit will be deemed to be the weighted average of the types and amounts of consideration actually received, per Ordinary Share, by the holders of Ordinary Shares. The Company will notify Holders of such weighted average as soon as practicable after such determination is made.

At or before the effective time of such Ordinary Share Change Event, the Company and the resulting, surviving or transferee Person (if not the Company) of such Ordinary Share Change Event (the “Successor Person”) will execute and deliver to the Trustee a supplemental indenture pursuant to Section 8.01(F), which supplemental indenture will (x) provide for subsequent conversions of Notes in the manner set forth in this Section 5.09; (y) provide for subsequent adjustments to the Conversion Rate pursuant to Section 5.07(A) in a manner consistent with this Section 5.09; and (z) contain such other provisions as the Company reasonably determines are appropriate to preserve the economic interests of the Holders and to give effect to the provisions of this Section 5.09(A). If the Reference Property includes shares of stock or other securities or assets of a Person other than the Successor Person, then such other Person will also execute such supplemental indenture and such supplemental indenture will contain such additional provisions the Company reasonably determines are appropriate to preserve the economic interests of the Holders.

(B)          Notice of Ordinary Share Change Events. The Company will provide notice of each Ordinary Share Change Event to Holders no later than the effective date of such Ordinary Share Change Event.

(C)          Compliance Covenant. The Company will not become a party to any Ordinary Share Change Event unless its terms are consistent with this Section 5.09.

Section 5.10. Responsibility of Trustee.

(A)          The Trustee and any other Conversion Agent shall not at any time be under any duty or responsibility to any Holder to determine or calculate the Conversion Rate (or any adjustment thereto) or whether any facts exist that may require any adjustment (including any increase) of the Conversion Rate, or with respect to the nature or extent or calculation of any such adjustment when made, or with respect to the method employed, or herein or in any supplemental indenture provided to be employed, in making the same. The Trustee and any other Conversion Agent shall not be accountable with respect to the validity or value (or the kind or amount) of any Conversion Consideration; and the Trustee and any other Conversion Agent make no representations with respect thereto. Neither the Trustee nor any Conversion Agent shall be responsible or liable for any failure of the Company to make any cash payment to issue, transfer or deliver any Conversion Consideration upon the surrender of any Note for the purpose of conversion or to comply with any of the duties, responsibilities or covenants of the Company contained in this Article.

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Article 6. SUCCESSORS

Section 6.01. When the Company May Merge, Etc.

(A)          Generally. Neither the Parent nor the Company will consolidate with or merge with or into, or (directly, or indirectly through one or more of its Subsidiaries) sell, lease or otherwise transfer, in one transaction or a series of transactions, all or substantially all of the assets of the Parent or the Company and their respective Subsidiaries, taken as a whole, to another Person (a “Business Combination Event”), unless:

(i) the resulting, surviving or transferee Person either (x) is the Company or the Parent, as applicable, or (y) if not the Company or the Parent, as applicable, is a corporation (or equivalent entity) (the “Successor Corporation”) duly organized and existing under the laws of the United States of America, any State thereof or the District of Columbia, or, with respect to the Parent, the United Kingdom or Ireland that expressly assumes (by executing and delivering to the Trustee, at or before the effective time of such Business Combination Event, a supplemental indenture pursuant to Section 8.01(E)) all of the Company’s or the Parent’s obligations, as applicable, under this Indenture and the Notes (including, for the avoidance of doubt, the obligation to pay Additional Amounts pursuant to Section 3.04);and

(ii) immediately after giving effect to such Business Combination Event, no Default or Event of Default will have occurred and be continuing.

(B)          Delivery of Officer’s Certificate and Opinion of Counsel to the Trustee. Before the effective time of any Business Combination Event, the Company will deliver to the Trustee (x) an Officer’s Certificate stating that (i) such Business Combination Event (and, if applicable, the related supplemental indenture) complies with Section 6.01(A); and (ii) all conditions precedent to such Business Combination Event provided in this Indenture have been satisfied and (y) an Opinion of Counsel stating that all conditions precedent to such Business Combination Event provided in this Indenture have been satisfied.

Section 6.02. Successor Corporation Substituted.

At the effective time of any Business Combination Event that complies with Section 6.01, the Successor Corporation (if not the Company or the Parent, as applicable) will succeed to, and may exercise every right and power of, the Company or the Parent, as applicable, under this Indenture and the Notes with the same effect as if such Successor Corporation had been named as the Company or Parent, as applicable, in this Indenture and the Notes, and, except in the case of a lease, the predecessor Company or Parent, as applicable, will be discharged from its obligations under this Indenture and the Notes.

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Article 7. DEFAULTS AND REMEDIES

Section 7.01. Events of Default.

(A)          Definition of Events of Default. “Event of Default” means the occurrence of any of the following:

(i) a default in the payment when due (whether at maturity, upon Redemption or Repurchase Upon Fundamental Change or otherwise) of the principal of, or the Redemption Price or Fundamental Change Repurchase Price for, any Note;

(ii) a default for thirty (30) days in the payment when due of interest on any Note;

(iii) the Company’s failure to deliver, when required by this Indenture, a Fundamental Change Notice, or a notice pursuant to Section 4.02(E);

(iv) a default in the Company’s obligation to convert a Note in accordance with Article 5 upon the exercise of the conversion right with respect thereto, if such default is not cured within five (5) Business Days after its occurrence;

(v) a default in the Company’s or Parent’s obligations under Article 6;

(vi) a default in any of the Company’s obligations or agreements, or in any Guarantor’s obligations or agreements, under this Indenture or the Notes (other than a default set forth in clause (i), (ii), (iii), (iv) or (v) of this Section 7.01(A)) where such default is not cured or waived within sixty (60) days after notice to the Company and Parent by the Trustee, or to the Company, the Parent and the Trustee by Holders of at least twenty five percent (25%) of the aggregate principal amount of Notes then outstanding, which notice must specify such default, demand that it be remedied and state that such notice is a “Notice of Default”;

(vii) a default by the Parent, the Company or any of the Parent’s Significant Subsidiaries with respect to any one or more mortgages, agreements or other instruments under which there is outstanding, or by which there is secured or evidenced, any indebtedness for money borrowed of at least twelve million dollars ($12,000,000) (or its foreign currency equivalent) in the aggregate of the Parent, the Company or any of the Parent’s Significant Subsidiaries, whether such indebtedness exists as of the Issue Date or is thereafter created, where such default:

(1)          constitutes a failure to pay the principal of such indebtedness when due and payable at its stated maturity, upon required repurchase, upon declaration of acceleration or otherwise, in each case after the expiration of any applicable grace period; or

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(2)          results in such indebtedness becoming or being declared due and payable before its stated maturity,

in each case where such default is not cured or waived within sixty (60) days after notice to the Company by the Trustee or to the Company and the Trustee by Holders of at least twenty five percent (25%) of the aggregate principal amount of Notes then outstanding;

(viii) one or more final judgments being rendered against the Parent, the Company or any of the Parent’s Significant Subsidiaries for the payment of at least twelve million dollars ($12,000,000) (or its foreign currency equivalent) in the aggregate (excluding any amounts covered by insurance), where such judgment is not discharged or stayed within sixty (60) days after (i) the date on which the right to appeal the same has expired, if no such appeal has commenced; or (ii) the date on which all rights to appeal have been extinguished;

(ix) the Company, the Parent or any of the Parent’s Significant Subsidiaries, pursuant to or within the meaning of any Bankruptcy Law, either:

(1)          commences a voluntary case or proceeding;

(2)          consents to the entry of an order for relief against it in an involuntary case or proceeding;

(3)          consents to the appointment of a custodian of it or for any substantial part of its properly;

(4)          makes a general assignment for the benefit of its creditors;

(5)          takes any comparable action under any foreign Bankruptcy Law; or

(6)          generally is not paying its debts as they become due; or

(x) a court of competent jurisdiction enters an order or decree under any Bankruptcy Law that either:

(1)          is for relief against the Company, the Parent or any of the Parent’s Significant Subsidiaries in an involuntary case or proceeding;

(2)          appoints a custodian of the Company, the Parent or any of the Parent’s Significant Subsidiaries, or for any substantial part of the property of the Company, the Parent or any of the Parent’s Significant Subsidiaries;

(3)          orders the winding up or liquidation of the Company, the Parent or any of the Parent’s Significant Subsidiaries; or

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(4)          grants any similar relief under any foreign Bankruptcy Law,

and, in each case under this Section 7.01(A)(x), such order or decree remains unstayed and in effect for at least sixty (60) days. For the avoidance of doubt, no transactions or actions taken pursuant to and in accordance with the Plan shall constitute an Event of Default under clause (ix) or (x) of this Section 7.01(A)).

(B)          Cause Irrelevant. Each of the events set forth in Section 7.01(A) will constitute an Event of Default regardless of the cause thereof or whether voluntary or involuntary or effected by operation of law or pursuant to any judgment, decree or order of any court or any order, rule or regulation of any administrative or governmental body.

Section 7.02. Acceleration.

(A)          Automatic Acceleration in Certain Circumstances. If an Event of Default set forth in Section 7.01(A)(ix) or 7.01(A)(x) occurs with respect to the Company or any Guarantor (and not solely with respect to a Significant Subsidiary of the Company or any Guarantor), then the principal amount of, and all accrued and unpaid interest on, all of the Notes then outstanding will immediately become due and payable without any further action or notice by any Person.

(B)          Optional Acceleration. Subject to Section 7.03, if an Event of Default (other than an Event of Default to which Section 7.02(A) applies) occurs and is continuing, then the Trustee, by notice to the Company, or Holders of at least twenty five percent (25%) of the aggregate principal amount of Notes then outstanding, by notice to the Company and the Trustee, may declare the principal amount of, and all accrued and unpaid interest on, all of the Notes then outstanding to become due and payable immediately.

(C)          Rescission of Acceleration. Notwithstanding anything to the contrary in this Indenture or the Notes, the Holders of a majority in aggregate principal amount of the Notes then outstanding, by notice to the Company and the Trustee, may, on behalf of all Holders, rescind any acceleration of the Notes and its consequences if (i) such rescission would not conflict with any judgment or decree of a court of competent jurisdiction; and (ii) all existing Events of Default (except the non-payment of principal of, or interest on, the Notes that has become due solely because of such acceleration) have been cured or waived. No such rescission will affect any subsequent Event of Default or impair any right consequent thereto.

Section 7.03. Sole Remedy for a Failure to Report.

(A)          Generally. Notwithstanding anything to the contrary in this Indenture or the Notes, the Company may elect that the sole remedy for any Event of Default (a “Reporting Event of Default”) pursuant to Section 7.01(A)(vi) arising from the Company’s failure to comply with Section 3.02 will, for each of the first one hundred eighty (180) calendar days on which a Reporting Event of Default has occurred and is continuing, consist exclusively of the accrual of Special Interest on the Notes. If the Company has made such an election, then (i) the Notes will be subject to acceleration pursuant to Section 7.02 on account of the relevant Reporting Event of Default from, and including, the one hundred eighty-first (181st) calendar day on which a Reporting Event of Default has occurred and is continuing or if the Company

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fails to pay any accrued and unpaid Special Interest when due; and (ii) Special Interest will cease to accrue on any Notes from, and including, such one hundred eighty-first (181st) calendar day (it being understood that interest on any defaulted Special Interest will nonetheless accrue pursuant to Section 2.05(B)).

(B)          Amount and Payment of Special Interest. Any Special Interest that accrues on a Note pursuant to Section 7.03(A) will be payable on the same dates and in the same manner as the Stated Interest on such Note and will accrue at a rate per annum equal to one quarter of one percent (0.25%) of the principal amount thereof for the first ninety (90) days on which Special Interest accrues and, thereafter, at a rate per annum equal to one half of one percent (0.50%) of the principal amount thereof. For the avoidance of doubt, any Special Interest that accrues on a Note will be in addition to the Stated Interest that accrues on such Note and in addition to any Additional Interest that accrues on such Note.

(C)          Notice of Election. To make the election set forth in Section 7.03(A), the Company must send to the Holders, the Trustee and the Paying Agent, before the date on which each Reporting Event of Default first occurs, a notice that (i) briefly describes the report(s) that the Company failed to file or provide; (ii) states that the Company is electing that the sole remedy for such Reporting Event of Default consist of the accrual of Special Interest; and (iii) briefly describes the periods during which and rate at which Special Interest will accrue and the circumstances under which the Notes will be subject to acceleration on account of such Reporting Event of Default.

(D)          Notice to Trustee and Paying Agent; Trustee’s Disclaimer. If Special Interest accrues on any Note, then, no later than five (5) Business Days before each date on which such Special Interest is to be paid, the Company will deliver an Officer’s Certificate to the Trustee and the Paying Agent stating (i) that the Company is obligated to pay Special Interest on such Note on such date of payment; and (ii) the amount of such Special Interest that is payable on such date of payment. The Trustee will have no duty to determine whether any Special Interest is payable or the amount thereof.

(E)          No Effect on Other Events of Default. No election pursuant to this Section 7.03 with respect to a Reporting Event of Default will affect the rights of any Holder with respect to any other Event of Default, including with respect to any other Reporting Event of Default.

Section 7.04. Other Remedies.

(A)          Trustee May Pursue All Remedies. If an Event of Default occurs and is continuing, then the Trustee may pursue any available remedy to collect the payment of any amounts due with respect to the Notes or to enforce the performance of any provision of this Indenture or the Notes.

(B)          Procedural Matters. The Trustee may maintain a proceeding even if it does not possess any of the Notes or does not produce any of them in such proceeding. A delay or omission by the Trustee or any Holder in exercising any right or remedy following an Event of Default will not impair the right or remedy or constitute a waiver of, or acquiescence in, such Event of Default. All remedies will be cumulative to the extent permitted by law.

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Section 7.05. Waiver of Past Defaults.

An Event of Default pursuant to clause (i), (ii), (iv) or (vi) of Section 7.01(A) (that, in the case of clause (vi) only, results from a Default under any covenant that cannot be amended without the consent of each affected Holder), and a Default that could lead to such an Event of Default, can be waived only with the consent of each affected Holder. Each other Default or Event of Default may be waived, on behalf of all Holders, by the Holders of a majority in aggregate principal amount of the Notes then outstanding. If an Event of Default is so waived, then it will cease to exist. If a Default is so waived, then it will be deemed to be cured and any Event of Default arising therefrom will be deemed not to occur. However, no such waiver will extend to any subsequent or other Default or Event of Default or impair any right arising therefrom.

Section 7.06. Control by Majority.

Holders of a majority in aggregate principal amount of the Notes then outstanding, determined in accordance with Section 2.15, may direct the time, method and place of conducting any proceeding for exercising any remedy available to the Trustee or exercising any trust or power conferred on it. However, the Trustee may refuse to follow any direction that conflicts with law, this Indenture or the Notes, or that, subject to Section 11.01, the Trustee determines may be unduly prejudicial to the rights of other Holders or may involve the Trustee in liability, unless the Trustee is offered security and indemnity satisfactory to the Trustee against any loss, liability or expense to the Trustee that may result from the Trustee’s following such direction.

Section 7.07. Limitation on Suits.

No Holder may pursue any remedy with respect to this Indenture or the Notes (except to enforce (x) its rights to receive the principal of, or the Redemption Price or Fundamental Change Repurchase Price for, or interest on, any Notes; or (y) the Company’s obligations to convert any Notes pursuant to Article 5), unless:

(A)          such Holder has previously delivered to the Trustee notice that an Event of Default is continuing;

(B)          Holders of at least twenty five percent (25%) in aggregate principal amount of the Notes then outstanding deliver a request to the Trustee to pursue such remedy;

(C)          such Holder or Holders offer and, if requested, provide to the Trustee security and indemnity satisfactory to the Trustee against any loss, liability or expense to the Trustee that may result from the Trustee’s following such request;

(D)          the Trustee does not comply with such request within sixty (60) calendar days after its receipt of such request and such offer of security or indemnity; and

(E)          during such sixty (60) calendar day period, Holders of a majority in aggregate principal amount of the Notes then outstanding do not deliver to the Trustee a direction that is inconsistent with such request.

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A Holder of a Note may not use this Indenture to prejudice the rights of another Holder or to obtain a preference or priority over another Holder. The Trustee will have no duty to determine whether any Holder’s use of this Indenture complies with the preceding sentence.

Section 7.08. Absolute Right of Holders to Receive Payment and Conversion Consideration.

Notwithstanding anything to the contrary in this Indenture or the Notes (but without limiting Section 8.01), the right of each Holder of a Note to receive payment or delivery, as applicable, of the principal of, or the Redemption Price or Fundamental Change Repurchase Price for, or any interest on, or the Conversion Consideration due pursuant to Article 5 upon conversion of, such Note on or after the respective due dates therefor provided in this Indenture and the Notes, or to bring suit for the enforcement of any such payment or delivery on or after such respective due dates, will not be impaired or affected without the consent of such Holder.

Section 7.09. Collection Suit by Trustee.

The Trustee will have the right, upon the occurrence and continuance of an Event of Default pursuant to clause (i), (ii) or (iv) of Section 7.01(A), to recover judgment in its own name and as trustee of an express trust against the Company for the total unpaid or undelivered principal of, or Redemption Price or Fundamental Change Repurchase Price for, or interest on, or Conversion Consideration due pursuant to Article 5 upon conversion of, the Notes, as applicable, and, to the extent lawful, any Default Interest on any Defaulted Amounts, and such further amounts sufficient to cover the costs and expenses of collection, including compensation provided for in Section 11.06.

Section 7.10. Trustee May File Proofs of Claim.

The Trustee has the right to (A) file such proofs of claim and other papers or documents as may be necessary or advisable in order to have the claims of the Trustee and the Holders allowed in any judicial proceedings relative to the Company (or any other obligor upon the Notes) or its creditors or property and (B) unless prohibited by law or applicable regulations, collect, receive and distribute any money or other property payable or deliverable on any such claims. Each Holder authorizes any custodian in such proceeding to make such payments to the Trustee, and, if the Trustee consents to the making of such payments directly to the Holders, to pay to the Trustee any amount due to the Trustee for the reasonable compensation, expenses, disbursements and advances of the Trustee, and its agents and counsel, and any other amounts payable to the Trustee pursuant to Section 11.06. To the extent that the payment of any such compensation, expenses, disbursements, advances and other amounts out of the estate in such proceeding, is denied for any reason, payment of the same will be secured by a lien on, and will be paid out of, any and all distributions, dividends, money, securities and other properties that the Holders may be entitled to receive in such proceeding (whether in liquidation or under any plan of reorganization or arrangement or otherwise). Nothing in this Indenture will be deemed to authorize the Trustee to authorize, consent to, accept or adopt on behalf of any Holder any plan of reorganization, arrangement, adjustment or composition affecting the Notes or the rights of any Holder, or to authorize the Trustee to vote in respect of the claim of any Holder in any such proceeding.

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Section 7.11. Priorities.

The Trustee will pay or deliver in the following order any money or other property that it collects pursuant to this Article 7:

First:              to the Trustee and its agents and attorneys for amounts due under Section 11.06, including payment of all fees, compensation, expenses and liabilities incurred, and all advances made, by the Trustee and the costs and expenses of collection;

Second:          to Holders for unpaid amounts or other property due on the Notes, including the principal of, or the Redemption Price or Fundamental Change Repurchase Price for, or any interest on, or any Conversion Consideration due upon conversion of, the Notes, ratably, and without preference or priority of any kind, according to such amounts or other property due and payable on all of the Notes; and

Third:             to the Company or such other Person as a court of competent jurisdiction directs.

The Trustee may fix a record date and payment date for any payment or delivery to the Holders pursuant to this Section 7.11, in which case the Trustee will instruct the Company to, and the Company will, deliver, at least fifteen (15) calendar days before such record date, to each Holder and the Trustee a notice stating such record date, such payment date and the amount of such payment or nature of such delivery, as applicable.

Section 7.12. Undertaking for Costs.

In any suit for the enforcement of any right or remedy under this Indenture or the Notes or in any suit against the Trustee for any action taken or omitted by it as Trustee, a court, in its discretion, may (A) require the filing by any litigant party in such suit of an undertaking to pay the costs of such suit, and (B) assess reasonable costs (including reasonable attorneys’ fees) against any litigant party in such suit, having due regard to the merits and good faith of the claims or defenses made by such litigant party; provided, however, that this Section 7.12 does not apply to any suit by the Trustee, any suit by a Holder pursuant to Section 7.08 or any suit by one or more Holders of more than ten percent (10%) in aggregate principal amount of the Notes then outstanding.

Article 8. AMENDMENTS, SUPPLEMENTS AND WAIVERS

Section 8.01. Without the Consent of Holders.

Notwithstanding anything to the contrary in Section 8.02, the Company, the Guarantors and the Trustee may amend or supplement this Indenture or the Notes without the consent of any Holder to:

(A)          cure any ambiguity or correct any omission, defect or inconsistency in this Indenture or the Notes;

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(B)          add additional guarantees with respect to the Company’s obligations under this Indenture or the Notes;

(C)          secure the Notes or any Guarantees;

(D)          add to the Company’s or Guarantor’s covenants or Events of Default for the benefit of the Holders or surrender any right or power conferred on the Company or any Guarantor;

(E)          provide for the assumption of the Company’s or any Guarantor’s obligations under this Indenture and the Notes pursuant to, and in compliance with, Article 6 or Section 9.04, as applicable;

(F)           enter into supplemental indentures pursuant to, and in accordance with, Section 5.09 in connection with an Ordinary Share Change Event;

(G)          irrevocably elect or eliminate any Settlement Method or Specified Dollar Amount; provided, however, that no such election or elimination will affect any Settlement Method theretofore elected (or deemed to be elected) with respect to any Note pursuant to Section 5.03(A);

(H)          evidence or provide for the acceptance of the appointment, under this Indenture, of a successor Trustee;

(I)            provide for or confirm the issuance of additional Notes pursuant to Section 2.03(B);

(J)         comply with any requirement of the SEC in connection with any qualification of this Indenture or any supplemental indenture under the Trust Indenture Act, as then in effect;

(K)           to comply with the rules of the Depositary; or

(L)          make any other change to this Indenture or the Notes that does not, individually or in the aggregate with all other such changes, adversely affect the rights of the Holders, as such, in any material respect.

Section 8.02. With the Consent of Holders.

(A)          Generally. Subject to Sections 7.05, 7.08 and 8.01 and the immediately following sentence, the Company, the Guarantors and the Trustee may, with the consent of the Holders of a majority in aggregate principal amount of the Notes then outstanding, amend or supplement this Indenture or the Notes or waive compliance with any provision of this Indenture or the Notes. Notwithstanding anything to the contrary in the foregoing sentence, but subject to subject to Section 8.01, without the consent of each affected Holder, no amendment or supplement to this Indenture or the Notes, or waiver of any provision of this Indenture or the Notes, may:

(i) reduce the principal, or extend the stated maturity, of any Note;

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(ii) reduce the Redemption Price or Fundamental Change Repurchase Price for any Note or change the times at which, or the circumstances under which, the Notes may or will be redeemed or repurchased by the Company;

(iii) reduce the rate, or extend the time for the payment, of interest on any Note;

(iv) make any change that adversely affects the conversion rights of any Note;

(v) impair the rights of any Holder set forth in Section 7.08 (as such section is in effect on the Issue Date);

(vi) change the ranking of the Notes or the Guarantees;

(vii) modify or amend the terms and conditions of the obligations of the Guarantors, as guarantors of the Notes, in any manner that is adverse to the rights of the Holders, as such, other than (A) any elimination of a Guarantee in accordance with this Indenture or (B) to the extent changes are made to the terms and conditions of the Notes in accordance with this Indenture, changes made mutatis mutandis to the obligation of the Guarantors;

(viii) make any Note payable in a currency, or at a place of payment, other than that stated in this Indenture or the Note;

(ix) make any direct or indirect change to Section 3.04 in any manner that is materially adverse to the rights of the Holders;

(x) reduce the amount of Notes whose Holders must consent to any amendment, supplement, waiver or other modification; or

(xi) make any direct or indirect change to any amendment, supplement, waiver or modification provision of this Indenture or the Notes that requires the consent of each affected Holder.

For the avoidance of doubt, pursuant to clauses (i), (ii), (iii) and (iv) of this Section 8.02(A), no amendment or supplement to this Indenture or the Notes, or waiver of any provision of this Indenture or the Notes, may reduce the amount or change the type of consideration due on any Note (whether on an Interest Payment Date, Redemption Date, Fundamental Change Repurchase Date or the Maturity Date or upon conversion, or otherwise), or the date(s) or time(s) such consideration is payable or deliverable, as applicable, without the consent of each affected Holder.

(B)          Holders Need Not Approve the Particular Form of any Amendment. A consent of any Holder pursuant to this Section 8.02 need approve only the substance, and not necessarily the particular form, of the proposed amendment, supplement or waiver.

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Section 8.03. Notice of Amendments, Supplements and Waivers.

Promptly after any amendment, supplement or waiver pursuant to Section 8.01 or 8.02 becomes effective, the Company will send to the Holders and the Trustee notice that (A) describes the substance of such amendment, supplement or waiver in reasonable detail and (B) states the effective date thereof. The failure to send, or the existence of any defect in, such notice will not impair or affect the validity of such amendment, supplement or waiver.

Section 8.04. Revocation, Effect and Solicitation of Consents; Special Record Dates; Etc.

(A)          Revocation and Effect of Consents. The consent of a Holder of a Note to an amendment, supplement or waiver will bind (and constitute the consent of) each subsequent Holder of any Note to the extent the same evidences any portion of the same indebtedness as the consenting Holder’s Note, subject to the right of any Holder of a Note to revoke (if not prohibited pursuant to Section 8.04(B)) any such consent with respect to such Note by delivering notice of revocation to the Trustee before the time such amendment, supplement or waiver becomes effective.

(B)          Special Record Dates. The Company may, but is not required to, fix a record date for the purpose of determining the Holders entitled to consent or take any other action in connection with any amendment, supplement or waiver pursuant to this Article 8. If a record date is fixed, then, notwithstanding anything to the contrary in Section 8.04(A), only Persons who are Holders as of such record date (or their duly designated proxies) will be entitled to give such consent, to revoke any consent previously given or to take any such action, regardless of whether such Persons continue to be Holders after such record date; provided, however, that no such consent will be valid or effective for more than one hundred and twenty (120) calendar days after such record date.

(C)          Solicitation of Consents. For the avoidance of doubt, each reference in this Indenture or the Notes to the consent of a Holder will be deemed to include any such consent obtained in connection with a repurchase of, or tender or exchange offer for, any Notes.

(D)          Effectiveness and Binding Effect. Each amendment, supplement or waiver pursuant to this Article 8 will become effective in accordance with its terms and, when it becomes effective with respect to any Note (or any portion thereof), will thereafter bind every Holder of such Note (or such portion).

Section 8.05. Notations and Exchanges.

If any amendment, supplement or waiver changes the terms of a Note, then the Trustee or the Company may, in its discretion, require the Holder of such Note to deliver such Note to the Trustee so that the Trustee may place an appropriate notation prepared by the Company on such Note and return such Note to such Holder. Alternatively, at its discretion, the Company may, in exchange for such Note, issue, execute and deliver, and the Trustee will authenticate, in each case in accordance with Section 2.02, a new Note that reflects the changed terms. The failure to make any appropriate notation or issue a new Note pursuant to this Section 8.05 will not impair

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or affect the validity of such amendment, supplement or waiver.

Section 8.06. Trustee to Execute Supplemental Indentures.

Upon the written request of the Company, the Trustee will, and is hereby authorized to, execute and deliver any amendment or supplemental indenture authorized pursuant to this Article 8; provided, however, that the Trustee need not (but may, in its sole and absolute discretion) execute or deliver any such amendment or supplemental indenture that adversely affects the Trustee’s rights, duties, liabilities or immunities. In executing any amendment or supplemental indenture, the Trustee will be entitled to receive, and (subject to Sections 11.01 and 11.02) will be fully protected in relying on as conclusive evidence, an Officer’s Certificate and an Opinion of Counsel stating that (A) the execution and delivery of such amendment or supplemental indenture is authorized or permitted by this Indenture; and (B) in the case of the Opinion of Counsel, such amendment or supplemental indenture is valid, binding and enforceable against the Company in accordance with its terms.

Article 9. GUARANTEES

Section 9.01. Guarantees.

(A)          Generally. By its execution of this Indenture (or any amended or supplemental indenture pursuant to Section 8.01(B)), each Guarantor acknowledges and agrees that it receives substantial benefits from the Company and that such Guarantor is providing its Guarantee for good and valuable consideration, including such substantial benefits. Subject to this Article 9, each of the Guarantors hereby, jointly and severally, fully and unconditionally guarantees, to (x) each Holder of a Note authenticated and delivered by the Trustee and (y) to the Trustee and its successors and assigns on behalf of such Holder that:

(i) the principal of, any interest on, and any Conversion Consideration for, the Notes will be promptly paid in full when due, whether at maturity, by acceleration, on a Fundamental Change Repurchase Date, upon Redemption or otherwise, and interest on the overdue principal of, any interest on, or any Conversion Consideration for, the Notes, if lawful, and all other obligations of the Company to the Holders or the Trustee under this Indenture or the Notes (including the expense reimbursement and indemnification provisions of Section 11.06), will be promptly paid or delivered in full or performed, as applicable, in each case in accordance with this Indenture and the Notes; and

(ii) in case of any extension of time of payment or renewal of any Notes or any of such other obligations, that the same will be promptly paid in full when due or performed in accordance with the terms of the extension or renewal, whether at stated maturity, by acceleration, on a Fundamental Change Repurchase Date, upon Redemption or otherwise,

(collectively, the “Guaranteed Obligations”), in each case subject to Section 9.02.

Upon the failure of any payment when due of any amount so guaranteed, and upon the

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failure of any performance so guaranteed, for whatever reason, the Guarantors will be jointly and severally obligated to pay or perform, as applicable, the same immediately. Each Guarantor agrees that this is a guarantee of payment and not a guarantee of collection.

(B)          Guarantee Is Unconditional; Waiver of Diligence, Presentment, Etc. Each Guarantor agrees that its Guarantee of the Guaranteed Obligations is unconditional, regardless of the validity or enforceability of this Indenture, the Notes or the obligations of the Company under this Indenture or the Notes, the absence of any action to enforce the same, any waiver or consent by any Holder with respect to any provisions of this Indenture or the Notes, the recovery of any judgment against the Company, any action to enforce the same or any other circumstance that might otherwise constitute a legal or equitable discharge or defense of a guarantor. Each Guarantor waives diligence, presentment, demand of payment, filing of claims with a court in the event of insolvency or bankruptcy of the Company, any right to require a proceeding first against the Company, protest, notice and all demands whatsoever, and covenants that this Guarantee will not be discharged except by complete performance of the obligations contained in this Indenture and the Notes.

(C)          Reinstatement of Guarantee Upon Return of Payments. If any Holder or the Trustee is required by any court or otherwise to return, to the Company, the Guarantors or any custodian, trustee, liquidator or other similar official acting in relation to the Company or the Guarantors, any consideration paid or delivered by the Company or the Guarantors to such Holder or the Trustee, then each Guarantee, to the extent theretofore discharged, will be reinstated in full force and effect.

(D)          Subrogation. Each Guarantor shall be subrogated to all rights of the Holders of the Notes against the Company in respect of any amounts paid by such Guarantor on account of such Notes pursuant to the provisions of its Guarantee or this Indenture; provided that each Guarantor agrees that any right of subrogation, reimbursement or contribution it may have in relation to the Holders or in respect of any Guaranteed Obligations will be subordinated to, and will not be enforceable until payment in full of, all Guaranteed Obligations. Each Guarantor further agrees that, as between the Guarantors, on the one hand, and the Holders and the Trustee, on the other hand, (i) the maturity of the Guaranteed Obligations may be accelerated as provided in Article 7, notwithstanding any stay, injunction or other prohibition preventing such acceleration in respect of the Guaranteed Obligations; and (ii) if any Guaranteed Obligations are accelerated pursuant to Article 7, then such Guaranteed Obligations will, whether or not due and payable, immediately become due and payable by the Guarantors.

Section 9.02. Limitation on Guarantor Liability.

Each Guarantor, and, by its acceptance of any Note, each Holder, confirms that each Guarantor and the Holders intend that the Guarantee of each Guarantor not constitute a fraudulent transfer or conveyance for purposes of Bankruptcy Law, the Uniform Fraudulent Conveyance Act, the Uniform Fraudulent Transfer Act or any similar federal or state law to the extent applicable to any Guarantee. Each of the Trustee, the Holders and each Guarantor irrevocably agrees that the obligations of each Guarantor under its Guarantee will be limited to the maximum amount that will, after giving effect to such maximum amount and all other contingent and fixed liabilities of such Guarantor that are relevant under such laws, and after

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giving effect to any collections from, rights to receive contribution from or payments made by or on behalf of any other Guarantor in respect of the obligations of such other Guarantor under its Guarantee, result in the obligations of such Guarantor under its Guarantee not constituting a fraudulent transfer or conveyance.

Section 9.03. Execution and Delivery of Guarantee.

The execution by each Guarantor of this Indenture (or an amended or supplemental indenture pursuant to Section 8.01(B)) evidences the Guarantee of such Guarantor, and the delivery of any Note by the Trustee after its authentication constitutes due delivery of each Guarantee on behalf of each Guarantor. A Guarantee’s validity will not be affected by the failure of any officer of a Guarantor executing this Indenture or any such amended or supplemental indenture on such Guarantor’s behalf to hold, at the time any Note is authenticated, the same or any other office at such Guarantor, and each Guarantee will be valid and enforceable even if no notation, certificate or other instrument is set upon or attached to, or otherwise executed and delivered to the Holder of, any Note.

Section 9.04. When Guarantors May Merge, Etc.

(A)          Generally. No Guarantor will consolidate with or merge with or into, or sell, lease or otherwise transfer, in one transaction or a series of transactions, all or substantially all of the assets of such Guarantor and its Subsidiaries, taken as a whole, to another Person (other than the Company, another Guarantor or one or more of the Company’s Wholly Owned Subsidiaries) (a “Guarantor Business Combination Event”), unless (i) such transaction otherwise complies with Section 3.12, (ii) such Guarantor is an Immaterial Subsidiary or (iii) the resulting, surviving or transferee Person is such Guarantor or, if not such Guarantor, expressly assumes (by executing and delivering to the Trustee, at or before the effective time of such Guarantor Business Combination Event, a supplemental indenture pursuant to Section 8.01(E)) all of such Guarantor’s obligations under this Indenture and the Notes.

(B)          Delivery of Officer’s Certificate and Opinion of Counsel to the Trustee. Before the effective time of any Guarantor Business Combination Event (unless such transaction otherwise complies with Section 3.12), the Company will deliver to the Trustee an Officer’s Certificate and Opinion of Counsel, each stating that (i) such Guarantor Business Combination Event (and, if applicable, the related supplemental indenture) comply with Section 9.04(A); and (ii) all conditions precedent to such Guarantor Business Combination Event provided in this Indenture have been satisfied.

(C)          Successor Corporation Substituted. At the effective time of any Guarantor Business Combination Event that complies with Section 9.04(A) and Section 9.04(B) (unless such transaction otherwise complies with Section 3.12), the surviving Person (if not the applicable Guarantor) will succeed to, and may exercise every right and power of, such Guarantor under this Indenture and the Notes with the same effect as if such surviving Person had been named as a Guarantor in this Indenture and the Notes, and, except in the case of a lease, the predecessor Guarantor will be discharged from its obligations under this Indenture and the Notes.

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Section 9.05. Future Guarantors.

(A)          If, after the Issue Date, any entity becomes a Subsidiary of the Company or any Guarantor (including by acquisition or creation), then the Company will, as soon as reasonably practicable but no later than ten (10) Business Days after such entity became such a Subsidiary, cause such Subsidiary to execute an amended or supplemental indenture pursuant to Section 8.01(B) causing such Subsidiary to become a Guarantor under this Indenture, unless (x) such supplemental indenture would result in material adverse tax consequences to the Parent, the Company or their Subsidiary, (y) such supplemental indenture would conflict with applicable law or (z) such Subsidiary is an Immaterial Subsidiary or organized within France.

(B)          The Company will, as soon as reasonably practicable after the Issue Date, cause each of the following Subsidiaries to execute an amended or supplemental indenture pursuant to Section 8.01(B) causing such Subsidiary to become a Guarantor under this Indenture, unless (x) such supplemental indenture would result in material adverse tax consequences to the Parent, the Company or their Subsidiary, (y) such supplemental indenture would conflict with applicable law or (z) such Subsidiary is an Immaterial Subsidiary:

(i) Aegerion Brasil Comercio e Importacao de Medicamentos LTDA, a limited liability company organized in Brazil;

(ii) Aegerion International Ltd., a limited company organized in Bermuda;

(iii) Aegerion Pharmaceuticals (Canada) Ltd., a limited company organized in Canada;

(iv) Aegerion Pharmaceuticals GmbH, a limited liability company organized in Germany;

(v) Aegerion Pharmaceuticals K.K., a stock corporation organized in Japan;

(vi) Aegerion Pharmaceuticals Ltd., a limited company organized in Bermuda;

(vii) Aegerion Pharmaceuticals S.r.l., a limited liability company organized in Italy;

(viii) Aegerion Pharmaceuticals SARL, a limited liability company organized in Switzerland;

(ix) Aegerion Pharmaceuticals Spain, S.L., a limited liability company organized in Spain; and

(x) Amryt Gmbh, a limited liability company organized in Germany.

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Section 9.06. Application of Certain Provisions to the Guarantors.

(A)          Officer’s Certificates and Opinions of Counsel. Upon any request or application by any Guarantor to the Trustee to take any action under this Indenture, the Trustee will be entitled to receive an Officer’s Certificate and an Opinion of Counsel pursuant to Section 12.02 with the same effect as if each reference to the Company in Section 12.02 or in the definitions of “Officer,” “Officer’s Certificate” or “Opinion of Counsel” were instead a reference to such Guarantor.

(B)          Notices and Demands. Any notice or demand that this Indenture requires or permits to be given by the Trustee, or by any Holders, to the Company may instead be given to any Guarantor.

Article 10. SATISFACTION AND DISCHARGE

Section 10.01. Termination of Company’s Obligations.

This Indenture will be discharged, and will cease to be of further effect as to all Notes issued under this Indenture, when:

(A)          all Notes then outstanding (other than Notes replaced pursuant to Section 2.12) have (i) been delivered to the Trustee for cancellation; or (ii) become due and payable (whether on a Redemption Date, a Fundamental Change Repurchase Date, the Maturity Date, upon conversion or otherwise) for an amount of cash and/or Ordinary Shares (or, if applicable, Reference Property) and cash (in lieu of fractional Ordinary Shares or, if applicable, Reference Property Units) (solely to satisfy amounts due and owing as a result of conversions of the Notes), as applicable, that has been fixed;

(B)          the Company has caused there to be irrevocably deposited with the Trustee, or with the Paying Agent (or, with respect to Conversion Consideration, the Conversion Agent), in each case for the benefit of the Holders, or has otherwise caused there to be delivered to the Holders, cash and/or Ordinary Shares (or, if applicable, Reference Property) and cash (in lieu of fractional Ordinary Shares or, if applicable, Reference Property Units) (solely to satisfy amounts due and owing as a result of conversions of the Notes) sufficient to satisfy all amounts or other property (including, if applicable, all related Additional Amounts) due on all Notes then outstanding (other than Notes replaced pursuant to Section 2.12);

(C)          the Company has paid all other amounts payable by it under this Indenture; and

(D)          the Company has delivered to the Trustee an Officer’s Certificate and an Opinion of Counsel, each stating that the conditions precedent to the discharge of this Indenture have been satisfied;

provided, however, that Article 11 and Section 12.01 will survive such discharge and, until no Notes remain outstanding, Section 2.14 and the obligations of the Trustee, the Paying Agent and the Conversion Agent with respect to money or other property deposited with them will survive such discharge.

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At the Company’s request, the Trustee will acknowledge the satisfaction and discharge of this Indenture.

Section 10.02. Repayment to Company.

Subject to applicable unclaimed property law, the Trustee, the Paying Agent and the Conversion Agent will promptly notify the Company if there exists (and, at the Company’s request, will promptly deliver to the Company) any cash, Conversion Consideration or other property held by any of them for payment or delivery on the Notes that remain unclaimed two (2) years after the date on which such payment or delivery was due. After such delivery to the Company, the Trustee, the Paying Agent and the Conversion Agent will have no further liability to any Holder with respect to such cash, Conversion Consideration or other property, and Holders entitled to the payment or delivery of such cash, Conversion Consideration or other property must look to the Company for payment as a general creditor of the Company.

Section 10.03. Reinstatement.

If the Trustee, the Paying Agent or the Conversion Agent is unable to apply any cash or other property deposited with it pursuant to Section 10.01 because of any legal proceeding or any order or judgment of any court or other governmental authority that enjoins, restrains or otherwise prohibits such application, then the discharge of this Indenture pursuant to Section 10.01 will be rescinded until such time as the Trustee, the Paying Agent or the Conversion Agent is permitted to apply such cash or other property pursuant to Section 10.01; provided, however, that if the Company pays or delivers any cash or other property due on the Notes to the Holders thereof, then the Company will be subrogated to the rights of such Holders to receive such cash or other property from the cash or other property, if any, held by the Trustee, the Paying Agent or the Conversion Agent, as applicable.

Article 11. TRUSTEE

Section 11.01. Duties of the Trustee.

(A)          If an Event of Default has occurred and is continuing, the Trustee will exercise such of the rights and powers vested in it by this Indenture, and use the same degree of care and skill in its exercise, as a prudent person would exercise or use under the circumstances in the conduct of such person’s own affairs.

(B)          Except during the continuance of an Event of Default:

(i) the duties of the Trustee will be determined solely by the express provisions of this Indenture, and the Trustee need perform only those duties that are specifically set forth in this Indenture and no others, and no implied covenants or obligations will be read into this Indenture against the Trustee; and

(ii) in the absence of willful misconduct on its part, the Trustee may conclusively rely, as to the truth of the statements and the correctness of the

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opinions expressed therein, upon Officer’s Certificates or Opinions of Counsel that are provided to the Trustee and conform to the requirements of this Indenture. However, the Trustee will examine the certificates and opinions to determine whether or not they conform to the requirements of this Indenture (but need not confirm or investigate the accuracy of mathematical calculations or other facts, statements, opinions or conclusions stated therein).

(C)        The Trustee may not be relieved from liabilities for its negligence or willful misconduct, except that:

(i) this paragraph will not limit the effect of Section 11.01(B) or Section 11.01(E);

(ii) the Trustee will not be liable for any error of judgment made in good faith by a Responsible Officer, unless it is proved that the Trustee was negligent in ascertaining the pertinent facts; and

(iii) the Trustee will not be liable with respect to any action it takes or omits to take in good faith in accordance with a direction received by it pursuant to Section 7.06.

(D)        Each provision of this Indenture that in any way relates to the Trustee is subject to this Section 11.01, regardless of whether such provision so expressly provides.

(E)        No provision of this Indenture will require the Trustee to expend or risk its own funds or incur any liability.

(F)        The Trustee will not be liable for interest on any money received by it, except as the Trustee may agree in writing with the Company. Money held in trust by the Trustee need not be segregated from other funds, except to the extent required by law.

(G)        The Trustee shall not be liable in respect of any payment (as to the correctness of amount, entitlement to receive or any other matters relating to payment) or notice effected by the Company or any Paying Agent or any records maintained by any co-Registrar with respect to the Notes.

(H)        If any party fails to deliver a notice relating to an event the fact of which, pursuant to this Indenture, requires notice to be sent to the Trustee, the Trustee may conclusively rely on its failure to receive such notice as reason to act as if no such event occurred.

(I)         In the absence of written investment direction from the Company, all cash received by the Trustee shall be placed in a non-interest bearing trust account, and in no event shall the Trustee be liable for the selection of investments or for investment losses incurred thereon or for losses incurred as a result of the liquidation of any such investment prior to its maturity date or the failure of the party directing such investments prior to its maturity date or the failure of the party directing such investment to provide timely written investment direction, and the Trustee shall have no obligation to invest or reinvest any amounts held hereunder in the absence of such written investment direction from the Company.

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(J)           In the event that the Trustee is also acting as Custodian, Registrar, Paying Agent or Conversion Agent hereunder, the rights, privileges, immunities, benefits and protections afforded to the Trustee pursuant to this Article 11 shall also be afforded to such Custodian, Registrar, Paying Agent or Conversion Agent.

(K)         The Trustee shall not be responsible for the application of any money by any Paying Agent other than the Trustee.

Section 11.02. Rights of the Trustee.

(A)         The Trustee may conclusively rely on any resolution, certificate, statement, instrument, opinion, report, notice, request, direction, consent, order or other document that it believes to be genuine and signed or presented by the proper Person, and the Trustee need not investigate any fact or matter stated therein.

(B)          Before the Trustee acts or refrains from acting, it may require an Officer’s Certificate, an Opinion of Counsel or both. The Trustee will not be liable for any action it takes or omits to take in good faith in reliance on such Officer’s Certificate or Opinion of Counsel. The Trustee may consult with counsel; and the advice of such counsel, or any Opinion of Counsel, will constitute full and complete authorization of the Trustee to take or omit to take any action in good faith in reliance thereon without liability.

(C)         Any request, direction, order or demand of the Company mentioned herein shall be sufficiently evidenced by a Company Order (unless other evidence in respect thereof be herein specifically prescribed); and any board resolutions may be evidenced to the Trustee by a copy thereof certified by the Company’s Secretary, Assistant Secretary or other duly authorized Officer.

(D)         The Trustee may act through its attorneys and agents and will not be responsible for the misconduct or negligence of any such attorney or agent appointed with due care.

(E)          The Trustee will not be liable for any action it takes or omits to take in good faith and that it believes to be authorized or within the rights or powers vested in it by this Indenture. The rights, privileges, protections, immunities and benefits given to the Trustee, including, without limitation, its right to be indemnified, are extended to, and shall be enforceable by, the Trustee in each of its capacities hereunder, and to each agent, custodian and other Person employed to act hereunder.

(F)          Unless otherwise specifically provided in this Indenture, any demand, request, direction or notice from the Company or any Guarantor will be sufficient if signed by an Officer of the Company or Guarantor, as applicable. The Trustee may request that the Company or any Guarantor deliver a certificate setting forth the names of individuals and/or titles of officers authorized at such time to take specified actions pursuant to this Indenture, which certificate may be signed by any person authorized to sign an Officer’s Certificate, including any person specified as so authorized in any such certificate previously delivered and not superseded.

(G)          The Trustee need not exercise any rights or powers vested in it by this Indenture at the request or direction of any Holder unless such Holder has offered the Trustee security or

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indemnity satisfactory to the Trustee against any loss, liability or expense that it may incur in complying with such request or direction.

(H)         Anything in this indenture notwithstanding, the Trustee will not be responsible or liable for any punitive, special, indirect or consequential loss or damage (including lost profits), even if the Trustee has been advised of the likelihood of such loss or damage and regardless of the form of action.

(I)          The Trustee shall not be required to give any bond or surety in respect of the performance of its powers and duties hereunder.

(J)          The Trustee shall be under no obligation to take or pursue any action that is not in accordance with applicable law.

(K)         The Trustee may execute any of the trusts or powers hereunder or perform any duties hereunder either directly or by or through agents, custodians, nominees or attorneys and the Trustee shall not be responsible for any misconduct or negligence on the part of any agent, custodian, nominee or attorney appointed by it with due care hereunder.

(L)         The permissive rights of the Trustee enumerated herein shall not be construed as duties.

Section 11.03. Individual Rights of the Trustee.

The Trustee, in its individual or any other capacity, may become the owner or pledgee of any Note and may otherwise deal with the Company or any of its Affiliates with the same rights that it would have if it were not Trustee; provided, however, that if the Trustee acquires a “conflicting interest” (within the meaning of Section 310(b) of the Trust Indenture Act), then it must eliminate such conflict within ninety (90) days or resign as Trustee. Each Note Agent will have the same rights and duties as the trustee under this Section 11.03.

Section 11.04. Trustee’s Disclaimer.

The Trustee will not be (A) responsible for, and makes no representation as to, the validity or adequacy of this Indenture or the Notes; (B) accountable for the Company’s use of the proceeds from the Notes or any money paid to the Company or upon the Company’s direction under any provision of this Indenture; (C) responsible for the use or application of any money received by any Paying Agent other than the Trustee; (D) responsible for any statement or recital in this Indenture, the Notes or any other document relating to the sale of the Notes or this Indenture, other than the Trustee’s certificate of authentication; (E) responsible to make any calculation with respect to any matter under this Indenture; or (F) under any duty to monitor or investigate the Company’s compliance with or the breach of, or cause to be performed or observed, any representation, warranty, or covenant, or agreement of any Person, other than the Trustee, made in this Indenture. For the avoidance of doubt, neither the Trustee nor any Note Agent shall have any obligation to make any calculation (including determinations of the Last Reported Sale Price, the Daily Conversion Value, the Daily Cash Amount, the Daily Share Amount, the Daily VWAP, accrued interest on the Notes and the Conversion Rate) or to determine whether the Notes may be surrendered for conversion, or to notify the Company, any

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Guarantor, the Depositary, or any Holders of the Notes if the Notes have become convertible.

Section 11.05. Notice of Defaults.

The Trustee shall not be deemed to have notice or be charged with knowledge of any Default or Event of Default unless a Responsible Officer receives written notice of such Default or Event of Default from the Company, the Guarantor or from Holders of Notes evidencing no less than twenty-five percent (25%) of the aggregate outstanding principal amount of Notes. If a Default or Event of Default occurs and is continuing and is known to the Trustee (as determined in accordance with the foregoing sentence), then the Trustee will send Holders a notice of such Default or Event of Default promptly (and in any event within ten (10) Business Days) after it becomes known to a Responsible Officer; provided, however, that, except in the case of a Default or Event of Default in the payment of the principal of, or interest on, any Note, the Trustee may withhold such notice if and for so long as it in good faith determines that withholding such notice is in the interests of the Holders.

Section 11.06. Compensation and Indemnity.

(A)          The Company will, from time to time, pay the Trustee reasonable compensation for its acceptance of this indenture and services under this Indenture. The Trustee’s compensation will not be limited by any law on compensation of a trustee of an express trust. In addition to the compensation for the Trustee’s services, the Company will reimburse the Trustee promptly upon request for all reasonable disbursements, advances and expenses incurred or made by it under this Indenture, including the reasonable compensation, disbursements and expenses of the Trustee’s agents and counsel.

(B)          The Company and each Guarantor will, on a joint and several basis, indemnify the Trustee and any predecessor Trustee and their respective officers, directors, agents and employees and any authenticating agent and hold them harmless against any and all losses, claims, damages, liabilities or expenses, including fees and expenses of counsel, including taxes (other than taxes based upon, measured by or determined by the income of the Trustee) incurred by it arising out of or in connection with the acceptance or administration of its duties under this Indenture, the Notes and any other document or transaction entered into in connection herewith or therewith, including the costs and expenses of enforcing this Indenture against the Company or any Guarantor (including this Section 11.06) and defending itself against any claim (whether asserted by the Company, any Guarantor any Holder or any other Person) or liability in connection with the exercise or performance of any of its powers or duties under this Indenture, except to the extent any such loss, liability or expense may be attributable to its gross negligence or willful misconduct as determined in a final non-appealable judgment by a court of competent jurisdiction. The Trustee will promptly notify the Company of any claim for which it may seek indemnity, but the Trustee’s failure to so notify the Company will not relieve the Company or any Guarantor of its obligations under this Section 11.06(B). The Company and each Guarantor will defend such claim, and the Trustee will cooperate in such defense. The Trustee may retain separate counsel, and the Company and each Guarantor will, on a joint and several basis, pay the reasonable fees and expenses of such counsel. The Company need not pay for any settlement of any such claim made without its consent, which consent will not be unreasonably withheld.

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(C)          The obligations of the Company and each Guarantor under this Section 11.06 will survive the resignation or removal of the Trustee, the satisfaction and discharge of this Indenture and the termination for any other reason of this Indenture. The Trustee’s right to receive payment of any amounts due under this Section 11.06 shall not be subordinate to any other liability or indebtedness of the Company.

(D)          To secure the Company’s payment obligations in this Section 11.06 and the Guaranteed Obligations with respect to the Company’s obligations in this Section 11.06, the Trustee will have a lien prior to the Notes on all money or property held or collected by the Trustee, except that held in trust to pay principal of, or interest on, particular Notes, which lien will survive the discharge of this Indenture.

(E)           If the Trustee incurs expenses or renders services after an Event of Default pursuant to clause (ix) or (x) of Section 7.01(A) occurs, then such expenses and the compensation for such services (including the fees and expenses of its agents and counsel) are intended to constitute expenses of administration under any Bankruptcy Law.

(F)           Each Note Agent will have the same rights and duties as the Trustee under this Section 11.06.

Section 11.07. Replacement of the Trustee.

(A)          Notwithstanding anything to the contrary in this Section 11.07, a resignation or removal of the Trustee, and the appointment of a successor Trustee, will become effective only upon such successor Trustee’s acceptance of appointment as provided in this Section 11.07.

(B)          The Trustee may resign at any time and be discharged from the trust created by this Indenture by so notifying the Company. The Holders of a majority in aggregate principal amount of the Notes then outstanding may remove the Trustee by so notifying the Trustee and the Company in writing. The Company may remove the Trustee if:

(i) the Trustee fails to comply with Section 11.09;

(ii) the Trustee is adjudged to be bankrupt or insolvent or an order for relief is entered with respect to the Trustee under any Bankruptcy Law;

(iii) a custodian or public officer takes charge of the Trustee or its property; or

(iv) the Trustee becomes incapable of acting.

(C)          If the Trustee resigns or is removed, or if a vacancy exists in the office of Trustee for any reason, then by written instrument, one copy of which instrument shall be delivered to the resigning Trustee and one copy to the successor Trustee (i) the Company will promptly appoint a successor Trustee; and (ii) at any time within one (1) year after the successor Trustee takes office, the Holders of a majority in aggregate principal amount of the Notes then outstanding may appoint a successor Trustee to replace such successor Trustee appointed by the Company.

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(D)          If a successor Trustee does not take office within sixty (60) days after the retiring Trustee resigns or is removed, then the retiring Trustee, the Company or the Holders of at least ten percent (10%) in aggregate principal amount of the Notes then outstanding may petition any court of competent jurisdiction for the appointment of a successor Trustee. Such court may thereupon, after such notice, if any, as it may deem proper and prescribe, appoint a successor Trustee.

(E)          If the Trustee, after written request by a Holder of at least six (6) months, fails to comply with Section 11.09, then such Holder may petition any court of competent jurisdiction for the removal of the Trustee and the appointment of a successor Trustee.

(F)          A successor Trustee will deliver a written acceptance of its appointment to the retiring Trustee and to the Company, upon which notice the resignation or removal of the retiring Trustee will become effective and the successor Trustee will have all the rights, powers and duties of the Trustee under this Indenture. The successor Trustee will send notice of its succession to Holders. The retiring Trustee will, upon payment of all amounts due to it under this Indenture, promptly transfer all property held by it as Trustee to the successor Trustee, which property will, for the avoidance ofdoubt, be subject to the lien provided for in Section 11.06(D).

(G)          Notwithstanding replacement of the Trustee pursuant to this Section 11.07, the Company’s obligations under Section 11.06 shall continue for the benefit of the retiring Trustee.

