Document:

EX-10.3

 Exhibit 10.3 

Execution Version 

INCREMENTAL AMENDMENT 

This INCREMENTAL AMENDMENT AND LENDER JOINDER AGREEMENT (this “Agreement”), dated as of August 17, 2020, is
entered into by and among JPMORGAN CHASE BANK, N.A., as an Incremental Revolving Lender and as an Issuing Bank (in such capacities, the “New Lender”), HORIZON THERAPEUTICS USA, INC., a Delaware corporation (the
“Borrower”), and CITIBANK, N.A., as Administrative Agent (in such capacity, together with its successors and assigns, “Administrative Agent”). Capitalized terms used herein and not otherwise defined shall have the
meanings set forth in the Credit Agreement (as defined below). 
 RECITALS 

WHEREAS, the Borrower and Administrative Agent are party to that certain Credit Agreement, dated as of May 7, 2015 (as amended by
Amendment No. 1, dated as of October 25, 2016, as further amended by Amendment No. 2, dated as of March 29, 2017, as further amended by Amendment No. 3, dated as of October 23, 2017, as further amended by Amendment
No. 4, dated as of October 19, 2018, as further amended by Amendment No. 5, dated as of March 11, 2019, as further amended by Amendment No. 6, dated as of May 22, 2019, as further amended by Amendment No. 7, dated
as of December 18, 2019, and as further amended, restated, supplemented or otherwise modified from time to time, the “Credit Agreement”), among the Borrower, the other Loan Parties from time to time party thereto, the Lenders
from time to time party thereto, the Issuing Banks from time to time party thereto, and Citibank, N.A., as Administrative Agent and Collateral Agent; 

WHEREAS, pursuant to Section 2.20 of the Credit Agreement, the Borrower and any Additional Borrower may request Incremental Revolving
Commitments; 
 WHEREAS, pursuant to Section 2.20(d)(ii)(B) of the Credit Agreement, the Borrower (a) has requested Incremental
Revolving Commitments in the amount of $75,000,000, and (b) has requested that the entire amount of such Incremental Revolving Commitments be allocated to the New Lender; 

WHEREAS, the Borrower will use the proceeds of Incremental Revolving Loans made pursuant to such Incremental Revolving Commitments (the
“New Incremental Revolving Loans”) from time to time for general corporate purposes; 
 WHEREAS, pursuant to
Section 2.20(e) of the Credit Agreement, this Agreement may, without the consent of any Agents or Lenders, amend the Credit Agreement and the other Loan Documents as may be necessary or appropriate, in the reasonable opinion of the
Administrative Agent and the Borrower, to effect the provisions of Section 2.20 of the Credit Agreement; 
 WHEREAS, the New Lender has
agreed to provide such Incremental Revolving Commitments on the Effective Date (as defined below) in the amount set forth opposite the New Lender’s name on Exhibit A hereto on the terms and conditions set forth herein and to become a Lender
under the Credit Agreement in connection therewith. 
 NOW, THEREFORE, IN CONSIDERATION of the premises and other good and valuable
consideration, the receipt and sufficiency of which are hereby acknowledged, the parties hereto agree as follows: 

  
 1 

 AGREEMENT 

1. Commitment Increase and Amendments. 

(a) Pursuant to Section 2.20 of the Credit Agreement, Borrower hereby (i) requests Incremental Revolving Commitments in the amount
of $75,000,000, and (ii) requests that the entire amount of such Incremental Revolving Commitments be allocated to the New Lender. 