(H)          The Company shall execute such written instruments as are reasonably request in writing by any successor Trustee to confirm such successor Trustee’s rights and powers under this Indenture.

Section 11.08. Successor Trustee by Merger, Etc.

If the Trustee consolidates, merges or converts into, or transfers all or substantially all of its corporate trust business (including the administration of this Indenture) to, another Person, then such Person will become the successor Trustee without any further act.

Section 11.09. Eligibility; Disqualification.

There shall at all times be a U.S. Trustee hereunder which shall be a Person that is eligible pursuant to the Trust Indenture Act to act as such and has a combined capital and surplus of at least the minimum amount required by the Trust Indenture Act.

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Article 12. MISCELLANEOUS

Section 12.01. Notices.

Any notice or communication by the Company or any Guarantor or the Trustee to the other will be deemed to have been duly given if in writing and delivered in person or by first class mail (registered or certified, return receipt requested), electronic transmission or other similar means of unsecured electronic communication or overnight air courier guaranteeing next day delivery, or to the other’s address, which initially is as follows:

If to the Company or any Guarantor:

Aegerion Pharmaceuticals, Inc.

90 Harcourt Street

Dublin 2, Ireland

Attention: Chief Financial Officer

with a copy to:

Gibson, Dunn & Crutcher LLP

200 Park Avenue, 47th Floor

New York, NY 10166

Attention: Andrew Fabens

If to the Trustee:

GLAS Trust Company LLC

3 Second Street, Suite 206

Jersey City, NJ 07311

Facsimile: (212) 202-6246

Attention: Administrator for Aegerion Pharmaceuticals, Inc.

The Company, any Guarantor or the Trustee, by notice to the others, may designate additional or different addresses (including facsimile numbers and electronic addresses) for subsequent notices or communications.

All notices and communications (other than those sent to Holders) will be deemed to have been duly given: (A) at the time delivered by hand, if personally delivered; (B) five (5) Business Days after being deposited in the mail, postage prepaid, if mailed; (C) when receipt acknowledged, if transmitted by electronic transmission or other similar means of unsecured electronic communication; and (D) the next Business Day after timely delivery to the courier, if sent by overnight air courier guaranteeing next day delivery.

All notices or communications required to be made to a Holder pursuant to this Indenture must be made in writing and will be deemed to be duly sent or given in writing if mailed by first class mail, certified or registered, return receipt requested, or by overnight air courier guaranteeing next day delivery, to its address shown on the Register; provided, however, that a

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notice or communication to a Holder of a Global Note may, but need not, instead be sent pursuant to the Depositary Procedures (in which case, such notice will be deemed to be duly sent or given in writing). The failure to send a notice or communication to a Holder, or any defect in such notice or communication, will not affect its sufficiency with respect to any other Holder.

If the Trustee is then acting as the Depositary’s custodian for the Notes, then, at the reasonable request of the Company to the Trustee, the Trustee will cause any notice prepared by the Company to be sent to any Holder(s) pursuant to the Depositary Procedures, provided such request is evidenced in a Company Order delivered, together with the text of such notice, to the Trustee at least one (1) Business Day before the date such notice is to be so sent. The Trustee will not have any liability relating to the contents of any notice that it sends to any Holder pursuant to any such Company Order.

If a notice or communication is mailed or sent in the manner provided above within the time prescribed, it will be deemed to have been duly given, whether or not the addressee receives it.

Notwithstanding anything to the contrary in this Indenture or the Notes, whenever any provision of this Indenture requires a party to send notice to another party, no such notice need be sent if the sending party and the recipient arc the same Person acting in different capacities.

The Trustee shall have the right, but shall not be required, to rely upon and comply with notices, instructions, directions or other communications sent by e-mail, facsimile and other similar unsecured electronic methods by persons believed by the Trustee to be authorized to give instructions and directions on behalf of the Company or the Guarantor. The Trustee shall have no duty or obligation to verify or confirm that the person who sent such instructions or directions is, in fact, a person authorized to give instructions or directions on behalf of the Company or the Guarantor; and the Trustee shall have no liability for any losses, liabilities, costs or expenses incurred or sustained by the Company or the Guarantor as a result of such reliance upon or compliance with such notices, instructions, directions or other communications. The Company and the Guarantor, as applicable, each agree to assume all risks arising out of the use of such electronic methods to submit notices, instructions, directions or other communications to the Trustee, including without limitation the risk of the Trustee acting on unauthorized instructions, and the risk of interception and misuse by third parties. The Company and the Guarantor shall use all reasonable endeavors to ensure that any such notices, instructions, directions or other communications transmitted to the Trustee pursuant to this Indenture are complete and correct. Any such notices, instructions, directions or other communications shall be conclusively deemed to be valid instructions from the Company or the Guarantor to the Trustee for the purposes of this Indenture.

- 95 -

Section 12.02. Delivery of Officer’s Certificate and Opinion of Counsel as to Conditions Precedent.

Upon any request or application by the Company to the Trustee to take any action under this Indenture (other than the initial authentication of Notes under this Indenture), the Company will furnish to the Trustee:

(A)          an Officer’s Certificate in form and substance reasonably satisfactory to the Trustee that complies with Section 12.03 and states that, in the opinion of the signatory thereto, all conditions precedent and covenants, if any, provided for in this Indenture relating to such action have been satisfied; and

(B)          an Opinion of Counsel in form and substance reasonably satisfactory to the Trustee that complies with Section 12.03 and states that, in the opinion of such counsel, all such conditions precedent and covenants, if any, have been satisfied.

Section 12.03. Statements Required in Officer’s Certificate and Opinion of Counsel.

Each Officer’s Certificate (other than an Officer’s Certificate pursuant to Section 3.05) or Opinion of Counsel with respect to compliance with a covenant or condition provided for in this Indenture will include:

(A)          a statement that the signatory thereto has read such covenant or condition;

(B)          a brief statement as to the nature and scope of the examination or investigation upon which the statements or opinions contained therein are based;

(C)          a statement that, in the opinion of such signatory, he, she or it has made such examination or investigation as is necessary to enable him, her or it to express an informed opinion as to whether or not such covenant or condition has been satisfied; and

(D)          a statement as to whether, in the opinion of such signatory, such covenant or condition has been satisfied.

Section 12.04. Rules by the Trustee, the Registrar and the Paying Agent.

The Trustee may make reasonable rules for action by or at a meeting of Holders. The Registrar or Paying Agent may make reasonable rules and set reasonable requirements for its functions.

Section 12.05. No Personal Liability of Directors, Officers, Employees and Shareholders.

No past, present or future director, officer, employee, incorporator or shareholder of the Company or any Guarantor, as such, will have any liability for any obligations of the Company or any Guarantor under this Indenture, the Notes or the Guarantees or for any claim based on, in

- 96 -

respect of, or by reason of, such obligations or their creation. By accepting any Note, each Holder waives and releases all such liability. Such waiver and release are part of the consideration for the issuance of the Notes.

Section 12.06. Governing Law; Waiver of Jury Trial.

THIS INDENTURE, THE GUARANTEES AND THE NOTES, AND ANY CLAIM, CONTROVERSY OR DISPUTE ARISING UNDER OR RELATED TO THIS INDENTURE, THE GUARANTEES OR THE NOTES, WILL BE GOVERNED BY AND CONSTRUED IN ACCORDANCE WITH THE LAWS OF THE STATE OF NEW YORK. EACH OF THE COMPANY, EACH GUARANTOR AND THE TRUSTEE IRREVOCABLY WAIVES, TO THE FULLEST EXTENT PERMITTED BY APPLICABLE LAW, ANY AND ALL RIGHT TO TRIAL BY JURY IN ANY LEGAL PROCEEDING ARISING OUT OF OR RELATING TO THIS INDENTURE, THE NOTES, THE GUARANTEES OR THE TRANSACTIONS CONTEMPLATED BY THIS INDENTURE , THE NOTES OR THE GUARANTEES.

Section 12.07. Submission to Jurisdiction.

Any legal suit, action or proceeding arising out of or based upon this Indenture or the transactions contemplated by this Indenture may be instituted in the federal courts of the United States of America located in the City of New York or the courts of the State of New York, in each case located in the City of New York (collectively, the “Specified Courts”), and each party irrevocably submits to the non-exclusive jurisdiction of such courts in any such suit, action or proceeding. Service of any process, summons, notice or document by mail (to the extent allowed under any applicable statute or rule of court) to such party’s address set forth in Section 12.01 will be effective service of process for any such suit, action or proceeding brought in any such court. Each of the Company, each Guarantor, the Trustee and each Holder (by its acceptance of any Note) irrevocably and unconditionally waives any objection to the laying of venue of any suit, action or other proceeding in the Specified Courts and irrevocably and unconditionally waives and agrees not to plead or claim any such suit, action or other proceeding has been brought in an inconvenient forum.

Section 12.08. No Adverse Interpretation of Other Agreements.

Neither this Indenture nor the Notes may be used to interpret any other indenture, note, loan or debt agreement of the Company or its Subsidiaries or of any other Person, and no such indenture, note, loan or debt agreement may be used to interpret this Indenture or the Notes.

Section 12.09. Successors.

All agreements of the Company in this Indenture and the Notes will bind its successors. All agreements of the Trustee in this Indenture will bind its successors.

Section 12.10. Force Majeure.

The Trustee and each Note Agent will not incur any liability for not performing, or any delay in performing, any act or fulfilling any duty, obligation or responsibility under this

- 97 -

Indenture or the Notes by reason of any occurrence beyond its control (including any act or provision of any present or future law or regulation or governmental authority, act of God or war, civil unrest, local or national disturbance or disaster, act of terrorism or unavailability of the Federal Reserve Bank wire or facsimile or other wire or communication facility).

Section 12.11. U.S.A. PATRIOT Act.

The Company acknowledges that, in accordance with Section 326 of the U.S.A. PATRIOT Act, the Trustee, like all financial institutions, in order to help fight the funding of terrorism and money laundering, is required to obtain, verify and record information that identifies each person or legal entity that establishes a relationship or opens an account with the Trustee. The Company agrees to provide the Trustee with such information as it may request to enable the Trustee to comply with the U.S.A. PATRIOT Act.

Section 12.12. Calculations; Determinations.

Unless otherwise provided in this Indenture, the Company will be responsible for making all calculations called for under this Indenture or the Notes, including determinations of the Last Reported Sale Price, the Daily Conversion Value, the Daily Cash Amount, the Daily Share Amount, the Daily VWAP, accrued interest on the Notes and the Conversion Rate and, in any event, neither the Trustee nor any Conversion Agent shall have any responsibility therefor.

The Company will make all calculations in good faith, and, absent manifest error, its calculations will be final and binding on all Holders. The Company will provide a schedule of its calculations to the Trustee and the Conversion Agent, and each of the Trustee and the Conversion Agent may rely conclusively on the accuracy of the Company’s calculations without independent verification. The Trustee will promptly forward a copy of each such schedule to a Holder upon its written request therefor at the sole cost and expense of the Company.

Section 12.13. Severability.

If any provision of this Indenture or the Notes is invalid, illegal or unenforceable, then the validity, legality and enforceability of the remaining provisions of this Indenture or the Notes will not in any way be affected or impaired thereby.

Section 12.14. Counterparts.

The parties may sign any number of copies of this Indenture. Each signed copy will be an original, and all of them together represent the same agreement. Delivery of an executed counterpart of this Indenture by electronically in portable document format or in any other format will be effective as delivery of a manually executed counterpart for all purposes.

Section 12.15. Table of Contents, Headings, Etc.

The table of contents and the headings of the Articles and Sections of this Indenture have been inserted for convenience of reference only, are not to be considered a part of this Indenture and will in no way modify or restrict any of the terms or provisions of this Indenture.

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Section 12.16. Withholding Taxes.

Each Holder of a Note agrees, and each beneficial owner of an interest in a Global Note, by its acquisition of such interest, is deemed to agree, that if the Company or other applicable withholding agent pays withholding taxes or backup withholding on behalf of such Holder or beneficial owner as a result of an adjustment to the Conversion Rate, then the Company or such withholding agent, as applicable, may, at its option, set off such payments against payments of cash or the delivery of other Conversion Consideration on such Note, any payments on the Ordinary Shares or sales proceeds received by, or other funds or assets of, such Holder or the beneficial owner of such Note.

Section 12.17. Trust Indenture Act Controls.

If any provision of this Indenture limits, qualifies or conflicts with another provision that is required to be included in this Indenture by the Trust Indenture Act, then required provision of the Trust Indenture Act will control.

[The Remainder of This Page Intentionally Left Blank; Signature Page Follows]

- 99 -

IN WITNESS WHEREOF, the parties to this Indenture have caused this Indenture to be duly executed as of the date first written above.

Aegerion Pharmaceuticals, Inc.
By:	/s/ Joseph Wiley
	Name: Joseph Wiley
	Title: President and Director

[Signature Page to Indenture]

Amryt Pharma Holdings plc
(to be renamed Amryt Pharma plc)
By:	/s/ Joe Wiley
	Name: Joe Wiley
	Title: Director

[Signature Page to Indenture]

Amryt Pharma plc
(to be renamed Amryt Pharma Holding Limited)
By:	/s/ Joe Wiley
	Name: Joe Wiley
	Title: Director

[Signature Page to Indenture]

AEGERION PHARMACEUTICALS HOLDINGS, INC.

/s/ Ailish Hogan

Ailish Hogan

President and Director

[Signature Page to Indenture]

Aegerion Pharmaceuticals Limited
By:	/s/ John McEvoy
	Name: John McEvoy
	Title: Director

[Signature Page to Indenture]

Amryt Pharma (UK) Limited
By:	/s/ Joe Wiley
	Name: Joe Wiley
	Title: Director

[Signature Page to Indenture]

GIVEN under the common seal of Amryt Pharmaceuticals DAC  and DELIVERED as a DEED
	By:	/s/ Joe Wiley
		Name: Joe Wiley
		Title:   Director
	By:	/s/ Ailish Hogan
		Name: Ailish Hogan
		Title:   Director/Secretary

[Signature Page to Indenture]

GIVEN  under  the common  seal of Amryt Research Limited and DELIVERED as a DEED
	By:	/s/ Joe Wiley
		Name: Joe Wiley
		Title:   Director
	By:	/s/ Ailish Hogan
		Name: Ailish Hogan
		Title:   Director/Secretary

[Signature Page to Indenture]

GIVEN under the common sealof Amryt Genetics Limited and DELIVERED as a DEED
	By:	/s/ Joe Wiley
		Name: Joe Wiley
		Title:   Director
	By:	/s/ Ailish Hogan
		Name: Ailish Hogan
		Title:   Director/Secretary

[Signature Page to Indenture]

GIVEN under the common seal of Amryt Lipidology Limited and DELIVERED as a DEED
	By:	/s/ Joe Wiley
		Name: Joe Wiley
		Title:   Director
	By:	/s/ Ailish Hogan
		Name: Ailish Hogan
		Title:   Director/Secretary

[Signature Page to Indenture]

GIVEN under the common seal of Amryt Endocrinology Limited and DELIVERED as a DEED
	By:	/s/ Joe Wiley
		Name: Joe Wiley
		Title:   Director
	By:	/s/ Ailish Hogan
		Name: Ailish Hogan
		Title:   Director/Secretary

[Signature Page to Indenture]

GLAS Trust Company LLC
By:	/s/ Yana Kislenko
Name: Yana Kislenko
Title:    Vice President

[Signature Page to Indenture]

EXHIBIT A

FORM OF NOTE

[Insert Global Note Legend, if applicable]

[Insert Private Placement Legend, if applicable]

AEGERION PHARMACEUTICALS, INC.

5.00% Convertible Senior Note due 2025

CUSIP No.:    [___] Certificate No. [____]

ISIN No.:        [___]

Aegerion Pharmaceuticals, Inc., a Delaware corporation, for value received, promises to pay to Cede & Co., or its registered assigns, the principal sum of [     ] dollars ($[     ]) [(as revised by the attached Schedule of Exchanges of Interests in the Global Note)]* on April 1, 2025 and to pay interest thereon, as provided in the Indenture referred to below, until the principal and all accrued and unpaid interest are paid or duly provided for.

Interest Payment Dates:	April 1 and October 1 of each year, commencing on April 1, 2020.
Regular Record Dates:	March 16 and September 16.

Additional provisions of this Note are set forth on the other side of this Note.

[The Remainder of This Page Intentionally Left Blank; Signature Page Follows]

*Insert bracketed language for Global Notes only.

A-1

IN WITNESS WHEREOF, Aegerion Pharmaceuticals, Inc. has caused this instrument to be duly executed as of the date set forth below.

	Aegerion Pharmaceuticals, Inc.
Date:	By:
		Name:
		Title:

A-2

TRUSTEE’S CERTIFICATE OF AUTHENTICATION

GLAS Trust Company LLC, as Trustee, certifies that this is one of the Notes referred to in the within-mentioned Indenture.

Date:	By:
		Authorized Signatory

A-3

AEGERION PHARMACEUTICALS, INC.

5.00% Convertible Senior Note due 2025

This Note is one of a duly authorized issue of notes of Aegerion Pharmaceuticals, Inc., a Delaware corporation (the “Company”), designated as its 5.00% Convertible Senior Notes due 2025 (the “Notes”), all issued or to be issued pursuant to an indenture, dated as of September 24, 2019 (as the same may be amended from time to time, the “Indenture”), among the Company, the Guarantors and GLAS Trust Company LLC, as trustee. Capitalized terms used in this Note without definition have the respective meanings ascribed to them in the Indenture.

The Indenture sets forth the rights and obligations of the Company, the Guarantors, the Trustee and the Holders and the terms of the Notes. Notwithstanding anything to the contrary in this Note, to the extent that any provision of this Note conflicts with the provisions of the Indenture, the provisions of the Indenture will control.

1.          Interest. This Note will accrue interest at a rate and in the manner set forth in Section 2.05 of the Indenture. Stated Interest on this Note will begin to accrue from, and including, September 24, 2019.

2.          Maturity. This Note will mature on April 1, 2025, unless earlier repurchased, redeemed or converted.

3.          Guarantees. The Company’s obligations under the Indenture and the Notes are fully and unconditionally guaranteed by the Guarantors as provided in Article 9 of the Indenture.

4.          Method of Payment. Cash amounts due on this Note will be paid in the manner set forth in Section 2.04 of the Indenture.

5.          Persons Deemed Owners. The Holder of this Note will be treated as the owner of this Note for all purposes.

6.          Denominations; Transfers and Exchanges. All Notes will be in registered form, without coupons, in principal amounts equal to any Authorized Denominations. Subject to the terms of the Indenture, the Holder of this Note may transfer or exchange this Note by presenting it to the Registrar and delivering any required documentation or other materials and any amounts required by the Indenture.

7.          Right of Holders to Require the Company to Repurchase Notes upon a Fundamental Change. If a Fundamental Change occurs, then each Holder will have the right to require the Company to repurchase such Holder’s Notes (or any portion thereof in an Authorized Denomination) for cash in the manner, and subject to the terms, set forth in Section 4.02 of the Indenture.

8.          Right of the Company to Redeem the Notes. The Company will have the right to redeem the Notes for cash in the manner, and subject to the terms, set forth in Section 4.03 of the Indenture.

A-4

9.           Conversion. The Holder of this Note may convert this Note into Conversion Consideration in the manner, and subject to the terms, set forth in Article 5 of the Indenture.

10.         When the Company May Merge, Etc. Article 6 of the Indenture places limited restrictions on the Parent’s and the Company’s ability to be a party to a Business Combination Event.

11.         Defaults and Remedies. If an Event of Default occurs, then the principal amount of, and all accrued and unpaid interest on, all of the Notes then outstanding may (and, in certain circumstances, will automatically) become due and payable in the manner, and subject to the terms, set forth in Article 7 of the Indenture.

12.         Amendments, Supplements and Waivers. The Company, the Guarantors and the Trustee may amend or supplement the Indenture or the Notes or waive compliance with any provision of the Indenture or the Notes in the manner, and subject to the terms, set forth in Article 8 of the Indenture.

13.         No Personal Liability of Directors, Officers, Employees and Shareholders. No past, present or future director, officer, employee, incorporator or shareholder of the Company or any Guarantor, as such, will have any liability for any obligations of the Company or any Guarantor under the Indenture, the Notes or the Guarantees or for any claim based on, in respect of, or by reason of, such obligations or their creation. By accepting any Note, each Holder waives and releases all such liability. Such waiver and release are part of the consideration for the issuance of the Notes.

14.         Authentication. No Note will be valid until it is authenticated by the Trustee. A Note will be deemed to be duly authenticated only when an authorized signatory of the Trustee (or a duly appointed authenticating agent) manually signs the certificate of authentication of such Note.

15.         Abbreviations. Customary abbreviations may be used in the name of a Holder or its assignee, such as TEN COM (tenants in common), TEN ENT (tenants by the entireties), JT TEN (joint tenants with right of survivorship and not as tenants in common), CUST (custodian), and U/G/M/A (Uniform Gift to Minors Act).

16.         Governing Law. THIS NOTE, AND ANY CLAIM, CONTROVERSY OR DISPUTE ARISING UNDER OR RELATED TO THIS NOTE, WILL BE GOVERNED BY AND CONSTRUED IN ACCORDANCE WITH THE LAWS OF THE STATE OF NEW YORK.

* * *

A-5

To request a copy of the Indenture, which the Company will provide to any Holder at no charge, please send a written request to the following address:

Aegerion Pharmaceuticals, Inc.

c/o Amryt Pharma pic

90 Harcourt Street

Dublin 2, Ireland

Attention: Chief Financial Officer

A-6

SCHEDULE OF EXCHANGES OF INTERESTS IN THE GLOBAL NOTE*

INITIAL PRINCIPAL AMOUNT OF THIS GLOBAL NOTE: $[____]

The following exchanges, transfers or cancellations of this Global Note have been made:

Date Amount of Increase
(Decrease) in
Principal Amount of
this Global Note Principal Amount of
this Global Note
After Such Increase
(Decrease) Signature of
Authorized
Signatory of Trustee

*Insert for Global Notes only.

A-7

CONVERSION NOTICE

AEGERION PHARMACEUTICALS, INC.

5.00% Convertible Senior Notes due 2025

Subject to the terms of the Indenture, by executing and delivering this Conversion Notice, the undersigned Holder of the Note identified below directs the Company to convert (check one):

 the entire principal amount of

 $____________* aggregate principal amount of

the Note identified by CUSIP No. ______________ and Certificate No. ______________.

The undersigned acknowledges that if the Conversion Date of a Note to be converted is after a Regular Record Date and before the next Interest Payment Date, then such Note, when surrendered for conversion, must, in certain circumstances, be accompanied with an amount of cash equal to the interest that would have accrued on such Note to, but excluding, such Interest Payment Date.

Date:
		(Legal Name of Holder)
	By:
		Name:
		Title:
	Signature Guaranteed:
	Participant in a Recognized Signature Guarantee Medallion Program
	By:
		Authorized Signatory

* Must be an Authorized Denomination.

A-8

FUNDAMENTAL CHANGE REPURCHASE NOTICE

AEGERION PHARMACEUTICALS, INC.

5.00% Convertible Senior Notes due 2025

Subject to the terms of the Indenture, by executing and delivering this Fundamental Change Repurchase Notice, the undersigned Holder of the Note identified below is exercising its Fundamental Change Repurchase Right with respect to (check one):

 the entire principal amount of

 $____________* aggregate principal amount of

the Note identified by CUSIP No. ______________ and Certificate No. ______________.

The undersigned acknowledges that this Note, duly endorsed for transfer, must be delivered to the Paying Agent before the Fundamental Change Repurchase Price will be paid.

Date:
		(Legal Name of Holder)
	By:
		Name:
		Title:
	Signature Guaranteed:
	Participant in a Recognized Signature Guarantee Medallion Program
	By:
		Authorized Signatory

* Must be an Authorized Denomination.

A-9

ASSIGNMENT FORM

AEGERION PHARMACEUTICALS, INC.

5.00% Convertible Senior Notes due 2025

Subject to the terms of the Indenture, the undersigned Holder of the within Note assigns to:

Name:

Address:

Social security or

tax identification

number:

the within Note and all rights thereunder irrevocably appoints:

as agent to transfer the within Note on the books of the Company. The agent may substitute another to act for him/her.

Date:
		(Legal Name of Holder)
	By:
		Name:
		Title:
	Signature Guaranteed:
	Participant in a Recognized Signature Guarantee Medallion Program
	By:
		Authorized Signatory

A-10

TRANSFEREE ACKNOWLEDGEMENT

The undersigned represents that it is purchasing the within Note for its own account, or for one or more accounts with respect to which the undersigned exercises sole investment discretion.

Dated:
(Name of Transferee)
By:
	Name:
	Title:

A-11

EXHIBIT B

FORM OF GLOBAL NOTE LEGEND

THIS IS A GLOBAL NOTE WITHIN THE MEANING OF THE INDENTURE HEREINAFTER REFERRED TO AND IS REGISTERED IN THE NAME OF THE DEPOSITARY OR A NOMINEE OF THE DEPOSITARY, WHICH MAY BE TREATED BY THE COMPANY, THE TRUSTEE AND ANY AGENT THEREOF AS THE OWNER AND HOLDER OF THIS NOTE FOR ALL PURPOSES.

UNLESS THIS CERTIFICATE IS PRESENTED BY AN AUTHORIZED REPRESENTATIVE OF THE DEPOSITORY TRUST COMPANY (“DTC”) TO THE COMPANY OR ITS AGENT FOR REGISTRATION OF TRANSFER, EXCHANGE OR PAYMENT, AND ANY CERTIFICATE ISSUED IS REGISTERED IN THE NAME OF CEDE & CO. OR IN SUCH OTHER NAME AS IS REQUESTED BY AN AUTHORIZED REPRESENTATIVE OF DTC (AND ANY PAYMENT HEREON IS MADE TO CEDE & CO. OR TO SUCH OTHER ENTITY AS IS REQUESTED BY AN AUTHORIZED REPRESENTATIVE OF THE DTC), ANY TRANSFER, PLEDGE OR OTHER USE HEREOF FOR VALUE OR OTHERWISE BY OR TO ANY PERSON IS WRONGFUL SINCE THE REGISTERED OWNER HEREOF, CEDE & CO., HAS AN INTEREST HEREIN.

TRANSFERS OF THIS GLOBAL NOTE WILL BE LIMITED TO TRANSFERS IN WHOLE, BUT NOT IN PART, TO NOMINEES OF DTC, OR TO A SUCCESSOR THEREOF OR SUCH SUCCESSOR’S NOMINEE, AND TRANSFERS OF PORTIONS OF THIS GLOBAL NOTE WILL BE LIMITED TO TRANSFERS MADE IN ACCORDANCE WITH THE RESTRICTIONS SET FORTH IN ARTICLE 2 OF THE INDENTURE HEREINAFTER REFERRED TO.

B-1

EXHIBIT C

FORM OF PRIVATE PLACEMENT LEGEND

THE NOTES EVIDENCED HEREBY HAVE NOT BEEN REGISTERED UNDER THE UNITED STATES SECURITIES ACT OF 1933, AS AMENDED (THE “SECURITIES ACT”), AND MAY NOT BE OFFERED, SOLD, PLEDGED OR OTHERWISE TRANSFERRED EXCEPT (A) (1) TO A PERSON WHOM THE SELLER REASONABLY BELIEVES IS A QUALIFIED INSTITUTIONAL BUYER WITHIN THE MEANING OF RULE 144A UNDER THE SECURITIES ACT PURCHASING FOR ITS OWN ACCOUNT OR FOR THE ACCOUNT OF A QUALIFIED INSTITUTIONAL BUYER IN A TRANSACTION MEETING THE REQUIREMENTS OF RULE 144A, (2) TO AN INSTITUTIONAL ACCREDITED INVESTOR IN A TRANSACTION EXEMPT FROM THE REGISTRATION REQUIREMENTS OF THE SECURITIES ACT, (3) PURSUANT TO AN EFFECTIVE REGISTRATION STATEMENT UNDER THE SECURITIES ACT, OR (4) TO THE COMPANY, ANY OF THE GUARANTORS OR ANY OF THEIR SUBSIDIARIES AND (B) IN ACCORDANCE WITH ALL APPLICABLE BLUE SKY LAWS OF THE STATES OF THE UNITED STATES, AND ANY SELLER AGREES THAT IT WILL DELIVER TO EACH PERSON TO WHOM THIS NOTE OR AN INTEREST HEREIN IS TRANSFERRED A NOTICE SUBSTANTIALLY TO THE EFFECT OF THIS LEGEND.

B-2

EX-10.7

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.7

Execution Version

University College Dublin, National University of Ireland

and-

AMRYT GENETICS LIMITED

LICENCE AGREEMENT

1

THIS LICENCE AGREEMENT is made BETWEEN:

(1) University College Dublin, National University of Ireland, dublin of Belfield, Dublin 4, Ireland (UCD) and

(2) AMRYT GENETICS LIMITED, a company registered in Ireland under number 622577 with the Companies Registration Office, whose registered office is 90 Harcourt Street, Dublin 2, Ireland (the Licensee),

(individually a Party and collectively, the Parties).

WHEREAS

1. The Parties wish to enter into this Agreement with effect from the Effective Date in accordance with the terms of this Agreement.

2. UCD has developed a polymer technology with applications in gene therapy, which is the subject of the Application (defined below).

3. The Licensee is a clinical stage specialty pharmaceutical company, which focuses on the development and commercialisation of medicines for the treatment for patients with rare and orphan diseases.

4. UCD has agreed to licence certain intellectual property to the Licensee on the terms set out in this Agreement.

5. The Parties intend to enter into a collaborative research agreement to further develop polymer technologies with applications in gene therapy subject to and in compliance with the terms of Clause 4 of this Agreement.

NOW, THEREFORE, IN CONSIDERATION OF THE MUTUAL COVENANTS SET FORTH HEREIN, THE PARTIES HEREBY AGREE AS FOLLOWS:

1. DEFINITIONS AND INTERPRETATION

1.1 In this Agreement (including the Schedules) and in the Recitals, unless the context otherwise requires or unless otherwise specified, the following terms shall have the following meanings:

Affiliate:	means in relation to either Party, any corporation, or other business entity which Controls, is Controlled by, or is under common Control with said Party.
Agreement:	means this document including its Schedules, as amended from time to time in accordance with Clause 13.12.
API:	means an active pharmaceutical ingredient of a dosage form intended to furnish pharmacological activity or other direct effect in the prevention, treatment or diagnosis of disease.
Application:	means the patent application as set out and further described at Part 1 of Schedule 1 attached hereto, and any amendments made to same in the course of the prosecution phase for such patent application, including any amendments required for the prosecution at

2

	nationalisation stage (as applicable), and any granted Patents issuing from such patent application.
Business Day:	means Monday to Friday (inclusive), except bank or public holidays in the Republic of Ireland and “Business Days” shall be construed accordingly.
Combination Product:	means a product that is sold in the form of a combination product containing (i) the Product and (ii) at least one other API, as separate molecular entity(ies) where such API does not comprise any component of the Product.
Commercially Reasonable Efforts	means exerting such efforts and employing such resources as would normally be exerted or employed by a reasonable Third Party company for a product of similar market potential at a similar stage of its product life, when utilising sound and reasonable scientific and business practice and judgment in order to develop and commercialise the Product in a timely manner.
“Control”, “Controlled by” and “under the common Control with”:	mean beneficial ownership of fifty percent (50%) or more of the share capital, stock or other participating interest having the right to vote or carrying the right to distribution of profits of that Party, as the case may be.
Effective Date:	14 March 2018.
Field:	means gene therapy products for the treatment of any and all disorders which, for the avoidance of doubt, will include all therapeutic uses in humans and animals.
First Commercial Sale:	means the first sale of the Product following the granting of a marketing authorisation by a regulatory agency in the applicable country of the Territory to a Third Party by Licensee, its sub-licensee or its Affiliates.
HPAE:	a Hyperbranched poly (β – amino ester).
Improvement:	means any IPR which constitutes an improvement, enhancement, alteration and/or modification in or to the Licensed IP in the Field.
IPR:	means any and all Patent, trade or other mark, registered design, design right, topography right, copyright, database right or any other right in the nature of any of the foregoing (or application, or right to apply for, any of the foregoing), and any invention, discovery, concept, idea, method improvement, design, technique, confidential process or information or know how, business or trade names, goodwill and all other intellectual property and rights of a similar or corresponding nature in each case subsisting anywhere in the world and whether registered, unregistered or unregisterable, and including all applications and the right

3

	to apply for any of the foregoing rights.
Know-How:	means the documented unpatented technical information, as set out and described at Part 2 of Schedule 1 attached hereto, insofar as same was created by Scientist and/or a Scientist’s Researcher (including [***]) prior to the Effective Date.
Licensed IP:	means (i) the Application, and (ii) the Know-How, insofar as same was created by or on behalf of UCD prior to the Effective Date.
Licensee Improvement:	means Improvements made by or on behalf of the Licensee during the Term, but for the avoidance of doubt excludes UCD Improvements.
Material Breach:	means a breach of this Agreement that is material in the widest sense of having a serious effect on the benefit which the terminating Party would otherwise derive from a substantial portion of this Agreement in respect to the performance of this Agreement in accordance with its terms.
Milestones	means the specific milestones events with respect to the to use the Licensed IP for the Purpose in the Field in the Territory, as set out at Schedule 2 hereto, as may be varied or amended on the written agreement of the Parties.
Net Receipts:	means the amount of the following payments (excluding value added tax where payable in respect of such amount) obtained by, or due to the Licensee, or its Affiliates from a Third Party in relation to: (i) the grant of sub-licences (including the grant of any option over a sub-license) of any of the Licensed IP such as: up-front, and milestone payments but for the avoidance of doubt excluding (a) milestone payments received by the Licensee or its Affiliates from a sub-licensee which constitute payment for research or development activities related to the Product, and (b) payments received by the Licensee or its Affiliates from a sub-licensee for the conduct of research and development activities related to the Product, in each case to the extent that such payments do not exceed a reasonable level of payment for the conduct of such research and development activities; and (ii) royalty payments in relation to the sale of the Products by a sub licensee. If the royalty payment received by the Licensee relates to a Combination Product, Net Receipts shall, subject to the

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provisions of Clause 6.1.3(e), be calculated by multiplying the royalties received by Licensee from the applicable sublicensee during the applicable reporting period by the fraction A/(A+B) where: “A” is the average sale price of the Product contained in the Combination Product when sold separately by the sublicensee in such country; and “B” is the average sale price of the other API included in the Combination Product when sold separately by its supplier in such country, in each case during the applicable reporting period or if sales of both the Product and/or other API did not occur in such country during such period, then in the most recent reporting period in which sales of both occurred. In the event that such average sale price cannot be determined for both the Product and the other API included in the Combination Product, the calculation of Net Receipts arising from such Combination Product shall subject to the provisions of Clause 6.1.3(e) be calculated by multiplying the royalties received by Licensee from the applicable sublicensee during the applicable reporting period by the fraction of C/(C+D) where: “C” is the fair market value of the Product, and “D” is the fair market value of the other API included in the Combination Product. In such event, the Parties shall negotiate in good faith to arrive at a determination of the respective fair market values of the Product and the other API included in the Combination Product. In circumstances where Net Receipts relate to license fees in relation to a Combination Product, the Parties shall negotiate in good faith to arrive at a determination of the respective fair market values of the Product and the other API included in the Combination Product.

Net Sales:

means the total of the gross amount invoiced for sales or other supply of the Product by the Licensee or its Affiliates to a Third Party (but for the avoidance of doubt excluding sales to Affiliates) in a usual arm’s length transaction from, less the following deductions from such gross amounts as are actually paid or accrued for by the Licensee, its sub-licensee or its Affiliates (as the case may be) including, without limitation: (i)            trade, cash or quantity discounts, retroactive price reductions or adjustments in each case actually granted, or billing corrections actually corrected and bad debts actually collected with respect to sales of Product; (ii)           amounts repaid, credits or allowances granted for damaged goods, defects, recalls, returns or

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rejections of the Product(s);

(iii)          taxes paid by or charged to the account of the Licensee or Affiliates including without limitation; rebates and similar payments made with respect to sales paid for by any Third Party, governmental or regulatory authority, sales tax, value added tax, import/export duties or other similar governmental taxes or charges imposed on sales of the Product(s) but excluding national, state or local taxes based on income);

(iv)          commercially reasonable fees payable to a distributor with respect to sales and distribution of the Products, subject to such fees being normal and standard within the Field in the applicable countries of the Territory; and

(v)           freight, postage, shipping, customs duties and insurance charges to the extent included in the gross amount invoiced.

In relation to a Combination Product, ‘Net Sales’ shall subject to the provisions of Clause 6.1.2(c) be calculated by multiplying the actual Net Sales of such Combination Product during the applicable reporting period, by the fraction A/(A+B) where: “A” is the average sale price of the Product contained in the Combination Product when sold separately by the Licensee or its Affiliates in such country; and “B” is the average sale price of the other API included in the Combination Product when sold separately by its supplier in such country, in each case during the applicable reporting period, or if sales of both the Product and/or other API did not occur in such country during such period, then in the most recent reporting period in which sales of both occurred.

In the event that such average sale price cannot be determined for both the Product and the other API included in the Combination Product, Net Sales for the purpose of determining royalty payments shall be calculated by multiplying the Net Sales of the Combination Products by the fraction of C/(C+D) where “C” is the fair market value of the Product and “D” is the fair market value of the other API included in the Combination Product. In such event, the Parties shall negotiate in good faith to arrive at a determination of the respective fair market values of the Product and the other API included in the Combination Product.

Products provided by the Licensee free of charge, for administration to patients enrolled in clinical trials or distributed through a not-for-profit foundation at no charge to eligible patients shall not be included in Net

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	Sales, provided that the Licensee receives no consideration from such not-for-profit foundation or from such clinical trials or such use of Products.
Orphan Drug Exclusivity:	means granted orphan drug exclusivity by the applicable governmental agency, where an application has been made by the Licensee or an Affiliate for orphan drug designation for the applicable Product.
Patents:	means design and utility patent applications and patents including, without limitation, provisional patent applications, continuations, continuations-in-part, divisional applications and patents, reissues, re-examinations and revalidations of same, and extensions of any patents, patents of addition, SPCs and the like, and any foreign counterparts or equivalent protection rights in respect thereof.
Patent Rights:	means the following Patents which are owned and/or become owned by UCD: (i)          the Application.
Phase I Study:	means a first in healthy human volunteer study.
Phase IIa Proof of Concept Study:	means a study in a small number of patients with the target disease that demonstrates the biological proof of concept.
Phase IIb Study:	means a dose ranging study conducted in patients with the target disease.
Product:	means one or more gene therapy products incorporating or using a HPAE, which incorporate or utilise, or the development, manufacture, use or supply of are covered by any Valid Claim, or which makes use of any of the Know-How, and “Products” shall be construed accordingly.
Purpose:	means to use the Licensed IP to develop, manufacture, have manufactured, use, and sell, market and/or otherwise supply the Product.
Reasonable Commercial Terms:	means such terms and conditions as would usually be found in a licence of the IPR in question in an equivalent arm’s length transaction.
Report:	means the report as set out and defined in Clause 5.4, detailing the progress made and steps taken by the Licensee during the previous twelve (12) months in respect of the Licensed IP in connection with the Purpose in the Field in the Territory, and which shall contain such financial, technical and other information, and in such a format, as is reasonably requested by UCD.

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Reporting Period:	means (i)            with respect to the first Reporting Period, the period commencing on the Effective Date and ending on 31 December 2018; and (ii)           with respect to each subsequent Reporting Period, a period of twelve (12) months commencing on 1 January 2019 and each subsequent anniversary of that date.
Scientist:	means [***] while employed by UCD and acting as principal investigator, and such principal investigator, appointed to replace [***] should [***] cease to act in such role.
Scientist’s Researcher:	means any researcher working under the direction of Scientist, while any such researcher is either employed or engaged by UCD (including as an employee, contractor or consultant).
SPC:	means a supplementary protection certificate.
Term:	means the term of this Agreement as set out in Clause 10.1.
Territory:	means worldwide.
Third Party:	means any individual or entity which is neither a Party, or an Affiliate of a Party, and “Third Parties” shall be construed accordingly.
UCD Improvement:	means any and all Improvements made by and/or on behalf of the Scientist and/or a Scientist’s Researcher during the Term, but which for the avoidance of doubt excludes Licensee Improvements.
Valid Claim:	means a claim of any issued, unexpired Patent Rights that has not been revoked or held unenforceable or invalid by a decision of a court or governmental agency of competent jurisdiction from which no appeal can be taken, or with respect to which an appeal is not taken within the time allowed for appeal, and that has not been disclaimed, dedicated to the public or otherwise rendered unenforceable.

1.2 Except as otherwise provided, any references in this Agreement to clauses, paragraphs, schedules and/or Parties are references to the clauses, paragraphs, schedules and/or Parties to this Agreement.

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1.3 Where applicable references to the singular shall include the plural and vice versa and reference to any gender shall include other genders.

1.4 The division of this Agreement to Clauses and sub-Clauses, and the headings used in this Agreement, are for convenience only, and shall not affect the interpretation of this Agreement.

1.5 In this Agreement, unless otherwise specified, any reference to writing includes fax transmission and email, but excludes SMS and similar means of communication.

1.6 This Agreement and all rights and obligations hereunder shall for all purposes be treated and construed as being separate and apart from any other agreement or agreements or any rights or obligations thereunder save only insofar as the express provision requires to the contrary.

1.7 In this Agreement, any phrase introduced by the words include, including, includes and such as are to be construed as illustrative, and shall not limit the sense of the words preceding those words.

1.8 In this Agreement, unless otherwise specified, any reference to a statute or statutory provision includes a reference to the statute or statutory provision as modified or re-enacted, or both, from time to time, and to any subordinate legislation made under it.

1.9 Any reference to a person shall be construed as a reference to any individual, firm, company, corporation, government, state or agency of a state or any association or partnership (whether or not having separate legal personality) of two or more of the foregoing.

2. GRANT OF LICENCE

2.1 Subject to Clause 2.5, UCD hereby grants to the Licensee, and the Licensee hereby accepts with effect from the Effective Date:

2.1.1 an exclusive licence to the Patent Rights for the Purpose in the Field in the Territory, subject to Clause 2.5;

2.1.2 a non-exclusive licence to the Know-How for the Purpose in the Field in the Territory;

2.2 During the period of [***] commencing on the Effective Date, subject to the provisions of Clause 2.5, UCD shall not grant a licence to the Know How to any Third Party to develop or commercialise a Product in the Field in the Territory.

2.3 On the expiry of the Term, on a Product by Product and country by country basis, the Licensee may at its option notify UCD that it wishes to elect to negotiate a non-exclusive licence to the Know-How in the Field and in the applicable countries of the Territory and such licence shall be on terms to be negotiated in good faith between the Parties, having regard to the applicable provisions of the Agreement, and subject to the negotiation and conclusion of a separate agreement in writing between the Parties.

2.4 The Licensee may grant sub-licenses of the rights set out at Clause 2.1, provided that:

2.4.1 the Licensee shall provide UCD with a copy of each sub-license within thirty (30) Business Days after its grant;

2.4.2 to the extent applicable the Licensee shall ensure that there are included in the terms of any sub-license the obligations on the sub-licensee which are equivalent to the obligations contained in this Agreement, including in particular (but not limited to) Clause 3.2 (confidentiality of Know-How) and Clause 7;

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2.4.3 all sub-licenses shall terminate immediately on the termination of this Agreement for any reason;

2.4.4 the payment terms of any sub-licenses shall be on terms equivalent to those that should be included in a customary arm’s length transaction;

2.4.5 all sub-licenses shall be personal to the sub-licensee and not capable of assignment or sub-sub-licensing without UCD's prior written consent; and

2.4.6 the Licensee shall at all times indemnify UCD and keep UCD indemnified against any and all actions, proceedings, costs, claims, damages, expenses and/or liabilities arising any act or omission of any sub-licensee.

2.5 Notwithstanding (i) the exclusive licence granted by UCD to the Licensee pursuant to Clauses 2.1.1 and (ii) the provisions of Clause 2.2, the Licensee agrees and acknowledges that, subject to the provisions of Clause 3.3, UCD will at all times have an irrevocable, perpetual, royalty-free, worldwide right to use the Licensed IP for the purposes of academic teaching, publication (subject to Clauses 4.3.2 and 7.3, as applicable) and non-commercial research, including the right for UCD to sub-license to research collaborators (whether pursuant to a research grant or otherwise) for non-commercial research, provided that such research collaborators shall be under equivalent obligations of confidentiality as Clause 7. Subject to the provisions of Clause 4.3, any other access to (i) the Patent Rights in the Field in the Territory, and (ii) the Know-How in the Field in the Territory for five (5) years commencing on the Effective Date in accordance with Clause 2.2 will require the prior written consent of the Licensee which it may withhold at its sole discretion.

2.6 No licence is granted to the Licensee other than as expressly stated in this Clause 2. UCD reserves all other rights under the Licensed IP.

2.7 The Licensee and UCD shall execute such licences as may be necessary or appropriate for registration with intellectual property offices, patent offices or other relevant authorities in particular countries or regions of the Territory. In the event of any conflict in meaning between any such licence and the provisions of this Agreement, the provisions of this Agreement shall prevail. Prior to the execution of the formal licence(s) (if any) referred to in this Clause 2.7 the Parties shall so far as possible have the same rights and obligations towards one another as if such licence(s) had been granted. The Parties shall use reasonable endeavours to ensure that, to the extent permitted by the relevant authorities, this Agreement shall not form part of any public record.

2.8 The Licensee may enter into one or more sub-contracts for the Products for the Purpose in the Field in the Territory, provided that:

2.8.1 any sub-contractor shall first enter into a confidentiality agreement with the Licensee and containing terms similar to, and no less onerous than, (and in respect to observing the obligations under) the provisions in Clause 7 of this Agreement, in relation to the disclosure of Confidential Information to the sub-contractor and the Licensee shall procure that any subcontractor duly observes and complies with such obligations;

2.8.2 the Licensee procures that the sub-contractor acts in accordance with the obligations on the Licensee pursuant to this Agreement.

2.8.3 the Licensee shall be liable for all acts and omissions of any sub-contractor.

3. PROVISION OF KNOW-HOW AND TECHNICAL ASSISTANCE

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3.1 UCD will provide the Know-How to the Licensee as soon as practicable after the Effective Date, subject to the Licensee complying with Clause 3.2.

3.2 In consideration of the disclosure by UCD of the Know-How, the Licensee undertakes and agrees:

3.2.1 to keep the Know-How secret and in strict confidence and to use the Know-How only for the Purpose in the Field in the Territory and in the manner as set out in this Agreement, and for no other purpose;

3.2.2 to only disclose the Know-How to such employees, contractors or sub-licensees as require same for the Purpose in the Field in the Territory and provided said employees, contractors or sub-licensees have undertaken in writing to comply with the terms of this Agreement.

3.2.3 not to make or use any copies, synopses or summaries of any of the Know-How, except such as are strictly necessary for the Licensee’s internal communications in connection with the Purpose in the Field in the Territory, or as are strictly necessary for the Purpose;

3.2.4 at UCD’s written request, and in any event upon termination or expiration of this Agreement, to return to UCD (or, if requested, destroy or erase) all documents (and copies thereof) and other materials concerning or incorporating, or in any way recording any of the Know-How, in whatever form, which are in its possession or control, and shall make no further use or disclosure of any of the Know-How;

3.2.5 to give notice to UCD of any unauthorised misuse, disclosure, theft or loss of the Know-How as soon as possible and, in any event, no later than five (5) Business Days after becoming aware of the same; and

3.2.6 that the obligations in this Clause 3.2 shall continue to be binding on it with respect to each piece of Know-How, for so long as it remains secret and confidential, it being recognised that any particular Know-How shall not be deemed to be publicly known merely because other Know-How contained in the same document or embodiment becomes publicly known.

3.3 UCD shall keep the Know-How secret and in strict confidence and shall procure that other licensees of the Know-How shall also do so.

4. IMPROVEMENTS

4.1 UCD shall own all rights in and to the UCD Improvements.

4.2 It is envisaged as at the Effective Date, that the Parties intend to enter into good faith negotiations during the Term with a view to the negotiation and conclusion of a collaborative research agreement as between the Parties, to further develop HPAE polymer technologies developed by the Scientist with application in the Field and that such an agreement shall regulate such collaborative research project and the access rights of Licensee to the foreground intellectual property generated pursuant to such collaborative research project. The provisions of this Clause 4.2 shall not be construed to obligate either Party to enter into such a collaborative research agreement. Furthermore, any failure on the part of the Parties, or either Party, to so enter into such an agreement following good faith negotiations shall not constitute a breach of this Agreement.

4.3 Strictly subject to Clause 4.3.4 below, it is agreed that during the Term:

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4.3.1 in the event that UCD is carrying out research in relation to HPAE polymer technology developed by the Scientist with application (including in conjunction with one or more additional technologies) in the Field, UCD shall prior to disclosure to any Third Party (other than a research collaborator) notify the Licensee of and, if and/or requested by Licensee, provide the Licensee with details in respect to IPR and data generated pursuant to such research and such other information as is reasonably required by Licensee for the Licensee to be able to evaluate such IPR and data, and that such information and/or access to and/or evaluation of such IPR and data shall be subject to the good faith negotiation and conclusion of a separate evaluation licence between the Parties in writing;

4.3.2 in the event that UCD proposes to publish the output of all or some of such research, UCD shall provide a copy of the proposed publication material to Licensee for review and comment at least thirty (30) Business Days prior to the proposed submission date for publication. UCD shall in good faith consult with Licensee and its patent advisers to consider recommended alterations or deletions to the proposed publication material that are reasonably appropriate in order to enable UCD to seek appropriate legal protection or registration of the IPR in respect of such output, or to facilitate the negotiation of a further agreement between the Parties. For the avoidance of doubt, UCD shall be under no obligation whatsoever to act in accordance with such recommendations of the Licensee and UCD may make such alterations or deletions in and/or at its sole discretion; and

4.3.3 UCD shall in good faith consider proposals made by Licensee to enter in to an evaluation licence and/or a commercialisation licence with regard to all or part of the IPR in respect to the development of the Licensed IP by the Scientist in the Field and such licences shall be subject to negotiation in good faith between the Parties and the conclusion of separate agreements in writing between the Parties. The provisions of this Clause 4.3.3 shall not be construed to obligate either Party to enter into such a licence. Furthermore, any failure on the part of the Parties, or either Party, to so enter into such a licence following good faith negotiations shall not constitute a breach of this Agreement.

4.3.4 The provisions of this Clause 4.3 shall be strictly subject to the extent that UCD is not prohibited by (i) law or (ii) any contractual or other obligation which UCD may have to any Third Party (including without limitation in respect to any contractual or other obligations in such funding agreements with one or more state agencies as may be applicable).

4.4 The Licensee shall own all rights in and to the Licensee Improvements. To the extent that it is not prohibited by law or by any contractual obligation to any Third Party, Licensee shall in good faith consider a request by UCD to grant to UCD a non-exclusive licence to use applicable Licensee Improvements for non-commercial research purposes only, on terms to be negotiated in good faith to include obligations of confidentiality consistent with the provisions of this Agreement.

5. OBLIGATIONS ON THE Licensee

5.1 The Licensee shall use Commercially Reasonable Efforts to develop, use and exploit the Licensed IP for the Purpose in the Field in the Territory.

5.2 Notwithstanding and without prejudice to the generality of Clause 5.1, the Licensee shall use Commercially Reasonable Efforts to achieve the Milestones by the dates as described in Schedule 2.

5.3 The Licensee shall consult with the UCD in relation to the progress being made to achieve the Milestones. Any updates and/or amendments to the Milestones shall be discussed in advance

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with UCD and any such updates and/or amendments shall be incorporated into this Agreement with the express written agreement of both Parties.

5.4 Without prejudice to the generality of the Licensee’s obligations pursuant to Clause 5.1, the Licensee shall provide annually to UCD an updated, written statement (the Report), at the same time as the royalty statements outlined in Clauses 6.9 and 6.10, which shall include details of:

5.4.1 the progress made and steps taken by the Licensee, its Affiliates and the sub-licensees since the Effective Date or the date of the previous Report (as defined and pursuant to this Clause 5.4) in respect to the Purpose in the Field in the Territory, including in respect to regulatory approvals and/or regulatory applications;

5.4.2 set out the dates on which the applicable Milestone(s) have been achieved and, where applicable, report on the differences between such dates and the dates as described in Schedule 2 (as may be amended) and the reasons for those differences; and

5.4.3 Licensee’s plans for the next twelve (12) months in relation to the Product.

5.5 Licensee shall meet UCD to discuss the contents of such Report, and will answer such questions as may be asked by UCD in respect of the progress made by the Licensee and the steps taken pursuant to this Agreement. The Licensee acknowledges and agrees that UCD’s receipt or approval of any Report shall not be taken to waive or qualify the Licensee’s obligations under Clause 5.1.

5.6 Referral to Expert. If UCD reasonably considers on the basis of the Report that Licensee has, without legitimate reason having put Licensee on notice that it considers Licensee to have failed to comply with its obligations pursuant to Clause 5.1 and/or 5.2 and given Licensee reasonable time to address UCD’s concerns, failed to comply with its obligations pursuant to Clause 5.1 and/or 5.2, UCD shall be entitled to refer the following questions to an Expert (as defined in Schedule 3):

5.6.1 whether taking into account the provisions of Clause 5 and Schedule 2 the Licensee has complied with its obligations pursuant to Clause 5.1 and/or Clause 5.2 (as the case may be); and if not

5.6.2 what specific action the Licensee should have taken (Specific Action) in order to have so complied.

5.7 Appointment of Expert. The Expert shall be appointed in accordance with the provisions of Schedule 3 and his decision shall be final and binding on the Parties.

5.8 Consequences of Expert’s decision. If the Expert determines that the Licensee has failed to comply with its obligations pursuant to Clause 5.1 taking into account the provisions set out in Schedule 2, and if the Licensee fails to take the Specific Action within six (6) months of the Expert giving his decision, UCD shall be entitled, by giving, at any time within three (3) months after the end of that six (6) month period, not less than three (3) months’ notice to terminate this Agreement and the licences granted to the Licensee pursuant to Clause 2.

5.9 Licensee shall report to UCD the dates of the First Commercial Sale of a Product and the First Commercial Sale of each and every subsequent Product (that is each and every subsequent Product requiring a separate marketing authorisation such as pursuant to a New Drug Application) in each country within the Territory within [***] of their occurrence.

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5.10 The Licensee will ensure that the Products and the packaging associated with them are appropriately marked with any relevant Patent or patent application numbers to comply with the laws of each of the countries in which the Products are sold.

5.11 The Licensee shall procure that the manufacture of the Products are to the standard of quality and workmanship as is required by and to comply with all applicable laws and regulations in each country of the Territory, and the Licensee shall be responsible for compliance with all relevant regulatory and legal requirements in respect of the Licensed IP and Products.

6. PAYMENTS

6.1 Subject to Clause 6.2 hereto, in consideration of the rights granted pursuant to Clause 2 and Clause 3, the Licensee shall make payments to UCD in accordance with this Clause 6.1 as follows:

6.1.1 Upfront and Milestone Payments:

(a) an upfront payment of forty thousand Euro (€40,000) [***] of the Effective Date;

(b) development milestone payment(s) of:

(i) one hundred thousand Euro (€100,000) on the successful completion of the first Phase IIa Proof of Concept Study; and

(ii) one hundred thousand Euro (€100,000) on successful completion of the first Phase IIb Study; and

(c) commercial milestone payment(s) of:

(i) two hundred thousand Euro (€200,000) when the First Commercial Sale of a Product has been achieved; and

(ii) two hundred thousand Euro (€200,000) in the case of the First Commercial Sale of each and every subsequent Product (that is each and every subsequent Product requiring a separate marketing authorisation such as pursuant to a New Drug Application).

All upfront and milestone payments as set out and specified in this Clause 6.1.1 are non-creditable and non-refundable.

(d) The milestone payments in Clause 6.1.1(b) shall only be payable if the applicable Product is covered by a Valid Claim in France, Germany, Italy, Spain, the United Kingdom, or the United States.

(e) The milestone payments in Clause 6.1.1(c) shall only be payable if (i) the sale of the applicable Product is covered by a Valid Claim, or (ii) there is Orphan Drug Exclusivity for such a Product in the country of sale.

6.1.2 Royalty Payments on Net Sales:

(a) Net Sales of Product as follows:

(i) two per cent (2%) on Net Sales in a calendar year up to one hundred million Euro (€100,000,000); and

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(ii) three per cent (3%) on Net Sales in a calendar year in excess of one hundred million Euro (€100,000,000).

(b) A royalty on Net Sales of the Product shall only be payable:

(i) if at the time of sale the sale of the Product is covered by a Valid Claim in the country of sale,

(ii) if at the time of sale there is Orphan Drug Exclusivity for the Product in the country of sale; or

(iii) in relation to the first Product to achieve First Commercial Sale in any country of the Territory, until the last to occur of (a) the expiration of the last Valid Claim covering the Product in the country of sale, (b) the expiration of Orphan Drug Exclusivity for the Product in the country of sale, or (c) fifteen years (15) from the date of the First Commercial Sale of such Product in any country of the Territory, provided that such period follows the expiration of the last Valid Claim in the applicable country of sale.

(c) If the royalty on Net Sales relates to a Combination Product, (i) the royalty rate in Clause 6.1.2(a)(i) shall not be reduced below [***], and (ii) the royalty rate in Clause 6.1.2(a)(ii) shall not be reduced below [***].

(d) The determination as to the applicable royalty rates in this Clause 6.1.2 shall be determined during every calendar year of the period set out in Clause 6.1.2.

6.1.3 Net Receipts:

(a) thirty per cent (30%) of Net Receipts received during that period of the Term prior to the initiation of a Phase I Study;

(b) twenty per cent (20%) of Net Receipts received during that period of the Term commencing upon the initiation of the Phase I Study and before the completion of a Phase IIa Proof of Concept Study;

(c) ten per cent (10%) of Net Receipts received during that period of the Term commencing upon the completion of a Phase IIa Proof of Concept Study.

(d) A royalty on Net Receipts in relation to royalties received by Licensee or an Affiliate from a sub-licensee shall only be payable:

(i) if at the time of sale by the sub-licensee the sale of the Product is covered by a Valid Claim in the country of sale;

(ii) if at the time of sale by the sub-licensee there is Orphan Drug Exclusivity for the Product in the country of sale; or

(iii) in relation to the first Product to achieve First Commercial Sale in any country of the Territory, until the last to occur of (a) the expiration of the last Valid Claim covering the Product in the country of sale, (b) the expiration of Orphan Drug Exclusivity for the Product in the country of sale; or (c) fifteen years (15) from the date of the First Commercial Sale of such Product in any country of the Territory, provided that such period follows the expiration of the last Valid Claim in the applicable country of sale.

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(e) If the royalty on Net Receipts relates to a Combination Product, the royalty rates in Clauses 6.1.3(a), (b) and (c) shall not be reduced by more than [***].

General Terms re Payments:

6.2 The consideration which the Licensee and/or its Affiliates shall charge Third Parties for the supply of Product shall be on an arm’s length transaction basis on Reasonable Commercial Terms. If any prices are not charged on this basis, the Net Sales shall be calculated based on the price which would have been charged on an arm’s length transaction basis.

6.3 Subject to the provisions of Clause 6.4, UCD shall be responsible for and shall bear all taxes levied upon payments received by it, and UCD hereby authorises Licensee to withhold such taxes from the payments which are payable to UCD in accordance with this Agreement if Licensee is either required to do so pursuant to the applicable tax laws or directed to do so by any agency of the relevant government. Upon the written request of UCD, Licensee shall, with respect to the laws of the country from which the payments are made, reasonably support UCD in its legal efforts to minimise any such withholding taxes, and provide UCD with information about any and all documentation reasonably required to enable UCD to obtain appropriate relief in respect to the payment in question and, if this is not possible, to facilitate the reduction of the withholding to a legal minimum and on request to provide UCD with receipts and any other evidence from relevant revenue authorities which may be required by UCD for its own tax affairs in this respect.

6.4 In the event that the Licensee ceases to be tax resident in Ireland, the Parties shall in good faith review the provisions of Clause 6.3 and negotiate such amendments to Clause 6.3 as are fair and equitable between the Parties.

6.5 All amounts payable by the Licensee pursuant to of this Agreement shall be made in Euro, and shall be payable by cheque or by bank transfer to the account of UCD as set out in Annex 1:

6.6 All amounts payable to UCD pursuant to Clauses 6.1.2 and 6.1.3 shall be payable annually within [***] from the end of each Reporting Period and include payment of all sums payable or receivable in respect of that Reporting Period.

6.7 Any exchange of currency made to calculate sales or payments for the purpose of this Clause 6 will be determined as at the last Business Day of each Reporting Period with respect to which the sales or payments are made, using the average of the average daily buying and selling rates of such other currency as quoted by Bank of Ireland in Dublin during that Reporting Period.

6.8 If the Licensee fails to make any payment due to UCD pursuant to this Agreement by the due date, without prejudice to any other right or remedy available to UCD, UCD may charge interest both before and after any judgment) on the amount outstanding, on a daily basis at a rate equivalent to of [***] per annum above the ECB Rate from time to time in force. Such interest will be calculated from the payment due date to the actual date of payment, both dates inclusive. The Licensee will pay such interest to UCD on demand.

Financial Reporting:

6.9 When making any payments to UCD pursuant to this Agreement, the Licensee shall also contemporaneously notify NovaUCD as per UCD’s perspective representative as in accordance with clause 13.2 in writing of said payment, and said notification shall be accompanied by a report sent to NovaUCD (hereinafter Financial Reports), certified as true and accurate by an authorised officer of the Licensee, showing such information as UCD may reasonably request to enable UCD to calculate the sums due to UCD pursuant to this Agreement, including, without

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limitation, details of how the following were calculated in respect of the payments made for the Reporting Period in question: gross revenue receivable by the Licensee, the sources of that revenue, the deductions made to calculate Net Sales, and all sums due to UCD.

6.10 The Licensee shall, and shall procure that its Affiliates and its sub-licensees (as applicable), keep accurate records and books of account (hereinafter Records and Accounts) showing details of all activities in respect of the Licensed IP for the Purpose in the Field in the Territory and such other information as would be reasonably necessary for UCD to be able to confirm the calculation of Net Sales and Net Receipts including, without limitation, details of sums receivable by the Licensee in respect of the Licensed IP for the Purpose in the Field in the Territory, (including in respect of Products made or sold), for at least [***] following the dates of the underlying transaction, and will permit UCD or its agents to inspect and audit these records and books, who shall also be entitled to take copies of or extracts from the same. In the event that any such inspection or audit reveals a discrepancy of greater than [***] between the sums paid from those payable to UCD pursuant to this Agreement, the Licensee shall, save where the determination of such inspection or audit is disputed in good faith by Licensee (acting reasonably), promptly (i) make up any shortfall plus interest at a rate of [***] per annum above the ECB Rate from time to time in force, and (ii) reimburse UCD in respect of any reasonable professional charges incurred for such audit or inspection. The provisions of this Clause 6.10 shall continue to apply notwithstanding termination or expiry of this Agreement for whatever reason, until the payment of all outstanding sums due to UCD.

7. CONFIDENTIALITY

7.1 Confidential Information shall mean:

7.1.1 the provisions of this Agreement and information, know-how or data that is disclosed by one Party to the other Party for the purposes of this Agreement before, on or after the date of this Agreement relating to the operations, business, research of a Party which if disclosed in writing shall be marked ‘Confidential’, or if first disclosed otherwise, shall within thirty (30) Business Days of such disclosure be reduced to writing by the Party, as the case may be, and sent to the other Party. For the avoidance of doubt Know-How shall be treated as Confidential Information of UCD and shall be subject to the applicable confidentiality restrictions in Clause 3. Furthermore, all information relating to the Application disclosed by UCD to the Licensee shall be treated as Confidential Information of UCD and the provisions of this Clause 7 shall be construed accordingly;

7.1.2 Confidential Information shall exclude information which:

(a) is publicly available or subsequently becomes publicly available other than in a breach of this Agreement;

(b) the recipient can demonstrate by way of contemporaneous written documentation was already lawfully known to the recipient on a non-confidential basis before being disclosed by the recipient; or

(c) is rightly acquired from a Third Party who is not in breach of an agreement to keep such information confidential

7.1.3 Subject to the provisions of Clause 7.2, each Party shall (i) keep the Confidential Information confidential at all times; (ii) not disclose the Confidential Information or allow it to be disclosed in whole or in part to any Third Party without the prior written consent of the other Party such consent not to be unreasonably withheld, conditioned or delayed, except in accordance with

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the provisions of this Agreement; (iii) not to use the Confidential Information in whole or in part for any purpose, other than for the sole purpose of performing its obligations under this Agreement; (iv) to take all proper and reasonable measures to ensure the confidentiality of the Confidential Information; (v) to inform the other Party immediately if it becomes aware of the possession, use, or knowledge of any of the Confidential Information by an unauthorized person, and to provide any reasonable assistance in relation to such unauthorized possession, use, or knowledge that the other Party shall require; (vi) to refrain from using the Confidential Information for any purposes outside the scope of this Agreement either for the other Party’s benefit or for the benefit of any of its Agents; and (vii) not disclose Confidential Information of the other Party to others (except to its employees, agents or consultants who are bound to the applicable Party by a like obligation of confidentiality) without the express written permission of other Party, except that the Party shall not be prevented from using or disclosing any of the Confidential Information of the other Party that:

(a) such Party can demonstrate by written records was previously known to it;

(b) is now, or becomes in the future, public knowledge other than through acts or omissions of such Party;

(c) is lawfully obtained by such Party from sources independent of the other Party; or

(d) is required to be disclosed by law, a court of competent jurisdiction or a relevant stock exchange, in which case the Party will (i) inform the other Party of the full circumstances of the disclosure and the information that shall be disclosed; (ii) consult with the other Party prior to such disclosure as to possible steps to avoid or limit disclosure and take those steps where they would not result in significant adverse consequences; (iii) ask the court or other public body to treat the Confidential Information as confidential; and (iv) in the event disclosure of the Confidential Information is made, only disclose the minimum amount necessary to comply with the court or public body’s request.

7.2 The Financial Reports, Records and Accounts and Report shall be treated as Confidential Information of the Licensee, but notwithstanding the provisions of Clause 7.1, UCD may identify the sums received from the Licensee in UCD’s annual report and similar publications, in consideration of the disclosure by the Licensee of the Financial Reports, Records and Accounts and Report to UCD, provided that UCD agrees:

7.2.1 to keep such Financial Reports, Records and Accounts and Report strictly confidential and to use such Financial Reports, Records and Accounts and Reports solely in connection with and for the purposes of this Agreement and in the manner as set out in this Agreement, and for no other purpose;

7.2.2 to only disclose such Financial Reports, Records and Accounts and Report (in relation to the financial aspects of the Report only) (and for the avoidance of doubt, unless otherwise required by a funding agency, ensuring all technical and other information relating to the Licensed IP is not disclosed) to such employees, contractors or sub-licensees as require same only for the purposes of this Agreement;

7.3 In relation to the proposed publication of material relating to the Licensed IP:

7.3.1 UCD shall provide a copy of proposed publication material relating to the Licensed IP to the Licensee for review and comment at least [***] prior to the proposed submission date for publication (Review Period);

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7.3.2 during the Review Period UCD shall in good faith consult with Licensee and its patent advisers to consider recommended alterations or deletions to the proposed publication material that are reasonably appropriate in order to enable UCD to seek appropriate legal protection or registration of such Licensed IP, but UCD shall not be obliged whatsoever to make such alterations or deletions, and such alterations or deletions may be made by UCD in and/or at UCD’s sole discretion;

7.3.3 if, during the Review Period, the Licensee (acting reasonably) requests alterations to the proposed publication material so that it does not disclose any (i) Confidential Information of the Licensee, or (ii) proprietary information owned by Licensee, the publication will be amended as requested; and

7.3.4 if, during the Review Period, the Licensee reasonably requests it and to the extent the proposed publication material contains IPR owned by the Licensee, UCD may also be required by the Licensee to delay publication for a period not exceeding [***] in order to enable the Licensee (as the case may be in accordance with this Agreement) to seek appropriate legal protection or registration of such IPR as owned by the Licensee.

7.4 The obligations of each Party with respect to Confidential Information shall continue for a period ending [***] from the termination or expiry of this Agreement, excepting in respect to Know-How, which shall be treated as Confidential Information of UCD and which is and shall be subject to the confidentiality and secrecy restrictions as set out in and in accordance with Clause 3 and which obligations shall continue to be binding on the Parties (as applicable) in accordance with the relevant provisions of Clause 3.

8. MANAGEMENT AND PROTECTION OF LICENSED IP

Management of Licensed IP:

8.1 Within [***] of the Effective Date, Licensee shall reimburse UCD in respect of all of the vouched Third Party costs and expenses UCD has incurred for the filing, prosecuting, maintaining, protecting and all other actions and costs in respect of the Application, including the professional and associated costs therewith (Past Patent Costs). Such Past Patent Costs shall be paid by the Licensee to the UCD within [***] of receiving an invoice from and/or on behalf of the UCD, dated after the Effective Date. During the Term, the Licensee will be entitled to and shall be responsible for taking, and shall take, all actions and pay all costs associated with the filing, prosecution, maintenance, protection and all other actions and costs in respect of the Application, including the professional and associated costs therewith.

8.2 During the Term, the Licensee, shall appoint and be the point of contact with patent agents mutually agreed between the Licensee and UCD in respect of all aspects of filing, prosecuting and maintaining the Patent Rights, but. Licensee shall ensure UCD receives a copy of material correspondence to and from the relevant patent offices and shall consult with UCD in respect of the filing, prosecution and maintenance of the Patent Rights, including the designation of all countries, unless the Parties agree otherwise in writing.

8.3 UCD shall provide the Licensee all reasonable assistance in the filing, and prosecution of the Patent Rights, and the maintenance of the Patent Rights when granted, at the expense of the Licensee.

8.4 The Licensee and UCD shall cooperate fully in the preparation, filing, prosecution and maintenance of the Patent Rights in the Territory by executing all papers and instruments so as

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to enable the Licensee to apply for, to prosecute and to maintain patent applications and Patents in UCD’s name in any country, including the execution of documents by applicable employees and students of UCD. Each Party shall promptly notify the other Party as to all matters which come to its attention and which may affect the filing, prosecution, maintenance and protection of the Patent Rights.

8.5 UCD shall provide such assistance as is reasonably required by the Licensee to obtain all available SPC(s) in relation to the Patent Rights. If a Party has an option to extend the patent term for only one of several granted Patents pursuant to the Patent Rights, the Parties will consult before making the election. If more than one granted Patent pursuant to the Patent Rights is eligible for extension, SPC or patent term restoration, the Parties will discuss and agree upon a strategy that will maximise patent protection for the Patent Rights. All filings of such SPC(s) will be the responsibility of the Licensee with all reasonable assistance from UCD and shall be owned by UCD.

8.6 The Licensee shall pay the costs of all SPC filings and payment shall be made by the Licensee directly to the patent agent mutually agreed between the Licensee and UCD to be responsible for the drafting and filing of such SPCs.

8.7 Licensee agrees and acknowledges that, save as specified in this Clause 8, UCD shall be under no other obligation to do any other acts or things, or pay any sums, to prosecute, maintain, defend or in any other way protect the Patent Rights.

8.8 Notwithstanding Clause 8.2, Licensee shall not permit any Patent Rights (or if granted, any Patent) to lapse or be abandoned in [***], without giving UCD at least thirty (30) Business Days’ notice prior to any such lapse or abandonment, of its intention to do so, in order that UCD has an opportunity to assume full responsibility for the continued prosecution and maintenance of same in the applicable country or countries, including the payment of all costs associated therewith. In the event that the Licensee elects not to do any particular act specified in this Clause 8.8 and UCD elects (in its sole discretion) to take said action, then UCD’s rights in the Patent Rights for the applicable country or countries shall be deemed to be excluded from the licence granted to the Licensee in this Agreement, the Licensee’s responsibility for the future costs will terminate in respect of the Patent Rights in question in the applicable country or countries on the date of UCD’s receipt of the Licensee’s notice, and UCD shall have the sole and exclusive right to use and exploit all rights in the applicable Patent Rights in the applicable country or countries. It is understood that UCD shall have the right, but not the obligation, to assume such responsibility under this provision.

8.9 Infringement by Third Parties

8.9.1 The Licensee shall promptly give UCD full information of any actual, threatened or suspected infringement by a Third Party of the Licensed IP or of any declaratory judgment, opposition, or similar action alleging the invalidity, unenforceability or non-infringement of any of the Licensed IP including as follows: (i) that any aspects of the Licensed IP is being attacked whether through validity challenge, revocation application, nullity action, opposition, interference or otherwise; or (ii) that any application for a Patent is made by, or any Patent is granted to, a Third Party by reason of which the Third Party in a Party’s opinion may be granted or may have been granted rights which conflict with any of the rights granted to the Licensee under the Licensed IP; or (iii) of any infringement/misappropriation or potential infringement/misappropriation of any of the Licensed IP; or (iv) that any application is made for a compulsory licence under any granted Patent (collectively Third Party Infringement) of which the Licensee becomes aware.

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8.9.2 If a Third Party Infringement occurs, then the Parties will consult in good faith with each other as to the appropriate course of action to be taken and to decide the best way to respond to such alleged infringement. The Licensee may, in its sole discretion, take such action as is commercially and legally reasonable in accordance with the advice and resources available to it with respect to the Third Party Infringement. In any event, before starting any legal action under this Clause 8.9.2, the Licensee shall consult with UCD as to the advisability of the action or settlement, its adverse effect on the good name of UCD, how the action should be conducted, and in addition, if the alleged infringement is both within and outside the Field, the Parties shall also co-operate with UCD’s other licensees (if any) in relation to any such action. Such action will be brought in the Licensee’s own name and at the Licensee’s cost, and the Licensee will retain all damages awarded or settlement amounts received to reimburse Licensee for all costs and expenses incurred relating to such proceedings which have not already been reimbursed in such court judgment. At the request of the Licensee, UCD shall provide reasonable assistance to the Licensee in connection therewith, including by executing reasonably appropriate documents, cooperating in discovery and joining as a party to the action.

8.9.3 The Licensee indemnifies UCD against all actions, claims, loss, damage, cost or expenses and awards, which UCD may incur, suffer or become liable to, arising out of or in connection with any proceedings or action taken by the Licensee in relation to a Third Party Infringement.

8.9.4 The Licensee shall keep UCD advised of the progress of such proceedings and provide UCD with copies of documents used in or prepared for such proceedings (subject to ensuring that any legal professional privilege in any documents is maintained).

8.9.5 If UCD wishes, in its sole discretion, to join or assist the Licensee in prosecuting such proceedings, UCD will notify the Licensee of such wish and in such event shall contribute to the costs and expenses of the proceedings as agreed between the Parties, and if no agreement is reached as to costs and expenses as between the Parties, then UCD shall have no obligation to contribute to the costs and expenses of the proceedings.

8.9.6 If the Licensee:

(a) determines it will not take action or, once some action has been engaged in, not commence proceedings, the Licensee will promptly notify UCD in writing that it is unwilling to take action or proceedings against a Third Party with respect to a Third Party Infringement; or

(b) fails to take any action or agree a course of action with UCD, within a period of [***] after notification of the Third Party Infringement pursuant to Clause 8.9.1,

unless the Parties agree alternative arrangements, then UCD may institute and prosecute an action in its own name, in which case UCD will retain all damages awarded or settlement amounts received.

8.10 Third Party claims

8.10.1 If proceedings are threatened or commenced by a Third Party against UCD, or the Licensee in the Territory on the ground that the exploitation of any Licensed IP or Product pursuant to this Agreement infringes IPR owned by a Third Party, then:

(a) the Party threatened or sued shall promptly notify the other Party and provide full information. The Parties shall promptly consult with each other in good faith;

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(b) the Parties will refer the matter to the Licensee’s patent lawyer for advice on the action, or settlement thereof and how the action should be conducted;

(c) each Party will co-operate in providing the necessary information and documents and attend meetings to assist the preparation of that advice; and

(d) the Licensee will pay for the cost of that advice.

8.10.2 Subject to Clause 8.10.1, if proceedings are threatened or commenced against the Licensee, the Licensee will, in consultation with UCD, be responsible for the defence of those proceedings.

8.10.3 A Party will not be required to defend any infringement proceedings brought by a Third Party where advice obtained pursuant to Clause 8.10.1(b) is to the effect that it would be imprudent to defend such proceedings.

8.10.4 Each Party agrees to keep the other Party advised of the progress of any proceedings in which it is a defendant and (subject to ensuring that any legal professional privilege in any documents is maintained) provide the other Party with copies of documents used in or prepared for such proceedings.

8.11 If during the Term of this Agreement, the Licensee acting in a commercially reasonable manner considers it necessary to obtain a licence from any Third Party (a Third Party Licence) in order to avoid infringing a Third Party’s Patent(s) resulting from the exploitation of the Patent Rights, the Licensee shall be entitled to offset [***] of the amount paid pursuant to the Third Party Licence against the royalty payments otherwise payable to UCD pursuant to Clauses 6.1.2 and 6.1.3 of this Agreement, provided that such royalty payments payable to UCD shall not be reduced by more than [***] of the royalty payment otherwise payable pursuant to Clauses 6.1.2 and 6.1.3 of this Agreement. The offset referred to in this Clause 8.11 shall only be made where the infringement of the Third Party’s Patents arises from the use of the Patent Rights in accordance with the provisions of this Agreement, and not from the use of any other IPR that the Licensee chooses to use in the manufacture or sale of any Product.

8.11.1 In the event that the Licensee is required to make a payment of any amount to a Third Party in connection with a Third Party claim resulting from the exploitation of the Patent Rights in order to avoid infringing a Third Party’s Patent(s), by way of damages, pursuant to a court order, settlement or otherwise, the Licensee shall be entitled to offset [***] of the amount paid pursuant to the Third Party Licence against the royalty payments otherwise payable to UCD pursuant to this Agreement, provided that the royalty payments payable to UCD shall not be reduced by more than [***] of the royalty payments otherwise payable.

8.11.2 The Licensee shall have the right to carry forward any amounts of the credit which are not or have not been off set against the royalty payments paid in any given calendar year to the following or subsequent calendar years (if applicable) as against payments otherwise payable by Licensee pursuant to Clause 6.1.2 or 6.1.3.

9. WARRANTIES AND LIABILITY

9.1 Each of the Parties acknowledges that, in entering into this Agreement, it has not relied on any warranty, representation or undertaking and each Party waives any claim for breach of any warranty, representation or undertaking which is not expressly contained in this Agreement as a warranty.

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9.2 Subject to the provisions of Clause 9.4 and Clause 9.6, Licensee acknowledges that UCD is providing the Licensed IP on an ‘as is’ basis, without warranty or representations, including, by way of example, without any warranty or representation:

9.2.1 as to merchantability, fitness for a particular purpose, accuracy, efficacy, completeness, capabilities or safety; or

9.2.2 as to the efficacy or usefulness of any of the Licensed IP; or

9.2.3 that the Application will proceed to grant or, if granted, shall be valid; or

9.2.4 that the use of any of the Licensed IP, or the manufacture, use or supply of any Products, or the exercise of any of the rights granted pursuant to this Agreement will not infringe any IPR or other rights of any other person; or

9.2.5 that any Products will be of satisfactory quality or fit for the purpose for which the Licensee (or its sub-licensees or customers) intended, and all warranties, conditions, terms, undertakings and obligations with respect to the Licensed IP and any Products, whether express or implied by statute, common law, custom, trade usage, course of dealing or in any other way, are hereby excluded to the greatest extent permitted by law.

9.3 Subject to the provisions of Clause 9.12, the total aggregate liability of UCD in respect of the Licensed IP or arising in any other way out of the subject matter of this Agreement shall not exceed the total amounts paid by Licensee to UCD pursuant to this Agreement.

9.4 Warranties by UCD

UCD represents and warrants to the Licensee that, except as otherwise disclosed by UCD in writing, and as far as UCD is aware (but without having carried out any searches or investigations in this regard, including without limitation in respect to the existence of any Third Party rights that may affect any of the Licensed IP, each of the following warranties is correct as at the Effective Date:

9.4.1 UCD is the owner of the Patent Rights and has the right to grant the licences pursuant to this Agreement to the Licensee;

9.4.2 all necessary licenses, consents, permits and authorities (public and private), have been obtained to enable UCD to properly perform its obligations pursuant to this Agreement;

9.4.3 the execution and performance of this Agreement by UCD does not breach any agreements that it has with Third Parties;

9.4.4 in relation to the agreement entered into by UCD on [***] with a Third Party pursuant to which UCD granted a non-exclusive licence to the certain of the Licensed IP to the Third Party to facilitate a project to be conducted in conjunction with [***] (as an SFI Fellow)(Fellowship Agreement), and such non-exclusive licence in respect to such Licensed IP granted to such Third Party will be terminated in accordance with and pursuant to the terms and conditions of such Fellowship Agreement, and that following such termination the said Third Party shall have no rights to the Licensed IP;

9.4.5 there is no action or proposed action by any other person to challenge, threaten or cancel any IPR in the Licensed IP;

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9.4.6 there is no claim against any person relating to or alleging infringement of any of the Licensed IP;

9.4.7 no claim or legal proceeding has been served on UCD, nor, is any claim or legal proceeding threatened, by any person alleging (and there is no basis for any claim, whether or not asserted) that:

(a) the Licensed IP are invalid or unenforceable; or

(b) the Licensed IP violates or infringes the IPR of any Third Party;

9.4.8 there are no judgments against UCD or settlements to which UCD is a party concerning the Licensed IP;

9.4.9 the Know-How was generated by employees of UCD, and in each case the terms of employment vested in UCD, all right, title, and interest in and to any intellectual property generated by them in respect of such Know-How; and UCD is not aware that any Third Party has rights in any Know-How that would adversely affect the Licensee’s rights pursuant to this Agreement.

9.5 Mutual warranties

Each Party warrants that at the Effective Date:

9.5.1 it has full corporate power and authority to enter into, perform and observe its obligations and duties pursuant to this Agreement and that the execution and performance of this Agreement by it has been duly and validly authorised by all necessary corporate action;

9.5.2 this Agreement and the transactions contemplated by this Agreement do not contravene its constituent documents or any law, regulation or official directive or any of its obligations or undertakings by which it or any of its assets are bound or cause a limitation on its powers or of its directors and officers to be exceeded; and

9.5.3 its obligations pursuant to this Agreement are valid and binding and enforceable against it in accordance with their terms.

9.6 Licensee’s warranties

9.6.1 The Licensee acknowledges and agrees that the Licensee, its Affiliates, and its Sub-licensees exploit the Licensed IP at their own risk.

9.6.2 The Licensee warrants to UCD that each sub-licence agreement will be enforceable in accordance with its terms.

9.7 Neither Party shall be liable to the other Party for any death or injury unless it is caused by the negligence of that Party or its agents.

9.8 Indemnity by the Licensee

The Licensee agrees to indemnify and hereby indemnifies and keeps indemnified UCD and its respective directors, officers, employees and agents (each an Indemnified Party) against all liability, loss, costs, damages or expense (including legal costs and expenses) incurred or suffered by UCD (except any claim caused by breach of this Agreement, negligence, malpractice, wrongful acts or omissions or breach of statutory duty of UCD), as a result of:

9.8.1 wilful misconduct, negligent act or omission or wilful failure to act on the part of the Licensee;

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9.8.2 the development, manufacture, use, marketing or sale of, or any other dealing in any of the Products by the Licensee or other entity involved in the development, manufacture, use, marketing or sale of, or any other dealing in any of the Products under its authority;

9.8.3 the use by a Third Party of any Product manufactured by the Licensee or other entities involved in the manufacture by the Licensee;

9.8.4 the use of any of the Licensed IP by the Licensee; and

9.8.5 and/or arising out of any act or omission of any sub-contractor, including (i) pursuant to the provisions of Clause 2.8, and (ii) in relation to any product liability claim relating to Products manufactured by the subcontractor.

9.9 Continuing obligations

The indemnities in this Agreement are enforceable as debts and are continuing obligations, independent from the other obligations pursuant to this Agreement and continue after this Agreement ends. Upon UCD becoming aware of any claim or other circumstance that may give rise to it seeking to rely on an indemnity set out in this clause, UCD shall provide the Licensee with full details of the action, claim, proceeding, or demand.

9.10 Enforcement of indemnities and conduct of claims

It is not necessary for UCD to incur expense or make payment before enforcing a right of indemnity conferred by this Agreement. Before making any demand for performance of the indemnity UCD shall allow the Licensee sufficient time as is reasonable in the circumstance to investigate its alleged liability and to negotiate a settlement of or defend the action, claim, proceeding or demand. In particular, UCD will permit the Licensee and its insurer, at Licensee’s expense, to assume or participate in the defence of any such claims or suits. UCD will co-operate with Licensee and its insurers in such defence when reasonably requested to do so and will not compromise or settle the claim or suit without Licensee’s prior written consent.

9.11 Limitation of Liability

Subject to Clause 9.12 and notwithstanding anything to the contrary in this Agreement and excluding the indemnity provided by the Licensee to UCD pursuant to Clause 9.8, neither Party shall not be liable to the other Party by reason of any representation or warranty, indemnity, condition or other term or any duty of common law, or under the express terms of this Agreement, for any consequential or special or incidental or punitive loss or damage (whether for loss of current or future profits, loss of enterprise value or otherwise), or loss of opportunity, and whether occasioned by the breach of contract or the negligence or other wrongdoing of the responsible Party, its employees or agents or otherwise.

9.12 Nothing in this Agreement shall limit or exclude the liability of either Party for:

9.12.1 death or personal injury resulting from negligence;

9.12.2 fraud or fraudulent misrepresentation; and

9.12.3 any breach of this Agreement that results from the wilful act or wilful omission of that Party or its employees, agents or sub-contractors.

9.13 On the written request of UCD, the Licensee shall provide a copy of the insurance cover secured by the Licensee. Licensee shall obtain and maintain at its own cost and expense, the same level

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	of insurance during the Term, and for any period thereafter where any Product utilising any of the Licensed IP is being commercially developed or sold.
10.	TERM AND TERMINATION

10.1 This Agreement shall commence on the Effective Date and will continue in full force on a Product-by-Product and country-by-country basis until the later of (a) the expiration of the last to expire Valid Claim of the Patent Rights; (b) the expiration of Orphan Drug Exclusivity; or (c) fifteen (15) years after the First Commercial Sale of the first Product in the Territory by the Licensee, its Affiliates or sub-licensees (the Term), unless terminated earlier in accordance with the remainder of this Clause 10.

10.2 UCD may terminate this Agreement by giving at least thirty (30) Business Days’ prior written notice to the Licensee if:

10.2.1 the Licensee is in Material Breach of any provision of this Agreement and after receiving written notice from UCD identifying a Material Breach by Licensee of its obligations pursuant to this Agreement, fails to cure such Material Breach within [***], then UCD may give written notice of default (Notice of Default) to Licensee. If Licensee fails to cure the default [***] of the Notice of Default, UCD may terminate this Agreement and the license granted herein by a second written notice (Notice of Termination) to Licensee; or

10.2.2 the Licensee becomes insolvent, or if an interim order is applied for or made, or a voluntary arrangement approved, or a voluntary arrangement is proposed or approved or an administration order is made, or a receiver or administrative receiver is appointed of any of the Licensee's assets or undertaking or a winding-up resolution or petition is passed or presented (otherwise than for the purposes of reconstruction or amalgamation), or if any circumstances arise which entitle the court or a creditor to appoint a receiver, administrative receiver or administrator or to prevent a winding-up petition or make a winding-up order, or other similar or equivalent action is taken against or by the Licensee by reason of its insolvency or in consequence of debt, or if the Licensee makes any arrangement with its creditors;

10.2.3 the Licensee shall dispose of all or a substantial part of its business involving the licensing of the Licensed IP, for the Purpose in the Field in the Territory in circumstances where it does not enter into a novation agreement pursuant to Clause 13.5;

10.2.4 the Licensee or its Affiliates challenge the validity of the Application(s) when granted),or assists any Third Party to commence legal proceedings to challenge such validity;

10.2.5 pursuant to Clause 5.8; or

10.2.6 if the Licensee fails to pay any amount due pursuant to this Agreement (including payments due pursuant to Clause 6 of this Agreement) within [***] from Licensee receiving written notice from UCD of its failure to pay, and such failure to pay is not subject to a good faith dispute between the Parties.

10.3 The Licensee may terminate this Agreement by giving UCD not less than sixty (60) Business Days’ written notice at any time, provided that the Licensee shall continue to pay any invoices received within sixty (60) Business Days’ after the effective date of termination.

11. CONSEQUENCES OF TERMINATION and expiry

11.1 On termination or expiry, of this Agreement for whatever reason:

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11.1.1 all rights and licenses granted to the Licensee pursuant to this Agreement shall terminate forthwith and the Licensee shall not use any of the Licensed IP for any purpose;

11.1.2 subject to the provisions of Clause 11.1.5 the Licensee shall procure that all sub-licenses of any of the Licensed IP shall terminate forthwith;

11.1.3 the Licensee shall co-operate with UCD and provide such assistance as is necessary to cancel all or any licences registered pursuant to this Agreement;

11.1.4 the Licensee shall not be reimbursed for any payments made pursuant to this Agreement, and shall pay all outstanding payments properly accrued and/or payable to UCD up to and including the date of termination or expiry within [***] of the date of termination or expiry in accordance with Clause 6.1 (Payments); and

11.1.5 the Licensee and any sub-licensee shall have the right to dispose of all stocks of the Products in its possession and all Products in the course of manufacture at the date of termination, provided that any sums payable pursuant to the provisions of Clause 6.1 (as if such stocks shall have been supplied at the date of termination) shall be received within a period of [***] following termination.

11.2 Termination or expiry of this Agreement for whatever reason shall not extinguish or otherwise affect any obligations and/or rights of either Party to this Agreement which:

11.2.1 accrued prior to the date of termination or expiry; or

11.2.2 otherwise relate to or may arise at any future time from any breach or non-observance of obligations pursuant to this Agreement which arose prior to the date of termination or expiry.

11.3 Notwithstanding expiry or termination of this Agreement for whatever reason, the following terms shall continue to be binding on the Parties (to the extent applicable for the applicable period): Clause 2.3 (on expiry), Clause 3.2, Clause 4.1, Clause 4.4 (first sentence), Clause 6.10, Clause 7, Clause 8.11.2, Clause 9.2, Clause 9.6.1, Clauses 9.8 to 9.13, Clause 11, and Clause 13 as applicable.

12. FORCE MAJEURE

If the performance by either Party of any of its obligations pursuant to this Agreement (except a payment obligation) is made impossible, delayed or prevented by circumstances beyond its reasonable control including but not limited to war, riot, and insurrection; laws, proclamations, edicts, ordinances, or regulations; strikes, lockouts, or other serious labour disputes; and floods, fires, explosions, or other natural disasters , that Party will not be in breach of this Agreement because of that delay in performance. When such events have abated, the non-performing Party’s obligations herein shall resume.

13. GENERAL

13.1 Publicity: Neither UCD nor the Licensee will use the other’s name or logo in any press release or product advertising, or for any other promotional purpose, without first obtaining the other's written consent, except that UCD may identify the sums received from the Licensee in UCD’s annual report and similar publications.

13.2 Notices: Any notice to be given pursuant to this Agreement shall be in writing, may be delivered to the other Party by any of the methods set out in the left hand column below, and will be deemed to be received on the corresponding day set out in the right hand column:

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Method of service	Deemed day of receipt
By hand or courier	the day of delivery
By pre-paid first class post	the second Business Day after posting
By recorded delivery post	the next Business Day after posting

The Parties’ respective representatives for the receipt of notices are, until changed by notice given in accordance with this Clause 13.2, as follows:

For UCD:	For the Licensee:
Name: Director of NovaUCD Address: NovaUCD, University College Dublin, Dublin 4	Name: Dr. Joseph Wiley Address: Amryt Genetics Limited, 90 Harcourt Street, Dublin 2.

13.3 Headings: The headings in this Agreement are for ease of reference only; they do not affect its construction or interpretation.

13.4 Assignment by either Party: The rights of each Party pursuant to this Agreement are personal. Subject to the provisions of this Clause 13.4 and Clause 13.5, neither Party may assign, charge, transfer or otherwise encumber or dispose of any of their rights pursuant to this Agreement unless the prior written consent of the other Party has been obtained (such consent not to be unreasonably conditioned, withheld or delayed) SAVE THAT UCD may assign its rights and obligations pursuant to this Agreement to an entity that acquires its business or assets by way of public sector reorganization.

13.5 Novation by Licensee

Excepting where the Licensee is insolvent or any other circumstance described in Clause 10.2 applies to the Licensee:

13.5.1 The Licensee may novate all (but not a portion) of its rights and obligations pursuant to this Agreement to an Affiliate, subject to entering into a deed of novation with that Affiliate which binds the Affiliate to the relevant terms and conditions of this Agreement; and

13.5.2 The Licensee may novate all (but not a portion) its rights and obligations pursuant to this Agreement together with its rights in the Licensed IP in an arm’s length transaction to any company to which it transfers all or substantially all of its assets or business or all or substantially all of its assets or business to which this Agreement relates, provided that the company is in a position to enter into (and actually enters into) a deed of novation which binds such company to the relevant terms and conditions of this Agreement.

13.6 Illegal/unenforceable provisions: Each of the provisions of this Agreement is separate and severable and enforceable accordingly. If at any time any of the provisions is held to be void or unenforceable in law, including the laws of the European Union, the remainder of such provision and all other provisions of this Agreement shall remain valid and enforceable to the

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fullest extent permissible by law, and such provision shall be deemed to be omitted from this Agreement to the extent of such invalidity or unenforceability and the Parties shall negotiate in good faith to replace the invalid or unenforceable provision with a valid, legal and enforceable provision which achieves to the greatest extent possible the economic, legal and commercial objectives of the invalid or unenforceable provision.

13.7 Construction: This Agreement will be considered drafted by all Parties and it will not be construed against any one Party because that Party or its solicitors drafted it.

13.8 Waiver of rights: If a Party fails to enforce, or delays in enforcing, an obligation of the other Party, or fails to exercise, or delays in exercising, a right pursuant to this Agreement, that failure or delay will not affect its right to enforce that obligation or constitute a waiver of that right, nor shall any single or partial enforcement or exercise of any obligation or right prevent any further or other enforcement or exercise thereof. Any waiver of any provision of this Agreement will not, unless expressly stated to the contrary, constitute a waiver of that provision on a future occasion.

13.9 No agency: Nothing in this Agreement creates, implies or evidences any partnership or joint venture between the Parties, or the relationship between them of principal and agent. Neither Party has any authority to make any representation or commitment, or to incur any liability, on behalf of the other.

13.10 Entire agreement: The Parties agree that this Agreement constitutes the entire agreement between the Parties relating to its subject matter and supersedes all previous agreements, discussions and arrangements between the Parties relating to the subject matter hereof.

13.11 Further Assurances: Each Party undertakes, at the reasonable request of the other Party at any time in the future, and at the requesting Party’s expense, to execute all such documents, give such assistance and do such acts and things as may in the opinion of the requesting Party be necessary to give effect to the terms of this Agreement.

13.12 Amendments: No variation or amendment of this Agreement will be effective unless it is made in writing and signed by each Party's representative.

13.13 Governing law: This Agreement is governed by and is to be construed in accordance with Irish law. Subject to the provisions of Clauses 5.6, 5.7, 5.8, and 13.14, the Irish Courts will have exclusive jurisdiction to deal with any dispute which has arisen or may arise out of, or in connection with, this Agreement.

13.14 Disputes: If the Parties are unable to reach agreement on any issue concerning this Agreement within [***] after one Party has notified the other of that issue, the matter shall be referred to the Director of NovaUCD and the CEO of the Licensee. In the event these two (2) individuals cannot reach agreement within [***] of the matter being referred to them, the matter shall be subject to the jurisdiction of the Irish Courts. The provisions of this Clause 13.14 shall not apply in the event that a Party considers it necessary to seek injunctive relief.

13.15 Certain changes of Control: In the event of a change of Control of Licensee which results in a Third Party being in a position to change the composition of the board of directors of the Licensee, the Licensee shall give written notice thereof to UCD as soon as is practicable following such change of Control.

13.16 Counterparts. This Agreement may be executed in two or more counterparts, each of which shall be deemed to be an original, and which together shall constitute one and the same

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Agreement. This Agreement shall become effective and be dated (and each counterpart shall be dated) on the date first written above between the parties which have executed and delivered a counterpart. Immediate evidence that a counterpart engrossment has been executed may be provided by transmission of such counterpart engrossment by fax machine or a scanned version thereof by email with the original executed counterpart engrossment to be put in the post as soon as practicable thereafter.

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Schedule 1

Licensed IP

31

Schedule 2

MILESTONES

1. As of the Effective Date and as part of the Licensee’s obligations pursuant to Clause 5.2, the Licensee agrees to meet the following milestones, either by itself and/or either directly or indirectly through its Affiliate(s) and/or its sub-licensee(s):

1.1.1 [***];

1.1.2 initiate the first Phase IIa clinical trial of a Product by [***];

1.1.3 initiate the first Phase IIb clinical trial of a Product by [***];

1.1.4 initiate the first pivotal clinical study of a Product by [***];

1.1.5 [***].

2. The Parties acknowledge and accept that the estimated timelines set out above may have to be adjusted for a number of reasons, such as, the requirements of one or more regulatory agencies, supply chain issues or issues in connection with the conduct of the clinical study. In such event any updates and/or amendments to the Milestones shall be discussed in advance with UCD and any such updates and/or amendments shall be incorporated into this Agreement with the express written agreement of both Parties.

3. For the purposes of this Schedule 2, initiation of a “clinical trial” and/or “clinical study” (as the case may be) shall mean the effective date of the clinical trial and/or clinical study research agreement (as the case may be) in which the first patient or subject is to be treated with Product pursuant to a protocol approved both by an appropriate institutional review board and by an appropriate drug regulatory agency with a therapeutic agent or process that has been manufactured according to the applicable good manufacturing practices (GMP) guidelines provided by the relevant regulatory agency.

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Schedule 3

Appointment of expert

1. Pursuant to Clause 5.5, UCD may serve notice on the Licensee (Referral Notice) that it wishes to refer to an expert who shall be independent, impartial and a leading authority in the Field, (the Expert) the questions set out in Clause 5.5.

2. The Parties shall mutually agree the identity of the Expert who shall have at least ten (10) years’ experience of at least senior at vice president level in a engaged in the development and commercialisation of innovative pharmaceutical or biotechnology products. In the absence of such agreement within sixty (60) Business Days of the Referral Notice, the questions shall be referred to an Expert appointed by the President of Law Society of Ireland who shall have the experience referred to in this paragraph 2.

3. Sixty (60) Business Days after the giving of a Referral Notice, both parties shall exchange simultaneously statements of case, and each side shall simultaneously send a copy of its statement of case to the Expert.

4. Each Party may, within thirty (30) Business Days of the date of exchange of statement of case pursuant to paragraph 3 of this Schedule 3, serve a reply to the other side's statement of case of not more than 10,000 words, or alternatively not to exceed such other word count as may be mutually agreed between the Parties in writing. A copy of any such reply shall be simultaneously sent to the Expert.

5. The Expert shall make his decision on the said questions on the basis of written statements and supporting documentation only and there shall be no oral hearing. The Expert shall issue his decision in writing within thirty (30) Business Days of the date of service of the last reply pursuant to paragraph 4 of this Schedule 3, or in the absence of receipt of any replies, within sixty (60) Business Days of the date of exchange pursuant to paragraph 3 of this Schedule 3.

6. The Expert's decision shall be final and binding on the Parties.

7. The Expert's charges shall be borne equally by the Parties.

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Annex 1

Bank Account Details

 

34

IN WITNESS WHEREOF, the Parties have hereto have signed this Agreement on the date set out above:

SIGNED on behalf of University College Dublin, National University of Ireland, DUBLIN:	SIGNED on behalf of AMRYT GENETICS LIMITED:
Ciaran O’Beirne	Joseph Wiley
Name	Name
Manager, Technology Transfer
Position	Position
/s/ Ciaran O’Beirne	/s/ Joseph Wiley
Signature	Signature

Dated:  14^th March 2018 Dated: 14^th March 2018

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EX-10.11.1

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.11.1

CONFIDENTIAL

Contract Manufacturing Agreement

- Metreleptin SLD-

BETWEEN

Amylin Pharmaceuticals, Inc. and

Sandoz GmbH

1.	DEFINITIONS	5
2.	PROCESS CHARACTERIZATION PHASE	11
3.	PROCESS INTERMEDIATES SUPPLY ACTIVITIES	12
4.	ANALYTICAL ACTIVITIES	12
5.	OPTION FOR PILOT STUDY	14
6.	PROCESS VALIDATION CAMPAIGN	14
7.	JOINT STEERING COMMITTEE	17
8.	RAW MATERIALS	18
9.	COMMERCIAL PHASE	19
10.	FORECAST AND SUPPLY	20
11.	FIRM ORDERS	22
12.	DELIVERY	23
13.	PERSON-IN-PLANT; AUDITS AND INSPECTIONS; SAFETY ISSUES	23
14.	DURATION	25
15.	RESERVATION AND COST	25
16.	PRICING AND TERMINATION FEES	25
17.	INTELLECTUAL PROPERTY	27
18.	PAYMENT	30
19.	LIMITATION OF LIABILITY	31
20.	SPECIFICATION	31
21.	WARRANTIES AND INDEMNITIES	32
22.	INSURANCE	34
23.	CONFIDENTIALITY	34
24.	RECALLS	36
25.	TERMINATION	36
26.	CONSEQUENCES OF TERMINATION	37

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27.	ACCEPTANCE	38
28.	FORCE MAJEURE	39
29.	SPECIFIC INVESTMENTS	39
30.	NOTICES	40
31.	ENTIRE AGREEMENT	40
32.	MISCELLANEOUS PROVISIONS	41
33.	GOVERNING LAW AND JURISDICTION	42
34.	DISPUTE RESOLUTION	42

Schedules:

1.Delivery Procedure

2. Product Specifications

3. Process Transfer Materials

4. Capacity and Pricing Estimates, Fees

5. Product Description

6. Facility Modifications

7. Pricing Model for Commercial Supply

8. Regulatory Support

9. Initial five (5) year forecast

10. Analytical Methods Description

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CONFIDENTIAL

THIS AGREEMENT is made as of September 30, 2010 (the “Effective Date”)

BETWEEN:

(1) Amylin Pharmaceuticals, Inc, whose principal office is at 9360 Towne Centre Drive, San Diego, California 92121 (“Amylin”) and

(2) Sandoz GmbH, whose principal office is at Biochemiestrasse 10, A6250 Kundl, Austria (“Sandoz”)

WHEREAS:

(A) Sandoz carries on the business of, inter alia, development and bulk manufacture of biopharmaceutical products; and

(B) Amylin and Sandoz have entered into a Process Optimization and Manufacturing Agreement dated June 25, 2010 regarding the optimization and demonstration of the manufacturing process for metreleptin drug substance in the pilot plant, and scale up of such process to [***] w.v. facility at Sandoz, which drug substance is intended by Amylin to be used as active substance for a finished product for the treatment of obesity developed by Amylin; and

(C) Amylin wishes to establish a separate manufacturing process for metreleptin drug substance as active substance for a finished product for the treatment of severe lipodystrophy at [***] w.v. scale at Sandoz’ RPP4 facility (the “RPP4 Facility”), and to engage Sandoz as a manufacturer for commercial quantities of such drug substance according to the terms and conditions of this Agreement; and

(D) Sandoz is willing to conduct the following activities:

a) Process characterization activities at lab scale and pilot scale (Process Characterization Phase as defined below);

b) Generation of intermediates at pilot scale for use in process characterization (Process Intermediates Supply as defined below);

c) Analytical method validation and method transfer activities to enable process validation for the product (Analytical Activities as defined below);

d) Optional performance of selected studies at pilot scale (Pilot Study as defined below); and

e) Process validation at [***] w.v. scale at the RPP4 Facility (Process Validation as defined below); and

f) Manufacture of commercial quantities of the Product (as defined below) at the [***] w.v. scale at the RPP4 Facility (Commercial Supply as defined below)

under the terms and conditions set out in this Agreement;

IT IS AGREED as follows:

1. DEFINITIONS

(1) In this Agreement:

“Additional Specifications” means the initial Additional Specifications as explicitly identified as such in Schedule 2 as they may be amended by mutual written Agreement according to Clause 32(9).

“Additional Campaign” has the meaning as set forth in Clause 6(4).

“Additional Campaign Option” has the meaning as set forth in Clause 6(4).

“Additional Campaign Option Fee” has the meaning as set forth in Schedule 7 (pricing option “B”).

“Agreement” means this contract manufacturing agreement and all Schedules attached thereto including all amendments subsequently agreed to by the parties, as the case may be.

“Affiliates” means, with respect to either party to this Agreement, any company, partnership or other entity that directly or indirectly controls, is controlled by or is under common control with such party. For the purpose of this definition, “control” means direct or indirect beneficial ownership or voting control of at least fifty percent (50%) of the issued share capital in such company, partnership or other entity.

“Amylin” has the meaning set forth in the opening paragraph of this Agreement.

“Amylin Assigned Technology” has the meaning set forth in Clause 17(4).

“Amylin Background Technology has the meaning set forth in Clause 17(1).

“Amylin Finished Product” means a pharmaceutical product containing the Product as the active ingredient and currently developed by or on behalf of Amylin for the therapeutic indication severe lipodistrophy.

“Amylin Foreground Technology” has the meaning set forth in Clause 17(3).

“Analogs” means any peptides for which the sequence of such peptide was derived from the Product by insertions of, or substitutions by, one or more naturally-occurring amino acids and/or by deletions from the amino acid sequence of the Product, and such peptide maintains seventy percent (70%) sequence identity with the amino acid sequence identity of the Product.

“Analytical Activities” means the activities related to the transfer, implementation and validation of analytical methods, including without limitation the analytical method transfer, analytical method development and analytical validation, as set out in Clause 4.

“Analytical Activities Fee” has the meaning as set forth in Schedule 4.

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“Analytical Activities Master Plan” mean a document describing the concept and the strategy (including the estimates for timeline and capacities) for the Analytical Activities.

“Analytical Activities Protocol” means the mutually agreed protocols based on Schedule 10 and describing (a) the analytical methods to be transferred to Sandoz analytical labs and (b) the additional analytical methods to be developed by Sandoz and implemented into the Process and (c) the analytical validation to be carried out by Sandoz and (d) the expected schedule of the Analytical Activities.

“Audit” means an audit and inspection of all documents, processes and facilities of Sandoz or of any approved subcontractor applicable to the Manufacture of the Product.

“Bankruptcy” has the meaning as set forth in Clause 25(1)(b).

“Batch” means uniquely identified or identifiable Product that has been processed in one process or series of processes.

“Certificate of Analysis” means a document signed by an authorized employee of Sandoz stating and confirming the record of tests and test methods performed on Product, and the results of each such test.

“Certificate of Compliance” means a document signed by an authorized employee of Sandoz stating and confirming that the Product to which such document refers has been Manufactured in accordance with this Agreement, including the Sandoz Release Specifications and cGMP.

“cGMP” means the United States’ current Good Manufacturing Practices pursuant to the U.S. Federal Food, Drug, and Cosmetic Act, as amended (21 U.S.C. Sect. 301 et seq.), and pursuant to relevant regulations found in Title 21 of the U.S. Code of Federal Regulations (including Parts 210, 211, 600 and 611) and any comparable European Union and Austrian laws, directives, rules or regulations, and of any agreed-upon foreign jurisdiction, as each may be amended from time to time. At the time of this Agreement, this includes compliance with European Union, Austrian, and United States regulatory and statutory requirements relating to good manufacturing, laboratory, warehousing and distribution practices and adherence to any applicable product license requirements and relevant current U.S., E.U., and Austrian guidance documents.

“CMC” means the Chemistry, Manufacturing, and Controls content of regulatory applications for the Product including all INDs and NDAs (and amendments thereto) filed by Amylin.

“Collaboration IP Rights” shall mean (i) any and all IP Rights consisting of or resulting from inventions or improvements that are made, conceived or reduced to practice by one or both parties as a result of the services performed according to a specific Schedule under this Agreement.

“Collaboration Know-How” shall mean any Know-How that is generated by one or both parties as a result of the services performed according to a specific Schedule under this Agreement.

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“Commercial Batches” means any Batches of Product Manufactured after completion of the Process Validation Campaign.

“Commercial Phase” means the time period starting with the first manufacture of Commercial Batches.

“Confidential Information” has the meaning as set forth in Clause 23.

“Defective” or “Defect” means, with respect to any Product, that the Product (i) does not meet the Product Specifications and/or (ii) has not been manufactured in compliance with cGMP requirements.

“Discloser” has the meaning as set forth in Clause 23(1).

“Effective Date” has the meaning as set forth above in the introductory paragraph of this Agreement.

“EMA” means the European Medicine Agency.

“Engineering Batch” means a Batch of Product identified as an Engineering Batch on Schedule 7.

“Excess Order” has the meaning set in Clause 10(6).

“Facility Modifications” has the meaning as set forth in Clause 29(1).

“FDA” means the United States Food and Drug Administration.

“Firm Order” has the meaning set forth in Clause 11(3).

“IP Rights” shall mean (i) any and all inventions or improvements, (ii) any intellectual property rights covering such inventions or improvements, including but not limited to patent applications, patents, utility models and all divisionals, continuations, substitutions, continuations-in-part, re-examinations, reissues, additions, renewals, extensions, registrations, and supplemental protection certificates and the like of any of the foregoing, and (iii) all other intellectual property rights and equivalent or similar forms of protection existing anywhere in the world, but (iv) excluding any Know-How.

“Joint Foreground Technology” has the meaning set forth in Clause 17(3).

“JSC” has the meaning as set forth in Clause 7(1).

“Know-How” shall mean (i) any and all proprietary and confidential technology, trade secrets and other information (including data, results, reports, expertise, techniques, methods, processes, assays, developments, standard operating procedures, formulas, and specifications), and (ii) any physical, chemical or biological materials, which are generated by one or both parties in connection with the activities under this Agreement and owned or controlled by a party to this Agreement; provided, however, for the avoidance of doubt, Know-How shall not include any portion or entirety thereof which does not constitute Confidential Information pursuant to the exclusions set forth in Clause 23(2)(a) through (c) inclusive hereof.