(b) The Borrower represents and warrants to each of the New Lender and the Administrative Agent that the representations and warranties of
Irish Holdco, the Borrower and their respective Restricted Subsidiaries set forth in Article III of the Credit Agreement are true and correct in all material respects (or, if qualified as to “materiality” or “Material Adverse
Effect”, in all respects) on and as of the Effective Date, except in the case of any such representation and warranty that expressly relates to an earlier date, in which case such representation and warranty is true and correct in all material
respects (or, if qualified by materiality or “Material Adverse Effect”, in all respects) as of such earlier date. 
 (c) At the
time of and immediately after giving effect to this Agreement, no Default or Event of Default exists. 
 (d) Except as expressly set forth
herein, this Agreement shall not by implication or otherwise limit, impair, constitute a waiver of, or otherwise affect the rights and remedies of the Lenders, Administrative Agent or any other Agent under the Credit Agreement or any other Loan
Document, and shall not alter, modify, amend or in any way affect any of the terms, conditions, obligations, covenants or agreements contained in the Credit Agreement or any other Loan Document, all of which are ratified and affirmed in all respects
and shall continue in full force and effect. Each Loan Party reaffirms its obligations under the Loan Documents to which it is party and reaffirms and confirms the validity of its guarantees pursuant to the Guaranty and its grant of Liens to secure
the Secured Obligations pursuant to the Loan Documents. Except as expressly set forth herein, nothing herein shall be deemed to entitle any Loan Party to a consent to, or a waiver, amendment, modification or other change of, any of the terms,
conditions, obligations, covenants or agreements contained in the Credit Agreement or any other Loan Document in similar or different circumstances. After the Effective Date, any reference to the Credit Agreement shall mean the Credit Agreement as
modified hereby. This Agreement shall constitute a “Loan Document” for all purposes of the Credit Agreement and the other Loan Documents. Each of the Loan Parties hereby (i) consents to this Agreement, (ii) confirms that
all obligations of such Loan Party under the Loan Documents to which such Loan Party is a party shall continue to apply and (iii) reaffirms its prior grant and validity of the security interests pursuant to any Loan Document and agrees that all
such security interests shall secure the Secured Obligations (including without limitation, the New Incremental Revolving Loans, the Swingline Exposure and the LC Exposure). Neither the modification of the Credit Agreement effected pursuant to this
Agreement nor the execution, delivery, performance or effectiveness of this Agreement impairs the validity, effectiveness or priority of the Liens granted pursuant to any Loan Document, and after giving effect to this Agreement, such Liens continue
unimpaired with the same priority to secure repayment of all Secured Obligations (including without limitation, the New Incremental Revolving Loans, the Swingline Exposure and the LC Exposure), whether heretofore or hereafter incurred. 

(e) This Agreement shall constitute an Incremental Amendment. This Agreement shall not constitute a novation of the Credit Agreement or any of
the Loan Documents. 
 (f) The Incremental Revolving Commitments established hereunder shall have terms identical to, and shall be an
increase of, the Revolving Commitments existing prior to the Effective Date. 

  
 2 

 (g) The Borrower shall use the proceeds of the New Incremental Revolving Loans as set forth
in the recitals to this Agreement. 
 (h) The definition of “Issuing Bank” in the Credit Agreement is hereby amended to add
“, JPMorgan Chase Bank, N.A.” after each instance of “Morgan Stanley Senior Funding, Inc.”. 
 (i) Clause (A)(iii) of
the last sentence of Section 2.06(b) of the Credit Agreement is hereby amended and restated as follows: 
 “(iii) subject to
Sections 2.04 and 2.11(b), the Dollar Amount of the LC Exposure in respect of such Issuing Bank shall not exceed, in the case of (I) Citibank, N.A., $13,635,000, (II) Morgan Stanley Senior Funding, Inc., $13,635,000, (III) JPMorgan Chase Bank,
N.A., $13,635,000 and (IV) Goldman Sachs Bank USA, $9,095,000; provided that the LC Sublimit may be increased from time to time upon agreement between the Administrative Agent and Borrowers, so long as any increase has been appropriately
committed by a Lender (that is or shall be an Issuing Bank), on terms and conditions satisfactory to the Administrative Agent.” 
 2.
New Lender Joinder. 
 (a) The New Lender hereby agrees to provide Incremental Revolving Commitments in the amount set forth on
Exhibit A attached hereto. 
 (b) New Lender (i) represents and warrants that (A) it has full power and authority, and has
taken all action necessary, to execute and deliver this Agreement and to consummate the transactions contemplated hereby and to become a Lender under the Credit Agreement, (B) it satisfies the requirements, if any, specified in the Credit
Agreement that are required to be satisfied by it in order to become a Lender, (C) from and after the Effective Date, it shall be bound by the provisions of the Credit Agreement as a Lender thereunder and shall have the obligations of a Lender
thereunder, (D) it has received a copy of the Credit Agreement, together with copies of the most recent financial statements delivered pursuant to Section 5.01 or 4.01(r) thereof, as applicable, and such other documents and information as
it has deemed appropriate to make its own credit analysis and decision to enter into this Agreement on the basis of which it has made such analysis and decision independently and without reliance on the Administrative Agent or any other Lender, and
(E) attached to this Agreement is any documentation required to be delivered by it pursuant to the terms of the Credit Agreement, duly completed and executed by the New Lender; and (ii) agrees that (A) it will, independently and
without reliance on the Administrative Agent 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 Loan
Documents, and (B) it will perform in accordance with their terms all of the obligations which by the terms of the Loan Documents are required to be performed by it as a Lender. 