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“Manufacture” means the production of the Product in bulk from the Raw Materials using the Process and shall, where relevant, include manufacturing, formulating, assembling, packaging, storing, handling, testing and quality control, and “Manufactured” and “Manufacturing” shall be interpreted accordingly.

“Manufacturing Records” means all documents relating to the Manufacture of a Batch, including manufacturing instructions, deviations, test results, out of specification results, investigations, bills of materials, Certificates of Compliance, and Certificates of Analysis.

“Master Batch Records” means a detailed, step-by-step description of the entire production process for the Product, including an explanation of the Product is produced and indicating, among other things, specific types and quantities of components and Raw Materials, processing parameters, in-process quality controls.

“Minimum Fee” has the meaning as defined in Clause 9(3).

“Minimum Fee Period” shall mean each consecutive time period of [***] during, with the first Minimum Fee Period starting upon the earlier of (i) FDA approval of the Product and (ii) [***], and each following Minimum Fee Period starting on every [***], until expiry or termination of this Agreement.

“Modification” means any modification, addition, improvement, or adaptation, material or otherwise.

“NDA” means a New Drug Application submitted to the FDA, or any successor application or procedure.

“Optional Activities” means the activities based on Schedule 4 to be optionally performed during Process Characterization Phase by Sandoz upon written request of Amylin.

“Ordered Amount Fee” has the meaning as defined in Clause 9(3).

“Pilot Studies” has the meaning as set forth in Clause 5(1).

“Pilot Study Option Fee” has the meaning set forth in Schedule 4.

“Pilot Study Option” has the meaning as set forth in Clause 5(1).

“Pilot Study Work Plan” means an outline of activities, timing and sampling to be performed in connection with the Pilot Studies upon Amylin’s exercise of the Pilot Study Option.

“Process” means the series of methods and techniques provided by Amylin by which the Product is Manufactured, as defined in the Manufacturing Records, and “Processed” and “Processing” shall be interpreted accordingly. The Process will be optimized and changed from time to time by written agreement of the parties and in accordance with the Quality Agreement.

“Process Characterization Phase” means the period of time during which Sandoz will conduct the process characterization as set out in Clause 2.

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“Process Characterization Phase Fee” has the meaning as set forth in Schedule 4.

“Process Characterization Phase Master Plan” means a document describing the concept and the strategy for the activities to be performed under the Process Characterization Phase.

“Process Characterization Phase Protocol” means the protocols describing (a) a detailed workplan to be conducted by Sandoz during the Process Characterization Phase and (b) the expected duration of the Process Characterization Phase.

“Process Intermediate Supply Activities” has the meaning set forth in Clause 3(2).

“Process Intermediate Supply Fee” has the meaning set forth in Schedule 4.

“Process Intermediate Work Plan” means an outline of the activities, timing and sampling to be performed under the Process Intermediate Supply Activities.

“Process Transfer Information” means all know-how, registration data, experience, instructions, standards, methods, test and trial results, manufacturing processes, hazard assessments, quality control standards, formulae, specifications, storage data, samples, drawings, designs, analytical methods, validation reports of analytical methods and all other relevant information relating to the Product, the Process, Manufacture or storage of the Product, including the information and the Process Transfer Materials as defined herein or in that certain Process Transfer and Initial Manufacturing Agreement, dated November 1, 2006, between Amylin and Sandoz as delivered from Amylin to Sandoz.

“Process Transfer Materials” means the cell banks, reference standards and other materials to be provided by Amylin to Sandoz for the Process Characterization Phase, Analytical Activities, Process Intermediate Supply Activities, Pilot Studies, Process Validation Campaign and Commercial Phase, as listed in Schedule 4.

“Process Validation Campaign” has the meaning set forth in Clause 6(2).

“Process Validation Campaign Fee” has the meaning as set forth in Schedule 4.

“Process Validation Master Plan” means an outline of activities to be performed in connection with the Process Validation Campaign.

“Process Validation Protocol” means the protocols describing (a) a detailed work plan, including an outline of sampling, to be conducted by Sandoz during the Process Validation Campaign and (b) the expected duration of the Process Validation Campaign.

“Product” means the drug substance metreleptin, which is a recombinant protein having the amino acid sequence set forth in Schedule 5.

“Product Specifications” means the Sandoz Release Specifications and the Additional Specifications.

“Quality Agreement” means the separate agreement executed by Sandoz and Amylin and constituting an integrated part of this Agreement, which defines the

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quality assurance and regulatory responsibilities of the parties as they relate to this Agreement as it may be amended in accordance with its terms. In the event of any conflict between the terms of this agreement and the Quality Agreement, the terms of this Agreement shall control.

“Raw Materials” means the raw materials which are required by Sandoz to conduct the Manufacture as specifically listed in the Quality Agreement.

“Recipient” has the meaning as set forth in Clause 23.

“Regulatory Authority” means the EMA or the FDA or any equivalent competent regulatory body in each non-U.S. jurisdiction in which the Product is approved for marketing or distribution.

“Released” means the mechanism by which Sandoz states that the Product was Manufactured in accordance with this Agreement, including cGMP, and conforms with the Sandoz Release Specifications based on the results of all Product tests listed in Schedule 2 to be performed by Sandoz (Sandoz Release Specifications). Release is demonstrated by issuance of a Certificate of Analysis and Certificate of Compliance.

“Requested Optional Activities” means such Optional Activities that Amylin has requested in writing to be performed.

“Rolling Commerical Forecast” has the meaning set forth in Clause 10(4).

“RPP4 Facility” has the meaning set forth in the recitals of this Agreement.

“Sandoz” has the meaning set forth in the opening paragraph of this Agreement.

“Sandoz Background Technology” has the meaning set forth in Clause 17(2).

“Sandoz Foreground Technology” has the meaning set forth in Clause 17(3).

“Sandoz Release Specifications” means the initial Sandoz Release Specifications as explicitly identified as such in Schedule 2 as they may be amended by mutual written Agreement according to Clause 32(9).

“w.v.” means working volume scale.

(2) Words importing the singular shall include the plural and vice versa. Words denoting persons shall include bodies corporate and unincorporated associations of persons and vice versa.

(3) Clauses 1(1) and 1(2) apply unless the contrary intention expressly appears elsewhere in this Agreement.

(4) Any reference, express or implied, to any law includes references to:

(a) that law as amended, extended or applied by or under any other law (before or after the Effective Date) and

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(b) any subordinate legislation or regulations made (before or after the Effective Date) under that law, as amended, extended or applied as described in Clause 1(4)(a).

(5) The headings in this Agreement are for convenience of reference only and do not affect the interpretation or scope of this Agreement or any provision herein.

(6) Wherever any provision of this Agreement uses the term “including” (or “includes”), such term shall be deemed to mean “including, without limitation” and “including, but not limited to” (or “includes, without limitation” and “includes, but is not limited to”) regardless of whether the words “without limitation” or “but not limited to” actually follow the term “including” (or “includes”).

(7) The Schedules attached hereto are an integral part of this Agreement and are incorporated herein by reference.

2. PROCESS CHARACTERIZATION PHASE

(1) Upon both parties executing this Agreement, and not later than [***] in advance of commencing the Process Characterization Phase, Amylin will provide Sandoz with a draft Process Characterization Phase Master Plan in accordance with the planned activities outlined on Schedule 4 and all Process Transfer Materials that are reasonably required for Sandoz to conduct the Process Characterization Phase. After review by Sandoz and mutual agreement by both parties on the final Process Characterization Phase Master Plan, Sandoz will prepare a draft Process Characterization Phase Protocol. Not less than [***] in advance of commencing the Process Characterization Phase, Amylin and Sandoz will agree upon the final Process Characterization Phase Protocol in writing.

(2) The Process Characterization Phase is planned for the [***] through the [***], provided this Agreement has been signed by both parties not later than [***].

(3) Sandoz will perform all activities for which it is responsible under the Process Characterization Phase Protocol at its facilities, but it may subcontract tests to laboratories which have been qualified by Sandoz, in each case subject to Amylin’s prior written approval, such approval not to be withheld unreasonably.

(4) In consideration for the services provided by Sandoz as part of the Process Characterization Phase, Amylin shall pay to Sandoz the Process Characterization Phase Fee as set out in Schedule 4. The Process Characterization Phase Fee shall be paid on the basis of the number of weeks needed for the services and the price per week as set out in Schedule 4. Sandoz is entitled to invoice, and Amylin shall pay, the Process Characterization Phase Fee in several installments at certain time milestones as described in Schedule 4. Upon completion of the entire Process Characterization Phase, Sandoz shall provide written reports (in the English language) to Amylin for the experiments performed during the Process Characterization Phase together with an invoice for the last installment of the Process Characterization Phase Fee.

(5) Sandoz shall provide Amylin with regular updates regarding the progress of the Process Characterization Phase. If during the Process Characterization Phase the

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parties identify an unforeseeable technical problem, which prevents Sandoz from conducting its activities under the Process Characterization Phase Protocol, then the following procedure shall apply:

(a) Amylin or its representative may provide Sandoz with technical assistance as reasonably appropriate. Upon request of Sandoz, Amylin shall provide Sandoz with technical assistance as reasonably appropriate.

(b) Sandoz shall use its commercially reasonable endeavors to resolve the problem and Amylin shall be entitled to have a representative present at the Sandoz site and to fully participate in solving such problem to ensure that this provision is satisfied.

(c) The additional cost of resolving the problem shall be borne by Amylin, unless Sandoz has not conducted the Process Characterization Phase with care and diligence consistent with industry standards and in a professional and workmanlike manner with employees that have sufficient technical expertise. Any additional cost shall be subject to Amylin’s prior written approval.

3. PROCESS INTERMEDIATES SUPPLY ACTIVITIES

(1) Sandoz will provide Amylin with a draft Process Intermediate Work Plan in accordance with Schedule 4. Not less than [***] in advance of commencing the Process Intermediate Supply Activities, Amylin and Sandoz will agree upon the final Process Intermediate Work Plan in writing.

(2) Sandoz will perform all activities for which it is responsible under the Process Intermediate Work Plan (the “Process Intermediate Supply Activities”) in accordance with the Process Intermediate Work Plan and Schedule 4.

(3) Amylin will provide the Process Transfer Materials required for the Process Intermediate Supply Activities as set out in Schedule 4 free of charge.

(4) Upon completion of the Process Intermediate Supply Activities according to the final Process Intermediate Work Plan, Sandoz shall issue an invoice for the Process Intermediate Supply Fee.

4. ANALYTICAL ACTIVITIES

(1) Upon both parties executing this Agreement, and not later than [***] in advance of commencing the Analytical Activities, Amylin will provide Sandoz with a draft Analytical Activities Master Plan in accordance with Schedule 4. After review by Sandoz and mutual agreement by both parties on the final Analytical Activities Master Plan, Sandoz will prepare a draft Analytical Activities Protocol.

(2) Not later than [***] in advance of commencing the Analytical Activities, Amylin and Sandoz will agree upon the Analytical Activities Protocol in writing. If agreed by Amylin and Sandoz, separate Analytical Activities Protocols may be generated for each analytical method.

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(3) Not later than [***] in advance of commencing the Analytical Activities, Amylin will begin supplying and transferring to Sandoz all Process Transfer Information and Process Transfer Materials that are reasonably required for Sandoz to perform the Analytical Activities.

(4) Sandoz shall perform the Analytical Activities as outlined in Schedule 4 and according to the agreed Analytical Activities Protocol. Amylin and Sandoz will reasonably modify and/or confirm Schedule 4 not less than [***] in advance of commencing the Analytical Activities. Schedule 10 outlines certain responsibilities of the parties with respect to the validation and transfer of the Analytical Activities and primary authorship responsibilities for related protocols and reports.

(5) Subject to prior agreement by the parties of the Analytical Activities Protocol, Sandoz shall carry out analytical testing, analytical development and analytical validation in accordance with the Analytical Activities Protocol. Sandoz will perform all such activities at its facilities, but it may subcontract tests to laboratories which have been qualified by Sandoz, in each case subject to Amylin’s prior written agreement, such consent not to be withheld unreasonably.

(6) Sandoz shall provide Amylin with regular updates regarding the progress of the Analytical Activities. If during the Analytical Activities the parties identify an unforeseeable technical problem, which prevents Sandoz from conducting, developing or validating analytical tests pursuant to the Analytical Activities Protocol, then the following procedure shall apply:

(a) Amylin or its representative may provide Sandoz with technical assistance as reasonably appropriate. Upon request of Sandoz, Amylin shall provide Sandoz with technical assistance as reasonably appropriate.

(b) Sandoz shall use its commercially reasonable endeavors to resolve the problem and Amylin shall be entitled to have a representative present at the Sandoz site and to fully participate in solving such problem to ensure that this provision is satisfied.

(c) The additional cost of resolving the problem shall be borne by Amylin, unless Sandoz has not conducted the Analytical Activities with care and diligence consistent with industry standards and in a professional and workmanlike manner with employees that have sufficient technical expertise. Any additional cost shall be subject to Amylin’s prior written approval.

(7) Upon completion of the Analytical Activities, Sandoz will provide to Amylin written reports for each analytical method (in English language) on the results of the Analytical Activities.

(8) With respect to any Analytical Activities conducted by Sandoz as part of or in connection with the Process Characterization Phase, Sandoz shall be entitled to invoice the Analytical Activities Fee for all Analytical Activities performed until the date of the invoice together with the relevant invoice for the Process Characterization Phase Fee. With respect to all Analytical Activities performed but 13

not so invoiced, Sandoz shall be entitled to invoice the relevant Analytical Activities Fee together with the reports provided according to Clause 4(7).

(9) Information obtained from the Analytical Activities may, at Amylin’s sole discretion, be shared by Amylin with other Amylin development projects.

5. OPTION FOR PILOT STUDY

(1) Amylin shall have an option to order pilot studies as set forth in Schedule 4 (the “Pilot Studies”) and as described below (the “Pilot Study Option”) subject to the terms and conditions of this Clause 5. The Pilot Study Option may be exercised by Amylin by written notice to Sandoz, which notice may be given (i) only once, and (ii) not later than [***]. The exercise of the Pilot Study Option shall be irrevocable.

(2) If the Pilot Study Option has been validly exercised by Amylin, (i) Amylin will provide Sandoz with a draft Pilot Study Work Plan in accordance with Schedule 4. and (ii) after agreement of a final written Pilot Study Work Plan between the Parties (not less than [***] prior to the start of the activities for the Pilot Studies), Sandoz will start to conduct the Pilot Studies according to the final Pilot Study Work Plan and Schedule 4 using applicable protocols contained in the final Process Characterization Phase Protocol.

(3) Upon completion of the Pilot Studies, Sandoz will provide to Amylin a written report (in English language) on the results of the Pilot Studies and an invoice for the Pilot Study Option Fee.

6. PROCESS VALIDATION CAMPAIGN

(1) Not later than [***] in advance of commencing the Process Validation Campaign, Amylin will provide Sandoz with a draft Process Validation Master Plan in accordance with Schedule 4. After review by Sandoz and mutual agreement by both parties on the final Process Validation Master Plan, Sandoz will prepare a draft Process Validation Protocol. Not less than [***] in advance of commencing the Process Validation Campaign, Amylin and Sandoz will agree upon the final Process Validation Protocol in writing.

(2) Subject to (i) completion of the final Process Validation Protocol and (ii) achievement of all relevant milestones of Process Characterization Activities that are a prerequisite for the Process Validation Characterization Phase as defined in the agreed Process Characterization Phase Master Plan, Sandoz will start the Process Validation Campaign, and will deliver the Product produced therefrom to Amylin (or Amylin’s designee) after completion of such campaign. The parties agree that the “Process Validation Campaign” means a production campaign in which Sandoz shall Manufacture at the [***] w.v. scale at the RPP4 Facility:

(a) [***] Engineering Batches (fermentation and isolation) and [***] Engineering Batches (purification);

(b) [***] cGMP validation Batches (fermentation and isolation) that meet validation acceptance criteria; and

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(c) [***] cGMP validation Batches of Product (purification) that meet the Product Specifications and validation acceptance criteria.

In consideration for Sandoz Manufacturing the Product pursuant to the Process Validation Campaign, Amylin shall pay Sandoz the Process Validation Campaign Fee based on actual facility occupation and materials at prices according to Schedule 4.

(3) Commencement of the Process Validation Campaign is planned for the [***]. The parties agree to use their commercially reasonable endeavors to ensure that the timeline for the Process Validation Campaign is met. Upon completion of the Process Validation Campaign, Sandoz shall invoice Amylin for the Process Validation Campaign Fee and applicable reports.

(4) Amylin shall have the option to order an additional campaign (the “Additional Campaign”) consisting of up to [***] cGMP Batches of Product that meet the Product Specifications (purification only using inclusion bodies from the Process Validation Campaign) (the “Additional Campaign Option”) to be produced as an extension to, and directly following, the production of the Process Validation Campaign. The Additional Campaign Option may be exercised by Amylin by written notice to Sandoz, which notice may be given no later than [***]. The exercise of the Additional Campaign Option shall be binding and irrevocable. If the Additional Campaign Option has been timely exercised, Sandoz will Manufacture for Amylin, and Amylin shall purchase [***] cGMP Batches of Product in the RPP4 Facility. Sandoz will use commercially reasonable efforts to reach the target of an average quantity of [***] kg Product per cGMP Batch in the Additional Campaign. It is agreed that with respect to any target amounts of Product set out in this Clause, Sandoz shall only be obliged to use commercially reasonable efforts to achieve such target amounts, but does not guarantee, and shall have no obligation whatsoever, to achieve such target amounts.

(5) For the avoidance of doubt, if an Engineering Batch is released by Sandoz by the issuance of a Certificate of Analysis and a Certificate of Compliance, the total Batches of Product to be Manufactured by Sandoz and to be compensated by Amylin according to this Clause 6 shall not be reduced as a result thereof.

(6) Sandoz covenants that:

(a) It has and will maintain during the term of this Agreement, all licenses, registrations, certificates, approvals, authorizations or permits required under applicable law, which are necessary to perform the services hereunder and shall bear all costs and expenses associated therewith. Sandoz shall provide copies thereof to Amylin upon reasonable request by Amylin and shall operate in compliance therewith. Sandoz shall promptly provide Amylin with notice, confirmed in writing, in the event of revocation or modification of such documents, or with regard to any other event or regulatory action or involvement such as an order or notice, which in any way impacts Sandoz’ ability to provide services under this Agreement.

(b) It will Manufacture the Product in accordance with this Agreement, including utilizing the Process and complying with cGMP and the Product Specification, except for any Engineering Batches. With respect to

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Engineering Batches, Sandoz will use commercially, scientifically and technically reasonable efforts to achieve compliance of Engineering Batches with cGMP requirements and Product Specifications (including, for the avoidance of doubt, documentation required by cGMP and Sandoz quality systems), it being understood and agreed that (except for the aforesaid obligations to use commercially, scientifically and technically reasonable efforts) Sandoz shall have no obligation and/or liability whatsoever with respect to compliance of Engineering Batches with cGMP requirements and/or Product Specifications.

(c) Any Raw Materials employed by Sandoz in the Manufacture and not supplied by or on behalf of Amylin will at the time of use conform to the requirements stated in Clause 8(2).

(d) It will maintain complete and accurate records pertaining to its Manufacture, analysis and distribution of the Product (including the Manufacturing Records) in accordance with the types of records to be maintained as described in cGMP, the format and content of such records to follow Sandoz’ standard form in German language. In addition to the foregoing, Sandoz shall maintain all site-relevant and CMC-relevant documents necessary for the filing of a NDA for the drug product containing the Product by Amylin, and samples of the Product as are required by cGMP. Amylin shall have access to all such records and samples during normal business hours after reasonable advance written notice, subject to the confidentiality obligations under this Agreement.

(e) The Product will be labeled, prepared and packed for shipment in accordance with labeling requirements as specified by Amylin from time to time.

(f) It will be responsible for the treatment and/or disposal of all waste generated as a result of Manufacturing the Product in accordance with all applicable laws, regulations and directives, and the maintenance of detailed and complete records related thereto.

(g) It will not incorporate any Modifications into the Process without being identified as such by Sandoz and without Amylin’s prior written consent, provided that the parties will in advance agree upon a reasonable adaptation in the fees of the respective phase as shown in Schedule 4, if applicable, and the terms of a license to Amylin and/or its designees for the use of the Sandoz Background Technology pursuant to Clause 17(12) upon termination of this Agreement.

(7) Sandoz may at any time undertake any change (including routine maintenance and repair work) to any of its premises or facilities or equipment directly used in the Manufacture of the Product; provided that any such change is in accordance with the terms of the Quality Agreement and will not be a detriment to the Product or Sandoz’ obligations under this Agreement. Sandoz will notify Amylin of any such changes in accordance with the terms of the Quality Agreement.

(8) After three (3) months prior written notice from Amylin, Sandoz shall provide Amylin with Manufacturing Records and all documentation in relation to the Manufacture of

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the Product as necessary for Amylin for the submission of a NDA with the FDA and a marketing authorization application with the EMA in respect of the Amylin Finished Product and will use commercially reasonable efforts to requested in writing by Amylin, Sandoz will provide Amylin with regulatory support for drafting such NDA, including providing data and reports requested by Amylin, each to the extent as described in Schedule 8. Amylin shall compensate Sandoz for such services provide such support for comparable filings in other jurisdictions. If at a rate of [***], based on actual hours worked for such services. Sandoz will provide Amylin all manufacturing process documentation for purposes of updating regulatory filings on an ongoing basis.

(9) Amylin covenants that:

(h) It has and will maintain throughout the term of this Agreement any appropriate regulatory approvals for the Product and any product containing the Product required in any jurisdiction or territory in which Amylin or its sublicensee is marketing, distributing or using such products.

(i) Any Raw Materials provided by Amylin shall conform to the requirements set forth in Clause 8(3).

(j) It shall, as soon as reasonably possible after it becomes aware of the same, notify Sandoz of any hazards to the health or safety of any personnel of Sandoz or the possibility of cross contamination of any other products being manufactured or stored by Sandoz.

(k) Non-cGMP Batches willused for clinical trials in humans, for commercialization of Product or any product containing Product, or otherwise in humans.

(10) The parties shall update the Quality Agreement between Amylin and Sandoz dated [***] on terms as may be mutually agreed between the parties as soon as possible after the Effective Date of this Agreement but in any case not later than [***].

7. JOINT STEERING COMMITTEE

(1) The parties hereby establish a Joint Steering Committee (the “JSC”) which is intended to facilitate communication between the parties, decision-making and management of the parties’ activities under this Agreement, and each party agrees to use good faith and cooperative efforts to facilitate and assist the efforts of the JSC.

(2) The JSC shall be composed of [***] members, [***] members appointed by each party, but no member shall be the Chief Executive Officer of a party. Within [***] after the Effective Date, each party shall notify the other party of the identity of its initial [***] representatives to the JSC. Each party may replace any or all of its JSC representatives at any time upon written notice to the other party. Notwithstanding of the foregoing, each party may, at its discretion, have present at meetings of the JSC such other employees as it may deem necessary to aid it in participating in meetings of the JSC.

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(3) Sandoz will designate one of its representatives as the initial Chairperson of the JSC. The Chairperson shall be responsible for scheduling meetings, preparing and circulating an agenda in advance of each meeting, and preparing and issuing draft minutes of each meeting within [***] thereafter. The Chairpersonship of the JSC shall alternate between Sandoz and Amylin on a rotating basis, with each party’s Chairperson fulfilling such role for a period of [***].

(4) The parties shall endeavor to schedule meetings of the JSC at least [***] in advance. In-person meetings shall alternate between the places of business of the parties, unless otherwise agreed by the parties. During the term of this Agreement, the JSC shall endeavor to meet in person at least [***] and shall, in any event, meet in person [***]. A meeting may be held by audio or video teleconference.

(5) A quorum for a meeting of the JSC shall require the presence of at least [***] of each party in person or by telephone. All decisions made or actions taken by the JSC shall be made by [***] of the members, with the members of [***] cumulatively having [***] and the members of the [***] cumulatively having [***]. The JSC shall have no power to amend or overrule the terms of this Agreement, which may only be amended in accordance with Clause 32(9) hereof.

(6) The parties agree that the minutes of the JSC meetings shall not be deemed final unless and until they shall have been approved by the JSC.

8. RAW MATERIALS

(1) Except for those Raw Materials that Amylin agrees it will supply to Sandoz, Sandoz will contract with third parties and pay (subject to Amylin’s reimbursement as described below) for the supply of all Raw Materials necessary to Manufacture and package the Product in accordance with this Agreement. A complete list of Raw Materials to be used by Sandoz including vendor and grade is attached to the Quality Agreement. For all Raw Materials used by Sandoz in connection with activities under this Agreement, [***]. Sandoz will maintain written records of all purchases and use of Raw Materials and will provide copies of all such records to Amylin upon request. Raw Materials purchased according to this Clause 8(1) and reimbursed by Amylin which are not used hereunder will, at Amylin’s option and expense, be shipped by Sandoz to Amylin or Amylin’s designated representative. Such request shall be made known by Amylin to Sandoz not later than [***] after finalization of the Process Validation Campaign.

(2) All Raw Materials obtained by Sandoz from a third party and employed in the Manufacture of the Product will at the time of use (a) comply with the relevant Raw Material specifications as agreed upon in the Quality Agreement and (b) with respect of ruminant-sourced Raw Materials, will conform with the Note for Guidance on Minimizing the Risk of Transmitting Animal Spongiform Encephalopathy Agents via Medicinal Products (EMEA/410/01 Rev. 2 - October 2003; published in the Official Journal of the European Union on 28.1. 2004).

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(3) All Raw Materials provided by Amylin as agreed upon by the parties shall be (a) accompanied by a Certificate of Analysis where applicable and agreed upon by the parties and (b) with respect of ruminant-sourced Raw Materials, will conform with the Note for Guidance on Minimizing the Risk of Transmitting Animal Spongiform Encephalopathy Agents via Medicinal Products, (EMA/410/01 Rev. 2 - October 2003; published in the Official Journal of the European Union on 28.1. 2004).

9. COMMERCIAL PHASE

(1) After successful completion of the Process Validation Campaign, Amylin shall use commercially reasonable efforts to have Sandoz approved by the FDA as manufacturer of the Product. Subject to such approval, Amylin has the right and obligation to exclusively purchase from Sandoz, and Sandoz shall Manufacture for Amylin, at least [***] of the entire Product demand of Amylin, its Affiliates and Amylin’s Product licensees, pursuant and subject to the terms and conditions of this Agreement. For purposes of satisfying the exclusivity provisions of this Clause 9(1) and subject to the Minimum Fee provisions set forth in Clause 9(3), Amylin shall have the option to purchase Product from Sandoz that has been manufactured in accordance with the terms and conditions of that certain Process Optimization and Manufacturing Agreement, dated June 25, 2010, between Amylin and Sandoz and any other Product manufacturing and/or supply agreement that may be entered into between the parties in writing.

(2) For all Commercial Batches of Product ordered by Amylin, Amylin shall pay to Sandoz the purchase price set out in Schedule 7. Pricing model “A” on Schedule 7 shall apply for Firm Orders of [***] or more cGMP Batches per campaign. Pricing model “B” on Schedule 7 will apply for Firm Orders of [***] cGMP Batches per campaign. Notwithstanding the foregoing, if Amylin purchases Product under the terms of any other agreement as provided in Clause 9(1), Amylin shall pay the purchase price set out in such agreement.

(3) [***].

(4) Sandoz covenants that:

(a) It has and will maintain during the term of this Agreement, a manufacturer’s license.

(b) It will perform the Services in accordance with this Agreement. It will Manufacture the Product in accordance with this Agreement, including utilizing the Process and (except for the Engineering Batches) in compliance with cGMP and the Specifications. With respect to Engineering Batches, Sandoz will use commercially, scientifically and technically reasonable efforts to achieve compliance of Engineering Batches with

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cGMP requirements and Product Specifications (including, for the avoidance of doubt, documentation required by cGMP and Sandoz quality systems), it being understood and agreed that (except for the aforesaid obligations to use commercially, scientifically and technically reasonable efforts) Sandoz shall have no obligation and/or liability whatsoever with respect to compliance of Engineering Batches with cGMP requirements and/or Product Specifications.

(c) Any Raw Materials employed by Sandoz in the Manufacture of Product and not supplied by Amylin (directly or through an Amylin supplier) will at the time of use conform to the requirements stated in Clause 8(2).

(d) It will maintain complete, accurate and adequate records pertaining to its Manufacture, analysis and distribution of the Product (including the Manufacturing Records) in accordance with the types of records to be maintained as described in cGMP, the format and content of such records to follow Sandoz’ standard form in German language to the extent in compliance with Amylin’s regulatory filing requirements. The Master Batch Records and the site-relevant records will be in English language (or available as translation into English language). For any regulatory support in order to maintain approval in the United States and European Union during commercial phase, Sandoz will use commercially reasonable efforts to assist Amylin in the preparation of such records and documentation to the extent requested by Amylin, and Amylin will compensate Sandoz for such assistance at a rate of [***], based on actual hours worked. Sandoz will provide all manufacturing process documentation during the Commercial Phase for purposes of updating regulatory filings on an ongoing basis.

The parties understand that Amylin may license certain of its rights to third parties and such parties may require access to the described documents (including access to original copies of such documents on-site at Sandoz). Accordingly, such licensees shall be provided copies of such documents and prompt access to original copies of such documents on-site during regular business hours and after reasonable prior notice by Amylin, provided that (a) the number of such licensees on-site at Sandoz in any year is reasonable (not to exceed [***] visits in total), (b) Amylin, and not Sandoz, shall be responsible for providing copies of such documents to the licensees, and (c) Amylin shall accompany each licensee during any review of original documents on-site at Sandoz. In such event Amylin shall remain fully responsible to Sandoz regarding compliance of such third parties with the terms of this Agreement, including but not limited to confidentiality obligations.

(e) The Product will be labeled, prepared and packed for shipment in accordance with labeling requirements as specified by Amylin from time to time.

(f) It will be responsible for the treatment and/or disposal of all waste generated as a result of Manufacturing the Product in accordance with all applicable laws, regulations and directives, and the maintenance of detailed and complete records related thereto.

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(g) It will not incorporate any Sandoz IP Rights (including but not limited to any Sandoz Background Technology or Sandoz Collaboration IP Rights) into the Process without Amylin’s prior consultation and consent.

(5) Sandoz may at any time, with reasonable advance notice to Amylin and in accordance with any applicable provisions of the Quality Agreement, undertake any change (including routine maintenance and repair work) to any of its premises or facilities or equipment directly used in the Manufacture; provided that any such change shall not be a detriment to the Product.

(6) Amylin covenants that:

(a) It has and will maintain throughout the term of this Agreement appropriate Regulatory Approvals for the Product for any jurisdiction or territory in which Amylin is marketing, distributing or using the Product.

(b) Any Raw Materials provided by Amylin (directly or through an Amylin supplier), shall conform to the requirements set forth in Clause 8(3).

(c) It shall, as soon as it becomes aware of the same, notify Sandoz of any hazards to the health or safety of any personnel of Sandoz or the possibility of cross contamination of any other products being manufactured or stored by Sandoz.

(d) Pilot Batches and non-cGMP (Nonconforming) Batches will not be used for clinical trials.

10. FORECAST AND SUPPLY

(1) The parties shall work on the basic premise that Amylin shall at all times endeavor to give Sandoz as much advance notice as possible of any changes in its forecasts, and Sandoz shall use its commercially reasonable efforts to accommodate such changes.

(2) Amylin’s current non-binding [***] forecast for Product is attached as Schedule 9 for information purposes only.

(3) Amylin will use its best efforts in planning and estimating its requirements and will provide to Sandoz, a rolling [***] forecast for the Product by [***] and will update such forecast yearly.

(4) Upon [***] from the granting of regulatory approval of the Amylin Finished Product by FDA or EMA, and thereafter before [***] of each subsequent calendar year, Amylin will provide to Sandoz a rolling forecast of its entire demands for Product of Amylin, its Affiliates and licensees for the [***] following the year in which the forecast is provided (each such forceast a “Rolling Commercial Forecast”), provided that the amount of Product forecasted for any individual calendar year in the Rolling Commercial Forecast (i) will be comprised of a minimum campaign size of [***] Engineering Batch and [***] cGMP Batches, (ii) shall be suitable for Manufacture in [***] consecutive production campaign, and (iii) until otherwise agreed to by Amyln and Sandoz, will require a production capacity of no more than [***] per year at the RPP4 Facility.

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Each Rolling Commercial Forecast will consist of (i) Firm Order for Product for [***] forecast, and (ii) non-binding forecasted demands for Product for the subsequent [***]. If Amylin issues a Rolling Commercial Forecast that is not in line with the requirements set out above under (ii) and (iii), Sandoz agrees that it will use its commercially reasonable efforts to accommodate such deviating demands consistent with this Agreement (including, without limitation, upon financial terms no higher than described herein) in an attempt to meet Amylin’s requirements for the Product, and will inform Amylin whether it will fill such deviating demands within [***] of receiving the applicable order so as to permit Amylin to manage its inventory of Product and respond to market demand.

(5) Firm Orders placed by Amylin before Regulatory Approval shall be placed not later than [***] of the previous year, unless agreed otherwise by the parties.

(6) With respect to each Firm Order (a) Amylin will be obligated to purchase [***] of the quantity of Product set forth in such Firm Order and (b) Sandoz will be obligated to Manufacture [***] of the quantity of Product set forth in the Firm Order, except as justified by Batch volumes based on the number of kg of Product ordinarily produced per Batch, and provided that the Firm Order shall not exceed the previous year’s forecast for such calendar year by more than [***]. In the event Amylin requests Product from Sandoz in excess of (i) [***] of the Firm Order, (ii) the amounts of Product set forth in the applicable Rolling Commercial Forecast, or (iii) an amount of Product for which the Manufacture requires more than [***] of production capacity at the RPP4 Facility per calendar year (in each case an “Excess Order”), Sandoz will use its commercially reasonable efforts to accommodate such Excess Orders consistent with this Agreement (including, without limitation, upon financial terms no higher than described herein) in an attempt to meet Amylin’s requirements for the Product. Sandoz shall inform Amylin whether it will fill Excess Orders within [***] of receiving the applicable Firm Order so as to permit Amylin to manage its inventory of Product and respond to market demand.

(7) The parties agree that any Manufacture of Product under this Agreement will be conducted at the [***] w.v. scale. In planning the supply of Product for the following year, Sandoz shall agree in advance with Amylin which capacity of manufacture will be utilized, and in doing so, Sandoz shall use commercially reasonable efforts that Amylin’s supply requirements and all regulatory requirements (including the provision by Sandoz of validation and regulatory support if necessary) are met.

11. FIRM ORDERS

(1) Each Rolling Commercial Forecast, including the related Firm Order, shall be delivered by Amylin in writing. Each Firm Order (subject to the termination rights in this Agreement) shall constitute a binding obligation upon Sandoz to Manufacture and deliver the Product, and upon Amylin to accept and pay for the Product ordered therein.

(2) All Firm Orders shall be in writing and shall identify and reference the quantity of Product ordered the price to be paid for such Product packaging requirements,

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delivery schedule, delivery locations, invoice information and instructions. A Firm Order shall always constitute a binding obligation even if [***] of the items as aforementioned is missing.

(3) Sandoz shall confirm receipt of each order and shall within [***] of its receipt of the order provide Amylin with Manufacturing and delivery dates, depending on Sandoz’ available capacity accommodating as close as possible Amylin’s preferred delivery schedule, and, upon mutual agreement of the parties on the Manufacturing and delivery schedule, the order will become a “Firm Order”.

(4) Sandoz shall promptly notify Amylin by telephone and in writing (a) if Sandoz will not be able to fill the then most recent Firm Orders or (b) of any other production issues or other information of which Sandoz becomes aware that may affect the regulatory status of the Product or relate to the ability of Sandoz to supply Product in accordance with this Agreement and/or any Firm Orders.

12. DELIVERY

(1) Unless the parties otherwise agree from time to time with respect to specific Batches of Product, no Product (other than the Engineering Batches) will be delivered unless it has first been Released by Sandoz according to the Sandoz Release Specifications. Sandoz shall immediately notify Amylin of any Product being ready for dispatch, which shall be shipped as soon as possible upon Amylin’s demand for delivery and in no event later than [***] after Batch Release by Sandoz. Delivery of the Product, intermediates and samples shall be delivered by Sandoz to Amylin, or such nominee as designated by Amylin in writing, on an FCA Sandoz Kundl/Schaftenau facility basis (Incoterms 2000).

(2) Sandoz will prepare the Product for shipment and deliver it to the carrier designated by Amylin.

(3) All costs and expenses for the transportation of the Product including, but not limited to, dedicated transportation containers (including transportation insurance) from Sandoz’ premises to Amylin (or its designee) shall be borne by Amylin. All such costs shall be billed to and paid by Amylin directly.

(4) Title in the Product and any samples produced under this Agreement shall pass from Sandoz to Amylin upon receipt of full payment therefore by Sandoz.

(5) Sandoz shall deliver the Product according to the delivery procedure as outlined in Schedule 1.

13. PERSON-IN-PLANT; AUDITS AND INSPECTIONS; SAFETY ISSUES

(1) Upon Sandoz’ receipt of at least [***] prior notice, Amylin may, subject to Clause 13(3) and Clause 13(6) below, place Amylin employees or authorized representatives (with such authorized representatives being subject to Sandoz’ prior approval which will not be unreasonably withheld) on-site at Sandoz’ manufacturing facility as follows:

(a) up to [***] employees or representatives during the Process Characterization Phase and the Pilot Studies.

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(b) up to [***] employees or representatives during the Process Validation Phase, and the Manufacture of the Product in the RPP4 Facility hereunder free of charge, and

(c) up to [***] shall be at no cost to Amylin with a maximum of [***] visitors at the same time during the Commercial Phase.

(2) Subject to Clause 13(3), Amylin shall also have the right to perform “for cause” audits. “For cause” Audits are audits that Amylin is required to perform following significant Product quality concerns or customer complaints or Product recalls. Amylin will provide Sandoz with as much notice as possible if they wish to conduct a “for cause Audit”. Sandoz will make concerted efforts to schedule such Audit promptly. Amylin shall not be required to reimburse Sandoz for “for cause” audits.

(3) Amylin shall also have the right to perform [***] GMP compliance audit per year for up to [***] during Manufacture of the Product in accordance with provisions of the Quality Agreement at no cost to Amylin. [***] of Shionogi & Co., Ltd. shall be entitled to attend the GMP compliance audits.

(4) Sandoz’ obligation to allow visitors who are employees of Amylin or Amylin’s authorized representatives (with such authorized representatives being subject to Sandoz’ prior approval which will not be unreasonably withheld) is on condition that: (a) such visitors agree in writing to observe the reasonable customary requirements of Sandoz regarding security, health and safety, confidentiality or any other applicable regulations at the relevant premises; (b) any visit shall be under the specific supervision of Sandoz (without relieving any visitors of any obligations with respect to any damage or injury caused by them); and (c) Amylin uses reasonable endeavors to ensure that any visit and the number of visits is of minimal disruption to Sandoz’ day-to-day business. Amylin shall provide Sandoz with reasonable notice prior to each Amylin visit, communicate to Sandoz the reason for each Amylin visit, obtain Sandoz’ consent to the visit (which consent will not be unreasonably withheld) and ensure that Amylin visits are made by relevant personnel only. Amylin will, in consultation with Sandoz, develop an agenda for each Amylin visit.

(5) Amylin’s representatives may not be present in development labs. Amylin’s representatives visiting Sandoz’ facilities shall accept Sandoz’ procedures regulating external customer relationships (including cGMP training, hygiene, confidentiality and controlled access to facilities and documents) and will obtain Sandoz’ consent (which will not be unreasonably withheld) prior to any active participation in the Process or analytical testing.

(6) If Sandoz is required by any governmental authority to have inspected or approved the site of Manufacturing or storing the Product or any Raw Materials, Sandoz shall permit and cooperate with officials of the governmental authority to inspect such sites. Amylin will grant any consent needed for such inspection, if any. In the event of any such audit or inspection by a governmental authority, Sandoz will notify Amylin within one (1) business day by telephone followed by written notice not less than three (3) business days from its knowledge of any such audit or inspection. Sandoz shall cooperate with such governmental authority and allow them access to relevant information. Sandoz shall Provide Amylin with any and all copies of information provided, received, or generated by Sandoz prior to, in the course of, or

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as a result of any such Agency audit or inspection, to the extent such information relates to the Product.

(7) Visits by Amylin in excess of the [***], and presence of such persons in plant and Audits or inspections by government and Regulatory Authority representatives for purposes of obtaining regulatory approval of Product will be charged by Sandoz to Amylin per visit or presence at a rate of [***] or [***], whichever is less, provided, however, that there shall be no charge for persons in plant for one cGMP audit per calendar year or any “for-cause” audits.

(8) Sandoz shall provide Amylin with prompt notice of any information it receives relating to the safety of the Product, including any confirmed or unconfirmed information on adverse, serious or unexpected events associated with the use of the Product regardless of the source. For serious (based on a good-faith evaluation) unexpected events, notice must be given by telephone within one (1) business day after receipt of the information and followed by written notice not less than three (3) business days thereafter. Amylin, with Sandoz’ cooperation, shall be responsible for responding to the FDA and filing any reports with the FDA concerning such events (including Drug Experience Reports) caused by the Product.

(9) Sandoz shall be responsible for ensuring the safe operation of the Manufacture of the Product in its premises and, in particular, that the Process Transfer Materials supplied by Amylin can be safely used in Sandoz’ premises.

14. DURATION

Subject to the provisions for earlier termination contained herein, this Agreement shall come into force on the Effective Date hereof and shall continue in force until the [***] on which the very first commercial launch of the Amylin Finished Product by Amylin, its Affiliates or any sublicensee of Amylin or its Affiliates has occurred in either Europe or the United States.

15. RESERVATION AND COST

Amylin hereby reserves, and Sandoz hereby confirms the reservation of the capacity at the RPP4 Facility set forth on Schedule 4, and this shall, subject to the terms and conditions of this Agreement, constitute a binding order for such capacity for (i) the Process Characterization Phase, (ii) the Process Intermediates Supply, (iii) the Analytical Activities, (iv) if the Pilot Study Option has been exercised, the Pilot Studies, (v) the Process Validation Activities, and (v) if the Additional Campaign Option has been exercised, the Additional Campaign.

The activities, estimated duration and estimated budget of the Process Characterization Phase, the Process Intermediates Supply, the Analytical Activities, the Pilot Studies and the Process Validation Activities are listed in Schedule 4. Any costs which exceed the budget shall be subject to the prior written agreement of Amylin and Sandoz as an amendment to this Agreement.

16. PRICING AND TERMINATION FEES

(1) The fees to be paid by Amylin for all activities for the Process Characterization Phase, the Process Intermediates Supply, the Analytical Activities, the Pilot Studies

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and the Process Validation Activities are set forth in Schedule 4 and subject to adjustment as expressly stated in this Agreement. A good faith estimated budget including all such activities and costs is set out in Schedule 4. The parties understand that the content and scope of the activities described herein may be changed by mutual written agreement as an amendment to this Agreement and that the budget for the Process Characterization Phase, the Process Intermediates Supply, the Analytical Activities, the Pilot Studies and the Process Validation Activities is based on current assumptions.

(2) All orders for the Commercial Batches of Product during the Commercial Phase will be at the price(s) as set out in Schedule 7 and subject to adjustment as expressly stated in this Agreement. Pricing model “A” will apply for Firm Orders of [***] or more cGMP Batches per year. Pricing Model “B” will apply for Firm Orders of [***] cGMP Batches per year.

(3) The parties agree to enter into good-faith negotiations to transfer from a Batch Price to a per gram price at the [***] w.v. as soon as the Manufacture has been stable and robust for at least [***] campaigns with [***] representative Batches in total; provided, however, that the parties are not obligated to make such transfer if they cannot reach agreement on the terms of a per gram price. The parties agree that the information in Schedule 4 and the following principles shall be taken into account in calculating the per gram price:

(a) The per gram price will be based on the mean yield of Product per Batch over an agreed number of Batches.

(b) In calculating the mean yield, Batches with a yield which is more than [***] standard deviations from the mean will not be included.

(c) Once agreed, the per gram price will be reviewed by the parties if there is a change in Raw Materials, Bulk Drug Specifications, facility and/or a material change in the mean yield at that w.v.

(4) In the event this Agreement is terminated prior to completion of the Process Validation Activities either (a) by Amylin for any reason, other than Sandoz’ Bankruptcy or uncured material breach or a force majeure event affecting Sandoz’ ability to perform or (b) by Sandoz as a result of Amylin’s Bankruptcy or uncured material breach of this Agreement, then Amylin shall pay to Sandoz (i) an amount of [***] (i.e. the total budgeted amount of the Process Characterization Fee as set out in Schedule 4), (ii) an amount of [***] (i.e. the total budgeted amount of the Process Intermediates Supply Activities Fee as set out in Schedule 4), (iii) an amount of [***] (i.e. the total budgeted amount of the Analytical Activities Fee as set out in Schedule 4), (iv) if the Pilot Study Option has been exercised, an amount of [***] (i.e. the total budgeted amount of the Pilot Study Fee as set out in Schedule 4), (v) an amount of [***] (i.e. the total budgeted amount of the Process Validation Campaign Fee as set out in Schedule 4), and (vi) if the Additional Campaign Fee Option has been exercised, an amount of [***] (i.e. the total budgeted amount of the Additional Campaign Option Fee as set out in Schedule 7), in each case excluding the budgeted cost for materials, to the extent as such fees have not already been paid by Amylin. Sandoz shall use commercially reasonably efforts to reschedule the unused capacity resulting from a termination of this Agreement pursuant to this Clause 16(4) for other clients and will, should this be

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possible, deduct such rescheduled weeks from Amylin’s payment under this clause but will be reimbursed instead for any additional cost resulting from such rescheduling.

(5) In the event of any termination as described in Clause 16(4), in addition to the termination fees, Amylin shall also pay for any Raw Materials purchased or under a binding order placed by Sandoz prior to termination in accordance with this Agreement, that are non-cancellable, and subject to Sandoz’ obligation to mitigate such costs as far as possible. Any Raw Materials purchased by Sandoz and paid for by Amylin pursuant to this Clause 16(5) will, at Amylin’s option and expense, be shipped by Sandoz to Amylin or Amylin’s nominated representative. Instead of having such Raw Material shipped back to Amylin, Amylin may also offer to Sandoz to purchase such excess Raw Material and Sandoz will consider in good faith whether it can use such Raw Material in other products. Sandoz shall be under no obligation to purchase such Raw Material from Amylin.

17. INTELLECTUAL PROPERTY

(1) Amylin owns or has rights to all IP Rights and Know-How which (i) are owned or controlled by Amylin as of the Effective Date, or which (ii) are generated solely by employees or agents of Amylin during or after the term of this Agreement but do not belong to Collaboration IP Rights or Collaboration Know-How (“Amylin Background Technology”).

(2) Sandoz owns or has rights to all IP Rights and Know-How which (i) are owned or controlled by Sandoz as of the Effective Date, or which (ii) are generated solely by employees or agents of Sandoz during or after the term of this Agreement, but do not belong to Collaboration IP Rights or Collaboration Know-How (“Sandoz Background Technology”).

(3) As between the Parties, Amylin shall own all rights, title and interest in all Collaboration IP Rights and all Collaboration Know-How generated solely by employees or agents of Amylin (“Amylin Foreground Technology”). Sandoz shall own all rights, title and interest in all Collaboration IP Rights and all Collaboration Know-How that (i) is generated solely by employees or agents of Sandoz and that (ii) does not belong to the Amylin Assigned Technology (“Sandoz Foreground Technology”). Amylin and Sandoz shall own jointly (with an undivided 50-50 share) all Collaboration IP Rights and all Collaboration Know-How that (i) is generated jointly by employees or agents of Amylin and employees or agents of Sandoz and that (ii) does not belong to the Amylin Assigned Technology (“Joint Foreground Technology”).

(4) Amylin shall own all rights, title and interest in any parts of any Collaboration IP Rights and Collaboration Know-How to the extent that it is specifically related to and covering the Product, its manufacture or use (“Amylin Assigned Technology”). Sandoz hereby assigns to Amylin all rights, title and interest in such Amylin Assigned Technology. Sandoz hereby covenants to require all of its employees, agents, and contractors engaged in performing any aspect of services (i) to assign all of their right, title, and interest in Amylin Assigned Technology to Sandoz such that Sandoz may assign all such right, title and interest in Amylin Assigned Technology to Amylin and (ii) to agree to execute all documents necessary to perfect Amylin’s; right, title and interest in Amylin Assigned Technology. As

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compensation for each Amylin Assigned Technology (i) which has been assigned to Amylin as described above, and (ii) for which a patent application has been filed, Amylin shall pay to Sandoz an amount of [***] only as a one-time payment. In the event that any invention and/or resulting patent application or patent contains in part Amylin Assigned Technology and in part Sandoz Foreground Technology, Clause 17(8) shall apply accordingly.

(5) Amylin Foreground Technology and Amylin Assigned Technology shall constitute Confidential Information of Amylin. Sandoz Foreground Technology shall constitute Confidential Information of Sandoz. Joint Foreground Technology shall constitute Confidential Information of both Amylin and Sandoz.

(6) Sandoz agrees to inform Amylin of any invention partially or completely constituting Amylin Assigned Technology and further agrees to assist Amylin in applying for patents and all other instruments in the nature of patent with respect to any Amylin Assigned Technology. Such assistance includes the prompt review and execution of all documents, including assignments.

(7) Amylin shall be the only party responsible for filing, prosecuting, and maintaining any patent application covering Amylin Background Technology, Amylin Foreground Technology or Amylin Assigned Technology at its own cost, provided that if any Collaboration IP Rights partially consist of Amylin Assigned Technology and partially of Sandoz Foreground Technology, Clause 17(8) shall apply accordingly with respect to the filing, prosecution and maintenance of such Collaboration IP Rights. Sandoz shall be solely responsible for filing, prosecuting, and maintaining any patent application covering Sandoz Background Technology or Sandoz Foreground Technology (other than Amylin Assigned Technology).

(8) Amylin and Sandoz shall file any applications for IP Rights that (i) belong to Joint Foreground Technology or (ii) contain claims belonging to Amylin Assigned Technology and claims belonging to Sandoz Foreground Technology in the name of Amylin and Sandoz, and the parties shall determine by mutual agreement which party shall be responsible for the filing, prosecution and maintenance of such IP Rights on a case-by-case basis. Subject to the following, the parties shall equally share the costs of filing, prosecution and maintenance of Joint Foreground Technology. In the event that a party desires to abandon any patent application or patent which belongs to Joint Foreground Technology or to the Amylin Assigned Technology or to the Sandoz Foreground Technology, such party shall provide reasonable prior written notice to the other party of its intention to abandon such patent application or patent. In such event, the other party shall have the right, but not the obligation, to assume such Joint Foreground Technology or such Amylin Assigned Technology or such Sandoz Foreground Technology by notifying the abandoning party in writing within thirty (30) days about its intention. If the other party has timely given such notification, the abandoning party shall, at the expense of the assuming party, assign and transfer the relevant Joint Foreground Technology or the relevant Amylin Assigned Technology or the relevant Sandoz Foreground Technology to the assuming party, and the assuming party shall have the sole right, but not the obligation, to file, prosecute and maintain any such Joint Foreground Technology or Amylin Assigned Technology or Sandoz Foreground Technology at its own cost and expense, provided that the abandoning party shall and hereby does retain a world-wide, irrevocable, perpetual, non-exclusive, sublicenseable, royalty-free right and license to use the intellectual property covered

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by such Joint Foreground Technology or Amylin Assigned Technology or Sandoz Foreground Technology without any restrictions.

(9) The parties hereby acknowledge the ongoing duty to disclose all information material to patentability of any such invention to the other party for purposes of disclosure to any governmental authority responsible for IP Rights. The parties shall cooperate with each other and execute such other documents as may be necessary or desirable to achieve the objectives of this Clause 17(9).

(10) In respect of any Joint Foreground Technology, Sandoz hereby grants to Amylin a perpetual, worldwide, non-exclusive, royalty-free license (which shall be assignable and sublicensable) under its interest in such Joint Foreground Technology to manufacture, have manufactured, sell, offer for sale or otherwise use any product, including the Product. In respect of any Joint Foreground Technology, Amylin hereby grants to Sandoz a perpetual, worldwide, non-exclusive, royalty-free license (which shall be assignable and sublicensable) under its interest in such Joint Foreground Technology to manufacture, have manufactured, sell, offer for sale or otherwise use any product other than the Product or any Analogs.

(11) Amylin hereby grants to Sandoz and Sandoz’ Affiliates a non-exclusive, fully paid-up, royalty-free license under the Amylin Background Technology, the Amylin Foreground Technology, Amylin’s share in the Joint Foreground Technology and the Amylin Assigned Technology, to manufacture the Product and to perform the services under this Agreement and the applicable Schedules during the term.

(12) Upon the earliest of (a) Sandoz’ termination of this Agreement for any reason other than Amylin’s Bankruptcy or uncured material breach or a force majeure event affecting Amylin’s ability to perform, (b) Amylin’s termination of this Agreement pursuant to Clause 25(2), or as a result of Sandoz’ Bankruptcy or uncured material breach or a force majeure event affecting Sandoz’ ability to perform, or (c) expiration of this Agreement as described in Clause 14 (or any extension thereof) and provided this Agreement is not substituted by a commercial manufacturing agreement between Amylin and Sandoz upon such expiration of this Agreement, Sandoz shall grant Amylin the following non-exclusive, worldwide, non-transferable, perpetual licenses, with the right to sublicense: (i) a royalty-free license in and to any Sandoz Foreground Technology to manufacture, have manufactured, sell, offer for sale, or otherwise use the Product or any Analogs, and (ii) a royalty-bearing license to use the Sandoz Background Technology for the sole purpose of manufacturing the Product or any Analogs, provided, that, at the time of termination or expiration of this Agreement, such Sandoz Background Technology is used by Sandoz for the manufacture of Product and is reasonably necessary to manufacture the Product or any Analogs. The license terms for such licenses shall be negotiated in good faith between Sandoz and Amylin.

(13) Except as expressly set out in this Agreement or as is otherwise necessary for the proper performance of this Agreement by the parties, no license, express or implied, is granted by this Agreement by either party to the other under, in or to any of its IP Rights and Know-How.

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18. PAYMENT

(1) Invoices for the fees will be submitted to Amylin

(a) for the Process Characterization Phase:

Upon completion of experimental work done under milestone 1a according to Schedule 4 (including all requested Optional Activities, all analytical activities performed and all materials used until this time point)

Upon completion of experimental work done under milestone 1b according to Schedule 4 (including all requested Optional Activities, all analytical activities performed and all materials used until this time point)

Upon completion of experimental work done under milestone 1c according to Schedule 4 (including all analytical activities performed and materials used until this time point)

Upon completion of experimental work done under milestone 1d according to Schedule 4 (including all analytical activities performed and materials used until this Clause 18(1)(a).

(b) for the Process Intermediate Supply Activities:

Upon completion of the Manufacture of Process Intermediate Supplies.

(c) for the Analytical Activities:

Upon completion the remainder of the Analytical Activities and materials not charged under (a) and (b) above.

(d) for the Pilot Studies:

Upon completion of all experimental activities to be conducted in connection with the Pilot Studies.

(e) for the Process Validation Campaign and Commercial Phase:

For Engineering Batches: Upon completion of analysis by Sandoz or, if applicable, delivery of such Batches.

For cGMP Batches: upon Release by Sandoz and delivery of the individual cGMP Batches.

(f) for resins:

Upon delivery and acceptance of the resins by Sandoz’ Quality Department

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(2) Any amounts invoiced according to this Agreement shall be paid in full to the bank account designated by Sandoz within [***] after the date of receipt of the invoice. Sandoz shall send invoices to Amylin either by (i) e-mail at [***] or (ii) by fax at [***]. All invoices shall include Amylin’s purchase order number and a description of the Product or completed development work. Sandoz shall maintain accurate and complete accounting records specifically relating to the Manufacture of the Product under this Agreement, in accordance with Austrian generally accepted accounting principles and practices consistently applied. To the extent such records may be relevant, in Amylin’s reasonable opinion, to determining whether Sandoz is complying with its obligations under this Agreement, Amylin may appoint an independent international public accounting firm reasonably acceptable to Sandoz to audit such records during Sandoz’ normal working hours subject to providing five (5) days written notice of such audit to Sandoz. For this purpose, Sandoz shall retain such records for a period of [***] from the date of payment of each invoice by Amylin.

19. LIMITATION OF LIABILITY

EXCEPT AS A RESULT OF WILLFUL AND KNOWING MISCONDUCT OR ILLEGAL ACTIVITY, NEITHER PARTY SHALL IN ANY CIRCUMSTANCES BE LIABLE TO THE OTHER FOR ANY INCIDENTAL, CONSEQUENTIAL, SPECIAL, PUNITIVE OR INDIRECT LOSSES OR DAMAGES, OF ANY NATURE, INCLUDING DAMAGE TO GOODWILL, LOSS OF MARKET SHARE OR LOST PROFITS, WHETHER EXISTING OR PROSPECTIVE, OR THE COSTS ASSOCIATED WITH A DELAY IN ANY REGULATORY FILING. EXCEPT FOR CASES OF WILLFUL MISCONDUCT OR GROSS NEGLIGENCE AND NOTWITHSTANDING THE FOREGOING LIMITATION, THE LIABILITY OF SANDOZ WITH RESPECT TO NON-CONFORMANCE OF PRODUCT MANUFACTURED UNDER THIS AGREEMENT WITH SPECIFICATIONS AND/OR GMP REQUIREMENTS SHALL NOT EXCEED [***] PER OCCURRENCE AND [***] IN AGGREGATE PER CALENDAR YEAR.

20. SPECIFICATION

(1) The initial agreed Product Specifications are identified and listed in Schedule 2 under the headings ‘Sandoz Release Specifications’ and ‘Additional Specifications’. The parties acknowledge that they may adjust the Product Specifications in accordance with the terms of the Quality Agreement, but not less than [***] prior to the planned commencement of the Process Validation Campaign. Amylin will establish the Product Specifications in mutual agreement with Sandoz in writing (taking into account the information relating to the Product learned during the Process Characterization Phase) prior to the Process Validation Campaign. The Product Specifications, as amended from time to time by written agreement of the parties as set forth in the Quality Agreement, shall apply for the purposes of the obligations set out in this Agreement.

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21. WARRANTIES AND INDEMNITIES

(1) Each party hereby represents and warrants to the other party that (a) the person executing this Agreement is authorized to execute this Agreement; (b) this Agreement is legal and valid and the obligations binding upon such party are enforceable by their terms; and (c) the execution, delivery and performance of this Agreement does not conflict with any agreement, instrument or understanding, oral or written, to which such party may be bound, and does not violate any law or regulation of any court, governmental body or administrative or other agency having jurisdiction over it.

(2) Sandoz represents and warrants that (a) the cGMP Batches of Product (but not the Engineering Batches unless released by Sandoz by the issuance of a Certificate of Compliance) will be Manufactured in compliance with the Process, cGMP and the requirements of the FDA or the EMA (as applicable), (b) the cGMP Batches of Product (but not the Engineering Batches unless released by Sandoz by the issuance of a Certificate of Analysis indicating such conformity) will be in conformity with the Product Specifications when delivered to Amylin (c) it will comply with all applicable environmental and other laws and regulations relevant for the Manufacture, (d) it has and shall maintain all necessary and applicable licenses, permits and other authorizations for the Manufacture of the Product, (e) it shall deliver with the Product a Certificate of Analysis and, with respect to cGMP Batches of Product (but not the Engineering Batches unless released by Sandoz), a Certificate of Compliance, each such certificate in a form to be agreed upon by the parties, and (f) it is not debarred and has not and will not knowingly use in any capacity the services of any person debarred under Section 306(a) or (b) of the U.S. Generic Drug Enforcement Act of 1992 or any comparable law of any EU jurisdiction, as each may be amended from time to time.

(3) Sandoz represents and warrants that it performs internal cGMP audits no less than once per year in order to ensure compliance with the requirements of the Product Specifications and agrees to give a written certification of compliance with cGMP to (a) Amylin upon Amylin’s request, (b) an independent third party appointed by Amylin and reasonably acceptable to Sandoz, upon Amylin’s request, and/or (c) Regulatory Authorities upon such Regulatory Authorities’ request. Subject to Clause 13, Amylin employees or authorized representatives shall have the right upon reasonable notice during business hours to audit the quality systems of Sandoz that are related to the Product, including inspecting the facilities used in Manufacture, warehousing and shipping of the Product. If Sandoz becomes aware that any shipment of the Product to Amylin does not meet all the requirements of the Product Specifications, Sandoz will promptly notify Amylin.

(4) Sandoz represents and warrants that Sandoz will not implement any Sandoz Background Technology or Sandoz Foreground Technology as a Modification into the Process for the Manufacture of the Product if, at the time of such implementation, Sandoz is aware of any third party patent rights, under which Sandoz is not already licensed, that may be asserted to be valid and infringed by Sandoz’ use of such implemented Sandoz Background Technology or Sandoz Foreground Technology.

(5) Amylin represents and warrants that it is not aware of any third party patent rights, under which Amylin is not already licensed with the right to sub-license, that may be

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asserted to be valid and infringed by the Manufacture of the Product by Sandoz under the terms of this Agreement.

(6) Each party shall notify the other party in the event that such party becomes aware of information or action which would make any representation, warranty or covenant of such party inaccurate or untrue.

(7) EXCEPT FOR THE EXPRESS WARRANTIES CONTAINED IN THIS AGREEMENT, THE PARTIES PROVIDE NO OTHER WARRANTIES, EXPRESS OR IMPLIED, REGARDING THE PRODUCT AND DISCLAIM ALL OTHER EXPRESS AND IMPLIED WARRANTIES, INCLUDING (A) THE IMPLIED WARRANTIES OF MERCHANTABILITY, FITNESS FOR A PARTICULAR PURPOSE, AND NONINFRINGEMENT, AND (B) ANY IMPLIED WARRANTIES ARISING FROM ANY COURSE OF DEALING, USAGE OR TRADE PRACTICE, OR (C) ANY WARRANTIES ARISING UNDER APPLICABLE LAW.

(8) Sandoz shall indemnify and hold Amylin, its Affiliates and their respective directors, officers, employees and agents harmless from and against all losses, liabilities, damages and expenses (including reasonable attorneys’ fees and costs) resulting from all claims, demands, actions and other proceedings by any third party to the extent arising from (a) breach of any representation, warranty or covenant of Sandoz under this Agreement or (b) the gross negligence, recklessness or willful misconduct of Sandoz in the performance of its obligations and its permitted activities under this Agreement and based on applicable law, including the Manufacture of the Product, except to the extent Amylin has an obligation to indemnify Sandoz, its Affiliates and their respective directors, officers, employees and agents pursuant to Clause 21(9) below.

(9) Amylin shall indemnify and hold Sandoz, its Affiliates and their respective officers, employees and agents harmless from and against all losses, liabilities, damages and expenses (including reasonable attorneys’ fees and costs) resulting from all claims, demands, actions and other proceedings by any third party to the extent arising from (a) the breach of any representation, warranty or covenant of Amylin under this Agreement, (b) the research, development, commercialization or marketing of the Product or any product containing Product by or on behalf of Amylin, or (c) the gross negligence, recklessness or willful misconduct of Amylin in the performance of its obligations and permitted activities under this Agreement and based on applicable law, or (d) product liability, except to the extent Sandoz has an obligation to indemnify Amylin, its Affiliates and their respective directors, officers, employees and agents pursuant to Clause 21(8) above.

(10) Amylin shall indemnify and hold Sandoz, its Affiliates and their respective officers, employees and agents harmless from and against all losses, liabilities, damages and expenses (including reasonable attorneys’ fees and costs) resulting from all claims, demands, actions and other proceedings by any third party arising from any infringement by Sandoz of any third party intellectual property rights arising from its use of the Process and the Process Transfer Information for Manufacture of the Product, except to the extent that such infringement results from any Sandoz Modification to the Process or the Process Transfer Information implemented by Sandoz in the Manufacture of Product without the consent of Amylin; provided, however, that such indemnity shall not apply to the extent Sandoz has an obligation

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to indemnify Amylin, its Affiliates and their respective directors, officers, employees and agents pursuant to Clause 21(8) above.

(11) The party claiming the benefit of any indemnity hereunder must promptly notify the other of any claim as soon as it becomes aware of any claim, permit the other party to control the defense of the action, not accept any compromise or settlement of such claim or take any material steps in relation to such claim without the prior consent of the other party (not to be unreasonably withheld or delayed) and shall fully cooperate with the other party in the handling of any such claim. The party that controls the defense of the action shall bear the costs.

22. INSURANCE

(1) Amylin shall have in place product liability insurance with a reputable insurer in an amount appropriate for its business and products of the type that are the subject of this Agreement, and for its obligations under this Agreement. At Sandoz’ request, Amylin shall provide Sandoz with evidence of the existence and maintenance of such cover.

(2) Sandoz shall have in place public liability insurance with a reputable insurer or self-insurance coverage in an amount appropriate for its business and products of the type that are the subject of this Agreement, and for its obligations under this Agreement. At Amylin’s request, Sandoz shall provide Amylin with evidence of the existence and maintenance of such cover.

(3) In no event will the liability of either party be limited to that which is recoverable by insurance, notwithstanding any other limitation of liability as set out in this Agreement.

23. CONFIDENTIALITY

(1) Each party (the “Recipient”) undertakes to the other to treat as confidential all information relating to the other’s business operations, research, trade secrets and affairs (whether marked “confidential” or not) received from the other party (the “Discloser”) and all other information obtained from the Discloser that the Discloser has designated as confidential, both pursuant to this Agreement and prior to and in contemplation of it, either directly or from any person, firm, company or organization associated with the Discloser (“Confidential Information”). The Recipient shall respect and keep confidential the Discloser’s Confidential Information, use the same exclusively for the purpose of this Agreement and for no other use and disclose the same only as set forth in Clause 23(3) below.

(2) Confidential Information shall not include information that as demonstrated by competent evidence:

(a) was known to the Recipient prior to the disclosure, or is independently developed by or on behalf of the Recipient without use of the Discloser’s Confidential Information;

(b) is subsequently lawfully disclosed to the Recipient without any obligations of confidentiality by a third party who has not derived it directly or indirectly from the Discloser or any of the Discloser’s Affiliates;

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(c) was in the public domain or enters the public domain other than as a breach of this Clause 23; or

(d) the Discloser is required to disclose by law or by any competent Regulatory Authority or other governmental body authorized to require such disclosure.

(3) The Recipient shall allow access to the Discloser’s Confidential Information exclusively to those employees of itself, its Affiliates, its professional advisers and its licensees or subcontractors, as such are permissible pursuant to this Agreement, who have reasonable need to see and use it for the purposes of this Agreement, and shall inform each of said employees of the confidential nature of the Confidential Information and of the obligations of the Recipient in respect thereof. The Recipient shall ensure that the employees of it and its licensees or subcontractors, as such are permissible pursuant to this Agreement, having access to the Confidential Information are contractually bound by obligations of confidentiality and shall take such steps as may be necessary to enforce such obligations.

(4) Sandoz shall not submit for written or oral publication or presentation any manuscript, abstract, writing, printed material or the like that includes data of a scientific or technical nature containing any Confidential Information owned by Amylin without first obtaining Amylin’s prior written consent. Amylin shall not submit for written or oral publication or presentation any manuscript, abstract, writing, printed material or the like that includes data of a scientific or technical nature containing any Confidential Information owned by Sandoz without first obtaining Sandoz’ prior written consent.

(5) Nothing contained herein shall be construed as precluding Amylin from making, in its discretion, any disclosure of information of any type that relates to the Product; provided that such disclosure does not contain any Confidential Information of Sandoz.

(6) Each party shall maintain the confidentiality of all provisions of this Agreement and, without the prior consent of the other party, neither party shall make any press release or other public announcement of or otherwise disclose this Agreement or any of its provisions to any third party (other than to its officers and employees and attorneys, accountants, investment bankers and other professional advisers whose duties require familiarity with this Agreement), except for such disclosures as may be required by applicable law or government regulation in which case such party agrees to promptly notify the other party of the impending disclosure, providing sufficient time for the other party to take steps to protect such information.

(7) The Recipient’s obligation of confidentiality shall expire [***] from expiration or termination of this Agreement, or prior thereto with the written consent of the Discloser.

(8) Given the nature of the Confidential Information and the competitive damage that would result to a party upon unauthorized disclosure, use or transfer of its Confidential Information to any third party, the parties agree that monetary damages may not be a sufficient remedy for any breach of this Clause 23. In addition to all other remedies, a party shall be entitled to seek specific performance and injunctive

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and other equitable relief as a remedy for any breach or threatened breach of this Clause 23.

24. RECALLS

(1) If Sandoz or Amylin is required or requested by any government authority, or if Amylin in its sole discretion otherwise elects, to recall or dispose of any Product for any reason, Amylin shall be responsible for conducting any recall or disposal of such Product, and Sandoz shall cooperate with and give all reasonable assistance to Amylin in conducting any such recall or disposal, at Amylin’s expense. If Sandoz determines a recall or disposal may be necessary, then Sandoz will promptly notify Amylin in writing. Replacement of any recalled or disposed Product shall be subject to Article 27.

(2) Amylin shall keep Sandoz informed of any information it receives involving the Product that relates to any hazards to the health or safety of any personnel of Sandoz.

25. TERMINATION

(1) Each party may, without prejudice to its other rights and remedies, terminate this Agreement immediately by written notice to the other party if the other party

(a) is in breach of any of its material obligations under the Agreement and either that breach is incapable of remedy or the other party has failed to remedy the breach within [***] after receiving written notice requiring it to remedy that breach or

(b) becomes unable to pay its debts or becomes insolvent, or an order is made or a resolution passed for the liquidation, administration, winding-up, bankruptcy or dissolution of the other party (otherwise than for the purposes of a solvent amalgamation or reconstruction), or an administrative or other receiver, manager, trustee, liquidator, administrator or similar officer is appointed over all or any substantial part of the assets of the other party, or the other party enters into or proposes any composition or arrangement with its creditors generally, or anything analogous to any of these events occurs in any applicable jurisdiction (collectively, “Bankruptcy”).

(2) Amylin may terminate this Agreement for any scientific, regulatory, safety or economic reason. For the avoidance of doubt, it is agreed that in the event of such termination, the provisions on consequences of termination as set out in Clauses 16(4) and 16(5) shall apply.

(3) In the event Sandoz becomes aware of or receives written notice of a threatened patent infringement claim relating to the Manufacture of Product by Sandoz, and such patent infringement claim does not relate to technical information or know-how incorporated into the Manufacturing process by Sandoz, except for those incorporations requested or approved by Amylin, Sandoz shall promptly notify Amylin in writing of such claim. As soon as practicable after Amylin receives such written notice, the parties and their respective patent legal counsel will meet to discuss in good faith the validity of such infringement claim, applicable legal Precedence and all possible resolutions of the matter, including Amylin’s proposals.

36

If an outside patent attorney reasonably selected by Sandoz determines that any continued performance by Sandoz of its obligations under this Agreement will be held to constitute an infringement of a third party’s valid patent rights, then Sandoz may terminate this Agreement on ninety (90) days’ prior written notice to Amylin. For the avoidance of doubt, neither party shall be obliged to perform any of its obligations under this Agreement if and to the extent it is prevented from doing so by an injunction or court order asserted against such party based on an infringement or alleged infringement of any third party intellectual property right or based on any other government order precluding the manufacture, supply or distribution of the Product.

26. CONSEQUENCES OF TERMINATION

(1) Upon expiration of this Agreement or its termination for any reason:

(a) Each party shall return to the other all information and materials supplied by the other party; in particular, the Process Transfer Materials supplied by Amylin under this Agreement shall promptly be returned to Amylin;

(b) Sandoz shall continue to maintain information about the Product and its production for such time and in such manner as required by any Regulatory Authority in the US and EU and shall continue to respond in a timely manner to all queries and requests for information from Regulatory Authorities;

(c) Sandoz shall, at Amylin’s option, immediately cease Manufacture;

(d) Subject to Clause 26(1)(b), each party shall deliver to the other all materials, reports, and other documents (including copies thereof) in its possession or control containing Confidential Information of the other party, and each will cease to make use of the other’s Confidential Information;

(e) Sandoz shall, at Amylin’s option, either (i) arrange for transportation, at Amylin’s expense, of any materials paid for by Amylin to Amylin or Amylin’s designated representative or (ii) dispose of such materials on Amylin’s behalf.

(2) Upon expiry of this Agreement, its termination by Amylin pursuant to Clause 25(1) or its termination by Sandoz pursuant to Clause 25(3), then Sandoz shall provide information, support and assistance, including technical assistance, as is reasonably necessary for the effective transfer of the Manufacturing of the Product and Process to Amylin or to another entity nominated by Amylin. Amylin shall bear the reasonable costs of any technical assistance provided pursuant to this clause. Assistance provided by Sandoz shall be limited to [***]. Amylin will compensate Sandoz for such assistance at a rate of [***], based on actual hours worked.

(3) Termination of this Agreement shall not affect any accrued rights or liabilities of either party, nor shall it affect the coming into force or the continuation in force of any provision of this Agreement that is expressly or by implication intended to come into force or continue in force on or after termination.

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27. ACCEPTANCE

(1) Sandoz shall notify Amylin in writing when each Batch of the Product has been Released according to the Sandoz Release Specifications and cGMPs and is ready for delivery.

(2) Within [***] after the later to occur of i) delivery of the pre-defined test samples from a Batch of Product and ii) delivery of the Certificate of Analysis and Certificate of Compliance, Amylin or its representatives may, at Amylin’s option, analyze each Batch.

(3) Any complaints by Amylin that a Batch does not comply with the requirements of the Product Specifications shall be delivered to Sandoz in writing within [***] after the later to occur of (i) delivery of the pre-defined test samples from a Batch of Product and (ii) delivery of the Certificate of Analysis and Certificate of Compliance, with such complaint being accompanied by supporting data for purposes of consideration and verification by Sandoz. Any complaints by Amylin that a Batch is otherwise Defective shall be delivered to Sandoz in writing within [***] after discovery by Amylin, but in no event later than [***] after the later to occur of (i) delivery of the pre-defined test samples from a Batch of Product and (ii) delivery of the Certificate of Analysis and Certificate of Compliance, with such complaint being accompanied by supporting data for purposes of consideration and verification by Sandoz.

(4) If Sandoz receives a complaint under Clause 27(3) within the applicable time period for any Batch of Product other than an Engineering Batch, the following procedures shall apply:

(a) If the alleged Defect concerns the quantity or quality of the Product delivered and Sandoz accepts the details submitted by Amylin as to the Product’s noncompliance, Sandoz shall supply to Amylin (at Sandoz’ cost, including the cost of Raw Materials, components and shipment related thereto) the replacement quantity of the Product that was allegedly missing or Defective from the original Batch within [***].

(b) If the alleged Defect concerns the quantity or quality of the Product delivered and Sandoz does not accept the details submitted by Amylin, then within [***] from the date on which the details of Amylin’s complaint are received by Sandoz, Sandoz will appoint an independent scientific and technical expert acceptable to Amylin to review Amylin’s details supporting its complaint of noncompliance. Amylin shall not unreasonably refuse to accept the appointment of the independent scientific and technical expert identified by Sandoz. The findings of the expert shall be final and conclusively binding on the parties as to whether the Product is Defective. If the expert holds that the Product is Defective, all the fees and costs of the expert and the independent laboratory appointed by the expert to analyze the Product shall be paid by Sandoz, and Amylin shall have no obligation to pay for such replacement quantities of Product and Sandoz shall be responsible for all costs associated therewith (including the cost of Raw Materials, components and shipment related thereto). If the expert rejects Amylin’s complaint and determines that the Product is not Defective, all such fees and costs of the laboratory and the expert will be paid by

38

Amylin, Amylin shall pay for any replacement quantities delivered by Sandoz in addition to the original quantities delivered, and Amylin shall be considered to have finally and completely accepted such Batch of the Product.

28. FORCE MAJEURE

Neither party shall be liable to the other for any delay or nonperformance of its obligations under this Agreement if such non fulfillment is due to strikes, riots, war, invasion, acts of God, fire, explosion, floods, delay of carrier, acts of government agencies, judicial action, labor disturbance and/or other contingencies beyond its reasonable control, subject to the affected party promptly notifying the other party in writing of the cause and the likely duration of the delay or nonperformance and provided that the affected party shall use reasonable endeavors to limit the effect of such event on the other party; the performance of the affected party’s obligations, to the extent affected by the cause, shall be suspended during the period that the cause persists. Sandoz shall have no obligation to obtain supplies, Raw Materials, energy, utilities, labor and Products from a third party in order to supply Sandoz’ excused shortfall under this Agreement. The failure to be granted either U.S. FDA or EMA approval of Product shall not be deemed a Force Majeure situation according to the terms of this Agreement.

29. SPECIFIC INVESTMENTS

(1) Sandoz shall use commercially reasonable efforts to implement the modifications to its manufacturing facility required for the Manufacture of Product as identified in Schedule 6, collectively the “Facility Modifications”.

(2) The assets acquired for the Facility Modifications will be purchased by Sandoz on its own behalf, and Sandoz shall become the sole owner of such assets. Amylin shall reimburse Sandoz for all costs and expenses, including without limitation capital expenditures, reasonably incurred by Sandoz for the Facility Modifications and for any assets acquired as part of the Facility Modifications as follows: Sandoz shall be entitled to invoice for such costs as a surcharge on each individual Batch of the process Validation Campaign. Sandoz shall bear the costs of maintaining such assets to keep them in acceptable working condition.

(3) If (i) the specific attributes of the Product, (ii) Amylin and/or (iii) a Regulatory Authority (because of an inspection of Sandoz’ facilities or otherwise) require a change in the Process of Manufacture or to the Product Specifications that affects the cost of Manufacturing the Product, then upon written notification by either party to the other, Sandoz and Amylin will meet to negotiate in good faith (a) any change to the fees, (b) the date upon which such fee change will take effect, and (c) any other consequences arising from such change (including but not limited to investments). In the event no agreement can be reached, Sandoz shall be under no obligation to Manufacture Product.

(4) Any acquisition of fixed or moveable assets or any other investments that may be required for Sandoz to perform the activities under this Agreement shall require prior written agreement between the parties, and Sandoz shall have no obligations whatsoever to make such investments and/or to bear any costs, expenses or capital expenditures with respect to such investments.

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30. NOTICES

(1) Any notice or other information required or permitted to be given under this Agreement shall be in writing and shall be delivered in person, or sent to the other by first-class registered pre-paid post (return receipt requested), fax or comparable means of communication addressed as follows:

1.	if to Amylin, address to:	Amylin Pharmaceuticals, Inc.
		9360 Towne Centre Drive
		San Diego, CA 92121
		Fax No: [***]
		Attention:
		[***]
		[***]
	With a copy to:	Amylin Pharmaceuticals, Inc.
		9360 Towne Centre Drive
		San Diego, CA 92121
		Fax No: [***]
		Attention: General Counsel
(b)	if to Sandoz, address to:	Sandoz GmbH
		Biochemiestrasse 10
		A-6250 Kundl
		Austria
		Fax No: [***]
		Attention: Head of Biotech Cooperations

or to other such address or addresses as may be specified from time to time in a written notice.

(2) Any notice, request, approval or other document shall be deemed to have been served at the time of delivery or if sent by fax, two (2) hours after the time of dispatch, if dispatched before 3:00 p.m. (local time at the place of destination) on a business day, and in any other case at 10:00 a.m. (local time at the place of destination) on the next business day after the date of dispatch.

31. ENTIRE AGREEMENT

(1) This Agreement and any documents referred to herein together contain the entire agreement between the parties with respect to its subject matter and supersede all previous agreements, understandings, representations and statements between the parties relating to the subject matter of this Agreement.

(2) Each party acknowledges that, in agreeing to enter into this Agreement, it has not relied on any representation, warranty, collateral contract or other assurance made by or on behalf of any other party, other than those representations and warranties expressly made by the parties in this Agreement.

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32. MISCELLANEOUS PROVISIONS

(1) Assignment. Unless otherwise expressly permitted hereunder, neither party may assign any of its rights or delegate any of its duties under this Agreement without the express prior written consent of the other party; provided, however, that either party may assign its rights and obligations under this Agreement without the other party’s consent to a third party in the event of (a) a merger with or acquisition by such third party or (b) such third party’s purchase of all or a substantial part of the business to which this Agreement relates. Without limitation to the foregoing restrictions on assignment, this Agreement shall be binding upon and shall inure to the benefit of each of the parties hereto and its successors and permitted assignees.

(2) Relationship. Nothing in this Agreement shall create, or be deemed to create, a partnership, employment, agency or joint venture between the parties, and, except as expressly set forth herein, neither party shall have any right by virtue of this Agreement to bind the other party in any manner whatsoever.

(3) Survival of Provisions. The termination of this Agreement, howsoever occasioned, shall be without prejudice to any obligations or rights on the part of either party that accrued prior to or upon termination. The following Clauses of this Agreement shall survive termination hereof: 1, 16(4), 16(5), 17, 18, 19, 21(8) through 21(11), 22, 23, 24, 26, 27(2) through 27(4), 28, 29(2), 30, 31, 32, 33 and 34.

(4) Waivers. The failure of either party at any time to enforce any of the terms, provisions or conditions of this Agreement or to exercise any right under this Agreement shall not constitute a waiver of the same or affect that party’s right thereafter to enforce the same.

(5) Severability. If any provision of this Agreement is held to be illegal, invalid or unenforceable under present or future laws effective while this Agreement remains in effect, the legality, validity and enforceability of the remaining provisions shall not be affected thereby, and in lieu of each such illegal, invalid or unenforceable provision, there shall be added automatically as a part of the document a provision that is legal, valid and enforceable, and as similar in terms to such illegal, invalid or unenforceable provision as may be possible while giving effect to the benefits and burdens for which the parties have bargained hereunder.

(6) English Language. Unless otherwise specified in this Agreement, all documentation, records, reports and other written information shall be provided by Sandoz to Amylin in the English language. The English language version of this Agreement shall be controlling, notwithstanding any translation thereof into another language.

(7) Counterparts. This Agreement is executed simultaneously in two counterparts. These two counterparts shall collectively constitute one and the same Agreement.

(8) Subcontracting. If Sandoz determines that proper Manufacturing of the Product requires the retention of one or more subcontractors or consultants, Sandoz will obtain the written approval of Amylin and of the Regulatory Authority (if required) before using any subcontractors or consultants. Sandoz will be fully responsible to Amylin for any portion of the services performed by any subcontractor or consultant

41

to the same extent as if such portion of the services was performed directly by Sandoz.

(9) Amendments. No modification or alteration of any of the terms of this Agreement shall be of any effect unless in writing signed by both parties.

(10) Headings. Headings included in this Agreement are for convenience only, and shall not be used to construe this Agreement.

33. GOVERNING LAW AND JURISDICTION

This Agreement is governed by and shall be construed in accordance with Austrian law. Any dispute arising under or relating to this Agreement between the parties hereto shall be finally settled through binding arbitration under the rules of arbitration of the International Chamber of Commerce. The arbitration shall be confidential and take place in Vienna, Austria in the English language. The prevailing party may enter such decision in any court having competent jurisdiction.

34. DISPUTE RESOLUTION

The parties agree to attempt to settle any dispute, controversy or difference that may arise between them out of, in relation to, or in connection with this Agreement, including the breach thereof, by good-faith discussions. Any such dispute which cannot be settled by mutual understanding of the parties shall be submitted for resolution to a senior executive officer of each party having authority to resolve the dispute who shall promptly meet and endeavor to reach resolution through good-faith negotiations. In the event the parties cannot reach resolution within [***], then either party may commence arbitration (pursuant to the arbitration rules described in Clause 33 as it deems necessary to resolve the dispute.

[Remainder of this page intentionally left blank]

42

 

IN WITNESS WHEREOF, each party has caused this Manufacturing Agreement to be executed by its duly authorized representatives as of the date first written above.

Amylin:	SANDOZ:
Amylin Pharmaceuticals, Inc.	Sandoz GmbH

By: [***] By: [***]

Name: [***] Name: [***]

Title: [***] Title: [***]

Date Signed: 4 Oct. 2010 Date Signed:

By:	[***]
Name:	[***]
Title:	[***]

Date Signed: October 19, 2010

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EX-10.11.2

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.11.2

Confidential

First Amendment to

Contract Manufacturing Agreement (Metreleptin SLD) dated September 30, 2010

THIS AMENDMENT (“Amendment”) entered into as of September 01, 2011 is by and between Amylin Pharmaceuticals, Inc., whose principal office is at 9360 Towne Centre Drive, San Diego, California 92121 (“Amylin”) and Sandoz GmbH, whose principal office is at Biochemiestrasse 10, A6250 Kundl, Austria (“Sandoz”).

WHEREAS, Amylin and Sandoz have entered into a Contract Manufacturing Agreement (Metreleptin SLD) effective as of September 30, 2010 (the “Agreement”), and desire to amend the Agreement as described below.

For good and valuable consideration, the receipt and sufficiency of which is hereby acknowledged, the parties agree as follows:

1. The third item in the table of contents to the Agreement is hereby amended and restated in its entirety as follows: “Post-Validation Characterization Activities.”

2. Clause D(b) under the heading “WHEREAS” of the Agreement is hereby amended and restated in its entirety as follows:

“Performance of experimental activities to be conducted in connection with the Post-Validation Characterization Activities” (Post-Validation Characterization Activities defined below).”

3. Clause 1(1) of the Agreement is hereby amended as follows:

The following defined terms are hereby deleted in their entirety: (i) “Process Intermediate Supply Activities,” (ii) “Process Intermediate Supply Fee,” and “Process Intermediate Work Plan.”

The definition of “Process Transfer Materials” is hereby restated in its entirety as follows: “‘Process Transfer Materials’ means the cell banks, reference standards and other materials to be provided by Amylin to Sandoz for the Process Characterization Phase, Analytical Activities, Post-Validation Characterization Activities, Pilot Studies, Process Validation Campaign and Commercial Phase, as listed in Schedule 4.”

The following defined terms and definitions are hereby added to Clause 1(1):

“‘Post-Validation Characterization Activities’ has the meaning set forth in Clause 3(2).”

“‘Post-Validation Characterization Supply Fee’ has the meaning set forth in Schedule 4.”

“‘Post-Validation Characterization Work Plan’ means an outline of the activities, timing and sampling to be performed under the Post-Validation Characterization Activities.”

1

Confidential

4. Clause 3 of the Agreement is hereby amended and restated in its entirety as follows:

“3. POST-VALIDATION CHARACTERIZATION ACTIVITIES

(1)Sandoz will provide Amylin with a draft Post-Validation Characterization Work Plan in accordance with Schedule 4. Not less than [***] in advance of commencing the Post-Validation Characterization Activities, Amylin and Sandoz will agree upon the final Post-Validation Characterization Work Plan in writing.

(2)Sandoz will perform all activities for which it is responsible under the Post-Validation Characterization Work Plan (the “Post-Validation Characterization Activities”) in accordance with the Post-Validation Characterization Work Plan and Schedule 4.

(3)Amylin will provide the Process Transfer Materials required for the Post-Validation Characterization Activities as set out in Schedule 4 free of charge.

(4)       Upon completion of the Post-Validation Characterization Activities according to the final Post-Validation Characterization Work Plan, Sandoz shall issue an invoice for the activities and raw materials of Post-Validation Characterization as described in table 2 of schedule 4 of the Amendment.

5. All references to “Process Intermediates Supply” contained in Clauses 15 and 16 of the Agreement are hereby deleted and replaced with the words “Post-Validation Characterization Activities”.

6. Clause 18(l)(b) of the Agreement is hereby amended and restated in its entirety as follows:

“(b) For the Post-Validation Characterization Process Activities:

Upon completion of the experimental activities to be conducted in connection with the Post-Validation Characterization Activities.”

7. Sandoz Release Specifications Table set forth in Schedule 2 of the Agreement is entirely amended and replaced by the following:

2

[***]

3

Confidential

For the avoidance of doubt, the “Additional Specifications” Tables contained in Schedule 2 of the Agreement shall continue to apply.

8. Table 2 of Schedule 4 of the Agreement is hereby amended and restated in its entirety as follows:

2. Planned activities and pricing for Post-Validation Characterization

Activity	Resource	Estimated duration [weeks]	Price per week [Euro]	Estimated price [Euro]
Resin Reuse Studies	*DSP Lab	[***]	[***]	[***]
Analytical Support	*Analytical Lab	[***]	[***]	[***]
Microbiology Support	*Microbiology Lab	[***]	[***]	[***]
Materials ** (Resins, disposables, columns, etc.)	NA	NA	NA	[***]
Total budget estimate (“Post-Validation Characterization Supply Fee *)				[***]

* Weekly costs for lab development microbiological and analytical support are based on lab units ([***] and [***])

** All raw materials, resins and disposables will be charged at cost plus [***] handling fee. Cost of subcontractors and necessary laboratory materials to be provided by Sandoz shall be charged to Amylin at cost plus [***].”

9. Schedule 4 of the Agreement is hereby further amended by adding the following footnote to Tables 1 through 5, as amended:
“Microbiology Lab Support will be performed at a rate of [***].

10. Except as expressly stated above, the Agreement remains in full force and effect in accordance with its terms. Unless otherwise stated in this Amendment each defined term herein shall have the same meaning ascribed to such term in the Agreement.

11. The terms and conditions of this Amendment are hereby incorporated into and made a part of the Agreement.

(the remainder of this page is intentionally left blank)

4

Confidential

IN WITNESS WHEREOF, the parties have caused this Amendment to be executed as of the Effective Date written above.

Sandoz GmbH		Amylin Pharmaceuticals, Inc.
By:	[***]	By:	[***]
Name:	[***]	Name:	[***]
Title:	[***]	Title:	[***]

5

EX-10.11.3

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.11.3

 

AMENDMENT NO. 2 TO
  CONTRACT MANUFACTURING AGREEMENT (Metreleptin SLD)

THIS AMENDMENT NO. 2 (the “Amendment”) is made as of 18 December 2012 (the “Amendment Effective Date”) by and between Amylin Pharmaceuticals, LLC (formerly known as and successor is interest to Amylin Pharmaceuticals, Inc.) with a principal place of business at 9360 Towne Centre Drive, San Diego, California 92121, USA (“Amylin”) and Sandoz GmbH, with a principal place of business at Biochemiestr. 10, A6250 Kundl, Austria (“Sandoz”).

WITNESSETH:

WHEREAS, Amylin and Sandoz are parties to a certain Contract Manufacturing Agreement in relation to Metreleptin SLD dated as of 30 September 2010, as amended (the “Agreement”); and

WHEREAS, pursuant to Clause 32(9) of the Agreement, the Agreement may be amended only by the written agreement between the Parties; and

WHEREAS, further to the “Consent to Disclosure” letter dated 30 March 2012 attached hereto as Exhibit A and incorporated herein by reference, Amylin desires that Sandoz supports one of Amylin’s lipodystrophy program licensees, Shionogi & Co., Ltd. 1-8, Dochomachi 3-chome, Chuo-ku, Osaka 541-0045, Japan (hereinafter “Shionogi”), in accordance with the terms and conditions set forth in this Amendment; and

WHEREAS, Amylin and Sandoz desire to integrate the Agreement to reflect mutually agreed upon terms in accordance with the provisions of this Amendment.

NOW, THEREFORE, in consideration of the mutual covenants and agreements set forth herein and for other good and valuable consideration, the receipt and adequacy of which are hereby acknowledged, Amylin and Sandoz agree as follows:

1. Definitions.

Capitalized terms used herein, but not otherwise defined, shall have the meanings ascribed to them in the Agreement.

2. Amendments.

2.1       Shionogi Pre-PAI Audit. Amylin hereby agrees to pay Sandoz for [***] GMP audit as requested by Amylin at Sandoz’s RPP4 Facility for purposes of preparation to Japanese health authorities’ regulatory inspections in support of Shionogi’s Japanese regulatory filings (“Shionogi Pre-PAI Audit”). Sandoz’s obligation to allow such audit is subject to Shionogi’s and Amylin’s acceptance of the conditions set forth in Clause 13(4) of the Agreement. The Shionogi Pre-PAI Audit team shall be limited to [***] Shionogi [***] and [***] Amylin [***].

 

1

In consideration for the Shionogi Pre-PAI Audit, which shall not last more than [***], Amylin agrees to pay Sandoz the sum of [***].

2.2.       Shionogi [***] Audit. [***] during the term of the Agreement, Amylin shall have the right to conduct (or have conducted) – in addition to the GMP compliance audit set forth in Clause 13.3 of the Agreement – [***] to support Shionogi regulatory approval in Japan (“Shionogi [***] Audit”). For the sake of clarity only, the expression [***] shall mean that, if a Shionogi [***] Audit is performed during a certain calendar year, no Shionogi [***] Audit may be performed in the following [***], unless otherwise agreed by the parties. Sandoz’s obligation to allow such audit is subject to Shionogi’s and Amylin’s acceptance of the conditions set forth in Clause 13(4) of the Agreement. The Shionogi [***] Audit team shall be limited to [***] and [***]. The Shionogi [***] Audit shall (i) be performed during the Manufacture of the Product and (ii) not last for more than [***]. In consideration for said Shionogi [***] Audit, Amylin agrees to pay Sandoz the sum of [***] or [***] (office hours, Monday to Friday), whichever is less, subject to the provisions and price adjustment of Section 6 (“General”) of Schedule 4 (“Capacity and Pricing Estimates, Fees”) of the Agreement. It is understood that this Clause 2.2 of this Amendment does not affect in any way whatsoever Amylin’s right to have one representative of Shionogi to attend the GMP compliance audit set forth in Clause 13.3.

2.3       Japanese Marketing Application. Upon Amylin’s request, Sandoz shall perform the preparation of documents to support a Japanese marketing application by Shionogi. As compensation for such services rendered under this Clause 2.3, Amylin agrees to pay Sandoz the sum of [***], based on actual hours worked, subject to the provisions and price adjustments of Section 6 (“General”) of Schedule 4 (“Capacity and Pricing Estimates, Fees”) of the Agreement.

2.4       Regulatory Inspection for Approval in Japan. Sandoz shall support one (1) Japanese Regulatory Authority inspection at the Sandoz’s RPP4 Facility to obtain regulatory approval in Japan. In consideration for the support under this Clause 2.4, Amylin agrees to pay Sandoz the sum of [***] for up to [***] and shall be limited to a maximum of [***] Shionogi participants in total. Should the inspection under this Clause 2.4 last more than [***], Amylin agrees to pay Sandoz, for each additional day, the sum of [***] or [***] (office hours, Monday to Friday), whichever is less, subject to the provisions and price adjustment under Section 6 (“General”) of Schedule 4 (“Capacity and Pricing Estimates, Fees”) of the Agreement.

Follow-up inspections by the Japanese Regulatory Authority (based on, and directly related to, the inspection indicated under this Section 2.4) shall be treated as follows:

(i) The first follow-up inspection will be free of charge if limited to [***]; for each additional day, the sum of [***] or [***] (office hours, Monday to Friday), whichever is less – subject to the provisions and price adjustment of Section 6 (“General”) of Schedule 4 (“Capacity and Pricing Estimates, Fees”) of the Agreement – shall apply;

(ii) For any subsequent follow-up inspections Amylin agrees to pay Sandoz the sum of  [***] or [***] (office hours, Monday to Friday), whichever is

2

less, subject to the provisions and price adjustment of Section 6 (“General”) of Schedule 4 (“Capacity and Pricing Estimates, Fees”) of the Agreement.

2.5       Post-Approval Regulatory Inspections in Japan. Sandoz’s support for Japanese Regulatory Authority’s post-approval inspections is subject to Clause 13.6 of the Agreement. For each any every Japanese Regulatory Authority’s post-approval inspections Amylin agrees to pay Sandoz the sums of [***] or [***] (office hours, Monday to Friday), whichever is less, subject to the provisions and price adjustment of Section 6 (“General”) of Schedule 4 (“Capacity and Pricing Estimates, Fees”) of the Agreement.

2.6       Preparation of Document to Maintain Japanese Regulatory Approval. Sandoz’s preparation of documents required by Shionogi to maintain regulatory approval in Japan during the commercial phase shall be subject to Clause 9.4(d) of the Agreement. For the sake of clarity only, as compensation for the services rendered under this Clause 2.6, Amylin agrees to pay Sandoz the sum of [***], based on actual hours worked, subject to the provisions and price adjustment of Section 6 (“General”) of Schedule 4 (“Capacity and Pricing Estimates, Fees”) of the Agreement.

2.7       Modifications. In the event that a Japanese Regulatory Authority requires modifications to Sandoz’s production process, equipment, or materials in order to grant or maintain regulatory approval to Shionogi, Amylin and Sandoz agree to negotiate specific investments and/or changes to fees in accordance with Clause 29.3 of the Agreement.

2.8       Confidentiality. Amylin hereby confirms and agrees that Shionogi is and shall be subject to confidentiality obligations as set forth in a separate confidentiality agreement (to which Sandoz shall be a party). It is hereby agreed and convened that Amylin shall be held responsible and liable towards Sandoz for any Shionogi’s breach of confidentiality relating to Sandoz’s business operations, research, trade secrets and affairs (whether marked “confidential” or not) and all other information obtained under this Agreement or any related agreements and/or documents.

3. Reference to and Effect on the Agreement.

3.1   On and after the Amendment Effective Date, each reference to “this Agreement,” “hereunder,” “hereof” “herein,” or words of like import shall mean and be a reference to the Agreement as amended hereby. No reference to this Amendment need be made in any instrument or document at any time referring to the Agreement, a reference to the Agreement in any of such instrument or document to be deemed to be a reference to the Agreement as amended or integrated hereby.

3.2   Except as expressly amended or integrated by this Amendment including the Exhibit A attached hereto, the provisions of the Agreement shall remain in full force and effect.

4. Counterparts.

This Amendment may be executed in any number of counterparts, each such counterpart shall be deemed to be an original instrument, and all such counterparts together shall constitute

3

but one agreement. Any such counterpart may contain one or more signature pages. This Amendment may be executed by facsimile signature pages.

IN WITNESS WHEREOF, the undersigned have caused this Amendment to be executed and delivered on the date first written above.

Sandoz GmbH		Amylin Pharmaceuticals, LLC
Date:	16.01.2013	Date:
By:	[***]	By:	[***]
Name:	[***]	Name:	[***]
Title:	[***]	Title:	[***]
Sandoz GmbH
Date:	16.01.2013
By:	[***]
Name:	[***]
Title:	[***]

4

Exhibit A

CONSENT TO DISCLOSURE LETTER

Attached

5

		CONFIDENTIAL
	Amylin Pharmaceuticals, Inc.	Tel     (858) 552 2200
	9360 Towne Centre Drive	Fax    (858) 552 2212
	San Diego, CA 92121  USA	www.amylin.com

March 30, 2012

Attn: [***]
[***]
Sandoz GmbH
Biochemiestrasse 10
A-6250 Kunndl / Tirol
AUSTRIA

Re:       Consent to Disclosure

Dear Friedrich:

Amylin Pharmaceuticals, Inc. (“Amylin”) has out-licensed its lipodystrophy program in certain countries and is presently contemplating the negotiation of additional licenses of its lipodystrophy program in other countries (collectively, such existing license and contemplated licenses are hereinafter referred to as the “Lipodystrophy Transaction”).

In connection with the Lipodystrophy Transaction, Amylin would like to share with existing and potential licensees/partners the existence of its relationship with Sandoz GmbH (“Sandoz”), including the existence and terms of the Process Transfer and Initial Manufacturing Agreement, dated November 1, 2006, as amended, the Contract Manufacturing Agreement, dated September 30, 2010, as amended, and the Quality Agreement dated March 13, 2007, as amended, each by and between Amylin and Sandoz (each an “Agreement” and collectively, the “Agreements”), certain Confidential Information (as defined in each Agreement) and all documents and agreements related thereto (collectively, the “Disclosure”). Amylin would also like to make the Disclosure to existing and new licensees/partners to support such parties’ filings of regulatory documents, and communications related thereto, with applicable regulatory authorities. It is understood that Amylin will promptly provide Sandoz with the names of the existing and potential licensees/partners to which the Disclosure and/or the Agreements will be disclosed.

The parties acknowledge that this Consent to Disclosure is not intended nor shall it be construed to amend the Agreements with respect to the specific regulatory or statutory requirements pursuant to which Sandoz has agreed to manufacture and/or release products. The parties further acknowledge that the compliance with the regulatory and statutory requirements of countries/jurisdictions in addition to those set forth in the Agreements will be evaluated by Sandoz and Amylin on a case-by-case basis and the relevant terms and conditions (including, but not limited to, the relevant financial provisions) will be subject to good faith negotiations.

In light of the above, Amylin hereby requests Sandoz’s consent to the Disclosure in connection with the Lipodystrophy Transaction and also for the purpose of permitting Amylin’s licensees/partners to disclose and use information obtained pursuant to the Disclosure to the extent necessary for regulatory documents and/or communications with regulatory authorities for the development and/or commercialization of lipodystrophy products. The Disclosure will be pursuant to confidentiality agreements between Amylin and its existing and potential licensees/partners, which will include obligations of confidentiality consistent with those of the

CONFIDENTIAL

Agreements. Such confidential disclosure agreements with potential partners shall include restrictions on further disclosure of Sandoz Confidential Information by such potential partners. It is however understood that any such potential partners will have the right to disclose information on the Lipodystrophy Transaction to their respective agents, advisors, or representatives who need to know such information for purposes of assisting or advising such potential partners with respect to the Lipodystrophy Transaction.

Please execute this consent and return a copy of the executed consent as soon as possible to [***] via e-mail at [***]. By signing below, you hereby confirm your grant of the requested consent. You should retain a duplicate copy of this letter for your records. Should you have any questions with respect to the foregoing, please call [***] at [***]. Your prompt attention to this matter is greatly appreciated.

Very truly yours,		ACCEPTED and AGREED:
Amylin Pharmaceuticals, Inc.		Sandoz GmbH
By:	[***]	By:	[***]

[***] Name: [***]

[***] Title: [***]

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EX-10.11.4

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.11.4

AMENDMENT NO. 3 TO
CONTRACT MANUFACTURING AGREEMENT (Metreleptin SLD)

THIS AMENDMENT NO. 3 (the “Amendment”) is made as of 8^th July 2013 (the “Amendment Effective Date”) by and between Amylin Pharmaceuticals, LLC (formerly known as and successor in interest to Amylin Pharmaceuticals, Inc.) with offices at 9625 Towne Centre Drive, San Diego, California 92121, USA (“Amylin”) and Sandoz GmbH, with a principal place of business at Biochemiestr. 10, A6250 Kundl, Austria (“Sandoz”).

WITNESSETH:

WHEREAS, Amylin and Sandoz are parties to a certain Contract Manufacturing Agreement in relation to Metreleptin SLD dated as of 30 September 2010, as amended (the “Agreement”); and

WHEREAS, Amylin and Sandoz desire to integrate the Agreement to reflect mutually agreed upon terms in accordance with the provisions of this Amendment.

NOW, THEREFORE, in consideration of the mutual covenants and agreements set forth herein and for other good and valuable consideration, the receipt and adequacy of which are hereby acknowledged, Amylin and Sandoz agree as follows:

1. Definitions.

Capitalized terms used herein, but not otherwise defined, shall have the meanings ascribed to them in the Agreement.

2. Amendments.

2.1       Sandoz Release Specifications Table set forth in Schedule 2 of the Agreement, as amended and replaced by Clause 7 of the First Amendment to the Agreement, is hereby amended and entirely replaced by the document CP-No. / CP-Version 7206.3, which is incorporated herein by reference.

2.2       Amylin’s address and contact information has changed; therefore Section 30(1)1. is amended and replaced with the following:

If to Amylin, address to:	Amylin Pharmaceuticals, LLC 9625 Towne Centre Drive San Diego, CA 921212 Attention: [***] [***]
With a copy to:	Vice President and Associate General Counsel, Transactions Contracts Center of Excellence Bristol-Myers Squibb Company

 

Route 206 & Province Line Road
Princeton, New Jersey 08543-4000

3. Reference to and Effect on the Agreement.

3.1       On and after the Amendment Effective Date, each reference to “this Agreement,” “hereunder,” “hereof,” “herein,” or words of like import shall mean and be a reference to the Agreement as amended hereby. No reference to this Amendment need be made in any instrument or document at any time referring to the Agreement, a reference to the Agreement in any of such instrument or document to be deemed to be a reference to the Agreement as amended or integrated hereby.

3.2       Except as expressly amended or integrated by this Amendment including the Exhibit A attached hereto, the provisions of the Agreement shall remain in full force and effect.

4. Counterparts.

This Amendment may be executed in any number of counterparts, each such counterpart shall be deemed to be an original instrument, and all such counterparts together shall constitute but one agreement. Any such counterpart may contain one or more signature pages. This Amendment may be executed by facsimile signature pages.

IN WITNESS WHEREOF, the undersigned have caused this amendment to be executed and delivered on the date first written above.

Sandoz GmbH Amylin Pharmaceuticals, LLC

Date:	Aug 08, 2013	Date:	08 Aug 2013
By:	[***]	By:	[***]
Name:	[***]	Name:	[***]
Title:	[***]	Title:	[***]

Sandoz GmbH

Date:	August 1, 2013
By:	[***]
Name:	[***]
Title:	[***]

EX-10.11.5

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

 Exhibit 10.11.5

AMENDMENT NO. 4 TO
CONTRACT MANUFACTURING AGREEMENT (Metreleptin SLD)

THIS AMENDMENT NO. 4 (the “Amendment”) is made as of 23^rd June 2014 (the “Amendment Effective Date”) by and between Amylin Pharmaceuticals, LLC (formerly known as and successor in interest to Amylin Pharmaceuticals, Inc.) with a principal place of business at 9625 Towne Centre Drive, San Diego, California 92121, USA (“Amylin”) and Sandoz GmbH, with a principal place of business at Biochemiestr. 10, A6250 Kundl, Austria (“Sandoz”).

WITNESSETH:

WHEREAS, Amylin and Sandoz are parties to a certain Contract Manufacturing Agreement in relation to Metreleptin SLD dated as of 30 September 2010, as amended (the “Agreement”); and

WHEREAS, Amylin and Sandoz desire to integrate the Agreement to reflect mutually agreed upon terms in accordance with the provisions of this Amendment.

NOW, THEREFORE, in consideration of the mutual covenants and agreements set forth herein and for other good and valuable consideration, the receipt and adequacy of which are hereby acknowledged, Amylin and Sandoz agree as follows:

1. Definitions.

Capitalized terms used herein, but not otherwise defined, shall have the meanings ascribed to them in the Agreement.

2. Amendments.

Sandoz Release Specifications Table set forth in Schedule 2 of the Agreement, as amended and replaced by Clause 7 of the First Amendment to the Agreement and clause 2.1 of the Third Amendment to the Agreement, is hereby amended and entirely replaced by the document CP-No. / CP-Version 7206.4 (Exhibit A), which is incorporated herein by reference.

3. Reference to and Effect on the Agreement.

3.1           On and after the Amendment Effective Date, each reference to “this Agreement,” “hereunder,” “hereof,” “herein,” or words of like import shall mean and be a reference to the Agreement as amended hereby. No reference to this Amendment need be made in any instrument or document at any time referring to the Agreement, a reference to the Agreement in any of such instrument or document to be deemed to be a reference to the Agreement as amended or integrated hereby.

3.2           Except as expressly amended or integrated by this Amendment including the Exhibit A attached hereto, the provisions of the Agreement shall remain in full force and effect.

 

1

4. Counterparts.

This Amendment may be executed in any number of counterparts, each such counterpart shall be deemed to be an original instrument, and all such counterparts together shall constitute but one agreement. Any such counterpart may contain one or more signature pages. This Amendment may be executed by facsimile signature pages.

IN WITNESS WHEREOF, the undersigned have caused this Amendment to be executed and delivered on the date first written above.

Sandoz GmbH		Amylin Pharmaceuticals, LLC
Date:	23 June 14	Date:	30 June 2014
By:	[***]	By:	[***]
Name:	[***]	Name:	[***]
Title:	[***]	Title:	[***]

Sandoz GmbH
Date:	23 June 2014
By:	[***]
Name:	[***]
Title:	[***]

2

Exhibit A

3

EX-10.11.6

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.11.6

AMENDMENT NO. 5 TO

CONTRACT MANUFACTURING AGREEMENT (Metreleptin SLD)

THIS AMENDMENT NO. 5 (the “Amendment”) is made as of October 13 2014 (the “Amendment Effective Date”) by and between Amylin Pharmaceuticals, Inc. with offices at 9360 Towne Centre Drive, San Diego, California 92121 (“Amylin Pharmaceuticals, LLC”) and Sandoz GmbH, with a principal place of business at Biochemiestr. 10, A6250 Kundl, Austria (“Sandoz”).

WITNESSETH:

WHEREAS, Sandoz and Amylin Pharmaceuticals, LLC (“Amylin”, formerly known as - and successor in interest to - Amylin Pharmaceuticals, Inc.) entered into a certain Contract Manufacturing Agreement in relation to Metreleptin SLD dated as of 30 September 2010, as amended (the “Agreement”); and

[WHEREAS, Amylin Pharmaceuticals, LLC is a party to the Agreement as successor in interest to Amylin;]

WHEREAS, Amylin Pharmaceuticals, LLC. and Sandoz desire to amend the Agreement to reflect mutually agreed upon terms in accordance with the provisions of this Amendment.

NOW, THEREFORE, in consideration of thess mutual covenants and agreements set forth herein and for other good and valuable consideration, the receipt and adequacy of which are hereby acknowledged, Amylin Pharmaceuticals, LLC (“Amylin”) and Sandoz agree as follows:

1. Definitions.

Capitalized terms used herein, but not otherwise defined, shall have the meanings ascribed to them in the Agreement.

2. Amendments.

The Parties hereby agree as follows:

(i) The RPP4 Facility price per week shall be [***], applicable from (and including) the [***] until the end of the Agreement term. For the avoidance of doubt, this amount is subject to the provisions and price adjustment of Section 6 (“General”) of Schedule 4 (“Capacity and Pricing Estimates, Fees”) of the Agreement starting on [***]. The amounts contained in the Agreement (including its Schedules) shall be recalculated accordingly.