(c) As of the Effective Date, the New Lender shall (i) be a party to the Credit Agreement and the other Loan Documents, (ii) be a
“Lender”, and “Issuing Bank”, a “Revolving Lender” and an “Incremental Revolving Lender” for all purposes of the Credit Agreement and the other Loan Documents, and (iii) to the extent of the interest
acquired by New Lender pursuant to this Agreement, have the rights and obligations of a Lender, and Issuing Bank, a Revolving Lender and an Incremental Revolving Lender under the Credit Agreement and the other Loan Documents. 

3. Conditions to Effectiveness. This Agreement shall not be effective until the date (the “Effective Date”) on which
each of the following conditions shall have been satisfied (or waived), as determined by Administrative Agent: 

  
 3 

 (a) Administrative Agent shall have received executed counterparts of this Agreement from
each of the intended parties hereto; 
 (b) The Administrative Agent shall have received (i) a copy of the certificate of
incorporation, memorandum of association or articles of incorporation and all applicable, if any, certificates of incorporation on a change of name or certificates of re-registration or other formation
documents, including all amendments thereto, of each Loan Party as of the Effective Date, certified as of a recent date by the Secretary of State of the state of its organization, and a certificate as to or of compliance evidencing (to the extent
the concept is applicable in such jurisdiction) the good standing of each such Loan Party as of a recent date, from such Secretary of State; (ii) a certificate of the secretary or assistant secretary of each Loan Party as of the Effective Date
dated the Effective Date and certifying (A) that attached thereto is a true and complete copy of the by-laws (or similar governing documentation) of such Loan Party as in effect on the Effective Date and
at all times since a date prior to the date of the resolutions described in sub-clause (B) below, (B) that attached thereto is a true and complete copy of resolutions duly adopted by the board of
directors or similar governing body of such Loan Party authorizing the execution, delivery and performance of the Loan Documents to which such Person is a party (in the case of the Borrower), the establishment of the Incremental Revolving
Commitments hereunder (in the case of each such Loan Party), the granting of the Liens contemplated to be granted by it under the Collateral Documents and (in the case of each Guarantor) the Guaranteeing of the Secured Obligations as contemplated by
this Agreement, joinders to any Loan Documents, and any other documents required to be executed by such Loan Party pursuant to this Section 3 (the “Amendment Documents”), and that such resolutions have not
been modified, rescinded or amended and are in full force and effect, (C) if applicable, that the certificate or articles of incorporation or other formation documents of such Loan Party have not been amended since the date of the last
amendment thereto shown on the certificate of good standing furnished pursuant to sub-clause (i) above (in which case such certification will be delivered in lieu of the documentation requested pursuant
to sub-clause (i) above and such certification is hereby deemed to satisfy the requirements of sub-clause (i) of this clause (f)) or where a certificate of
good standing is not applicable in its jurisdiction of incorporation that attach a true, up to date and correct copy of the certificate or articles of incorporation or other formation documents of each Loan Party duly certified as being true, up to
date and correct and (D) as to the incumbency and specimen signature of each officer executing any Amendment Document or any other document delivered in connection herewith on behalf of such Loan Party; and (iii) a certificate of another
officer as to the incumbency and specimen signature of the secretary or assistant secretary (or manager or director, if applicable) executing the certificate pursuant to (ii) above; 

(c) On the Effective Date, the representations and warranties set forth in Section 1(b) above shall be true and
correct on the basis set forth therein; 
 (d) At the time of and immediately after giving effect to this Agreement, no Default or Event of
Default shall exist; 
 (e) The Administrative Agent shall have received favorable written opinion of (i) Cooley LLP, New York counsel
for Irish Holdco, (ii) (a) Matheson, Irish counsel for the Loan Parties and (b) Arthur Cox, Irish counsel for the Administrative Agent and Collateral Agent in relation to validity and enforceability aspects and confirmation that the entry
by Irish Holdco and its Subsidiaries into this Agreement will not constitute financial assistance for the purposes of Section 82 of the Companies Act, (iii) Arendt & Medernach, Avocats à la Cour, Luxembourg counsel for
Irish Holdco in relation to the capacity of, authority of and execution by Horizon Therapeutics Finance S.à r.l., and the validity and enforceability of the Luxembourg Master Confirmation Agreement and (iv) Appleby (Bermuda) Limited,
Bermuda counsel for the Administrative Agent in relation to capacity, authority and execution aspects, in each case (A) addressed to the Administrative Agent and the Collateral Agent and in each case, each of