(ii) As of [***], Amylin hereby agrees to order a minimum amount of [***] per calendar year (“Minimum Order”). For the avoidance of doubt, [***] is the first year for which the Minimum Order shall apply. Unless otherwise agreed, the (annual) Product Manufacture shall be divided into [***] consisting of an equal number plus/minus [***]. No single campaign shall consist of less than [***] cGMP Batches. The definition of “Minimum Order” shall supersede the definitions of

 

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“Ordered Amount Fee”, “Minimum Fee” and “Minimum Fee Period”. The Parties agree that Amylin shall order and pay for - and Sandoz shall perform - [***] every time that: (i) a change is implemented into the Process, and (ii) the previous Product campaign ended more than [***] prior to the scheduled campaign start.

(iii) In the event that, with respect to any calendar year from [***] onwards, Amylin has not ordered the Minimum Order, Company shall pay to Sandoz - by [***] of the relevant year of Manufacture - the difference between the Minimum Order and the actually used capacity for Product Manufacture pursuant to a Firm Order as well as Raw Materials which were purchased or under a binding order placed by Sandoz. Any Raw Materials purchased/ordered by Sandoz and paid for by Amylin pursuant to this paragraph 2(iii) will, at Amylin’s option and expense, be shipped by Sandoz to Amylin or Amylin’s nominated representative. Instead of having such Raw Materials shipped back to Amylin, Amylin may also offer to Sandoz to purchase such excess Raw Materials and Sandoz will consider in good faith whether it can use such Raw Materials in other products. Sandoz shall be under no obligation to purchase such Raw Materials from Amylin. This paragraph 2(iii) supersedes Clause 9(3) of the Agreement.

(iv) Unless otherwise agreed, Sandoz shall be under no obligation to manufacture any Product exceeding [***] of Facility (RPP4) capacity [***], including product change over and Engineering Batches (“Maximum Order”).

(v) As of the Amendment Effective Date, Clause 10.4 of the Agreement shall be replaced by the following:

“Before [***] of each calendar year, Amylin will provide to Sandoz a rolling forecast of its entire demands for Product of Amylin, its Affiliates and licensees for the [***] following the year in which the forecast is provided (each such forecast a “Rolling Commercial Forecast”), provided that the amount of Product forecasted for any individual calendar year in the Rolling Commercial Forecast (i) shall not be less than the Minimum Order and (ii) unless otherwise agreed to by Amylin and Sandoz, shall not exceed the Maximum Order. Each Rolling Commercial Forecast shall consist of (i) the Firm Order for Product for calendar year 1 of such [***] forecast, and (ii) non-binding forecasted demands for Product for the subsequent [***].”

(vi) As of the Amendment Effective Date, Clause 10(6) of the Agreement shall be entirely replaced by the following:

“With respect to each Firm Order (a) Amylin will be obligated to purchase [***] of the quantity of Product set forth in such Firm Order and (b) Sandoz will be obligated to Manufacture [***] of the quantity of Product set forth in the Firm Order, except as justified by Batch volumes based on the number of kg of Product ordinarily produced per Batch. In the event Amylin requests Product from Sandoz in excess of (i) the Firm Order, (ii) the amounts of Product set forth in the applicable Rolling Commercial Forecast, or (iii) an amount of Product for which the Manufacture requires more than the Maximum Order (in each case, an “Excess Order”), Sandoz will use its commercially reasonable efforts

2

to accommodate such Excess Orders consistent with this Agreement in an attempt to meet Amylin’s requirements for the Product. Sandoz shall inform Amylin whether it will fill Excess Orders within [***] of receiving the applicable Firm Order so as to permit Amylin to manage its inventory of Product and respond to market demand.”

(vii) Any activity rendered under the Agreement and/or this Amendment shall be invoiced to, and paid by, Amylin – directly or through its affiliate [AstraZeneca LP, Accounts Payable [***]]. Nothing in the previous sentence shall relieve Amylin from its obligations under the Agreement or this Amendment, including but not limited to ensuring payment is made for activities rendered.

3. Reference to and Effect on the Agreement.

3.1          On and after the Amendment Effective Date, each reference to “this Agreement,” “hereunder,” “hereof,” “herein,” or words of like import shall mean and be a reference to the Agreement as amended hereby. No reference to this Amendment need be made in any instrument or document at any time referring to the Agreement, a reference to the Agreement in any of such instrument or document to be deemed to be a reference to the Agreement as amended or integrated hereby.

3.2          Except as amended or integrated by this Amendment the provisions of the Agreement shall remain in full force and effect.

4. Counterparts.

This Amendment may be executed in any number of counterparts, each such counterpart shall be deemed to be an original instrument, and all such counterparts together shall constitute but one agreement. Any such counterpart may contain one or more signature pages. This Amendment may be executed by facsimile signature pages.

IN WITNESS WHEREOF, the undersigned have caused this Amendment to be executed and delivered on the date first written above.

Sandoz GmbH		Amylin Pharmaceuticals, LLC
Date:	10/21/14	Date:	10/20/14
By:	[***]	By:	[***]
Name:	[***]	Name:	[***]
Title:	[***]	Title:	[***]

3

Sandoz GmbH		Amylin Pharmaceuticals, LLC
Date:	27 Oct 2014	Date:	10/14/14
By:	[***]	By:	[***]
Name:	[***]	Name:	[***]
Title:	[***]	Title:	[***]

4

EX-10.11.7

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.11.7

6^th Amendment 

to the Manufacturing Agreement

entered into as of September 30^th , 2010

This Amendment 6 (“Amendment 6”) is made effective as of June 1^st, 2017 (“Amendment 6 Effective Date”) by and between SANDOZ and Aegerion Pharmaceuticals, Inc., located at One Main Street, Cambridge, MA 02142, USA (“Aegerion”), and Sandoz GmbH, a company incorporated in Austria, with its office at Biochemiestrasse 10, A-6250 Kundl, Austria (“Sandoz”).

WITNESSETH:

WHEREAS, Aegerion and Sandoz are parties to a certain Contract Manufacturing Agreement in relation to Metreleptin SLD, dated as of September 30^th 2010, as amended (the “Agreement”);

WHEREAS, due to Aegerion’s increased demand and Sandoz’ limited storage capacity for Metreleptin, the parties wish to define the conditions upon which Sandoz may ship Metreleptin batches prior to Sandoz Batch Release for Aegerion to Fischer Bioservices, 1001 Aldridge Rd, Vacaville 98688 (USA) (“Aegerion’s Facilities”) or to other facilities designated by Aegerion;

WHEREAS, the number of Metreleptin Batches to be shipped prior to Batch Release will depend upon the number of Metreleptin batches Manufactured and the available storage capacity at Sandoz and will depend on authorization by Aegerion Quality Assurance;

WHEREAS, Aegerion and Sandoz desire to amend the Agreement to reflect mutually agreed upon revised terms in accordance with the provisions of this Amendment 6.

NOW, THEREFORE, in consideration of premises the mutual covenants and agreements set forth herein and for other good and valuable consideration, the receipt and adequacy of which are hereby acknowledged, Aegerion and Sandoz agree as follows:

1. Definitions.

Capitalized terms used herein, but not otherwise defined, shall have the meanings ascribed to them in the Agreement.

2. Amendments.

2.1. The parties hereby agree that Metreleptin batches may be shipped prior to Batch Release when authorized by Aegerion Quality Assurance;

2.2. The parties hereby agree that it may be necessary to ship Metreleptin Batches which have finished manufacturing prior to Batch Release, if it has been agreed mutually between both parties.

2.3. Shipments prior to Batch Release of Metreleptin Batches by Sandoz are subject to conditions defined in the Quality Agreement, as amended.

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2.4. Sandoz is and shall be entitled to issue the relevant commercial invoice(s) upon any Metreleptin shipment. The payment for each of the Metreleptin Batches delivered prior to Batch Release is due in accordance with the current payment terms of [***] after the relevant Sandoz Release has occurred.

2.5. Any delivery of a Metreleptin Batch prior to Batch Release shall be subject to the provisions of Section 12 (1) phrase 3 of the Agreement.

2.6. Each shipment prior to Batch Release shall meet the preconditions per the Quality Agreement, it shall be labeled, transported and stored clearly labeled and designated as “Quarantine” on relevant transport documentation, and where applicable, storage documentation, and be accompanied by the following documents: (i) commercial invoice, (ii) Aegerion’s request for transfer prior to release, and (iii) memorandum on deviations and at this time available release results (v) Packing List (vi) USDA statement (vii) End Use Letter (viii) Material Safety Data Sheet. Aegerion may request a copy of the shipping announcement for information by Aegerion QA, Supply Chain, Customs, and B80 at the time the shipment is sent.

2.7. Any Metreleptin quality disputes and/or non-conformance to the Specifications shall be subject to the provisions of Section 27 of the Agreement. For the avoidance of doubt, if the Metreleptin Batch(s) (delivered prior to Batch Release) do not pass the Release by Sandoz (after the material has arrived at Aegerion) due to reasons attributable to Sandoz in accordance with the Agreement, then Sandoz will be responsible for reimbursing Aegerion with the shipping, customs, other transportation and destruction charges; in addition, Sandoz shall cancel the invoice (or portion thereof), or issue credit if Aegerion has already paid the invoice, applicable to the nonconforming Batch(s). For the sake of clarity only, and consistent with the terms of delivery established in Section 12.1 of the Agreement, any risk of loss and other damage to any Metreleptin Batches shipped prior to Batch Release will pass to Aegerion once Sandoz has loaded said Batches onto Aegerion’s designated carrier.

2.8. The recitals constitute an integral and substantial part of this Amendment.

3. Reference to and Effect on the Agreement.

3.1          On and after the Amendment 6 Effective Date, each reference to “this Agreement,” “hereunder,” “hereof,” “herein,” or words of like import shall mean and be a reference to the Agreement as amended hereby. No reference to this Amendment need be made in any instrument or document at any time referring to the Agreement, a reference to the Agreement in any of such instrument or document to be deemed to be a reference to the Agreement as amended hereby.

3.2          Except as expressly amended by this Amendment 6, the provisions of the Agreement shall remain in full force and effect.

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4. Counterparts.

This Amendment 6 may be executed in any number of counterparts, each such counterpart shall be deemed to be an original instrument, and all such counterparts together shall constitute but one agreement. Any such counterpart may contain one or more signature pages. This Amendment 6 may be executed by facsimile signature pages.

[The remainder of this page has been intentionally left blank, signature page follows]

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IN WITNESS WHEREOF, the undersigned have caused this Amendment 6 to be executed and delivered on the date first written above.

Sandoz GmbH		Sandoz GmbH
Date:	01 Mar 2018	Date:	27 July 2017
By:	[***]	By:	[***]
Name:	[***]	Name:	[***]
Title:	[***]	Title:	[***]

Aegerion Pharmaceuticals, Inc.
Date:	24 July2017
By:	/s/ Joe Shulman
Name:	Joe Shulman
Title:	SVP, Global Technical Operations

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EX-10.11.8

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.11.8

7^th Amendment
to the Manufacturing Agreement
entered into as of September 30^th , 2010

This Amendment 7 (“Amendment 7”) is made effective as of August 1, 2017 (“Amendment 7 Effective Date”) by and between Aegerion Pharmaceuticals, Inc., located at One Main Street, Cambridge, MA 02142, USA (“Aegerion”), and Sandoz GmbH, a company incorporated in Austria, with its office at Biochemiestrasse 10, A-6250 Kundl, Austria (“Sandoz”).

WITNESSETH:

WHEREAS, Aegerion and Sandoz are parties to a certain Contract Manufacturing Agreement in relation to Metreleptin SLD, dated as of September 30^th 2010, as amended (the “Agreement”);

WHEREAS, due to increased demand of Product needed by Aegerion for clinical trials and later commercialization Aegerion wish Sandoz to scale up the Process in Sandoz’ RPP4 Facility and Sandoz is willing to perform such activities and Manufacture certain Batches as described herein;

NOW, THEREFORE, in consideration of premises, the mutual covenants and agreements set forth herein and for other good and valuable consideration, the receipt and adequacy of which are hereby acknowledged, Aegerion and Sandoz agree as follows:

1. Definitions.

Capitalized terms used herein, but not otherwise defined, shall have the meanings ascribed to them in the Agreement.

2. Amendments.

2.1. A new DEFINITION is added to Sec. 1(1) of the Agreement:

“RPP4+ Process” means the Process (as defined in Sec. 1 (1) of the Agreement) as adapted for the purpose of increasing the throughput in the RPP4 Facility.

2.2. Sandoz shall Manufacture Product based on the RPP4+ Process in RPP4 in quantities as defined in Schedule 1.

2.3. Sandoz shall use commercially reasonable efforts to implement in a timely manner the modifications to the RPP4 Facility as set forth in Schedule 2 required for the scale-up of the Process to become the RPP4+ Process (collectively the “4+ Facility Modifications”), including procuring, engineering, installing, testing and validating the applicable equipment and systems, in accordance with the budget set forth in Schedule 1. If the implementation and validation of the RPP4+ Process fails for reasons not attributable to Sandoz, remaining capacity, if any, shall be used for Manufacture of commercial quantities according to the currently established Process. Events of single out-of-specification results during the RPP4+ Process shall be

1

handled according to the current CMA and QAA, as amended. Costs payable to Sandoz under this amendment 7 shall not be reimbursed to Aegerion.

All assets for the 4+ Facility Modifications will be purchased by Sandoz on its own behalf. Sandoz shall become the sole owner of all assets. Sandoz shall be responsible for maintaining such assets and for all of the costs and expenses related thereto in order to keep them in acceptable working condition.

Aegerion shall reimburse Sandoz for all reasonable costs and expenses, including without limitation capital expenditures, incurred by Sandoz for obtaining and installing the assets, the Facility idle time required for installation and for the 4+ Facility Modifications (all of the foregoing, collectively, the “4+ Facility Modification Costs”) as follows: Sandoz shall be entitled to invoice, and Aegerion shall pay to Sandoz, such 4+ Facility Modification Costs estimated as set forth in Schedule 1, up to a maximum aggregate amount of [***].

2.4. Aegerion has paid [***] in [***] and [***] in [***] as a pre-payment for the campaign [***] and the 4+ Facility Modification Costs (purchase order [***] dated [***]). The remaining amount of the 4+ Facility Modification Costs shall be invoiced upon the start of the [***] campaign. For all future orders above [***], Aegerion shall pay in advance [***] of the difference between [***] and the actual value of the sum of all purchase order(s) of the same year. Sandoz shall issue an invoice for the Prepayment upon confirmation of the order by Sandoz and Aegerion shall pay not later than [***] after receipt of the invoice.

2.5. Upon delivery of the relevant Batch and the related certificates for the Batch to Aegerion, Sandoz shall invoice the full costs for each such Batch crediting [***] of the Prepayment (corresponding to the nine Batches to be Manufactured) to each such invoice or, for future campaigns, the pro rata amount of the prepayment based on the number of batches produced. The amounts to be prepaid, as described in the paragraph, is referred to individually and collectively as “Prepayment”.

2.6. The parties hereby agree that Metreleptin Batches may be shipped prior to Batch Release when authorized by Aegerion and Sandoz Quality Assurance according to Amendment 6.

2.7. If the campaign [***] (refer to purchase order [***], dated [***]) needs to be split into [***] due to production commitments for an alternate product, which cannot be rescheduled, cost for an additional change-over will be applicable for campaign [***] as outlined in Schedule 3.

2.8. The recitals constitute an integral and substantial part of this Amendment

3. Reference to and Effect on the Agreement.

3.1           On and after the Amendment 7 Effective Date, each reference to “this Agreement,” “hereunder,” “hereof,” “herein,” or words of like import shall mean and be a reference to the Agreement as amended hereby. No reference to this Amendment need be made in any instrument or document at any time referring to

2

the Agreement, a reference to the Agreement in any of such instrument or document to be deemed to be a reference to the Agreement as amended hereby.

3.2           Except as expressly amended by this Amendment 7, the provisions of the Agreement shall remain in full force and effect.

4. Counterparts.

This Amendment 7 may be executed in any number of counterparts, each such counterpart shall be deemed to be an original instrument, and all such counterparts together shall constitute but one agreement. Any such counterpart may contain one or more signature pages. This Amendment 7 may be executed by facsimile signature pages.

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IN WITNESS WHEREOF, the undersigned have caused this Amendment 7 to be executed and delivered on the date first written above.

Sandoz GmbH		Sandoz GmbH
Date:	19/02/2018	Date:	Feb 07, 2018
By:	[***]	By:	[***]
Name:	[***]	Name:	[***]
Title:	[***]	Title:	[***]

Aegerion Pharmaceuticals, Inc.
Date:	28 Feb 2018
By:	/s/ Joseph Shulman
Name:	Joseph Shulman
Title:	Senior Vice President, Global Technical Operations

4

EX-10.11.9

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.11.9

8^th Amendment

to the Manufacturing Agreement

entered into as of September 30^th, 2010

This Amendment 8 (“Amendment 8”) is made effective as of April 30, 2019 (“Amendment 8 Effective Date”) by and between Aegerion Pharmaceuticals, Inc., located at One Main Street, Cambridge, MA 02142, USA (“Aegerion”j, and Sandoz GmbH, a company incorporated in Austria, with its office at Biochemiestrasse 10, A-6250 Kundl, Austria (“Sandoz”).

WITNESSETH:

WHEREAS, Aegerion and Sandoz are parties to a certain Contract Manufacturing Agreement in relation to Metreleptin SLD, dated as of September 30^th 2010, as amended (the “Agreement”);

WHEREAS, Aegerion and Sandoz wish to amend the existing CMA with regard to its terms for pricing, contract expiry and contract termination.

NOW, THEREFORE, therefore Aegerion and Sandoz agree as follows:

1.             Definitions.

Capitalized terms used herein, but not otherwise defined, shall have the meanings ascribed to them in the Agreement.

2.             Amendments.

The Parties hereby agree as follows:

2.1. Price Adaptation:

The pricing model (previously laid down in Section 9 (2) of the Agreement and/or Section 2 of Amendment no. 5 to the Agreement) shall be changed as follows

The RPP4 Facility price per week shall be [***], applicable from (and including) the [***] large-scale (RPP4+) Manufacturing campaign of calendar year [***]. The RPP4 Facility price per week shall rise to [***] Euros for all large-scale (RPP4+) Manufacturing in [***] and to [***] in [***] and later years (Schedule 1). For the avoidance of doubt, the amounts in [***] and [***] (and later) are subject to the provisions and price adjustment of Section 9 (3) of the Agreement starting on 1 [***]. The amounts contained in the Agreement (including its Schedule) shall be recalculated accordingly.

The RPP4 Facility price per week shall be [***] Euros, applicable from (and including) the first small-scale (commercial) Manufacturing campaign after the effective date (Schedule 1).

Upon production of [***] large-scale batches after [***], the parties will enter into discussions to change from a weekly price to a gram-based price.

1

2.2. Duration:

Section 14 of the Agreement (Duration) shall be replaced by the following wording:

Subject to the provisions for earlier termination contained herein, this Agreement shall come into force on the Effective Date hereof and shall continue in force until [***] (expiry date). The parties will by the end of [***] enter into discussions as to whether or not the term of the Agreement shall be further extended.

2.3. Termination – Notice Period:

The parties wish to clarify that Aegerion's right under clause 25(2) to terminate the Agreement for scientific, regulatory, safety or economic reasons is subject to an adequate notice period of 24 months. Sandoz shall receive the same right for termination. Therefore, clause 25(2) of the Agreement shall be amended as follows:

Moreover, each of the parties may terminate this Agreement for any scientific, regulatory, safety or economic reason to the end of each calendar year by giving 24 months prior written notice.

3.             Reference to and Effect on the Agreement.

3.1       On and after the Amendment Effective Date, each reference to “this Agreement,” “hereunder,” “hereof,” “herein,” or words of like import shall mean and be a reference to the Agreement as amended hereby. No reference to this Amendment need be made in any instrument or document at any time referring to the Agreement, a reference to the Agreement in any of such instrument or document to be deemed to be a reference to the Agreement as amended or integrated hereby.

3.2       Except as amended or integrated by this Amendment the provisions of the Agreement shall remain in full force and effect.

4.             Counterparts.

This Amendment may be executed in two counterparts, each such counterpart shall be deemed to be an original instrument, and all such counterparts together shall constitute but one agreement. Any such counterpart may contain one or more signature pages. This Amendment may be executed by facsimile signature pages.

IN WITNESS WHEREOF, the undersigned have caused this Amendment 8 to be executed and delivered on the date first written above.

Sandoz GmbH		Sandoz GmbH
Date:	23.04.2019	Date:	29.04.2019
By:	[***]	By:	[***]
Name:	[***]	Name:	[***]
Title:	[***]	Title:	[***]

2

Aegerion Pharmaceuticais, Inc.
Date:	11 April 2019
By:	/s/ Joseph Shulman
Name:	Joseph Shulman
Title:	Senior Vice President, Global Technical Operations

3

Schedule 1 Pricing for Large-scale (RPP4+) and Small-scale Metreleptin Production

4

EX-10.11.10

 Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.11.10 

Confidential

9^th Amendment

to the Contract Manufacturing Agreement

entered into as of September 30^th, 2010

This Amendment 9 (“Amendment 9”) is made effective as of February 11, 2020 (“Amendment 9 Effective Date”) by and between Aegerion Pharmaceuticals, Inc., located at One Main Street, Cambridge, MA 02142, USA (“Aegerion”), and Sandoz GmbH, a company incorporated in Austria, with its office at Biochemiestrasse 10, A-6250 Kundl, Austria (“Sandoz”).

WITNESSETH:

WHEREAS, Aegerion and Sandoz are Parties to a certain Contract Manufacturing Agreement in relation to Metreleptin SLD, dated as of September 30^th 2010, as amended from time to time (the “Agreement”);

WHEREAS, As per Aegerion’s binding order issued on June 28, 2019 and Sandoz’ confirmation dated July 23, 2019, Sandoz had prepared for a DSP campaign including 11-12 large-scale batches, scheduled to start end of February 2020. However, Aegerion requested to revise its binding Firm Order dated June 28, 2019 and modify the Manufacturing Campaign of 2020 to include both large scale and small scale batches for the Product. Aegerion and Sandoz now have agreed to amend the existing CMA whereby Sandoz in good faith has agreed to accommodate the request, this being subject to the mutual agreement on the terms of this Amendment 9 by the Parties.

WHEREAS, Parties agree that this Amendment 9 is limited in its application to the Modified Campaign 2020 (defined below) and shall not extend to the following years’ Manufacturing Campaigns without further mutual written consent of the Parties.

NOW, THEREFORE, Aegerion and Sandoz agree as follows:

1. Definitions.

Capitalized terms used herein, but not otherwise defined, shall have the meanings ascribed to them in the Agreement.

“Modified Campaign 2020” shall have the meaning as provided in Schedule A sub-clause 1 as attached to this Amendment 9.

2. Amendments.

The Parties hereby agree as follows:

2.1.          Price Adaptation.

The pricing model (previously laid down in Section 9 (2) of the Agreement and/or Section 2 of Amendment no. 5 and/or Sec 2.1 of the Amendment no. 8 to the Agreement) to continue to apply unless otherwise agreed by the Parties in writing.

2.2.   Modified Campaign 2020. Pursuant to this Amendment 9, Parties agree to a modification to the Product’s Manufacturing Campaign for 2020 by Sandoz at Aegerion’s express request:

1

Confidential

(a)  Subject to this Amendment 9 and its Schedules, following Aegerion’s request to modify its binding Firm Order for the Product dated 28^th June, 2019, Sandoz in good faith, has agreed to modify the originally planned Manufacturing Campaign of 2020, subject to Aegerion’s acceptance of all risks and associated costs as provided in this Amendment 9.

(b)  As noted in Schedule A, Aegerion expressly consents to the Modification in the Process for the purpose of this Modified Campaign 2020.

(c)  Schedule A of this Amendment 9 contains the estimated modifications, risks and estimated costs and Sandoz agrees to keep Aegerion informed of any updates to the same as and when it becomes aware. Notwithstanding anything to the contrary in the Agreement elsewhere, Aegerion agrees to accept all risks including but not limited to regulatory, technical, quality that may arise due to modifications carried out under the Modified Campaign 2020 and Aegerion shall not withhold any payments, reimbursements to Sandoz if any such risks in the Modified Campaign 2020 lead to an adverse impact. Notwithstanding anything to the contrary in the Agreement, Aegerion agrees to hold Sandoz harmless and keep Sandoz indemnified for any regulatory, IP or other risks, third party claims, direct losses due to the modifications made to the Modified Campaign 2020.

(d)  Parties expressly agree that Aegerion is solely responsible at its own cost and risk for any regulatory submissions in relation to Product Manufactured under this Modified Campaign 2020 and Sandoz shall not be held responsible if any of these batches are not suitable for regulatory submission in the Territory. In accordance with the existing terms of the Agreement, Sandoz will continue to provide support to Aegerion for regulatory submissions, at Aegerion’s cost and risk.

(e) Termination

Aegerion accepts that it shall not have the right to terminate the Agreement under clause 25 of the Agreement (as amended) due to the modifications carried on by Sandoz hereunder the Modified Campaign 2020 as per this Amendment 9.

3. Reference to and Effect on the Agreement.

3.1             On and after the Amendment 9 Effective Date, each reference to “this Agreement,” “hereunder,” “hereof,” “herein,” or words of like import shall mean and be a reference to the Agreement as amended hereby. No reference to this Amendment need be made in any instrument or document at any time referring to the Agreement, a reference to the Agreement in any of such instrument or document to be deemed to be a reference to the Agreement as amended or integrated hereby.

3.2             Except and to the extent as amended or integrated by this Amendment 9, the provisions of the Agreement shall remain in full force and effect.

4. Counterparts.

This Amendment 9 may be executed in two counterparts, each such counterpart shall be deemed to be an original instrument, and all such counterparts together shall constitute but one agreement. Any such counterpart may contain one or more signature pages.

[SIGNATURE PAGE FOLLOWS]

2

Confidential

IN WITNESS WHEREOF, the undersigned have caused this Amendment 9 to be executed and delivered on the date first written above.

Sandoz GmbH		Sandoz GmbH
Date:	11.02.2020	Date:	11.02.2020
By:	[***]	By:	[***]
Name:	[***]	Name:	[***]
Title:	[***]	Title:	[***]
Aegerion Pharmaceuticals, Inc.
Date:
By:	/s/ Rory Nealon
Name:	Rory Nealon
Title:	Secretary

3

Confidential

Schedule A. The Modified Campaign 2020 and Estimated Modifications, Costs and Risk

1. Modified Campaign 2020. Further to Aegerion’s letter dated 22^nd January 2020, Aegerion requested Sandoz to evaluate a switch to small-scale production at short notice. Such change at short notice is associated with additional costs and risks as noted hereunder to the extent it can be commercially reasonably estimated at current stage.

Subject to Aegerion’s commitments in this Amendment 9 Section 2 above, Sandoz agrees that modifications shall be made to the regular and established Process for Manufacture of the Product with Aegerion’s consent and Sandoz’ considerable additional effort, in order to Manufacture certain batches in 2020 on a small-scale instead of as originally planned on large-scale. Aegerion expressly consents to the following structure for the Modified Campaign 2020:

●      Standard Change-over from alternate product to Metreleptin

●      Manufacture of [***] large-scale batches 

●      Additional Change-over from large-scale to small-scale

●      Manufacture of [***] small-scale batches

2. The typical cycle-time of a small-scale or large-scale batch is [***] days. The Modified Campaign 2020 is reasonably expected to utilize the contractual minimum order quantity of [***] weeks. 

Actual Facility occupational times and material cost will be invoiced to Aegerion in accordance with the existing mechanism provided in the Agreement.

3. Materials. 

●     Adaptation of Lynx Filter for filling vessel: Parties acknowledge that the appropriate Lynx filter ([***]µm Millipak 200 ([***]cm²) + Lynx adaptor) for the Metreleptin small scale will need to be procured and is not readily available.

●     Aegerion consents and requests Sandoz to implement the Lynx filter from Metreleptin large scale ([***]µm Durapore ([***]cm²) + Lynx adaptor) for the entire Modified Campaign 2020 including the small-scale batches at Aegerion’s risk and responsibility for the outcome.

●     Parties acknowledge and accept that from a technical standpoint, usage of the larger filter is feasible for both large scale and small scale production, however a higher than earlier yield loss can be expected due to a larger filter area. The replacement of filter will be started in the corresponding Process Change of the Modified Manufacturing Campaign 2020 in accordance with relevant Quality provisions. 

●     Since this is an Equipment change of the actual Validated Process for the Product, Aegerion agrees to perform the evaluation of any potential impact on the Regulatory submissions for the Product in the Territory, and notwithstanding any other contrary provision of the Agreement, Aegerion confirms and accepts any additional cost, risk with the associated Regulatory submission is retained by Aegerion and not Sandoz.

4. Automation 

●     The relevant computer programs are primarily programmed for Metreleptin large-scale, and a change has to be initiated for small-scale batches of the Modified Campaign 2020. Despite the short time-frame available. Sandoz agrees to make

Confidential 4

Confidential

commercially reasonable efforts to implement the change in a reasonable time, subject to additional costs and risks retained by Aegerion.

●     Estimated additional cost: €[***] (Any further revision to such estimate to be provided to Aegerion in due course).

5. Additional Efforts 

●    Sandoz expects significant increase in the time and effort required due to additional Manufacturing and QC documents (such as, Change request, Batch Records, Manufacturing Procedure) to be prepared for both Metreleptin large-scale and small-scale batches. Other than as noted in the Schedule B of Amendment 9 below, Sandoz shall charge to Aegerion the cost for any further additional documents as required for the Modified Campaign 2020.

●    Despite Sandoz’ diligent efforts, Aegerion acknowledges and accepts to bear risk and cost for any rejection or recall of batches manufactured under Modified Campaign due to an increase in potential deviations, human error notwithstanding anything contrary in the Article 27 of the Agreement; except if the same is due to Sandoz’ gross negligence.

6. Resins

●     In the event, CM-sepharose and Toyopearl backup resins (available with Sandoz) are used-up during large-scale batches’ production, there will be no resins remaining for initial packing for the small-scale columns or later back-up packing. Aegerion agrees to pay for additional resins to be purchased by Sandoz.

●     Estimated Quantity: [***] Liters CM-Sepharose FF (PBP) + [***] Liters Toyopearl BUTYL-650M (PBP). Estimated Cost: The Schedule B of this Amendment 9 provides for estimated resin cost expected. The cost of resins shall be invoiced to Aegerion in accordance with the existing mechanism provided in the Agreement.

5

Confidential

Schedule B. The Revised Estimated Quote for the Modified Campaign 2020 is provided below. 

6

EX-10.12.1

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.12.1

MASTER SERVICES AGREEMENT

- BMS COMMERCIAL-

For Services related to Metreleptin

This Master Services Agreement (this “Agreement”) is entered into on December 6, 2013 (the “Effective Date”) by and between BRISTOL-MYERS SQUIBB COMPANY, having an address at Route 206 and Province Line Road, Princeton, New Jersey 08543 (“BMS”) and Accredo Health Group, Inc. having an address at 6272 Lee Vista Boulevard, Orlando, FL 32822 and its Affiliates (“Service Provider”).

The parties agree as follows:

1 Services

1.1 Service Provider agrees to perform services as described in one or more statements of work (“SOW”) that reference this Agreement (the “Services”). Services shall not include standard services as described in Section 9.1(c).

1.2 BMS is entering into this Agreement for itself and for the benefit of its Affiliates. Services may be provided by an Affiliate of Service Provider provided Service Provider and the Affiliate are jointly and severally liable for performance hereunder. In the event of a conflict between this Agreement and a SOW, the terms of this Agreement shall prevail.

1.3 Service Provider represents and warrants that Service Provider shall not perform any Services under this Agreement or any SOW related to or involving:

1.3 (a) the advertising or promotion or communication to any third party about any of BMS’ product (including any Collaboration Products, as defined in Section 11.1 of this Agreement) (collectively, “Products”) unless such communication is in strict compliance with the terms of this Agreement.

1.3 (b) public relations related activities, including but not limited to the communication of information to third parties and/or the public about BMS.

1.3 (c) lobbying related activities.

1.3 (d) primary or secondary market research or competitive intelligence.

1.3 (e) health and/or economic outcomes research.

1.3 (f) the licensing of: data, information, materials or any other intellectual property that would be subject to the rights of a licensor – including where Service Provider is the licensor unless such Service is in strict compliance with the Additional Terms and Conditions For Data License And Analytics And Related Services, attached and incorporated herein by reference the provision of information technology related services, such as software maintenance, installation, configuration, upgrades or development; and/or the licensing of: software, software-as-a-service that would be subject to the rights of a licensor – including where Service Provider is the licensor.

1.3 (g) the provision of recruitment or employment related services.

1.3 (h) the provision of any leased worker to BMS including but not limited to a contracted sales organization,

1.3 (i) the provision of human resource/employee related training services to BMS personnel or third parties; and/or the licensing of: training modules that would be subject to the rights of a licensor – including where Service Provider is the licensor.

1.3 (j) the delivery, storage, shipment, sale or handling of any Products or any packaging, information, materials or equipment related to or used in conjunction with any Products.

1.3 (k) the reimbursement support, co-pay assistance, coupon, discount, rebate, voucher or any other pricing related activity of any Products – unless such Service is in strict compliance with the Additional Terms and Conditions For Benefits Investigation and Reimbursement Support Services, attached and incorporated herein by reference.

1.3 (I) patient assistance programs or related activities.

1.3 (m) medical and/or scientific content creation or advice related thereto.

1.3 (n) the provision of medical, nursing, or other healthcare services to patients intended to diagnose, treat or prevent any disease or condition. Notwithstanding the foregoing, nurses and pharmacists meeting the requirements under Section 9.1(f) of this Agreement may deliver specified BMS Product compliance and education-related information, in strict compliance with all of the terms and conditions of this Agreement, each applicable SOW, and all instructions of BMS.

1.3 (o) management, administrative and/or editorial or technical assistance with a scientific publication or publication planning.

1.3 (p) compliance, persistency, adherence or related services concerning a BMS Product – unless such Service is in strict compliance with the Additional Terms and Conditions For Compliance, Persistency and Adherence Program Services, attached and incorporated herein by reference.

1.3 (q) transporting, distributing or dispensing a BMS Product.

1.4 Service Provider agrees:

1.4 (a) that it shall not perform any of the services prohibited under Section 1.3 of this Agreement, unless they are described in an Additional Terms and Conditions attachment to this Agreement. Any such Additional Terms and Conditions Attachment must be either incorporated into this Agreement at execution, or incorporated via written amendment to this Agreement executed by both parties; and

1.4 (b) that performance of an SOW by Service Provider of any services prohibited by Section 1.3 of this Agreement (without an Additional Terms and Conditions attachment) is a material breach of this Agreement, notwithstanding execution of, performance by, or other consent to the SOW by BMS; and

1.4 (c) that BMS has no obligation to pay for any Services rendered under such an SOW entered (without an effective Additional Terms and Conditions attachment).

1.5 This Agreement does not create any exclusive relationship between the parties.

1.6 Services shall be deemed acceptably performed unless BMS promptly notifies Service Provider of an alleged violation of Service Provider’s obligations under this Agreement or under the specific performance standards or acceptance criteria defined in a SOW.

1.7 BMS issues service orders for accrual and internal controls purposes, which allows Service Provider to submit invoices to BMS. These service orders:

1.7 (a) shall not exceed the value of any SOW; and

1.7 (b) Service Provider shall not invoice BMS for Services in excess of the service order.

Any terms and conditions contained in or with the purchase order are null and void.

Compensation

2.1 In consideration of the Services, BMS will pay the fees to Service Provider as described in the applicable SOW (the “Compensation”).

2.2 All income related taxes on Compensation shall be the responsibility of Service Provider and not of BMS.

2.3 The Compensation intends to comply with the Federal Anti-Kickback Statute and its implementing regulations; and is fair market value, negotiated at arm’s length.

2.4 The Compensation has not been determined in a manner that takes into account the volume or value of any referrals of business otherwise generated or that may be generated between the parties for which payment may be made in whole or in part under any federal health care program.

2.5 Compensation is not overtly or covertly, directly or indirectly, a rebate or discount on the purchase of BMS Product(s).

2.6 Service Provider acknowledges that BMS is subject to applicable law related to the collection and reporting of any payments or transfers of value to certain healthcare providers and teaching hospitals, which includes, without limitation, the Affordable Care Act of 2010 and its implementing regulations (collectively, ‘Transparency Laws”). To comply with Transparency Laws, BMS may disclose for any lawful purpose, within its sole discretion, the terms of this Agreement, including without limitation, the compensation (including fees and expenses) payable or paid hereunder, or any other transfers of value made pursuant to this Agreement if required by applicable Transparency Laws. BMS reserves the right to identify in its disclosures any Service Provider personnel that is a health care provider (“HCP”) as BMS reasonably believes is required to satisfy its obligations under Transparency Laws. This identifying information will include, without limitation, names, NPI, licensure number(s), specialty and addresses. Service Provider acknowledges and agrees on behalf of itself and such Service Provider Personnel to these disclosures. Service Provider shall, and shall ensure that its Personnel, reasonably cooperate with BMS in meeting its obligations under Transparency Laws.

2.7 Reimbursable Expenses

2.7 (a) BMS will reimburse Service Provider for all reasonable and necessary business and travel expenses and pass-through costs actually incurred by Service

Provider in the course of performing Services at cost without mark-up (“Reimbursable Expenses”).

2.7 (b) Service Provider will adhere to BMS’ policy and procedure regarding travel, expenses and meetings (“BMS Travel Policy”) attached hereto as Attachment 3. BMS may update the “BMS Travel Policy” prospectively by providing written notice to Service Provider.

2.7 (c) Service Provider agrees to invoice BMS for Reimbursable Expenses, which invoice will be accompanied by any supporting documentation (e.g. receipts) required by BMS Travel Policy.

2.7 (d) BMS will have no obligation to reimburse Service Provider for any Reimbursable Expenses unless and until all documentation required by this Agreement and the BMS Travel Policy is received from Service Provider.

3 Payment

3.1 Subject to the terms and conditions of this Agreement, BMS will pay Service Provider for Services actually performed within [***] from receipt of invoices submitted by Service Provider to BMS through Web Electronic Data Interchange (“EDI”) or as otherwise instructed by BMS in writing. Service Provider agrees to establish EDI through XIGN, including but not limited to paying any applicable fee, and supplying banking information in order to establish the payment method. Service Provider shall invoice BMS for any applicable fees paid to XIGN for reimbursement on a monthly basis.

3.2 In the event of any good faith invoice-related dispute, BMS agrees to pay the undisputed portion in accordance with this section.

4 Term

4.1 This Agreement shall commence on the Effective Date and expires 3 years thereafter, unless terminated earlier in accordance with Section 5 below.

5 Termination

5.1 This Agreement is made in good faith based on the assumption that early termination shall not be required. Notwithstanding the foregoing, early termination shall be permissible as follows:

(a) By BMS with [***] written notice for any reason. By Service Provider with [***] written notice for any reason.

(b) By either Party with [***] written notice detailing a material breach, if the breaching Party fails to cure the breach within [***] of the date of the notice.

(c) Immediately upon notification or at any time thereafter, either Party may terminate this Agreement in the event that:

(i) the other Party shall file any petition under any bankruptcy, reorganization, insolvency or moratorium laws, or any other law or laws for the relief of or in relation to the relief of debtors;

(ii) there shall be filed against the other Party any involuntary petition under any bankruptcy statute or a receiver or trustee shall be appointed to take possession of all or substantial part of the assets of the party which has not been dismissed or terminated within [***] of the date of such filing or appointment;

(iii) the other Party shall make a general assignment for the benefit of creditors or shall become unable or admit in writing its inability to meet its obligations as they mature;

(iv) the other party shall institute any proceedings for liquidation or the winding up of its business other than for purposes of reorganization, consolidation or merger; or

(iv) the other party’s financial condition shall become such as to endanger completion of its performance in accordance with the terms and conditions of this Agreement.

(d) Immediately upon notification or at any time thereafter, by either Party if the terms of this Agreement are determined by either Party’s legal counsel, in good faith to be inconsistent with any applicable law. Such termination shall apply only to that portion of the Agreement for which Services are inconsistent with any applicable law.

(e) Upon termination of the Agreement or SOW, BMS shall be obligated to pay for all Services performed and Reimbursable Expenses incurred or that are non-cancellable as of the termination date.

6 Independent Contractor Relationship

6.1 All Services shall be rendered by Service Provider as an independent contractor. Nothing contained in this Agreement shall be construed to place the parties or their personnel in the relationship of employer and employee, partners, principal and agent, joint venturers or as an insurer or a representative of the other party to this Agreement. Neither party shall have the power to bind or obligate the other party nor shall either party hold itself out as having such authority.

6.2 Service Provider and any person performing Services:

6.2 (a) will not be eligible to participate in or receive any benefits or have any rights as an employee of BMS; and

6.2 (b) will not be covered by any BMS liability insurance policies unless otherwise contained herein.

7 Confidentiality

7.1 Definitions.

7.1 (a) “Affiliate(s)” means a business entity which directly or indirectly Controls, is under the Control of or under common Control with the party by a common parent company.

7.1 (b) “Control” means the possession, directly or indirectly, of the power to direct the management of a business entity, whether through ownership of voting securities or otherwise.

7.1 (c) “Disclosing Party” means a party that discloses Confidential Information to the Receiving Party under this Agreement.

7.1 (d) “Receiving Party” means a party that receives Confidential Information from the Disclosing Party under this Agreement.

7.1 (e) “Confidential Information” means Disclosing Party’s, Collaboration Partner’s (as defined in this Agreement) or their respective Affiliates’ owned or controlled information including but not limited to clinical data and research results, technical and non-technical data, formulae, ideas, know-how, materials, methods, operational information, patent applications, plans, procedures, pre-clinical data and results, processes, product information, projections, specifications, standards, strategies, technical information, techniques, trade secrets, tools, or other clinical, technical or business information of Disclosing Party, whether written, graphic, oral, visual, tangible or intangible, in any form or format (including machine or computer readable code), samples or specimens furnished directly or indirectly, There is no obligation to mark or otherwise identify information, nor reduce to writing any orally disclosed or information.

7.1 (f) Confidential Information does not include information to the extent that:

7.1 (f) (i) it is now in the public domain or subsequently enters the public domain through no breach of this Agreement;

7.1 (f) (ii) the Receiving Party lawfully receives it from any third party without restriction as to use or confidentiality as shown by written or other tangible evidence; or

7.1 (f) (iii) it is independently developed by or for the Receiving Party by persons without access to the Confidential Information.

7.1 (f) (iv) it is already known by the Receiving Party at the time of its disclosure as shown by written or other tangible evidence.

7.2 Required Disclosures. If Receiving Party receives a subpoena or other validly issued administrative or judicial process, order or government process demanding Confidential Information (“Subpoena”), Receiving Party will (a) promptly inform the party or entity issuing such Subpoena of the existence of this Agreement; (b) unless prohibited by law, promptly notify the Disclosing Party of the disclosure requirement (which will include a copy of any applicable Subpoena or documentation); (c) unless prohibited by law, afford the Disclosing Party a reasonable opportunity to oppose, limit or secure confidential treatment for the required disclosure; and (d) not oppose any effort by the Disclosing Party to quash any such Subpoena. If the Disclosing Party fails to intervene after being given notice and a reasonable opportunity to do so, or if such motion is denied by a court of competent jurisdiction, the Receiving Party will disclose only that portion of the Confidential Information that the Receiving Party is legally required to disclose, in the opinion of legal counsel. In the event that any Confidential Information is ordered produced in an action or proceeding, it will not lose its confidential status

through such production, and Receiving Party will take all reasonable and necessary steps to protect its confidentiality.

7.3 Return of Confidential Information. Upon the termination or expiration of this Agreement, or at any other time upon the written request of Disclosing Party, Receiving Party will promptly return to Disclosing Party or at Disclosing Party’s request, destroy all Confidential Information in Receiving Party’s possession or control, together with all copies, summaries and analyses, regardless of the format in which the information exists or is stored. In case of destruction, Receiving Party will promptly send a written certification that destruction has been accomplished to the Disclosing Party. However, Receiving Party is entitled to retain one copy of Confidential Information for the sole purpose of determining its obligations under law or this Agreement. With regard to Confidential Information stored electronically on backup tapes, servers or other electronic media, the parties agree to make commercially reasonable efforts to destroy such Confidential Information without undue expense or business interruption; however Confidential Information so stored is subject to the obligations of confidentiality and non-use contained in this Agreement for as long as it is stored.

7.4 Permitted Disclosures. Receiving Party agrees that it will not disclose Confidential Information to any third-party without the prior written consent of the Disclosing Party. Notwithstanding the foregoing, the Receiving Party may disclose Confidential Information to an agent performing services to the Receiving Party to the extent (a) such disclosure is related to the Services; and (b) the agent’s duties justify the need to know the Confidential Information; and (c) provided that such agent is under obligations of confidentiality and non-use at least as restrictive as those within this Agreement.

7.5 BMS may disclose the Confidential Information of Service Provider to any entity with which BMS co-develops, co-promotes or otherwise collaborates on certain unapproved or approved (by regulatory authorities) pharmaceutical and/or biological products (“Collaboration Partner”); to the extent (a) such disclosure is related to the Services; and (b) the Collaboration Partner’s duties justify the need to know the Confidential Information; and (c) provided that the Collaboration Partner is under obligations of confidentiality and non-use at least as restrictive as those within this Agreement.

7.6 Receiving Party will be liable for unauthorized disclosure of Confidential Information by its or its Affiliates’ officers, employees, agents or Collaboration Partner(s) (as the case may be).

7.7 Use of Confidential Information. Receiving Party shall use Confidential Information solely for the performance of Services. Without limiting the foregoing, Confidential Information shall not be (a) disclosed directly or indirectly by the Receiving Party to any financial, securities, or industry analyst, or to the media, except as authorized in writing by the Disclosing Party, or (b) used by Receiving Party in connection with purchase or sale of any securities.

7.8 Duration of Confidentiality. The obligations of confidentiality survive expiration or termination of this Agreement for 10 year(s).

8 Intellectual Property

8.1 Each party retains sole and exclusive ownership of any intellectual property already existing as of the Effective Date and of any derivative works of such intellectual property created after the Effective Date; provided that such derivative work (a) does not arise from the Confidential Information of the other party; and/or (b) is not created specifically for BMS by Service Provider.

8.2 Except as provided otherwise in this Agreement, all data, results, ideas, discoveries, inventions, reports and works of authorship, whether or not patentable or subject to copyright, which may be made, written, conceived or reduced to practice by Service Provider or any third-party, that does not arise from BMS’ Confidential Information and/or that is not created specifically for BMS by Service Provider (“Service Provider Intellectual Property”) will be owned solely by Service Provider.

8.3 Except as provided otherwise in this Agreement, all data, results, ideas, discoveries, inventions, reports and works of authorship, whether or not patentable or subject to copyright, which may be made, written, conceived or reduced to practice by Service Provider or by Service Provider together with BMS or any third-party, arising from BMS’ Confidential Information and/or that is created specifically for BMS by Service Provider (“BMS Intellectual Property”) will be owned solely by BMS.

8.4 All copyrightable works that are BMS Intellectual Property shall be considered “works made for hire” and shall be owned solely by BMS. All copyrightable works that are Service Provider Intellectual Property shall be considered “works made for hire” and shall be owned solely by Service Provider.

8.5 Service Provider represents and warrants that Service Provider has the right to use and sublicense, in connection with this Agreement, any materials that are owned by parties other than Service Provider (Third Party Materials”) and that are incorporated into the Services or any deliverables under this Agreement; and hereby sublicenses to BMS the right to use any such Third Party Materials in connection with the Services and deliverables only in a manner as expressly provided under this Agreement.

8.6 At BMS’ expense, Service Provider agrees to execute and have executed any applications, assignments, instruments or other documents, and perform such acts, as BMS may deem reasonably necessary or advisable to confirm and vest all rights, title and interests throughout the world to such BMS Intellectual Property in BMS, and to reasonably assist BMS in procuring, maintaining, enforcing and defending such BMS Intellectual Property throughout the world.

8.7 At Service Provider’s expense, BMS agrees to execute and have executed any applications, assignments, instruments or other documents, and perform such acts, as Service Provider may deem reasonably necessary or advisable to confirm and vest all rights, title and interests throughout the world to such Service Provider Intellectual Property in Service Provider, and to reasonably assist Service Provider in procuring, maintaining, enforcing and defending such Service Provider Intellectual Property throughout the world.

8.8 Nothing in the Agreement is intended to grant or create any right or license by one party to the other party with respect to any patent rights, copyrights, trademarks or other intellectual property rights owned or controlled by BMS, except as necessary to perform the Services.

9 Representations and Warranties

9.1 Service Provider represents and warrants on behalf of itself, its Affiliates and any person performing Services under this Agreement that:

9.1 (a) Service Provider is a duly licensed specialty pharmacy.

9.1 (b) All communications (oral, written, electronic) delivered as part of the Services shall strictly comply with approved Product’s labeling.

9.1 (c) Services are incremental to the services that are typically performed for patients. Service Provider typically provides a wide array of specialty pharmacy services, any of which may require separate service fees and/or discount arrangements in order for Service Provider to provide. Standard services provided by Accredo Health Group for patients without receiving a fee from BMS or any pharmaceutical manufacturer include:

9.1 (c) (i) Dispensing and shipping prescription drugs directly to patients or to patients’ designated healthcare professional (including cold-chain delivery, where applicable);

9.1 (c) (ii) Providing general, publically available information about third-party access to medication (benefits investigation and reimbursement requirements);

9.1 (c) (iii) Coordination of refills;

9.1 (c) (iv) Twenty-four (24) hour access to qualified healthcare professionals (i.e., licensed pharmacist and/or nurse) to answer patient questions, seven (7) days a week; and

9.1 (c) (v) Pharmacy Intake Services: prescription processing, basic benefits investigation, identification of payer Prior Authorization (“PA”) requirements and communication of such requirements to the healthcare providers (“HCPs”), and scheduling delivery of Product orders.

9.1 (d) BMS Product shall be dispensed strictly in accordance with all BMS requirements and applicable law. BMS Product subject to a REMS, including elements to assure safe use, or other additional requirements, such as authorization, management, certification or other documentation, shall be dispensed strictly in accordance with such REMS or additional requirements.

9.1 (e) Services shall only be performed for patients with a valid prescription for Product.

9.1 (f) Services for which a nurse or pharmacist perform under this Agreement shall be performed only through qualified, trained and competent nurses and pharmacists. Each such nurse and pharmacist shall (i) possess a current and valid license, certification, or legal authorization, as applicable, in his/her applicable profession in each state in which he/she performs Services; (ii) have no prior practice related infractions or disciplinary history from any governing organization (e.g. state boards of nursing); and (iii) be and remain in good standing in the applicable state. Notwithstanding the above, BMS may, in its sole discretion, preclude any individual nurse and/or pharmacist from performing Services which are outbound in nature pursuant to this Agreement. By way of example, Services which are outbound in nature may include, but are not limited to: calling or sending any written form of communication to a patient or HCP, visiting a patient’s home to conduct injection training, etc. This 9.1(f) shall not apply to the standard services as outlined in section 9.1(c).

9.1 (g) Services shall only be performed for patients whose prescription is within the FDA approved indication.

9.1 (h) The clinical judgment of the patient’s treating physician (or other healthcare provider) shall not be undermined or otherwise usurped in the performance of Services.

9.1 (i) Service Provider has the authority under law, including but not limited to the applicable board of pharmacy regulations to perform the Services.

9.1 (j) Service Provider shall perform Services solely to patients to whom disclosure has been made that BMS is providing financial support for the Services if such disclosure is required by applicable law.

9.1 (k) Service Provider will not implement any intervention, technique, counsel or encourage any patient or physician to use or prescribe BMS Product over any other product in its class.

9.1 (I) Service Provider shall not offer to physicians (nor to any healthcare provider) any financial inducement to prescribe or switch patients to Product(s).

9.1 (m) Service Provider shall not seek reimbursement from, or file any claim with, any third party (including but not limited to any government entity or program, any patient, or any third-party payor) with respect to the Services.

9.1 (n) No person performing Services under this Agreement shall identify themselves as employees of BMS to any person or entity.

9.1 (o) Service Provider performs similar services to the Services, for other pharmaceutical and biotechnology companies pursuant to written agreements, and all require compensation at fair-market value.

9.1 (p) To the extent Service Provider uses software or information systems to create, modify, maintain, archive, retrieve or distribute electronic records or electronic signatures in connection with the Services, any such software or information system subject to 21 C.F.R. Part 11 (i) shall be maintained in a fully validated state; and (ii) shall comply with, through electronic and/or manual means (e.g., paper copies, personnel access controls), the standards contained 21 C.F.R. Part 11

9.1 (q) Service Provider shall not purchase, for itself or any third-party, any Product under this Agreement.

9.1 (r) In the course of performing Services, Service Provider shall (i) not communicate to any third-party information about BMS or any BMS product except to the extent such communication is consistent with the FDA-approved prescribing information, is balanced and complete (within the approved package insert), and presents all relevant information; (ii) not suggest that a BMS product is safer or more efficacious than the data in the product package insert demonstrates nor make comparative claims to other products that are not supported by the product package insert; and (iii) obtain BMS’ prior written approval of the content of any such communication.

9.1 (s) Service Provider (i) has facilities, personnel, experience, expertise, certifications, rights, licenses, and membership in professional organization(s) necessary and sufficient in quality and quantity to perform the Services; and (ii) will devote commercially reasonable efforts to perform the Services ethically, promptly and diligently and commensurate with relevant professional standards.

9.1 (t) Service Provider’s performance of this Agreement does not conflict with any obligations or duties that Service Provider may have to third parties.

9.1 (u) Unless prohibited by applicable law, prior to performing any Services, any person providing Services (i) on site at BMS owned or controlled property on an average of 2 or more days per calendar week during the term of this Agreement; and/or (ii) with access to BMS computer networks and systems, BMS study database(s) or controlled substances must, at Service Provider’s expense, have passed a background check and drug screening test comparable to what BMS employees are subject, such requirements to be provided to Service Provider in advance.

9.1 (v) Upon notice from BMS, Service Provider will replace any person performing Services which are outbound in nature, if BMS and Service Provider mutually agree that the performance of services by such person is unacceptable. Any costs associated with such replacement shall be borne by Service Provider. By way of example, Services which are outbound in nature may include, but are not limited to: calling or sending any written form of communication to a patient or HCP, visiting a patient’s home to conduct injection training, etc. This 9.1 (v) shall not apply to the standard services as outlined in section 9.1(c).

9.1 (w) Service Provider will not subcontract any of the Services hereunder without BMS’ prior written consent, such consent shall not be unreasonably withheld or delayed. Service Provider is liable for acts and omissions of each subcontractor and each subcontractor is bound in writing by obligations at least as strict as those in this Agreement for all relevant purposes. Notwithstanding the foregoing, Service Provider may subcontract Services under this Agreement to an Affiliate without the prior consent of BMS.

9.1 (x) With regard to personal data, Service Provider agrees to and will comply with the obligations set forth in Attachment – Personal Data.

9.1 (y) Service Provider will comply with HIPAA and its implementing regulations, as applicable.

9.1 (z) With regard to BMS’ audit rights, Service Provider agrees to and will comply with the obligations set forth in Attachment – Audit.

9.1 (aa) With regard to Adverse Events identified in the course of performing Services, for clarity not during standard services as defined in 9.1(c), Service Provider shall report to BMS within one (1) business day of receiving information about an event that meets the following BMS’ definition of an “Adverse Event”: Any untoward medical occurrence in a product user or a patient/consumer administered a BMS product, whether or not a causal relationship is demonstrated. An Adverse Event can therefore be any unfavorable or unintended sign (for example, an abnormal laboratory finding), symptom, death, injury, or disease temporarily associated with a use of a product, whether or not considered related to the product. For the avoidance of doubt, BMS requires pregnancy, overdoses, lack of efficacy, medication errors, including potential medical errors and transmissions of infectious agents, to be reported as an Adverse Event. Service Provider agrees that no person shall perform Services unless such person has been trained, to BMS’ reasonable satisfaction, on pharmacovigilance and adverse event reporting. BMS understands

that when Service Provider prices each SOW there are certain costs that must be considered and included within a fair market value price structure. BMS understands that these costs may relate to the work necessary for reporting Adverse Events to BMS under this section and may be included in the overall costs of the SOW.

9.1 (bb) No person performing Services is debarred under the U.S. Generic Drug Enforcement Act of 1992, 21 U.S.C. §§335(a) and as it may be amended from time to time (“GDEA”), in any capacity. Service Provider shall notify BMS promptly in the event any person so used becomes debarred under the GDEA during the term of this Agreement.

9.1 (cc) Neither Service Provider nor any person performing Services is currently under any loss or restriction of any professional license, nor of any related certifications, rights, or privileges that is necessary or required for the performance of Services.

9.1 (dd) Neither Service Provider nor any person performing Services is currently excluded, debarred, suspended, or otherwise ineligible to participate in Federal health care programs or in Federal procurement or non-procurement programs.

9.1 (ee) Neither Service Provider nor any person performing Services has been convicted of or is currently under investigation for a criminal offense that falls within the scope of 42 U.S.C. § 1320a-7(b), but has not yet been excluded, debarred, suspended, or otherwise declared ineligible.

9.1 (ff) Neither Service Provider nor any person performing Services appears on either the Department of Human Health & Services/Office of Inspector General List of Excluded Individuals/Entities, found at http://exclusions.oig.hhs.gov/ or the General Services Administration’s List of Parties Excluded from Federal Programs, found at https://www.sam.qov/portal/public/SAM/

9.1 (gg) Service Provider will, throughout the term of the Agreement and for a period of [***] from termination or expiration, maintain insurance coverage as identified in Attachment – Insurance; and agrees to provide to BMS a certificate evidencing such coverage upon request.

9.1 (hh) In accordance with the best interests and objectives of BMS, including efficiency, cost-effectiveness, and compliant and lawful performance of the Services, Service Provider will coordinate and work with any supplier of goods or services to BMS that relates to the Services, whether or not Service Provider has any relationship with such supplier. If Service Provider’s relationship with such suppliers affects any of the terms or obligations of Service Provider herein, the Parties agree to amend the Agreement to reflect any such changes and, if necessary, modify the fees to ensure compliance with fair market value (“FMV”).

9.2 Both parties represent and warrant that:

9.2 (a) Each will comply, to the extent applicable, with the Federal Anti-Kickback Statute, the Foreign Corrupt Practices Act, the Food Drug and Cosmetic Act, the Stark Act, the False Claims Act (and each of their implementing regulations), Affordable Care Act and any other applicable law, ordinance, rule, regulation and final or draft guidance document generally accepted as having the force of law in any country in which the Services are performed.

9.2 (b) Nothing in this Agreement shall be construed and none of the terms are overtly or covertly, directly or indirectly, in exchange for, or to induce, the referrals of patients to BMS or to Service Provider.

9.2 (c) Nothing prohibits Service Provider from also complying with its own standards, policies and procedures with regard to adverse events and product quality complaints.

9.2 (d) Product Promotion. Service Provider will not promote BMS’s Products, but Service Provider will promote its own specialty pharmacy services to its customers in accordance with Service Provider’s standard business practices, which typically include (but are not necessarily limited to) informing its customers of pricing available for products distributed by Service Provider. Accordingly, Service Provider shall not distribute or generate any promotional material containing claims relating to the Product. Service Provider may, however, provide its customers with educational information concerning the Product but only those that are approved by BMS in advance and distributed in the manner as approved by BMS in advance without modification of any kind, nor oral or other messaging accompanying such material, unless approved by BMS in advance. BMS represents and warrants that any materials relating to the Product that it provides to Service Provider, and approved for distribution: (i) are compliant with all applicable law; and (ii) if required under applicable law, have received the appropriate regulatory approval(s) prior to use (e.g., FDA approval); and (iii) do not involve the counseling or promotion of any off-label use.

10 Use of Name

10.1 Neither party shall make, place or disseminate any advertising, public relations, press release, promotional material or any material of any kind or any public statement, using the name of the other party and/or an Affiliate or a Collaboration Partner or use their trademark(s), without the prior written approval of the other party.

11 Collaborations and Collaboration Products

11.1 BMS co-develops, co-promotes or otherwise collaborates on certain unapproved and approved (by regulatory authorities) pharmaceutical and/or biological products, (“Collaboration Products”) with Collaboration Partners pursuant to certain agreements (“Collaboration Agreement(s)”).

11.2 Service Provider agrees that with regard to Services related to a Collaboration Product, the rights and obligations of confidentiality and non-use; and the rights and obligations regarding intellectual property shall apply to both BMS and the applicable Collaboration Partner for the particular Collaboration Product only.

11.3 BMS represents and warrants that BMS shall assume all liability for the actions of Collaboration Partner as they relate to the obligations, warranties, representations, and duties contained in this Agreement.

11.4 Service Provider represents and warrants that no person performing Services related to a Collaboration Product will share any information regarding Collaboration Partner’s products, other than the Collaboration Product, with employees or agents of BMS.

11.5 Service Provider represents and warrants that no person performing Services related to a Collaboration Product will share any information regarding BMS’ products, other than the Collaboration Product, with employees or agents of the applicable Collaboration Partner.

11.6 Service Provider agrees that BMS may deliver this Agreement and any and all SOW(s) that relate to a Collaboration Product to the applicable Collaboration Partner only after securing a confidentiality agreement with Collaboration Partner with terms at least as stringent as the terms contained herein (which may satisfied through the Collaboration Agreement).

12 Indemnification and Limitation of Liability

12.1 Service Provider will indemnify, defend and hold harmless BMS, its Affiliates, and its and their officers, directors, employees, agents, and Collaboration Partners (“BMS Indemnitees”) from and against any loss, expense, cost (including reasonable attorneys’ fees), liability, damage, or claim by a third party (collectively, “Claims”) made against BMS Indemnitees to the extent arising in connection with Service Provider’s (including, without limitation, any of its or its Affiliates and its and their officers, directors, employees, agents or subcontractors): (a) negligence or willful misconduct; (b) infringement of any third party intellectual property; (c) breach of any representation or warranties made under this Agreement (d) employment related claims made by any employee of Service Provider (or any subcontractor).

12.2 BMS will indemnify, defend and hold harmless Service Provider, its Affiliates, and its and their officers, directors, employees and agents (“Service Provider Indemnitees”) from and against any loss, expense, cost (including reasonable attorneys’ fees), liability, damage, or claim by a third party (collectively, “Claims”) made against Service Provider Indemnitees to the extent arising in connection with BMS’s (including, without limitation, any of its or its Affiliates and its and their officers, directors, employees, agents: (a) manufacturing of the Products; (b) any recall, quarantine, warning, or withdrawal of any Products; (c) the use by any third party of any Product; (d) negligence or willful misconduct; (e) infringement of any third party intellectual property; (f) breach of any representation or warranties made under this Agreement; or (g) employment related claims made by any employee of BMS.

12.3 EXCEPT FOR DAMAGES THAT ARISE FROM A BREACH OF CONFIDENTIALITY, GROSS NEGLIGENCE, WILLFUL MISCONDUCT, OR FOR WHICH A PARTY HAS AN OBLIGATON TO INDEMNIFY, DEFEND OR HOLD HARMLESS, IN NO EVENT SHALL EITHER PARTY BE LIABLE FOR ANY SPECIAL, COLLATERAL, INDIRECT, PUNITIVE, INCIDENTAL, CONSEQUENTIAL, OR EXEMPLARY DAMAGES IN CONNECTION WITH OR ARISING OUT OF THIS AGREEMENT OR THE USE OF THE DELIVERABLES PROVIDED HEREUNDER, REGARDLESS OF WHETHER SUCH PARTY HAS BEEN ADVISED OF THE POSSIBILITY OF SUCH DAMAGES. EXCEPT FOR DAMAGES THAT ARISE FROM A BREACH OF CONFIDENTIALITY, GROSS NEGLIGENCE, WILLFUL MISCONDUCT, OR FOR WHICH A PARTY HAS AN OBLIGATON TO INDEMNIFY, DEFEND OR HOLD HARMLESS, IN NO EVENT SHALL SERVICE PROVIDER’S AGGREGATE LIABILITY FROM ANY WARRANTY, OR OTHER OBLIGATION ARISING OUT OF OR UNDER THIS AGREEMENT, OF ANY USE OF ANY OF THE DELIVERABLE OR SERVICES PROVIDED HEREUNDER, EXCEED FIVE TIMES THE TOTAL FEES AND/OR OTHER AMOUNTS PAID BY BMS TO SERVICE PROVIDER FOR THE APPLICABLE SERVICE.

12.4 Notwithstanding the foregoing, the indemnifying party shall have no indemnity obligations, duties, or liability under this Section or this Agreement unless the indemnified party: (i) delivers prompt written notice of any claim made; (ii) tenders and provides to the indemnifying party, the

opportunity to control the defense of any such action, and take over, negotiate any settlement, settle, or defend any claim through counsel of indemnifying party’s choice and under and in indemnifying party’s sole direction; and (iii) fully cooperates with indemnifying party in the defense of any such action and makes available to indemnifying party all information, materials, witnesses, defenses, and the like, against any such claim known to or available to the indemnified party.

12.5 Service Provider’s indemnity obligations, duty and liability that apply to intellectual property shall not apply to or for any claim of infringement, liability, damages, costs, expenses, or the like, based on and/or relating to any claims arising out of: (i) any modification(s) of any of the deliverables and/or Services made by BMS, BMS’ Affiliates, agents or Collaboration Partners (and its agents and affiliates); (ii) any manufacture, use, offer for sale, sale, importation and/or exportation of any of the deliverables in combination with any products, software, equipment, information, materials, systems, methods, processes, apparatus, data, steps, or other material or information not specifically furnished and provided by Service Provider for use on and in connection with the deliverables and Services; (iii) any use of any of the deliverables and/or Services by any person or entity other than BMS, BMS’ Affiliates, Collaboration Partners (and its affiliates) and its and their agents who perform services to them; (iv) any use of the deliverables in a manner or for an application that is not a lawful business purposes or reasonably contemplated by the Agreement.

13 Business Conduct and Ethics

13.1 BMS takes seriously its compliance and ethics responsibilities and seeks to do business only with third parties who share high standards of ethical behavior. To that end, BMS has adopted Standards of Business Conduct and Ethics for Third Parties (3P Standards). BMS encourages Service Provider to comply with the elements of the 3P Standards that apply to Service Provider. The 3P Standards are available at http://www.bms.com/ourcompany/compllance ethics/Pages/default.aspx.

14 Notices

14.1 Any notices must be in writing and will be deemed effective when (a) delivered personally; or (b) delivered by reputable overnight courier with proof of delivery to the party and address set forth below or such other address(es) of which such party shall have given written notice.

For BMS:

Vice President and Associate General Counsel, Transactions
Contracts Center of Excellence
Bristol-Myers Squibb Company
Route 206 & Province Line Road
Princeton, New Jersey 08543-4000

For Accredo Health Group, inc.:

Express Scripts, Inc.
c/o Accredo Health Group, Inc.
One Express Way
St, Louis, MO 63121
Attn: Legal Department

With Copy to:

Accredo Health Group, Inc.

6272 Lee Vista Blvd.
Orlando, FL 32822
Attn: Contracting Department

15 Miscellaneous

15.1 Survival. The provisions of sections 2.3, 5.2, 5.3, 6, 7, 8, 9, 10, 11, 12, 14 and 15 shall survive expiration or termination of this Agreement.

15.2 No Waiver. No breach of any provision of this Agreement will be waived except with the express written consent of the party not in breach.

15.3 Severability. The invalidity or unenforceability of any provision of this Agreement shall not affect the validity or enforceability of any other provision of this Agreement. Any invalid, illegal or unenforceable term or provision of this Agreement shall be deemed replaced by a term or provision that is valid and enforceable and that comes closest to expressing the intention of the parties.

15.4 Entire Agreement. This Agreement and any attachments hereto set out the entire agreement of the parties and supersedes all prior agreements and understandings relating to its subject matter. This Agreement and any attachments hereto may not be altered, modified, or waived in whole or in part, except in writing signed by both parties.

15.5 Governing Law. This Agreement and any claim, controversy or dispute related to this Agreement or the relationship of the parties, will be governed by the laws of the State of New Jersey and shall be construed and governed by laws of that State without regard to the provisions governing conflict of laws.

15.6 Assignment. Neither party may assign any of its rights or delegate any of its duties under this Agreement without the prior written consent of the other party which consent will not be unreasonably withheld or delayed; provided, however, that either party may assign its rights and/or delegate its duties, without the consent of the other, to an Affiliate or to a purchaser of all or substantially all of its assets or stock without the prior written consent of the other party; and in the case of BMS, BMS may assign that portion of the Agreement (or the entire Agreement, if applicable), including all relevant SOWs related to a Collaboration Product to the applicable Collaboration Partner without the consent of Service Provider. Any unauthorized, attempted assignment by either party shall be null and void.

15.7 Force Majeure. Notwithstanding anything to the contrary herein, neither Party shall be liable in any manner for any delay to perform its obligations hereunder which are beyond a Party’s reasonable control, including, without limitation, any delay or failure due to strikes, labor disputes, riots, earthquakes, storms, hurricanes, floods or other extreme weather conditions, fire, explosions, acts of God, embargoes, war or other outbreak of hostilities, government acts or regulations, necessary to enable a Party to perform its obligations hereunder. In any such circumstance, the Party unable to perform its obligations shall notify the other Party of such

circumstance, and said other Party shall have the right to terminate this Agreement immediately upon provision of written notice if the Party of the first part continues to be unable to perform its obligations hereunder for a period of thirty (30) days.

15.8	Equal Employment Opportunity. To the extent applicable, the equal employment opportunity and affirmative action requirements set forth in 41 C.F.R. Part 60-1.4 (women and minorities), 41 C.F.R. Part 60-300-5 (covered veterans) and 41 C.F.R. Part 60-741.5 (individuals with disabilities) are hereby incorporated by reference into this Agreement.
15.9	Counterparts. This Agreement may be executed in counterparts each of which shall be deemed to be an original and all of such counterparts shall together constitute one and the same Agreement.

In order to demonstrate their agreement, the parties have executed this Agreement as of the Effective Date.

Bristol-Myers Squibb Company		Accredo Health Group, Inc.
By:	/s/ Cheryl L. Fassak	By:	/s/ Bill Martin
Name:	Cheryl L. Fassak	Name:	Bill Martin
Title:	Executive Director, Diabetes	Title:	VP
Date:	12/7/2013	Date:	12/4/13

ATTACHMENT - PERSONAL DATA

ADDITIONAL TERMS AND CONDITIONS FOR THE COLLECTION, USE, DISCLOSURE, RETENTION OR
PROCESSING OF PERSONAL DATA

With regard to the Agreement, Service Provider may gain access to “Personal Data,” which shall mean any information which identifies or is capable of identifying a living individual, or as otherwise defined as “Personal Data” by applicable laws, including, without limitation, information relating to BMS or BMS’ customers, consumers, patients, employees, personnel, shareholders, physicians, suppliers, consultants and competitors, whether verbal or recorded in any form or medium, disclosed to Service Provider, or collected by Service Provider on BMS’ behalf in order to provide the Services, including, without limitation, (a) an individual’s name, address, phone number, e-mail address, Social Security number or other country identifier, driver’s license number, bank account information, or credit card information; and (b) all information, data and materials, including without limitation, demographic, medical and financial information, that relate to (i) the past, present, or future physical or mental health or condition of an individual; (ii) the provision of health care to an individual; or (iii) the past, present, or future payment for the provision of health care to an individual.

Service Provider agrees that, in the event any of the Services that Service Provider provides to BMS related to Personal Data are subcontracted out to a third-party subcontractor, Service Provider shall obtain a written certification from said third-party subcontractor that it agrees to comply with each and every provision of this Attachment. Service Provider shall provide a copy of the written certification to BMS.

Collection, use, disclosure, retention and other processing of Personal Data may be regulated by certain privacy and data security laws.

When collecting, using, disclosing, retaining or otherwise processing Personal Data pursuant to the Agreement, Service Provider agrees to comply with all applicable privacy and data security laws, rules and regulations in those respective jurisdictions where Service Provider provides the Services and/or collect, use, disclose or otherwise process Personal Data pursuant to the Agreement. For purposes of this Attachment, applicable privacy and data security laws, rules, and regulations include, but are not limited to, any law related to the transmission, communication or storage of Personal Data via mail, telephone, computer, wireless technology, facsimile, or other such means.

Service Provider acknowledges and agrees that, as required and as appropriate under applicable laws, Service Provider will furnish notices to and/or obtain the prior and freely given, specific and informed consents of the individuals to whom the Personal Data relate to collect, use, disclose, retain or otherwise process such data in order to provide the Services.

Without limiting the foregoing and in addition to any other obligations under the Agreement, Service Provider further agrees as follows:

a. Service Provider agrees to collect, use, disclose, retain and otherwise process Personal Data solely as required by the Agreement and limited to that which is necessary for the purpose of performing Service Provider’s obligations under the Agreement, or as permissible under applicable laws;

b. Access to data: only authorized persons should have access to the Personal Data; Service Provider shall restrict such access to only those of its employees and subcontractors who have a specific, identifiable need for access to the Data in order to perform activities authorized and contemplated by the Agreement.

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c. Service Provider shall notify BMS of any request for disclosure of the Personal Data by a law enforcement authority, unless such disclosure is otherwise prohibited.

d. Service Provider shall not disclose Personal Data without prior approval by BMS other than as permitted by law.

e. Obligations to cooperate: Service Provider commits to cooperate with regard to BMS’ right to monitor processing operations, facilitate the exercise of data subjects’ rights to access/correct/erase their data, where applicable.

When collecting, using, disclosing, retaining or otherwise handling Personal Data for or on behalf of BMS, Service Provider will comply with BMS’s privacy policy and any other policies related to the protection of Personal Data confidentiality and security, as directed by BMS, so long as such policies comply with applicable law.

Any and all consents collected by Service Provider from individuals on behalf of BMS relating to collection, use, disclosure, retention and other processing of the individual’s Personal Data must be retained by Service Provider in compliance with applicable data retention policies and as requested by BMS. This data retention requirement shall survive termination or expiration of the Agreement.

Service Provider agrees to establish commercially adequate controls to prevent unauthorized access, use or disclosure of Personal Data. Service Provider will implement all safeguards that reasonably and appropriately protect the confidentiality, integrity, and security of Personal Data. Such safeguards shall include the encryption of sensitive Personal Data that includes United States Social Security numbers (or the comparable identifiers in other countries), driver’s license numbers, medical information (including all Personal Data pertaining to an individual’s health or the provision of or payment for the individual’s health care), bank account numbers and/or credit card information. Service Provider agrees to provide BMS and/or its representatives with a right to audit, at BMS’ discretion, but no more than once per year and only after giving Service Provider thirty (30) business days prior written notice, accompanied by a detailed scope, that includes the scope of the audit, Service Provider’s business policies, processes, books, records, and practices relating to collection, use, disclosure, retention and other processing of Personal Data. BMS shall be responsible for BMS’ costs associated with the audit. In no event shall BMS be responsible for paying any of Service Provider’s fees and expenses associated with the audit. If a designated agent of the Manufacturer conducts the audit, the designated agent shall enter into a confidentiality agreement with Service Provider. Audits during the months of December and January are limited to regulatory needs, which may include request by the FDA, “for cause” audits, or “breach of contract” audits.

Duty of cooperation with law enforcement authorities: Parties agree to cooperate with law enforcement authorities, especially in case of requests for information or during inspections.

Data portability: At any time, upon BMS request, Service Provider and subcontractors will provide BMS with a copy of all Personal Data in the same format as the one used by BMS to transfer the Personal Data to Service Provider, or in another structured and usual format, if permissible under HIPAA and other applicable privacy laws.

Traceability: Service Provider will keep, at BMS’s disposal, connection logs of processing operations performed by the provider and its subcontractors authorized personnel, for a period of 6 months.

Business continuity: Service Provider commits to implement necessary measures to ensure availability and integrity of Personal Data processed during the performance of the Agreement.

Upon expiration or termination of the Agreement, or at any time upon request of BMS, Service Provider shall return to BMS and/or destroy or otherwise handle as required by applicable law all Personal Data held in any

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form or medium whatsoever, collected or received from or on behalf of BMS. Service Provider shall promptly send BMS a written certification acknowledging that all Personal Data has been returned and/or destroyed or otherwise handle as required by applicable law. In the event that it is infeasible to return or destroy any Personal Data, Service Provider shall accord to such Data all the protections required by this Attachment regardless of the expiration or termination of the Agreement, for so long as Service Provider maintains the Personal Data.

Any collection, use, disclosure, retention and other handling of Personal Data other than as contemplated by this Attachment, permissible by law, and the Agreement will be deemed a material breach of the Agreement and subject to the applicable provisions of the Agreement related to a material breach; and

Service Provider agrees to work in good faith to mutually amend this Attachment at any time as requested by BMS in order to comply with any applicable law relating to privacy and data security in those respective jurisdictions where Service Provider provides services and/or collect, use, disclose or otherwise process Personal Data pursuant to the Agreement.

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ATTACHMENT
AUDIT

  

Service Provider shall:

1. maintain during the term of this Agreement and for the period of six years thereafter sufficient documentation necessary to demonstrate compliance with the requirements of this Agreement;

2. allow BMS (or its designee), upon [***] written notice, accompanied by a detailed scope, to validate Service Provider’s compliance with this Agreement during the term of this Agreement and for the period of two years thereafter, via an audit of Service Provider’s and Service Provider’s agents’ and subcontractors’ documents, procedures, systems and facilities and interviews with people under their control; any such audit to be conducted not more than once per calendar year, except (A) in the event of an audit based on BMS’ reasonable belief that a violation has occurred; (B) for audits related to quality control standards, for which no maximum number shall apply; and (C) to confirm resolution of adverse findings from a prior audit. If a third party is used to conduct any audit, such third-party with sign a confidentiality agreement with the audited party. Audits during the months of December and January are limited to regulatory needs, which may include request by the FDA, “for cause” audits, or “breach of contract” audits. Each such audit shall be subject to, and wholly covered by, the mutual confidentiality protections of this Agreement;

3. bear the expense, up to $[***], of any such audit if the audit reveals that Service Provider (or any agent or subcontractor of Service Provider) materially violated this Agreement, which caused damage to BMS. If the costs of such audit exceeded $[***], both parties will discuss and mutually agree on the allocation of any such costs in excess of $[***];

4. with regard to audits that reveal a compliance with law or breach of compliance related provision of the Agreement, bear the expense up to $[***], of any such audit if the audit reveals that Service Provider (or any agent or subcontractor of Service Provider) materially violated this Agreement, which caused damage to BMS. If the costs of such audit exceeded $[***], both parties will discuss and mutually agree on the allocation of any such costs in excess of $[***];

5. promptly reimburse BMS the amount of any overcharge revealed by any such audit;

6. be responsible for the cost of correcting any deficiencies revealed by any such audit, unless the parties otherwise agree, in writing;

7. notify BMS promptly after Service Provider’s receipt of notice of any proposed inspection by a government or regulatory authority that relates to this Agreement or the transactions contemplated by it; and

8. notify BMS promptly after Service Provider’s self-identification of any material violation of this Agreement and/or any failure to comply with any laws applicable to this Agreement or the transactions contemplated by this Agreement by Service Provider or any of its agents or subcontractors.

21

ATTACHMENT
INSURANCE

1 Service Provider shall, throughout the term of the Agreement maintain in full force and effect from a third party that is rated “A” or “A-” in Best’s Insurance Guide, or otherwise acceptable to BMS, the insurance coverages for Service Provider’s operations, as set forth below.

2 Service Provider agrees to maintain a policy of workers’ compensation insurance (as required by the applicable state statute) on its employees. Such policy shall provide statutory limits and contain Employer’s Liability coverage in an amount not less than $[***] per occurrence.

In addition, Service Provider agrees to maintain throughout term of the Agreement, the following insurance coverage for its operations worldwide:

Type of Coverage Amount	Amount
Automobile liability covering all vehicles owned non-owned, hired and leased	Not less than $[***] per claim (combined single limit for bodily injury and property damage)
Commercial general liability insuring against bodily injury, property damage, contractors’ completed operations, and contractual liability (including covering Service Provider’s indemnification obligations contained herein)	A combined single limit of not less than $[***] per claim
Professional liability and errors and omissions insurance	Not less than $[***] per claim

3 Service Provider may comply with the above insurance requirements through the combined purchase of primary, excess, or umbrellas policies. Service Provider shall furnish BMS with certificates of insurance evidencing the above coverages (including the effective and expiration dates of policies) and any exclusions to policies that are not part of the standard form and Service Provider will provide at least thirty (30) days prior written notice to BMS of cancellation or material modification. Insurance carried on claims made basis shall be carried for a sufficient period of at least five (5) years after termination of services under this Agreement or any amendment thereof.

4 The insurance coverage shall be primary, and all coverage shall be non-contributing with respect to any other insurance or self insurance which may be maintained by BMS with respect to Service Provider’s indemnification obligations contained herein.

5 Service Provider shall require each of Service Provider’s agents or subcontractors to comply with this Attachment as applicable to the services provided by such agent or subcontractor (as if each reference to “Service Provider” in this Attachment was a reference to such subcontractor).

6 For the avoidance of doubt, any policy amounts or limitations shall not in any event be construed as limitations or expansions on Service Provider’s liability under the Agreement.

7 The professional liability and errors and omissions insurance coverage carried by Service Provider shall include coverage against any malpractice or other claims brought in connection with Services provided by pharmacists, nurses, or other healthcare providers under this Agreement.

22

ATTACHMENT - DATA LICENSE AND ANALYTICS

ADDITIONAL TERMS AND CONDITIONS FOR DATA LICENSE AND ANALYTICS AND RELATED SERVICES

IN THE EVENT OF A CONFLICT BETWEEN THE AGREEMENT AND THIS ATTACHMENT, THIS ATTACHMENT SHALL GOVERN BUT SOLELY WITH REGARD TO DATA LICENSE AND ANALYTICS AND RELATED SERVICES. IN ALL OTHER CASES, THE AGREEMENT SHALL CONTROL OVER THIS ATTACHMENT.

WITHOUT LIMITING, AND IN ADDITION TO THE TERMS AND CONDITIONS OF THE AGREEMENT, THE FOLLOWING SHALL ALSO APPLY TO DATA LICENSE AND ANALYTICS AND RELATED SERVICES:

1 Licensed Material and Services

1.1 Service Provider owns or controls certain data or other materials (the “Licensed Material”) and agrees to license such Licensed Material to BMS pursuant to the terms of this Agreement.

1.2 Licensed Material shall be identified in one or more Purchase Order(s) (“PO(s)”) or SOWs. Each PO or SOW shall identify the following:

1.2 (a) Name/Title of the Licensed Material

1.2 (b) Duration of license to the Licensed Material

1.2 (c) Format of delivery of Licensed Material

1.2 (d) Schedule of delivery of Licensed Material

1.2 (e) License restrictions to the use of Licensed Materials

1.2 (f) Fee for Licensed Materials

1.3 Service Provider hereby grants BMS a license to the Licensed Material identified on each PO and SOW subject to this Agreement.

1.4 Notwithstanding anything to the contrary in Section 1.2(e) or a PO or SOW, the scope of the license that Service Provider grants to BMS shall include BMS’ Affiliates and BMS’ and its Affiliates’ agents who perform services to them for all Licensed Material.

1.5 Notwithstanding anything to the contrary in Section 1.2(e) or a PO or SOW, the geographic region of the license that Service Provider grants to BMS shall be worldwide, subject to applicable export law.

1.6 Section 1.4 and Section 1.5 are conditioned on BMS’ Affiliates and BMS’ and its Affiliates’ agents who perform services to them being subject to obligations of confidentiality, non-use and scope of license grant at least as restrictive as those contained herein.

1.7 Section 1.4 and Section 1.5 are conditioned on BMS remaining liable for any breach of this Agreement by BMS’ Affiliates and/or BMS’ and/or its Affiliates’ agents.

23

2 Termination

2.1 Upon termination of the Agreement, PO, or SOW, BMS is only obligated to pay for (a) Services actually and property performed and Reimbursable Expenses actually incurred or that are non-cancellable as of the termination or expiration date; and (b) pro rata adjusted fees for Licensed Materials actually received.

3 Intellectual Property

3.1 Licensed Materials licensed hereunder are the property of Service Provider and are protected by copyright, trademark, and other intellectual property laws.

4 Software

4.1 In the event any software is required to access the Licensed Material (the “Software”), Service Provider hereby grants BMS and its Affiliates and BMS’ and its Affiliates’ agents who perform services to them a non-exclusive, royalty free, worldwide license to access and use the Software for such term as is required to give effect to any license granted hereunder.

5 Representations and Warranties

5.1 All Licensed Material, the Software (if any) and materials and systems used in connection with the Services do not infringe the intellectual property rights of any third party.

5.2 Service Provider represents and warrants that (i) it has all necessary rights, title, licenses, certifications, registrations, permissions, and approvals required by law to grant the rights and licenses to the Licensed Material and the Software (if any), granted under this Agreement, and (ii) with regard to all Licensed Material and the Software (if any), Service Provider has not received actual or constructive notice that Service Provider may be infringing the copyright, patent, trademark, trade secret, or other intellectual property or other proprietary rights of any third party.

6 Collaborations and Collaboration Products

6.1 Service Provider agrees that with regard to Services related to a Collaboration Product, the rights to use any data, software or other materials that are licensed, shall apply to both BMS and the applicable Collaboration Partner for the particular Collaboration Product only.

7 Releases

7.1 Any materials furnished by Service Provider in performance of Services that are subject to the rights of third parties will be specifically identified to BMS in writing. Service Provider will obtain (and deliver to BMS upon request) releases for all names, photographs, illustrations, testimonials and any and all other materials used in such materials. All such releases will run directly to BMS.

24

ATTACHMENT - BENEFITS INVESTIGATION AND REIMBURSEMENT SUPPORT

ADDITIONAL TERMS AND CONDITIONS FOR BENEFITS INVESTIGATION AND REIMBURSEMENT SUPPORT SERVICES

IN THE EVENT OF A CONFLICT BETWEEN THE AGREEMENT AND THIS ATTACHMENT, THIS ATTACHMENT SHALL GOVERN BUT SOLELY WITH REGARD TO BENEFITS INVESTIGATION AND REIMBURSEMENT SUPPORT SERVICES. IN ALL OTHER CASES, THE AGREEMENT SHALL CONTROL OVER THIS ATTACHMENT.

WITHOUT LIMITING, AND IN ADDITION TO THE TERMS AND CONDITIONS OF THE AGREEMENT, THE FOLLOWING SHALL ALSO APPLY TO BENEFITS INVESTIGATION AND REIMBURSEMENT SUPPORT SERVICES:

1. BMS may choose to engage Service Provider to perform certain benefits investigation and/or reimbursement support services related to Products that are incremental to the services that Service Provider typically performs for patients. Any such services shall be described in one or more SOWs.

2. In no event shall Service Provider perform, nor shall BMS provide any compensation for, the following activities:

2.1. reviewing, preparing, drafting, completing, submitting, or assisting any healthcare professional or patient with the preparation or drafting of, any claims, prior authorization forms, medical exception documents, or appeals documents; and

2.2. contacting an insurer regarding a particular claim, requesting prior authorization or medical exception once such claim or request is submitted, or negotiating with the insurer in an attempt to secure prior authorization or medical exception approval, or payment of a claim or an appeal of a denied claim.

3. Service Provider represents and warrants that:

3.1. It shall perform any benefits investigation and/or reimbursement support services that are the subject of an SOW in a professional, careful and diligent manner and in accordance with the best practices in the specialty pharmacy industry;

3.2. It shall not perform any benefits investigation and/or reimbursement support services with respect to any patient unless Service Provider has received a written prescription from that patient’s prescribing physician; and

3.3. It shall not offer patients or healthcare professionals any reimbursement guarantees with respect to any Product.

4. The parties mutually represent and warrant that:

4.1. Any benefits investigation and/or reimbursement support services that are the subject of an SOW shall not (a) be intended to serve, either directly or indirectly, as a means for marketing any Product; (b) be intended to diminish the objectivity or professional judgment of a patient’s prescribing physician; and (c) involve the counseling or promotion of any off-label use of BMS’ products; and

4.2. Any fees for services related to benefits investigation and/or reimbursement support services that are the subject of an SOW shall: (a) represent fair market value for such services, based on arms-length negotiations; (b) not be intended in any way as remuneration for referrals or for other business generated; and (c) not be intended in any way as a payment related to a drug formulary or drug formulary activities and shall not have been negotiated or discussed between the parties in connection with any such drug formulary or formulary activities.

25

ATTACHMENT - COMPLIANCE, PERSISTENCY AND ADHERENCE

ADDITIONAL TERMS AND CONDITIONS FOR COMPLIANCE, PERSISTENCY AND ADHERENCE PROGRAM SERVICES

IN THE EVENT OF A CONFLICT BETWEEN THE AGREEMENT AND THIS ATTACHMENT, THIS ATTACHMENT SHALL GOVERN BUT SOLELY WITH REGARD TO COMPLIANCE, PERSISTENCY AND ADHERENCE PROGRAM SERVICES. IN ALL OTHER CASES, THE AGREEMENT SHALL CONTROL OVER THIS ATTACHMENT.

WITHOUT LIMITING, AND IN ADDITION TO THE TERMS AND CONDITIONS OF THE AGREEMENT, THE FOLLOWING SHALL ALSO APPLY TO COMPLIANCE, PERSISTENCY AND ADHERENCE PROGRAM SERVICES:

1. BMS may choose to engage Service Provider to perform certain compliance, persistency and/or adherence program services related to Products, for patients who have been prescribed Products within FDA approved indications that are incremental to the services that Service Provider typically performs for patients. Any such services shall be described in one or more SOWs.

2. Service Provider represents and warrants that:

2.1. It shall perform any compliance, persistency and/or adherence program services that are the subject of an SOW in a professional, careful and diligent manner and in accordance with the best practices in the specialty pharmacy industry;

2.2. It shall not perform any compliance, persistency and/or adherence program services with respect to any patient unless Service Provider has (a) received a written prescription from that patient’s prescriber, and (b) disclosed to the patient that BMS is providing financial support for the compliance, persistency and/or adherence program services;

2.3. It shall not seek reimbursement from or file any claim with any third party (including, without limitation, any government entity or program, any patient, or any third-party payor) with respect to any compliance, persistency and/or adherence program services;

2.4. It shall not develop, use or distribute any materials regarding BMS, or its Affiliates or Collaboration Partners, or any Product without BMS’ prior written approval. All materials that Service Provider develops with BMS’ written approval must contain a clear and conspicuous statement disclosing that BMS is providing financial support to Service Provider to perform the compliance, persistency and/or adherence program services; and

2.5. It shall not implement any intervention technique, counsel or encourage any patient, physician, or any other healthcare professional to use or prescribe a Product over any other pharmaceutical product in its therapeutic class.

3. The parties mutually represent and warrant that:

3.1. Any compliance, persistency and/or adherence programs that are the subject of an SOW shall not (a) be intended to serve, either directly or indirectly, as a means for marketing any Product; (b) be intended to diminish the objectivity or professional judgment of a patient’s prescribing physician; and (c) involve the counseling or promotion of any off-label use of BMS’ products; and

3.2. Any fees for services related to compliance, persistency and/or adherence programs that are the subject of an SOW shall: (a) represent fair market value for such services, based on arms-length negotiations; (b) not be intended in any way as remuneration for referrals or for other business generated; and (c) not be intended in any way as a payment related to a drug formulary or drug formulary activities and shall not have been negotiated or discussed between the parties in connection with any such drug formulary or formulary activities.

26

EX-10.12.2

Exhibit 10.12.2

1^st AMENDMENT

This 1^st AMENDMENT (“Amendment”) is entered by and between Bristol-Myers Squibb Company (“BMS”) and Accredo Health Group, Inc. (“Company”) as of January 19, 2014 (the “Effective Date of this Amendment”).

PRELIMINARY STATEMENT

A. The parties desire to amend the MASTER SERVICES AGREEMENT FOR SERVICES RELATED TO METRELEPTIN effective December 6, 2013 (the “Agreement”).

The parties agree:

1. That “Attachment – Compliance, Persistency and Adherence” is hereby deleted in its entirety and replaced with “Amended and Restated – January 19, 2014 – Attachment – Compliance, Persistency and Adherence”, attached and made a part hereof.

2. That except as amended hereby, the Agreement remains in full force and effect.

(In order to demonstrate their agreement, the parties have executed this Amendment as of the Effective Date of this Amendment.

Agreed and Accepted:		Agreed and Accepted:
BRISTOL-MYERS SQUIBB COMPANY		Accredo Health Group, Inc.
By:	/s/ Cheryl Fassalk	By:	/s/ Bill Martin
Name:	Cheryl Fassalk	Name:	Bill Martin
Title:	Executive Director	Title:	VP
	Diabetes Alliance

AMENDED AND RESTATED - JANUARY 19, 2014
ATTACHMENT - COMPLIANCE, PERSISTENCY AND ADHERENCE

ADDITIONAL TERMS AND CONDITIONS FOR COMPLIANCE, PERSISTENCY AND
ADHERENCE PROGRAM SERVICES

IN THE EVENT OF A CONFLICT BETWEEN THE AGREEMENT AND THIS ATTACHMENT, THIS ATTACHMENT SHALL GOVERN BUT SOLELY WITH REGARD TO COMPLIANCE, PERSISTENCY AND ADHERENCE PROGRAM SERVICES. IN ALL OTHER CASES, THE AGREEMENT SHALL CONTROL OVER THIS ATTACHMENT.

WITHOUT LIMITING, AND IN ADDITION TO THE TERMS AND CONDITIONS OF THE AGREEMENT, THE FOLLOWING SHALL ALSO APPLY TO COMPLIANCE, PERSISTENCY AND ADHERENCE PROGRAM SERVICES:

1. BMS may choose to engage Service Provider to perform certain compliance, persistency and/or adherence program services related to Products, for patients who have been prescribed Products within FDA approved indications that are incremental to the services that Service Provider typically performs for patients. Any such services shall be described in one or more SOWs.

2. Service Provider represents and warrants that:

2.1. It shall perform any compliance, persistency and/or adherence program services that are the subject of an SOW in a professional, careful and diligent manner and in accordance with the best practices in the specialty pharmacy industry;

2.2. It shall not perform any compliance, persistency and/or adherence program services with respect to any patient unless Service Provider has (a) received a written prescription from that patient’s prescriber, and (b) disclosed to the patient that BMS is providing financial support for the compliance, persistency and/or adherence program services;

2.3. It shall not seek reimbursement from or file any claim with any third party (including, without limitation, any government entity or program, any patient, or any third-party payor) with respect to any compliance, persistency and/or adherence program services;

2.4. It shall not develop, use or distribute any materials regarding BMS, or its Affiliates or Collaboration Partners, or any Product without BMS’ prior written approval. All materials that Service Provider develops with BMS’ written approval must contain a clear and conspicuous statement disclosing that BMS is providing financial support to Service Provider to perform the compliance, persistency and/or adherence program services; and

2.5. It shall not implement any intervention technique, counsel or encourage any patient, physician, or any other healthcare professional to use or prescribe a Product over any other pharmaceutical product in its therapeutic class.

2.6. Service Provider represents and warrants that any compliance, persistency and/or adherence program services that are the subject of an SOW shall be conducted in compliance with 45 C.F.R. Parts 160 & 164, including with respect to (1) the amount of the reimbursement Service Provider receives from BMS for providing such services and (2) the disclosure of such reimbursement to patients.

3. The parties mutually represent and warrant that:

3.1. Any compliance, persistency and/or adherence programs that are the subject of an SOW shall not (a) be intended to serve, either directly or indirectly, as a means for marketing any Product; (b) be intended to diminish the objectivity or professional judgment of a patient’s prescribing physician; and (c) involve the counseling or promotion of any off-label use of BMS’ products; and

3.2. Any fees for services related to compliance, persistency and/or adherence programs that are the subject of an SOW shall: (a) represent fair market value for such services, based on arms-length negotiations; (b) not be intended in any way as remuneration for referrals or for other business generated; and (c) not be intended in any way as a payment related to a drug formulary or drug formulary activities and shall not have been negotiated or discussed between the parties in connection with any such drug formulary or formulary activities.

 

EX-10.12.3

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.12.3

SECOND AMENDMENT TO
MASTER SERVICES AGREEMENT

This Second Amendment (the “Amendment”) to the Master Services Agreement dated December 6, 2013 by and between Bristol-Myers Squibb Company (“BMS”), having an address at Route 206 and Province Line Road, Princeton, New Jersey 08543 and Accredo Health Group, Inc. (“Service Provider”) having an address at 6272 Lee Vista Boulevard, Orlando, FL 32822 (the “Agreement”) is made effective as of June 1, 2014 (the “Amendment Effective Date”) between AstraZeneca Pharmaceuticals LP, a Delaware limited partnership with offices at 1800 Concord Pike, Wilmington, Delaware 19803 (“AstraZeneca”); and Service Provider.

WHEREAS, Service Provider acknowledges and understands that AstraZeneca and BMS previously participated in a collaboration pursuant to co-development and co-promotion agreements between BMS and AstraZeneca with respect to multiple diabetes products, ONGLYZA™, KOMBIGLYZE™ XR, BUDUREON™, BYETTA™, SYMLIN™, FARXIGA™ and MYALEPT™ (collectively, the “Diabetes Products”). The collaboration was expanded by BMS’ acquisition of Amylin Pharmaceuticals (“Amylin”) and, effective as of February 1, 2014, AstraZeneca acquired the rights to the BMS Diabetes Products. The Agreement was transferred as of February 1, 2014 from BMS to AstraZeneca.

WHEREAS, the parties desire to amend, modify and restate certain terms and conditions of the Agreement.

NOW, THEREFORE, in consideration of the mutual covenants contained in this Amendment, and other good and valuable consideration, the receipt and sufficiency of which are hereby acknowledged, the parties, intending to be legally bound, agree as follows:

1 Definitions

Any capitalized term not separately defined in this Amendment shall have the meaning ascribed to it in the Agreement.

2 Modifications

(a) All references to BMS in the Agreement are hereby changed to AstraZeneca and AstraZeneca hereby assumes all of the rights and responsibilities of BMS set forth in the Agreement.

(b) Section 3.1 of the Agreement, “Payment” is hereby deleted in its entirety and replaced with the following:

Service Provider shall deliver to AstraZeneca, within [***] after the end of each calendar month, an invoice for fees and authorized Pass-Through Expenses. The invoice shall contain a written progress report detailing the work performed by Company and a reasonable accounting of fees and reimbursable Pass-Through Expenses. Invoices and copies of receipts shall be submitted electronically (pdf, doc, xls or ppt format) to:

[***]

Service Provider shall keep original receipts for a period of five years for audit and reporting purposes.

AstraZeneca shall pay invoices within [***] following receipt. AstraZeneca shall have no obligation to reimburse Service Provider for Pass-Through Expenses that are not invoiced within [***] of the date that Service Provider incurred such expense.

(c) Section 9.1 (aa) of the Agreement is hereby deleted in its entirety and replaced with the following:

Adverse Event Reporting Requirements. Service Provider Employees shall be required to report Adverse Events to AstraZeneca in accordance with AstraZeneca policies and procedures, including training, compliance review and maintenance of records.

i.        Definition. An “Adverse Event” or “AE” is the development of an undesirable medical condition or the deterioration of a preexisting medical condition following or during exposure to a pharmaceutical product, whether or not considered causally related to the product. An undesirable medical condition can be symptoms (e.g., nausea, chest pain), signs (e.g., tachycardia, enlarged liver) or the abnormal results of an investigation (e.g., laboratory findings, electrocardiogram).

ii.       Adverse Event Training. Service Provider Employees must complete an AE reporting training program, developed and provided by AstraZeneca, prior to beginning designated projects on behalf of AstraZeneca. Successful completion and documentation of this training is required annually for those Employees supporting designated AstraZeneca projects. Additional training may be required at AstraZeneca’s discretion.

iii.      Procedure for Management of Adverse Event Information. AstraZeneca’s AE reporting process requires that Service Provider and its Employees shall collect and submit to the AstraZeneca Information Center (“AZIC) at 1-800-236-9933 (unless another entity to which the report is to be sent is specified in SOW) within one (1) business day any AE information involving any AstraZeneca product that is the subject of this Agreement and that Service Provider becomes aware of in the course of performing the Services. Service Provider shall attempt to warm transfer any Adverse Event or Product Complaint to AstraZeneca that meets AstraZeneca’s definition of an Adverse Event. In the event a patient does not remain on the phone for the warm transfer (i.e. dropped call prior to transfer), Service Provider shall notify AstraZeneca within one (1) business day, using the Service Provider Adverse Event Form, included as Exhibit A, by emailing the AZIC the completed form to [***].

iv.      Record Retention and Regulatory Inspections. Service Provider shall maintain records of all AE reports received on source documentation or entered into a Service Provider system. Service Provider shall also maintain records of successful AE training completion for all clinicians responsible for reporting AEs in support of this Agreement. AstraZeneca has the right to request that copies of such records be submitted to AstraZeneca within two (2) business days on an as-needed basis. In the event of a regulatory inspection, AstraZeneca has the right to request

copies of records to be submitted to AstraZeneca within 24 hours of an urgent mandated turnaround time request by a regulatory body. On the first day of each month, the AZIC will email an auto-generated report of cases received from Service Provider for reconciliation. Service Provider will compare this listing with Service Provider’s log of AEs to ensure that all dropped call cases were reported to AstraZeneca. Service Provider will send the reconciled report to the AZIC designee. If any discrepancy is noted by Service Provider, Service Provider will contact the AZIC designee within one (1) business day of Service Provider’s becoming aware of the discrepancy.

v.   Compliance Review. Service Provider shall, on an ongoing basis, review its compliance with AstraZeneca’s AE training requirements and reporting process. Company shall promptly notify AstraZeneca of any deviation from such training requirements or reporting process.

(d) Subsection 14.1 of Section 14, “Notices”, is hereby deleted in its entirety and replaced with the following:

Any notice that is required or permitted hereunder shall be deemed given only if delivered personally or sent by facsimile (with transmission confirmed) or by registered or certified mail, return receipt requested, or by a nationally recognized overnight delivery service, addressed as follows:

For:	AstraZeneca Pharmaceuticals LP
Address:	1800 Concord Pike Box 15437 Wilmington, DE 19803-5437
Facsimile:	001-302-886-2909
	For the attention of: Commercial Procurement
With a copy to:
Address:	AstraZeneca Pharmaceuticals LP 1800 Concord Pike Wilmington, DE 19803 Attention: General Counsel
If to Service Provider:
	Express Scripts, Inc.
Address:	One Express Way St. Louis, MO 63121 Attention: Legal Department, Accredo Health Group, Inc.
With a copy to:
	Accredo Health Group, Inc. 6272 Lee Vista Blvd. Orlando, FL 32822 Attn: Legal, Pharma Contracting

Notices shall be effective upon receipt. It is understood that this Section 14.1 is not intended to govern the day-to-day business communications between the parties in performing Services.

(e) Exhibit A is hereby added to the Agreement.

3 Counterparts

This Amendment may be executed in two or more counterparts, each of which shall be deemed an original and all of which shall together be deemed to constitute one agreement. The parties agree that execution of this Amendment by exchanging facsimile or PDF signatures shall have the same legal force and effect as the exchange of original signatures.

4 Entire Agreement

This Amendment, together with the Agreement, constitutes the entire agreement between the parties with respect to the subject matter of the Agreement. The Agreement together with this Amendment supersedes all prior agreements, whether written or oral, with respect to the subject matter of the Agreement, as amended. The parties hereby agree that subject to the modifications specifically stated in this Amendment, all terms and conditions of the Agreement, as amended, shall remain in full force and effect.

Each party is signing this Amendment on the date stated opposite that party’s signature.

Accredo Health Group, Inc.		AstraZeneca Pharmaceuticals LP
By:	/s/ Bill Martin	By:	/s/ Ellen Madford
Name:	Bill Martin	Name:	Ellen Madford
Title:	VP	Title:	Procurement Sourcing Manager
Date:	6/26/14	Date:	7/10/14

EXHIBIT A 

Accredo Adverse Event Form

 

1640 Century Center Parkway 

Memphis, TN 38134 

Phone: 1 800 235-8498 Ext. 83358

CSADCO Adverse Event (AE) Form

Reporter (person reporting the event to the pharmacy):
Last name:	First name:		Title (i.e. RN, MD, etc):
Relationship to patient (i.e. husband, wife, daughter, etc.)
Address:
City, state, zip:
Phone number:		Fax number:

Patient Information:
Patient name or initials:			Patient identifier:
Date of birth:	Sex:		Weight	lbs	kg	Height	in	cm
Occupation:		If female, pregnant at time of event? ☐Yea ☐No ☐NA			Report also sent to MedWatch?  ☐Yes ☐No

Suspect Drug Information:
Drug name:	Strength of dosage form:
Dose and route:	Frequency:
Lot #:	Expiration date:
Indication/diagnosis for use: (Please provide written diagnosis not ICD-9 code.)

Dates of Therapy:
Start date:	Stop date:
If therapy stopped and restarted provide restart date:
Restart dose and route:	Restart frequency:

Confidential and Proprietary Information	Updated: Feb-27-2013; Reviewed: Feb-27-2013
© 2013 Accredo Health Group, Inc. An Express Scripts Company. All Rights Reserved.	Form #: F-AHG-324

1640 Century Center Parkway 

Memphis, TN 38134 

Phone: 1 800 235-8498 Ext. 83358

CSADCO Adverse Event (AE) Form

List Adverse Experience(s)	Date of Event Onset	Summary or ADE. include description of severity (i.e. If hospitalized for event please specify)
1
		☐ Resolved ☐ Improved Not resolved ☐ Unknown
		Date event resolved:
2
		☐ Resolved ☐ Improved Not resolved ☐ Unknown
		Date event resolved:
3
		☐ Resolved☐ Improved Not resolved ☐ Unknown
		Date event resolved:

Other relevant information: Document other relevant history, inducing preexisting medical conditions (e.g. allergies, race, pregnancy, smoking and alcohol use, hepatic/renal dysfunction, etc.

List any medication(s) patient was using at or around the time of the adverse event and dates of therapy. (Exclude medications used to treat event) include OTC and herbals.

Confidential and Proprietary Information	Updated: Feb-27-2013; Reviewed: Feb-27-2013
© 2013 Accredo Health Group, Inc. An Express Scripts Company. All Rights Reserved.	Form #: F-AHG-324

 

 1640 Century Center Parkway

Memphis, TN 38134

Phone: 1 800 235-8498 Ext. 83358

CSADCO Adverse Event (AE) Form

List any relevant tests/laboratory data including dates.

Prescriber information:
Prescriber’s name:
Address:
Phone number:	Fax number:

Pharmacy Information: Pharmacy name, name and title of person completing this form, address, phone number, and date information reported to the pharmacy:
Pharmacy name and address:
Name of person completing form:	Title:(R.Ph, PharmD, R.N., etc.)	Date Information reported to pharmacy:	Time Information reported to pharmacy:
Phone number:		Fax number:

Confidential and Proprietary Information	Updated: Feb-27-2013; Reviewed: Feb-27-2013
© 2013 Accredo Health Group, Inc. An Express Scripts Company. All Rights Reserved.	Form #: F-AHG-324

EX-10.12.4

Exhibit 10.12.4

THIRD AMENDMENT TO
MASTER SERVICES AGREEMENT

THIS THIRD AMENDMENT TO MASTER SERVICES AGREEMENT (“3rd Amendment”) dated as of June 20, 2016 (“Effective Date”) is entered into by and between Accredo Health Group, Inc. (“Service Provider”) and Aegerion Pharmaceuticals, Inc., a Delaware corporation with offices at One Main Street Suite 800, Cambridge, MA 02142 (“Aegerion”) as successor in interest to AstraZeneca Pharmaceutical LP, a Delaware limited partnership with offices at 1800 Concord Pike, Wilmington, Delaware 19803 (“AstraZeneca”).

WITNESSETH:

WHEREAS, the predecessor in interest to Aegerion and Service Provider entered into a MASTER SERVICES AGREEMENT with an effective date of December 6, 2013 (the “Agreement”); which was later amended by the 1^st Amendment dated January 19, 2014, and the Second Amendment dated June 1, 2014; and

WHEREAS, the Agreement was assigned to Aegerion by AstraZeneca effective January 9, 2015; and

WHEREAS, Aegerion and Service Provider desire to amend the terms of the Agreement as set forth herein.

NOW, THEREFORE, THE PARTIES, intending to be legally bound, agreed to amend the Agreement as follows:

1. Notwithstanding anything in the Agreement, to the contrary, the parties mutually agree to extend the term of the Agreement until July 31, 2018.

2. Except as modified herein, all other terms of the Agreement remaining full force and effect.

IN WITNESS WHEREOF, the parties have hereunto signed this 3rd Amendment effective as of the date set forth above.

AEGERION PHARMACEUTICALS, INC. ACCREDO HEALTH GROUP, INC.

By: /s/ Christine Strobele  By: /s/ Bill Martin 

Name: Christine Strobele
Name: Bill Martin

Title: VP Pt Engagement  Title: VP

Date: 6/23/16 Date: 6/20/16

EX-10.12.5

Pursuant to 17 CFR 229.601(b)(10)(iv), confidential information (indicated by [***]) has been omitted from this exhibit because it is both not material and would likely cause competitive harm to the registrant if publicly disclosed.

Exhibit 10.12.5

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FOURTH AMENDMENT TO
MASTER SERVICES AGREEMENT

This Fourth Amendment (the “Amendment”) to the Master Services Agreement dated December 6, 2013, as amended, by and between Aegerion Pharmaceuticals, Inc. (“Aegerion”), as assignee of AstraZeneca Pharmaceuticals LP, and Accredo Health Group, Inc. (“Service Provider”) (the “Agreement”) is effective as of October 19, 2017 (the “Amendment Effective Date”). Any capitalized terms used but not defined in this Amendment shall have the meanings ascribed to them in the Agreement.

WHEREAS, the parties desire to amend certain terms of the Agreement to reflect its assignment from AstraZeneca to Aegerion.

NOW, THEREFORE, in consideration of the mutual covenants contained in this Amendment and other good and valuable consideration the receipt and sufficiency of which is hereby acknowledged, the parties agree as follows:

1. All references to AstraZeneca in the Agreement are hereby changed to Aegerion and Aegerion hereby assumes all of the rights and responsibilities of AstraZeneca set forth in the Agreement.

2. Section 1.30(j) is hereby deleted in its entirety and replaced with the following:

“The delivery, storage, shipment, sale, or handling of any Product.”

3. Section 1.3(1) is hereby deleted in its entirety and replaced with the following:

“Reserved”

4. Section 2.7(a) is hereby deleted in its entirety and replaced with the following:

“Aegerion will reimburse Service Provider for all reasonable out-of-pocket expenses incurred or paid by Service Provider in connection with the performance of Services by under this Agreement, but in each case solely to the extent such expenses have been authorized in advance in writing by an authorized representative of Aegerion or are otherwise specifically listed as reimbursable expenses on Attachment 2 attached hereto and incorporated herein by reference. Service Provider will furnish appropriate supporting documentation for authorized expenses to be reimbursed by Aegerion under this Agreement. All fees and expenses incurred by Service Provider in performing Services under this Agreement that are not specifically listed as part of fees and reimbursable expenses on Attachment 2 or otherwise expressly authorized in advance in writing by an authorized representative of Aegerion will be borne by Service Provider.”

5. Section 3.1of the Agreement - Payment - is hereby amended by replacing [***] with [***].

6. Section 4.1 - Term - is hereby deleted and replaced in its entirety with the following:

“This Agreement shall commence on the Effective Date and, unless terminated earlier in accordance with Section 5, below, shall expire on September 30, 2019 and shall automatically renew for an additional one year term ending September 30, 2020 unless

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either party gives written notice of non-renewal to the other at least 180 days prior to September 30, 2019.”

7. Section 9.1(f) of the Agreement is hereby deleted and replaced with the following:

“Services performed by a nurse or pharmacist under this Agreement shall be performed only through qualified, trained and competent nurses and pharmacists. Each such nurse and pharmacist shall (i) possess a current and valid license, certification, or legal authorization, as applicable, in his/her applicable profession in each state in which he/she performs services; and (ii) be and remain in good standing in the applicable state.”

8. Section 9.1(z) of the Agreement – Audit Rights – is hereby deleted and replaced with the following:

“Service Provider guarantees an acknowledgement response time within three (3) business days for all month end, quarter end, and other financial inquiries received from Aegerion. In addition, Service Provider agrees to and will comply with the obligations set forth in Attachment – Audit.”

9. The Agreement is hereby amended by (a) inserting new Exhibit B thereto as the same is attached hereto and incorporated herein by reference, and (b) deleting Section 9.1(aa) of the Agreement - Adverse Event Reporting - in its entirety and replacing it with the following:

“See Exhibit B attached hereto and incorporated herein by reference”.

10. Section 14.1 of the Agreement - Notice - is hereby amended by deleting in its entirety the Notice addresses for AstraZeneca and replacing them with the following:

“For:	Aegerion Pharmaceuticals, Inc.
Address:	One Main Street, Suite 800
	Cambridge, MA 02142
	Attn: President
With a copy to:
Address:	Aegerion Pharmaceuticals, Inc.
	One Main Street, Suite 800
	Cambridge, MA 02142
	Attn: Vice President, Legal”

11. Section 13.1 - Business Conduct and Ethics - is hereby replaced in its entirety with the following:

“Aegerion takes seriously its compliance and ethics responsibilities and seeks to do business only with third parties who share high standards of ethical behavior. To that end, Aegerion has adopted the Business Partner Guiding Principles a copy of which is attached hereto and incorporated herein as Attachment 1. Service Provider abides by its parent company’s Code of Conduct, available at https://express-scripts. com/aboutus/codeconduct/ExpressScriptsCodeOfConduct.pdf.

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12. Section 2.7 of the Agreement is hereby deleted in its entirety and replaced with the following:

“2.7 Reimbursable Expenses

2.7(a) Aegerion will reimburse Service Provider for all reasonable and necessary business and travel expenses and pass-through costs actually incurred by Service Provider in the course of performing Services at cost without mark-up (“Reimbursable Expenses”).

2.7(b) Service Provider shall obtain Aegerion’s written approval prior to incurring any travel expenses that will be invoiced to Aegerion under this Agreement as Reimbursable Expenses.

2.7(c) Service Provider agrees to invoice Aegerion for Reimbursable Expenses, which invoice will be accompanied by any supporting documentation (e.g. receipts) required by Aegerion.

2.7(d) Aegerion will have no obligation to reimburse Service Provider for any Reimbursable Expenses unless and until all documentation required by this Agreement and Aegerion is received from Service Provider.”

13. Section 15.5 of the Agreement - Governing Law - is hereby amended by replacing “State of New Jersey” with “State of New York”.

14. The Attachment to the Agreement entitled “ATTACHMENT – PERSONAL DATA” is amended and restated in its entirety as set forth in the new “ATTACHMENT – PERSONAL DATA” attached hereto and incorporated herein by reference.

15. The Agreement is hereby amended by inserting the following text as Section 16 of the Agreement:

“16. Compliance

16.1 General. The requirements of this Section 16 shall apply to all employees, agents, contractors and subcontractors of Service Provider that provide goods or services to Aegerion pursuant to this Agreement (collectively, “Personnel”). Service Provider shall ensure that all Personnel abide by the requirements set forth in this Section 16. To the extent that Service Provider reasonably expects that any Personnel will provide goods or services to Aegerion in excess of [***] during a contract year, then Service Provider shall specifically furnish the names of such Personnel to Aegerion (“Listed Personnel”) within [***] following the execution of the fourth amendment to this Agreement. Service Provider shall notify Aegerion with any changes to the Listed Personnel within [***] of Service Provider’s first knowledge thereof.

16.2 Training; Aegerion Compliance Policies and Procedures.

16.2(a) Within [***] of the execution of the fourth amendment to this Agreement, or a longer period if approved by Aegerion in writing, Service Provider shall ensure that all Listed Personnel have completed all compliance training required by Aegerion, which shall be limited to

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compliance training that Aegerion has agreed to ensure Listed Personnel complete. If requested by Aegerion for Listed Personnel, the training and certification will be provided by Aegerion through Aegerion’s corporate learning management system. In addition, Service Provider shall ensure that all Listed Personnel shall timely complete any compliance training required by Aegerion during the term of this Agreement.

16.2(b) Upon request from Aegerion, Service Provider shall certify to the completion of all required training by all Listed Personnel.

16.2(c) Service Provider shall ensure that its Listed Personnel promptly review any updated Aegerion compliance policies and procedures that are provided to Service Provider by Aegerion.

16.2(d) Upon request from Aegerion, Service Provider shall certify in writing, in the manner and form reasonably determined by Aegerion, that Listed Personnel received, read, understood and will abide by all required Aegerion compliance policies and procedures, including the Aegerion Code of Conduct.

16.3 Violations. Service Provider shall ensure Listed Personnel promptly report to the Aegerion Compliance Department any suspected or actual violations of criminal, civil, or administrative law or Aegerion compliance policies observed during the performance of this Agreement. Any such report can be made, on an attributed or anonymous basis, by writing to the Chief Compliance Officer c/o Aegerion Pharmaceuticals, Inc., One Main St., Ste. 800, Cambridge, MA 02142, calling 855-233-8089 or visiting https://novelioncompliance.tnwreports.com. Service Provider agrees to appropriately publicize to Personnel the ability to make such reports (e.g., via periodic e-mails to Personnel, or by posting the information in prominent common areas), and shall emphasize a nonretribution, nonretaliation policy regarding such reports.

16.4 Ineligible Persons. Service Provider represents and warrants that, as of the effective date of the fourth amendment to this Agreement, it is not, and no Personnel are, an “Ineligible Person,” defined as any individual or entity who: (i) is currently excluded from participation in Federal health care programs or (ii) has been convicted of a criminal offense that falls within the scope of 42 U.S.C. § 1320a-7(a), but has not yet been excluded. Service Provider shall screen all prospective Personnel against the HHS/OIG List of Excluded Individuals/Entities (LEIE) (available through the Internet at http://www.oig.hhs.gov) (“Exclusion List”)). Service Provider shall screen all Personnel against the Exclusion List in accordance with its policies (which shall be no less frequently than quarterly) and shall promptly notify Aegerion if Service Provider or any Listed Personnel become an Ineligible Person. Notwithstanding any other provision of this Agreement, Aegerion may terminate this Agreement immediately upon Service Provider becoming an Ineligible Person. If Service Provider has actual notice that Listed Personnel becomes an Ineligible Person, then Service Provider agrees to promptly notify Aegerion and to immediately remove such Personnel from any further responsibilities for, or involvement with, providing services to Aegerion.

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16.5 Records; Inspections; Requests for Information. Service Provider agrees to keep business records, including documents relating to (a) goods and services provided to Manufacturer under the Agreement and (b) compliance with the requirements of the fourth amendment to this Agreement, until six (6) years from the expiration or termination of the Agreement. Upon the expiration of such six (6) years, Service Provider will transfer such documents and records to Manufacturer if requested by Manufacturer at Manufacturer’s expense. Service Provider agrees to permit Manufacturer, or any third party person(s) designated by Manufacturer (including, but not limited to, Manufacturer’s CIA Independent Review Organization, Manufacturer’s Consent Decree Independent Auditor and Manufacturer Quality Assurance) to inspect such records according to the terms of ATTACHMENT – AUDIT to the Agreement. When Manufacturer is required to obtain information from Service Provider outside the annual audit, Service Provider agrees to respond promptly to any such requests for information from Manufacturer or any third party person(s) designated by Manufacturer (including, but not limited to, Manufacturer’s CIA Independent Review Organization, Manufacturer’s Consent Decree Independent Auditor and Manufacturer Quality Assurance). Such requests outside of the annual audit, unless they are for cause due to Service Provider’s fault, will be subject to a fee of $250 per hour.”

16. Schedule 16 to this Amendment is hereby attached to and incorporated into the Agreement as Schedule 16.

17. Paragraph 1 of “ATTACHMENT – AUDIT” to the Agreement is hereby deleted in its entirety and replaced with the following:

“1. maintain during the term of this Agreement and for the period of six years thereafter sufficient documentation necessary to demonstrate compliance with the requirements of this Agreement and provide for review to Aegerion during an on-site audit, policies, procedures, and records used or produced by Service Provider in relation to its performance of its obligations under this Agreement, and provide upon request to Aegerion, the FDA, or a third party acting on behalf of Aegerion or FDA documentation that all processes and procedures are in place and are being followed for any applicable Risk Evaluation Mitigation Strategy where required, which may include joint review during a WebEx session, for example to validate that changes have been made to a Product-specific SOP prior to implementation. The parties agree not to take screen shots of such WebEx sessions;”

18. Paragraph 2 of “ATTACHMENT – AUDIT” to the Agreement is hereby deleted in its entirety and replaced with the following:

“2. allow Aegerion (or its designee), upon (i) [***] written notice or (ii) for initial audits of new specialty pharmacies, [***] written notice, accompanied by a detailed scope, during the term of this Agreement and for the period of [***] thereafter, to audit Service Provider’s and Service Provider’s agents’ and subcontractors’ documents, books, records, procedures, systems and facilities and conduct interviews with people under their control. Aegerion may conduct such audits (i) without cause not more frequently than once per calendar year, (ii) for cause (including, without limitation, audits based on Aegerion’s reasonable belief that a violation has occurred, audits related to quality control standards, and audits to confirm resolution of adverse

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findings from a prior audit) at Aegerion’s reasonable discretion, and (iii) to assess compliance with requirements related to any applicable Risk Evaluation Mitigation Strategy (REMS) at Aegerion’s reasonable discretion. If a third party is used to conduct any audit, such third-party with sign a confidentiality agreement with the audited party. Each such audit shall be subject to, and wholly covered by, the mutual confidentiality protections of this Agreement;”

19. Paragraph 7 of “ATTACHMENT – AUDIT” is hereby deleted in its entirety and replaced with the following:

“notify Aegerion promptly (but in no event later than within [***]) after Service Provider’s receipt of notice of a proposed inspection by a government or regulatory authority that relates directly to this Agreement or the transactions contemplated by it and allow Aegerion on site for such on-site inspection that relates directly to any Product; and”

20. This Amendment may be executed in two or more counterparts, each of which shall be deemed an original and all of which shall together be deemed to constitute one agreement. The parties agree that execution of this Amendment by exchanging facsimile or PDF signatures shall have the same legal force and effect as the exchange of original signatures.

21. This Amendment, together with the Agreement, constitutes the entire agreement between the parties with respect to the subject matter of the Agreement. The Agreement together with this Amendment supersedes all prior agreements, whether written or oral, with respect to the subject matter of the Agreement. The parties hereby agree that subject to the modifications specifically stated in this Amendment, all terms and conditions of the Agreement shall remain in full force and effect.

IN WITNESS WHEREOF, the parties have caused this Fourth Amendment to be executed by their duly authorized representatives as of the Amendment Effective Date.

Accredo Health Group, Inc. Aegerion Pharmaceuticals, Inc.

By: /s/ Elizabeth Newport By: /s/ Joseph Shulman 

Name: Elizabeth Newport Name: Joseph Shulman

Title: VP Title:  Senior Vice President, Global Technical Operations

Date: 10/23/17 Date: 19 Oct 2017

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Attachment 1

Business Partner Guiding Principles

   Introduction

At Aegerion, we have an important mission – the development and commercialization of innovative therapeutics to treat debilitating and rare diseases. In working to achieve this goal, we set high standards for our own employees as well as for all who work on our behalf. Aegerion’s Business Partner Guiding Principles is based on our belief that working with business partners who follow ethical business practices is essential to our long-term success.

   Expectations of Our Business Partners

● Conduct business in an ethical and transparent manner that is compliant with applicable laws, regulations and industry codes.

● Develop and implement policies, systems and procedures that are consistent with these Business Partner Guiding Principles.

● Provide routine compliance training to your employees relevant to their job function.

● Maintain adequate compliance training records.

● Designate a person who is responsible for compliance in your organization.

● Establish a process for employees to report suspected wrongdoing and to publicize the process to your employees.

   Ethical Approach to Business

At a minimum, each Business Partner must conduct business in accordance with the following:

Anti-Bribery/Anti-Corruption

● You must not offer or make any bribe or improper payment or improper transfers of value either directly or indirectly, whether in cash or in kind, to secure any kind of business advantage for Aegerion.

● You must not encourage or allow others to offer or make improper payments.

Privacy/Data Protection

● Appropriately, and in compliance with all applicable laws and regulations, collect, use, and store personal information/data to ensure that privacy rights are protected for, among others, employees, healthcare professionals (HCPs) and patients.

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Product and Product-Related Materials

● Each Business Partner must comply with applicable laws and regulations related to the provision of information to HCPs, Government Officials (GOs), Government Entities (GEs), hospitals, payors and patients (if allowed and as required under local law/codes) about Aegerion’s products and Aegerion’s disease states of interest. This includes knowing when Aegerion’s products can be promoted and what constitutes promotion.

● Materials to be Used with HCPs, GOs, payors and patients (if permitted):

○ Materials must have been approved or provided by Aegerion.

○ Any materials used must be accurate and balanced; in addition, promotional materials must always be consistent with the local market authorization/label.

Interactions with Healthcare Professionals and Government Officials

Each Business Partner must be able to demonstrate that, in its interaction with third parties (including HCPs) on behalf of Aegerion, it has developed and implemented appropriate standards and policies to maintain compliance with applicable laws, regulations and industry codes.

Interactions may include the engagement of HCPs or GOs in a professional capacity for their expertise in particular areas that are relevant to Aegerion products or research.

● Prohibition of Inducements

○ Never offer, promise or provide inducements.

○ Inducements include payments or other items of value with the intent of, directly or indirectly, encouraging or influencing a decision, or influencing or encouraging the recipient to purchase, prescribe or recommend an Aegerion product.

● Legitimate Business Need and Written Agreement

○ Engage HCP/GOs to provide services only when there is a legitimate, documented business need and there is a written agreement in place.

● HCP/GO Selection Criteria

○ Base the selection of HCP/GOs as service providers on their qualifications (e.g. skills and expertise) to perform the required task.

○ Engage only the minimum number of HCP/GOs necessary to satisfy the legitimate business need.

● Fair Market Value

○ Ensure that payments are reasonable, based on prevailing rates of the HCP/GO’s home country, and only for bona fide services actually provided.

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● Disclosure of Payments and Transfers of Value

○ Payments and other transfers of value, including grants and donations, must be accurately recorded and reported to applicable governmental or industry agencies, as and when required by law or country code.

● Educational Grants:

○ Do not give Educational Grants to individual HCPs.

○ Never give a grant to encourage or influence a decision, or influence or encourage the recipient to purchase or prescribe an Aegerion product.

○ Award grants only to entities that have a legitimate right to receive Educational Grants, as determined by local tax law.

○ Establish a Grant review process that is void of commercial influence.

● Gifts, Hospitality and Travel

○ Ensure that hospitality and travel are only provided in conjunction with a legitimate business activity, and are not extravagant.

○ Select venues for business activities that are modest by local standards and conducive for business activities.

○ Follow local industry codes regarding gifts to HCP/GOs, but in all instances the gifts must be nominal in value and not for the personal benefit of the HCP/GO.

● No Political Donations

○ Do not provide political donations to government officials in connection with any activities related to Aegerion.

● Fair Competition

○ Business should be conducted consistent with fair and vigorous competition and in compliance with all applicable anti-trust laws.

Interactions with Patient Organizations and Patients

● Do not promote to Patient Organizations or patients.

● You should always respect the independence of a Patient Organization; do not use the organization to promote or recommend Aegerion products.

   Implementation

We expect our Business Partners to read and take reasonable steps to ensure its activities comply with the Aegerion Business Partner Guiding Principles and to take appropriate action in the event that a potential violation is discovered. These steps may include:

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● Developing, implementing and providing routine training on policies and procedures appropriate for the Business Partner’s business activities.

● Communicating to your employees Aegerion’s expectations of compliance with these Business Partner Guiding Principles.

● Developing and implementing appropriate business mechanisms to monitor for compliance with these Business Partner Guiding Principles.

It is each Business Partner’s responsibility to obtain its own legal counsel and its own legal advice, as required.

/ have read, understand and agree to comply with Aegerion’s Business Partner Guiding Principles.

Business Partner Legal Entity Name: Click here to enter text.

Name of Authorized Representative: Click here to enter text.

Signature:

Date: Click here to enter text. 

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ATTACHMENT – PERSONAL DATA

ADDITIONAL TERMS AND CONDITIONS FOR THE COLLECTION, USE, DISCLOSURE,
RETENTION OR
PROCESSING OF PERSONAL DATA

With regard to the Agreement, Service Provider may gain access to “Personal Data,” which shall mean any information which identifies or is capable of identifying a living individual, or as otherwise defined as Personal Data” by applicable laws, including, without limitation, (a) an individual’s name, address, phone number, e-mail address, Social Security number or other country identifier, driver’s license number, bank account information, or credit card information; and (b) all information, data and materials, including without limitation, demographic, medical and financial information, that relate to (i) the past, present, or future physical or mental health or condition of an individual; (ii) the provision of health care to an individual; or (iii) the past, present, or future payment for the provision of health care to an individual.

Service Provider agrees that, in the event any of the Services that Service Provider provides to AEGERION related to Personal Data are subcontracted out to a third-party subcontractor, Service Provider shall execute a written agreement with said third-party subcontractor that requires it to comply with privacy, security, and breach reporting requirements that are substantially similar to those set forth in this Attachment and to comply with all applicable laws.

Collection, use, disclosure, retention and other processing of Personal Data may be regulated by certain privacy, data security, and breach notification laws.

When collecting, using, disclosing, retaining or otherwise processing Personal Data pursuant to the Agreement, Service Provider agrees to comply with all applicable privacy, data security, and breach notification laws, rules and regulations that are applicable to its collection, use, disclosure or processing of Personal Data pursuant to the Agreement. For purposes of this Attachment, applicable privacy, data security, and breach notification laws, rules, and regulations include, but are not limited to, any law related to the transmission, communication or storage of Personal Data via mail, telephone, computer, wireless technology, facsimile, or other such means.

Service Provider acknowledges and agrees that, as required and as appropriate under applicable laws, Service Provider will furnish notices to and/or obtain the prior and freely given, specific and informed consents and/or authorizations of the individuals to whom the Personal Data relate to collect, use, disclose, retain or otherwise process such data in order to provide the Services.

Without limiting the foregoing and in addition to any other obligations under the Agreement, Service Provider further agrees as follows:

a.       Service Provider agrees to collect, use, disclose, retain and otherwise process Personal Data only as permissible under applicable laws;

b.       Access to data: only authorized persons should have access to the Personal Data; Service Provider shall restrict such access in accordance with applicable laws and its privacy policies.

Any and all consents or authorizations collected by Service Provider from individuals relating to collection, use, disclosure, retention and other processing of the individual’s Personal Data must be retained by Service Provider in compliance with applicable data retention policies. Service Provider agrees to establish commercially adequate controls to prevent unauthorized access, use or disclosure of Personal Data. Service Provider will implement safeguards that reasonably and appropriately protect the confidentiality, integrity, and security of Personal Data in accordance with applicable

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laws. Service Provider agrees AEGERION and/or its representatives may audit Service Provider’s business policies, processes, books, records, and practices relating to collection, use, disclosure, retention and other processing of Personal Data in accordance with “ATTACHMENT – AUDIT” to this Agreement.

Duty of cooperation with law enforcement authorities: Parties agree to cooperate with law enforcement authorities, especially in case of requests for information or during inspections.

Any collection, use, disclosure, retention and other handling of Personal Data other than as contemplated by this Attachment, permissible by law, and the Agreement will be deemed a material breach of the Agreement and subject to the applicable provisions of the Agreement related to a material breach; and

Service Provider agrees to work in good faith to mutually amend this Attachment at any time as requested by AEGERION to the extent required in order to comply with any applicable law relating to privacy, data security, and breach notification in those respective jurisdictions where Service Provider provides services and/or collect, use, disclose or otherwise process Personal Data pursuant to the Agreement.

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EXHIBIT E

Adverse Event Reporting, Product Complaint Reporting, and Medical Information Handling

I. Adverse Event (AE) Reporting

Service Provider (Accredo) shall report to Aegerion Pharmacovigilance (PV) vendor (United BioSource Corporation [UBC]) within one (1) business day after first becoming aware of any potential adverse event (AE). “Adverse Event” means any untoward event associated with the use of an Aegerion product, whether or not considered drug related, including events occurring in the normal course of use of a prescription drug; any drug overdose (whether accidental or intentional); any drug abuse, withdrawal, or lack of expected effect of the drug. AE also includes: drug exposure via a parent (during conception, pregnancy, breastfeeding, etc.), misuse, dependence, unintended beneficial effects, medication errors, off-label use* (all with or without an adverse drug experience), and suspected transmission of infectious agent via a medicinal product, occupational exposure, and drug-drug interactions.

The following table lists examples of additional events that should be documented and transferred in addition to AE (this table is not an all-inclusive list):

Additional Events	Clarification and/or Example
Abnormal test findings
Drug Abuse	Persistent or sporadic, intentional excessive use of medicinal products which is accompanied by harmful physical or psychological effects.
Lactation exposure (to infant)	Defined as the exposure of an infant/child to a medicinal product during breast-feeding/lactation.
Hospitalization
Lack of compliance	Any information suggesting the patient does not take the prescribed medication or follow a prescribed course of the treatment, such as failure of proper preparation, or using the correct dose, frequency, or route
Lack of efficacy/effect	Any information that questions the efficacy or therapeutic effect of a product, for example the product isn’t working as indicated in the product labelling (i.e. for licensed indications only).
Medication error	“medication error”- where the patient used the product “potential medication error”-patient noticed before the product was used A potential medication error is any unintentional error in the

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	prescribing, dispensing, or administration of a medicinal product while in the control of the healthcare professional, patient or consumer that was identified before the drug was administered to the patient or before the patient ingested any drug.
Missed dose	E.g., patient forgot, supply ran out
Misuse	Situations where the medicinal product is intentionally and inappropriately used not in accordance with the authorized product information.
Off-label use*	Situations where a medicinal product is intentionally used for a medical purpose not in accordance with the authorized product information (including an indication, dose, administration method, patient group, storage condition or legal status of supply) not on the license or against a contraindication.
Overdose	Occurs when a patient takes more than the prescribed amount. The product is ingested or administered in quantities and/or concentrations above the recommended/scheduled dosage large enough to overwhelm the homeostasis, disrupting wellbeing, causing severe illness and/or death.
Occupational Exposure	Drug exposure as a result of one’s occupation, with or without associated symptoms/event.
Pregnancy Exposure (to fetus)	Defined as an exposure of the male or female parent to a medicinal product at or around the time of conception (up to 3 months before conception) or at any time during pregnancy until birth and exposure of the fetus to a medicinal product (e.g., breastfeeding). Both intentional and unintentional exposures will be reported.
Prescribed/Accidental overdose	more than on license/packaging
Transmission, or suspected transmission, of an infectious agent
Unexpected benefit
Use of a product against a Contraindication

* The Juxtapid and Myalept REMS programs require the product to be dispensed only to patients who have a confirmed diagnosis of the indicated condition. “Off-label use” includes any reports of use for an ICD-10 code inconsistent with the approved indication for such product or reports that include information that is indicative with use for an indication other than the one that is approved. The

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Aegerion Compliance Department (novelioncompliance.tnwreports.com or 855-233-8089) must be notified within one (1) business day of receipt of all potential reports of off-label use.

Accredo shall capture the following information for each AE and report it to UBC on the Accredo Adverse Event Form attached to the Agreement as Exhibit A: (a) date information/complaint received, (b) patient identifiers, (c) reporter name and contact information, (d) date AE occurred, (e) suspect product name, NDC (when available), lot number (when available), dose, date of administration (when available), and (f) description of the AE. Accredo shall maintain records of all AE reports on source documentation or entered into an Accredo system for at least three years and shall make all such records available to Aegerion upon request within two (2) business days’ notice. In the event of a regulatory inspection, Aegerion has the right to request copies of records to be delivered to it within one (1) business day.

Accredo is responsible for:

● Identifying and recording details of any AEs and forwarding it to UBC PV for processing.

Ensuring that if the AE also involves a Product Quality Complaint (PC), the PC will also be reported

separately to Aegerion QA ([***]).

Accredo is not responsible for:

● Reporting an AE to the Regulatory Authorities.

● Proactively obtaining follow-up information on an AE.

● Reporting AEs from Aegerion sponsored clinical trials.

○ If any are inadvertently reported to Accredo, Accredo will obtain any details provided by the caller including the Clinical Trial number and forward back to UBC ([***]) to handle.

● Reporting AEs from the published literature, for example, from clinical papers, journal letters, newspapers, social media, etc.

A. Transmission and Reporting times of AE cases including any combination AE and Product Complaint (PC) cases to Aegerion Pharmacovigilance (PV)

i. Transmission of Reports

● All AEs will be posted to the UBC Aegerion portal.

○ An Accredo representative will post the AE form to the UBC Aegerion portal within the Accredo library.

○ Accredo will set-up from the portal automatic alerts to get the confirmation that the AE reports has correctly been posted to this portal. In case no alert would be received, the Accredo representative will investigate and send the AE documentation by email to UBC ([***]).

○ UBC Safety Scientist or designee will acknowledge by email to [***] the receipt of the AE report.

● Note: If a PC accompanies an AE, an AE and PC will each be reported. Refer to Product Complaint Reporting section A, ii.

● In the event that the UBC Aegerion portal is down, reports would be transmitted via email or e-fax to [***].

● In the event an AE or PC report is indicative a potential off label use of the product, Accredo will advise Aegerion Compliance of the report identifying number within one (1) business day.

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Execution Version

ii. Timelines

● For Accredo, all AE reports will be transferred to the UBC Aegerion portal within one (1) business day Monday to Friday.

○ AEs received by Accredo during non-business hours or holidays will be processed within the next business day.

● Acknowledgments of receipt will be received from the portal automatic alerts by the UBC representative following the receipt of the AE report.

iii. Reconciliation

● An Accredo representative will generate a weekly AE reconciliation report including a list of AE reports received for the week and post to the UBC Aegerion portal.

■ UBC Safety Scientist will set-up from the portal an automatic alert to get this list of AE reports from Accredo as soon as it is posted to the portal

● UBC Safety Scientist or designee will acknowledge by email to [***] the receipt of the weekly reconciliation report. A confirmation that reconciliation has been performed by UBC will be sent to [***] including identified discrepancies if any within two (2) business days.

II. Product Quality Complaint (“PC”)

Product Complaint – Any communication, written or oral, that alleges deficiencies relative to the identity, quality, stability, reliability, effectiveness or performance of a product after it is released for distribution. A product complaint includes any communication which alleges deficiencies relative to the packaging, labeling, immediate container, closure or contents of the drug, and/or any allegation of poor pharmaceutical quality such as chipped, split or otherwise faulty drug product capsule or drug product vial, aesthetic defects (off-color, off-shape, inconsistent surface texture, and off-flavor), or report of drug sub-potency or super-potency. “Sub-potency” only includes drug product lack of effect (including decreased, incomplete or delayed drug effect) reported at the established therapeutic dose.

Accredo shall also report to Aegerion Quality Assurance (QA) ([***]) within one (1) business day after first becoming aware of any PC.

A. PROCEDURE FOR INITIAL INTAKE OF PRODUCT COMPLAINT

i. PC Reporting Responsibilities

● Accredo is responsible for initial documentation of PCs and forwarding this information to Aegerion QA through the email address [***] for further follow up and evaluation.

● Accredo ensures that if a PC also involves an AE or special situation, this is also reported separately to Aegerion PV (see Section I.A.) and Aegerion QA is copied simultaneously on the Accredo AE form.

ii. PC Reporting Information to Obtain

As per the table below, the following information will be attempted to be obtained for the PC report.

Reporting Information to Obtain
Reporter	●Reporter name

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Execution Version

	●Telephone number
Product	●Product name
	●NDC
	●Lot number
	●Expiration date
	●Strength
	●Pack size
	●Quantity of product affected
	●Available for return to Aegerion?
	●Has the product been used in a patient?
Complaint	A detailed description of the PC

iii. Transmission and Reporting Times of PC cases to Aegerion

● All PCs are e-mailed to Aegerion QA ([***]) within one (1) business day.

● Accredo receives acknowledgement of receipt of case by Aegerion QA within 24 hours of receipt.

iv. Restitution

● Restitution of PCs will be handled by Aegerion QA.

v. Reconciliation

● Accredo sends a weekly reconciliation report to [***] which includes a list of the previous weeks PCs with the information set forth in the table below.

● Aegerion will acknowledge by email to Accredo ([***]) the receipt of the weekly reconciliation report.

● A confirmation that reconciliation has been performed by Aegerion QA is sent to Accredo including identified discrepancies if any, within two (2) business days.

● Any discrepancies noted by Aegerion QA is followed up with Accredo within one (1) business day and any missed PC report is sent immediately to Aegerion.

Field #	Data Element	Max Length	Required (Yes, No, If Available)	Notes
1	Drug Name	10	Yes
2	PC Date Reported to MFR	10	Yes	Date Format: MMDDYYYY
3	Unencrypted Patient ID	15	Yes	Not Encrypted
4	Patient Date of Birth	8	Yes	Not Encrypted

III. Training

Accredo shall ensure that its employees, subcontractors and agents who perform services under this Agreement or any associated Statement of Work, are trained in AE reporting and PC reporting before

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Execution Version

such employees, subcontractors or agents commence services. Accredo shall ensure that all such employees, subcontractors and agents are re-trained on an annual basis and shall maintain contemporaneous records of such training for all persons providing services hereunder or under an associated Statement of Work and shall send records to Aegerion at the time of completion and within two business days’ notice when requested (or sooner if requested for an inspection or audit). Aegerion point of contact for Accredo shall provide training materials, as needed.

Per the modification to the Juxtapid REMS Program approved by FDA on January 3, 2017, and subsequent revisions thereto, Accredo will designate an Authorized Representative for each pharmacy who must complete the Juxtapid online Training Module and complete the Pharmacist Enrollment Form (RXEF). The Training Certificate and the RXEF must be submitted to the Juxtapid REMS Coordinating Center by fax [***] or email [***] prior to the pharmacy certification and the dispensing of Juxtapid by the pharmacy. Pharmacy certification will be confirmed in writing by the Juxtapid REMS Program.

The Authorized Pharmacist for each Juxtapid dispensing pharmacy is responsible for ensuring training to the requirements of the REMS Supplement are completed by relevant pharmacy staff. The on-line module should be used for training of all of the pharmacy staff.

Accredo is required to verify every 12 months that the authorized representative’s name and contact information corresponds to that of the current designated authorized representative for DLSS, if different the pharmacy must be required to re-certify with a new authorized representative in a timely manner, and in no event later than thirty (30) calendar days after Accredo’s first knowledge thereof. The dispensing of Juxtapid by a pharmacy that is not certified in the Juxtapid REMS Program will be classified as a violation of the Juxtapid REMS Program requirements and may trigger corrective action or additional actions by Aegerion.

IV. Medical Information for Health Care Providers (HCPs) or Consumers not enrolled in the Accredo Patient Support Services Programs

A. Function

Medical Information is responsible to provide responses to unsolicited medical inquiries involving Aegerion products. Medical inquiries from HCPs that are not related to reimbursement or prior authorization needs should be transferred to the Medical Information Call Center (MICC). In addition, consumers who do not wish to enroll in the Product Patient Support Services program and have questions concerning Aegerion products can also be transferred to the MICC.

B. Medical Information Contact Information

i.	Medical Information: [***] Monday through Friday 8 am to 5:30 pm EST or [***].
ii.	Live caller:

1. During normal business hours: Provide phone number [***] and warm transfer caller. Provide contact details obtained to the MICC agent when call is connected.

2. After hours: provide phone number [***] and inform of business hours.

a. An after-hours call service is available for urgent inquiries only.

iii. Written request: Any requests for information received via email or in writing shall be transferred to [***].

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Execution Version

iv. Any exchange of adverse event (AE) or product complaint (PC) details prior to the transfer of the live call or written request shall be handled according to AE and PC reporting procedures.

1. Note in the AE or PC report that the case has been transferred to MICC to indicate a potential duplicate case.

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EX-10.13

Exhibit 10.13

AMRYT PHARMA PLC

EMPLOYEE SHARE OPTION PLAN 2019

Adopted on 25 September 2019

AMRYT PHARMA PLC

EMPLOYEE SHARE OPTION PLAN 2019

Rules

Definitions

1. The Plan is established by an ordinary resolution of the Shareholders passed on September 25^th 2019. The purpose of the Plan is to provide for the granting of share options to Directors and Employees of and Consultants to the Company and its subsidiary and associated companies in accordance with the provisions hereinafter contained.

2. In the Plan the following expressions bear the following meanings:-

(a)	"Associated Company"	means a company under the Control of the Company or any Subsidiary of the Company or any combination thereof or in which the Company and/or its Subsidiaries have a shareholding interest of 20% or greater;
(b)	"the Board"	means the Board of Directors for the time being of the Company or a duly constituted committee of the board of directors;
(c)	"the Company"	means Amryt Pharma Plc, a company registered in England and Wales under number 12107859
(d)	"Consultant"	means any individual or company who has a consultancy agreement with the Company or a Participating Company;
(e)	"Control"	has the same meaning as in Section 840 of Income and Corporation Taxes Act 1988;
(f)	“Date of Grant”	means the date on which an Option is granted as determined by the Board and specified in the Option Certificate;
(g)	"Directors"	means in relation to the Company or a Participating Company its board of directors and “Director” shall be construed accordingly and shall include non-executive members of any such board of directors;
(h)	"Employee"	means an employee of the Company or a Participating Company (other than one who is a Director of the Company or of a Participating Company);
(i)	"Final Option Date"	means in relation to an Option the last date upon which any part thereof may be exercised under Clause 7, which date shall be determined by the Board and specified in the Option Certificate, but in no event shall be later than the date preceding the tenth anniversary of the Date of Grant;

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(j)	“Market Value"	means the price which in the opinion of the Board represents the fair market value of a Share, having regard in circumstances where the Shares are traded on the Markets, to the prices prevailing in the Markets;
(k)	“Markets”	the AIM market operated by the London Stock Exchange plc and any recognised investment exchange (as defined by section 285 of the Financial Services and Markets Act 2000) the company is or will be listed on including for these purposes the National Association of Security Dealers Automated Quotation Market;
(l)	"Nominated Person"	means a person who shall have been nominated for the purpose of the Plan pursuant to Clause 3(a);
(m)	Notice of Exercise”	means a notice of exercise of an Option in the form set out in Schedule 2 to the Plan or such other form approved by the Board;
(n)	"Option"	means an option granted pursuant to the Plan;
(o)	"Option Certificate"	means a certificate executed under the seal of the Company evidencing the grant of an Option, in the form set out in Schedule 1 to the Plan or such other form approved by the Board;
(p)	"Option Price"	means the price at which a Share must be subscribed on exercise of an Option;
(q)	"Participant"	means any Nominated Person (or, in the event of his death, his personal representative) who is for the time being the holder of an Option;
(r)	"Participating Company"	means any company being the Company or an Associated Company to whom the Board has extended the Plan;
(s)	"the Plan"	means the Amryt Pharma Plc Employee Share Option Plan 2019, consisting of these rules, as amended from time to time in accordance with the provisions in that regard herein contained;
(t)	“Remuneration Committee”	means the committee of non-executive directors of the Board responsible for assessing and making recommendations concerning the remuneration of executive directors and senior employees of the Company and its Associated Companies;
(u)	"Shares"	means the Ordinary Shares of £0.06 each in the capital for the time being of the Company; and
(v)	“Subsidiary”	has the meaning assigned in Section 1159 of the Companies Act 2006;

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(w) “Tax Liability” the total of all and any tax, including income and employee NIC, (or their equivalents in any jurisdiction) for which any Participating Company is or may be liable to account.

The Rule headings are for ease of reference only and shall not in any way affect the interpretation hereof.

Eligibility for Participation

3. (a) Power of Board

The Plan is available for Directors, Employees or Consultants who shall be nominated for the purpose by the Board.

(b) Absolute Discretion

The Board shall at its absolute discretion determine whether or not a person is a Director, Employee or Consultant.

(c) No Right

No person shall be entitled as of right to participate in the Plan and the decision as to who shall have the opportunity of participating and the extent of his participation will, subject to the Plan, be made by the Board at its absolute discretion.

LIMITS

4. (a) Ten Year Limit

No Option shall be granted under the Plan on a date later than ten years after the date of adoption of the Plan by the members of the Company.

(b) Overall Limits for Plan at Adoption Date

Subject to Rules 13 and 14, the maximum number of Shares over which Options may be in issue at any one time under the Plan shall be 10% of the issued share capital in the Company.

5. (a) Power to Grant

Subject to the provisions of Rule 21(a), the Board may on behalf of the Company grant Options to Nominated Persons at any time or times within ten years of the date of adoption of this Plan by the members of the Company.

(b) Non-Assignable

Options shall be personal to the grantee and non-assignable, subject to Rule 8, unless the Board in its sole discretion consents to an assignment or transfer. Any purported transfer, assignment, mortgage or charge of an Option, without the consent in writing of the Board, shall cause the Option to lapse forthwith.

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(c) Certificates

An Option Certificate shall be issued to a Participant in respect of each Option.

(d) Right to Renounce

An individual to whom an Option has been granted may, by notice in writing within 30 days after receipt of the Option Certificate, renounce such Option, in which event it shall be deemed never to have been granted.

OPTION PRICE

6. The Option Price in relation to an Option shall be determined by the Board but shall not be less than the nominal value of a Share. If the option is to be granted at Market Value the Market Value per Share of the Shares comprised in the Option is to be computed as at the day prior to the Date of Grant.

PERIODS FOR EXERCISE OF OPTIONS

7. (a) Subject to Rules 7(b), 8, 9, 10 and 16, an Option may be exercised at any time or times prior to the Final Option Date. An Option shall expire immediately after the Final Option Date to the extent that it has not been exercised.

(b) The Board may when it grants an Option at its entire discretion attach a condition thereto such that the Option or portion(s) thereof may not be exercised until a period(s) (not exceeding four years) has elapsed from the Date of Grant. Such vesting condition(s) will be set out in the Option Certificate.

(c) The Board may when it grants an Option at its entire discretion attach a condition relating to future performance by the Director, Employee or Consultant such that the Option or portion(s) thereof may not be exercised until such conditions have been met. Such conditions will be set out in the Option certificate.

DEATH AND TRANSFER OF RIGHTS

8. (a) In the event of the death of a Participant before the Final Option Date the personal representative of such deceased Participant may, at any time and from time to time but no later than one year after the date of such death (or, if earlier, the Final Option Date), exercise the Option, to the extent exercisable on the date of the Participant’s death, in whole or in part.  Upon the expiration of such period the Option shall lapse to the extent that it shall not have been so exercised.  

(b) To the extent an Option is not exercisable on the date of a Participant’s death, the Option shall lapse on such date unless the Board in its sole discretion determines that such Option shall be exercisable in whole or in part after such date and if the Board does so determine, the Option shall be exercisable in accordance with Rule 8(a).

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RETIREMENT, RESIGNATION ETC.

9. (a) If a Participant ceases to be an Employee or Director or Consultant on account of:-

(i) retirement at normal retirement age; or

(ii) resignation or early retirement due to disability or ill-health (such matter to be determined by the Board in its absolute discretion);

the Participant may at any time and from time to time but no later than one year after he shall have so resigned or retired (or, if earlier, the Final Option Date), exercise the Option, to the extent exercisable on the date of such resignation or retirement, in whole or in part. Upon the expiration of such period the Option shall lapse to the extent that it shall not have been so exercised.

(b) To the extent an Option is not exercisable as of the date of a Participant’s resignation or retirement as described in Rule 9(a), the Option shall lapse on such date unless the Board in its sole discretion determines that such Option shall be exercisable in whole or in part after such date and if the Board does so determine, the Option shall be exercisable in accordance with Rule 9(a).

(c) If a Participant ceases on account of resignation, retirement, dismissal (subject to the provisions of Rule 9(d) or otherwise (except on death, retirement or resignation or early retirement due to disability or ill-health) to be an Employee or Director or Consultant, each Option held by the Participant, to the extent not exercisable at the date of such cessation, shall lapse on such date. To the extent an Option is exercisable at the date of such cessation, it may be exercised by the Participant in whole or in part within 90 days after such date (or, if earlier, until the Final Option Date), failing which it will lapse. Cessation shall be on completion of the appropriate notice period required from either the employee or Participating Company employing or engaging him to terminate the employment.

(d) If a Participant’s employment or office or consultancy is terminated summarily for serious misconduct by the Participating Company employing or engaging him, each Option held by the Participant shall lapse in full immediately upon such termination.

(e) Notwithstanding the foregoing provisions, the Board in its sole discretion may determine that if circumstances so warrant, an Option may be exercised after the Participant ceases to be an Employee, Director or Consultant during a longer period than the period provided under the foregoing provisions and/or that an Option, to the extent not exercisable on the date a Participant ceases to be an Employee, Director of Consultant, shall be exercisable in full or in part after such cessation and may be exercised within a period specified by the Board, but in no event may an Option be exercised later than the Final Option Date and an Option will lapse to the extent not exercised within the period specified by the Board.

(f) In no circumstances shall any Participant who ceases to serve as an Employee or Director or Consultant be entitled to any compensation for any loss of any right or

6

benefit or prospective right or benefit under the Plan which he might otherwise have enjoyed whether such compensation is claimed by way of damages for wrongful dismissal or other breach of contract or by way of compensation for loss of office or otherwise howsoever.

MERGER OR TAKEOVER

10. In the event that the Company is a party to a merger, takeover or other reorganisation, including but not limited to a court sanctioned compromise or scheme arrangement, pursuant to which a party or parties acting in concert obtain(s) Control of the Company, or the Board considers that this is about to occur, or notice is given of a resolution for the voluntary winding-up of the Company, each Option shall automatically accelerate and become exercisable in full as of a date specified by the Board, conditional upon such merger, takeover or other reorganisation or winding-up, and the Board shall, at its discretion, with respect to each Option:

(i) request the Participant to exercise the Option within such period and subject to such conditions as the Board may at its discretion determine and if the Participant does not comply with such request the Option shall lapse on a date specified by the Board; or

(ii) arrange for payment of a cash settlement to the Participant, in cancellation of the Option, equal per Share subject to the cancelled Option to the excess of the amount to be paid for a Share in the merger or takeover or reorganisation or winding up over the Option Price.

The Remuneration Committee may make such other comparable arrangements to replace any Options (whether exercisable or not) as it determines in its discretion.

For the avoidance of doubt, a reverse takeover by Amryt of another company will not result in an acceleration of share option vesting.

PROCEDURES ON EXERCISE OF OPTIONS

11. Upon the exercise of an Option in whole or in part the Participant shall deliver a Notice of Exercise and pay the Option Price in respect of the Shares for which the Option is being exercised to the Company, in cash or by cheque or by same-day sale exercise through a broker designated by the Company, or by any other means or arrangements reasonably approved by the Board, and shall deliver the Option Certificate to the Company and the Company shall issue the appropriate Shares to the Participant and deliver to the Participant any appropriate balance Option Certificate.

12. All Shares issued on any exercise of an Option shall rank pari passu in all respects with the Shares already in issue.

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TAX LIABILITIES

13. Each participant is responsible for all Tax Liability and will pay or enter into arrangements with the relevant Participating Company to pay all and any Tax Liability, failing which the Company shall not issue or transfer shares to the Participant on exercise of his Options.

14. If requested by the Participating Company, the Participant shall enter into a joint election under section 431(1) or 431(2) of the Income Tax (Earnings and Pensions) Act 2003 or other relevant legislation in respect of shares to be acquired on exercise of an Option.

BONUS, RIGHTS ISSUES, VARIATION IN SHARE CAPITAL

15. If a consolidation or subdivision of a reduction of the share capital of the Company or if any other variation in the share capital of the Company occurs, the Board may make such adjustment to the Option Price and/or the number and/or class of Shares subject each Option and to the share limit set out in Rule 4(b) as it deems appropriate.

16. If holders of Shares are granted rights to subscribe for further shares (such rights being related to the number of Shares held by them respectively) the Board shall at its absolute discretion decide whether the granting of such rights and the subscriptions made thereunder shall result in the depletion in the value of each Share and the Board may make such adjustment(s) to the Option Price and/or the number and/or class of Shares subject each Option and to the share limit set out in Rule 4(b) as it deems appropriate.

NO SHARE ISSUES AT A DISCOUNT

17. Notwithstanding anything herein contained no Option shall be granted to subscribe for any Shares at a discount to the nominal value of the Shares.

LIQUIDATION

18. In the event of a liquidator being appointed to the Company all Options shall ipso facto cease to be exercisable and (save to the extent, if at all, that the Board may prior to such liquidation at its absolute discretion determine) Participants shall not be entitled to damages or other compensation of any kind.

EXCHANGE QUOTATIONS

19. The Company will within five working days after the exercise of an Option apply for permission to deal in the Shares or securities of the Company (whichever one traded) issued pursuant to the exercise of Options on AIM or such other stock exchange upon which the Shares or securities are for the time being quoted.

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ALTERATIONS

20. The Company may at any time by resolution of the Board vary, amend or revoke any of the provisions of the Plan in such manner as it considers fit.

OBLIGATION TO KEEP UNISSUED CAPITAL

21. The Company shall take all necessary steps (including the passing of resolutions of the Company) to ensure that the directors of the Company shall, at all times, be generally and unconditionally authorised to allot Shares pursuant to Options to Nominated Persons.

TERMINATION

22. (a) The Plan may be terminated at any time by resolution of the Board.

(b) Subsequent to any termination of the Plan under paragraph (a) of this Rule 20 the Company shall not grant any further Options but no such termination shall affect or modify any subsisting rights or obligations of Participants in respect of any Options and notwithstanding such termination the Company shall continue to administer and manage the Plan in accordance with the rules of the Share Option Plan.

GENERAL

23 (a) If the Shares are listed on a stock exchange or securities market, the Company and each Participant shall be subject to such insider dealing policy as the Company may implement from time to time for its officers and employees imposing restrictions on transactions in the Shares during specified periods.

(b) In the event of any dispute or disagreement as to the interpretation of the Plan, or as to any question or right arising from or related to the Plan, the decision of the Board shall be final and binding upon all persons.

(c) Any notice or other communication under or in connection with an Option and/or the Plan may be given by personal delivery or by sending the same by prepaid post

(i) In the case of the Company to The Share Option Plan Administrator/ Company Secretary of the Company at its registered office;

(ii) In the case of a Nominated Person or Participant, to his last known address provided to the Company, or to the address of the place of business at which he performs the whole or substantially the whole of his duties of his employment or engagement; and

(iii) Where a notice or other communication is personally delivered, it shall be deemed to have been received at the time of delivery and where it is posted to an address within Ireland, it shall be deemed to have been received forty-eight (48) hours after it was put into the post properly addressed and stamped and where it is posted to an address outside Ireland, it shall be deemed to have been received on the fifth business day after

9

the date it was put into the post properly addressed and stamped. If a Participant is an employee and is not on extended leave from employment, notice to such Participant may be sent by email to the address at the Company or the Participating Company at which the Participant customarily receives email correspondence in connection with his employment and shall be deemed to have been received upon transmission.

(d) The Board shall be entitled to authorise any person to execute on behalf of a Participant, at the request of the Participant, any document relating to the Plan, insofar as such document is required to be executed pursuant thereto.

(e) By participating in the Plan, each Participant consents to the holding and processing of personal data relating to him by the Company or any Participating Company for all purposes relating to the operation of the Plan which purpose include, but are not limited to:

(i) administering and maintaining Participant records;

(ii) providing information to tax and regulatory authorities;

(iii) providing information to registrars, brokers and other third party administrators of the Plan; and

(iv) providing information, on a confidential basis, to potential purchasers of the Company or the business in which the Participant is employed.

(f) The Plan shall be governed by and construed and interpreted in accordance with English law and the Company and Participants agree to submit to the non-exclusive jurisdiction of the Courts of England in relation to any claim, dispute or difference which may arise hereunder.

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SCHEDULE 1

OPTION CERTIFICATE

AMRYT PHARMA PLC EMPLOYEE SHARE OPTION PLAN 2019

THIS DOCUMENT IS IMPORTANT

Name of Participant:

Address of Participant:

Date of Grant:

Number of Shares:

Option Price per Share:

Vesting Conditions:

Last Date on which Notice of Exercise of Option can be given (Final Option Date):

THIS IS TO CERTIFY that the Participant named above was on the above Date of Grant granted an option to subscribe for the above number of Ordinary Shares (“Shares”) in Amryt Pharma plc (the “Company”) at the above Option Price per Share. This Option may not be transferred, assigned, mortgaged or charged by the Participant and any purported transfer, assignment, mortgage or charge will cause this Option to lapse forthwith. This Option is exercisable subject to and in accordance with the terms and conditions of the Amryt Pharma plc Plan 2019, a copy of which accompanies this Option Certificate.

If the Participant wishes to renounce this Option, he may do so by notifying the Company in writing by 30 days after date of delivery of Option Certificate.

Please note that each employee is entirely responsible for the declaration and payment of tax on any profits which may arise from the exercise of share options. The company accepts no liability whatsoever in relation to the payment of tax in this regard.

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PRESENT when the common seal of AMRYT PHARMA PLC was affixed hereto:
	Signature
	Print name
	Signature
	Print name

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SCHEDULE 2

NOTICE OF EXERCISE

TO:	The Secretary
	Amryt Pharma plc
	90 Harcourt Street
	Dublin 2

Amryt Pharma plc ("The Company")

Amryt Pharma plc Employee Share Option Plan 2019

Date of Grant of Option:

Option Price per Share:

Total number of Shares subject to Option:

I hereby exercise the above option in respect of ______________** Ordinary Shares in the Company.

I enclose payment of the Option Price by [cheque][cash][cashless exercise][other method of payment].

Full Name:

Address:

Signature:

Date:

**Note

Insert the number of Shares in respect of which the option is exercised.

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EX-16.1

Exhibit 16.1

	Tel:+44 (0)20 7486 5888	55 Baker Street
	Fax:+44 (0)20 7487 3686 DX 9025 West End W1 www.bdo.co.uk	London W1U 7EU

Private and Confidential
Securities and Exchange Commission 100 F Street N.E. Washington, D.C. 20549 United States of America	23 June 2020

Ladies and Gentlemen

We have been furnished with a copy of the response to “Changes In Registrant’s Certifying Accountant” included in the Prospectus constituting a part of the Registration Statement on Form F-1 for the event that occurred on July 10, 2018, to be filed by our former client, Amryt Pharma plc.  We agree with the statements made in response to that disclosure insofar as they relate to our Firm.

Yours faithfully

/s/ BDO LLP

BDO LLP

BDO LLP, a UK limited liability partnership registered in England and Wales under number OC305127, is a member of BDO International Limited, a UK company limited by guarantee, and forms part of the international BDO network of independent member firms.  A list of members’ names is open to inspection at our registered office, 55 Baker Street, London W1U 7EU.  BDO LLP is authorised and regulated by the Financial Conduct Authority to conduct investment business.

EX-21.1

Exhibit 23.1

Aegerion Pharmaceuticals, Inc.	Delaware
Aegerion Pharmaceuticals Holdings, Inc.	Delaware
Aegerion Securities Corporation	Massachusetts
SomTherapeutics Corp.	Florida
Aegerion Argentina S.R.L.	Argentina
Aegerion International Ltd.	Bermuda
Aegerion Pharmaceuticals Ltd.	Bermuda
Aegerion Pharmaceuticals (Canada) Ltd.	British Columbia
Aegerion Pharmaceuticals Limited	England and Wales
Aegerion Pharmaceuticals SAS	France
Aegerion Pharmaceuticals GmbH	Germany
Aegerion Pharmaceuticals Srl	Italy
Aegerion Pharmaceuticals KK	Japan
Aegerion Pharmaceuticals B.V.	The Netherlands
Aegerion Pharmaceuticals Spain, S.L.	Spain
Aegerion Pharmaceuticals SARL	Switzerland
Aegerion İlaç Ticaret Limited Şirketi	Turkey
Aegerion Colombia S.A.S	Colombia
Aegerion Comercio E Importacao De Medicamentos LTDA	Brazil
Amryt Pharma Holdings Limited	England and Wales
Amryt Pharmaceuticals DAC	Ireland
Amryt Endocrinology Limited	Ireland
Amryt Research Limited	Ireland
Amryt Genetics Limited	Ireland
Amryt Lipidology Limited	Ireland
Amryt GmbH	Germany
Amryt Pharma France	France
Amryt Pharma Italy SRL	Italy
Amryt Pharma (UK) Ltd.	England and Wales
Amryt Pharma Spain	Spain
SomPharmaceuticals GmbH	Switzerland

EX-23.1

Exhibit 23.1

CONSENT OF INDEPENDENT REGISTERED PUBLIC ACCOUNTING FIRM

We have issued our report dated April 20, 2020 with respect to the consolidated financial statements of Amryt Pharma Plc included in the Registration Statement on Form F-1. We consent to the use of the aforementioned report in the Registration Statement on Form F-1 and to the use of our name as it appears under the caption “Experts”.

/s/ Grant Thornton

Dublin, Ireland

June 23, 2020

EX-23.2

Exhibit 23.2

CONSENT OF INDEPENDENT AUDITORS

We consent to the use in this Registration Statement on Form F-1 of our report dated February 18, 2020 relating to the financial statements of Aegerion Pharmaceuticals, Inc. We also consent to the reference to us under the heading "Experts" in such Registration Statement.

/s/ Deloitte & Touche LLP

Boston, Massachusetts

June 23, 2020