  
 4 

 
their permitted assigns (to the extent provided for, and allowed by, each opinion), (B) dated the Effective Date and (C) in form and substance reasonably satisfactory to the Administrative
Agent; 
 (f) All documents and instruments required to perfect the Collateral Agent’s security interest in (A) subject to the
Agreed Security Principles (in the case of the Equity Interests of any Foreign Subsidiary), all of the issued and outstanding Equity Interests of each Subsidiary Guarantor and (B) subject to the Agreed Security Principles (in the case of any
Foreign Subsidiary), substantially all of the assets of Irish Holdco, the Borrower and each Subsidiary Guarantor (in each case, to the extent included in the Collateral) shall have been executed and delivered and, if applicable, be in proper form
for filing; and 
 (g) Additional requirements for Foreign Subsidiary Guarantors: 

(1) The Administrative Agent shall have received, in respect of each Luxembourg Guarantor, (i) a master confirmation
agreement in a form satisfactory to the Administrative Agent and (ii) a manager’s certificate in a form satisfactory to the Administrative Agent, dated as of the Effective Date and signed by a manager of each such Luxembourg Guarantor,
certifying, inter alia, the following items: 
 (A) a true, correct, complete and up-to-date copy of its articles of association; 
 (B) a true, correct, complete
and up-to-date copy of the resolutions of the board of managers of each Luxembourg Guarantor approving, inter alia, its entry into the Amendment Documents to which it is
a party; 
 (C) a true, correct, complete and
up-to-date copy of an excerpt of the Luxembourg Companies Register dated on the Effective Date or at the earliest one Business Day before the Effective Date; 

(D) a true, correct, complete and up-to-date
copy of a certified true certificate of non-registration of judgments (certificat de non-inscription d’une décision judiciaire) dated on the Effective
Date or at the earliest one Business Day before the Effective Date, issued by the Luxembourg Companies Register; 
 (E) a
true, correct, complete and up-to-date copy of the specimen signatures of all authorised signatories; and 

(F) each Luxembourg Guarantor complies with, and adheres to, the provisions applicable to it of the Luxembourg Law dated
May 31, 1999 concerning the domiciliation of companies, as amended. 
 (2) The Administrative Agent shall have received,
in respect of each Guarantor organized under the laws of Ireland, (i) a deed of confirmation in a form satisfactory to the Administrative Agent and (ii) a director’s or secretary’s certificate in a form satisfactory to the
Administrative Agent, dated as of the Effective Date and signed by a director or secretary of such Guarantor, certifying, inter alia, the following items: 

(A) that the entry by such Guarantor into the Amendment Documents would: (I) not cause any borrowing, guaranteeing,
security or similar limit binding on such Guarantor to be exceeded; and (II) not constitute financial assistance for the purposes of Section 82 of the Companies Act; 

  
 5 

 (B) a true, correct and up-to-date list of the present directors and secretaries of such Guarantor; 
 (C)
that neither the Guarantor nor any of its directors or secretaries, is a company or a person to whom either Chapter 3 (Restrictions on directors of insolvent companies), Chapter 4 (Disqualification generally) or Chapter 5 (Disqualification and
restriction undertakings) of Part 14 (Compliance and Enforcement) of the Companies Act (as from time to time amended, replaced or re-enacted) applies; and 

(D) each copy document relating to it specified in the director’s certificate is correct, complete and in full force and
effect and has not been amended or superseded as at a date no earlier than the date of this Agreement. 
 (3) The
Administrative Agent shall have received, in respect of each Guarantor organized under the laws of Bermuda, (i) a deed of confirmation in a form satisfactory to the Administrative Agent and (ii) an officer’s certificate in a form
satisfactory to the Administrative Agent, dated as of the Effective Date and signed by an officer of each such Guarantor, certifying, inter alia, the following items: 

(A) a certified copy of the true, correct, complete and
up-to-date constitutional documents of each such Guarantor including, but not limited to, the certificate of incorporation (and any amendments thereto), memorandum of
association (and any amendments thereto), bye-laws, foreign exchange letter, tax assurance certificate, register of directors and officers and register of members; 

(B) a pdf copy of a Certificate of Compliance issued by the Registrar of Companies no later than 3 Business Days prior to the
execution hereof; and 
 (C) a true, correct, complete and
up-to-date copy of the resolutions of the Board of Directors approving the granting of the guarantee and the entry into the Amendment Documents to which it is a party.

 (h) All fees required to be paid on the Effective Date pursuant to the Fee Letter (as defined below) and reasonable out-of-pocket expenses required to be paid on the Effective Date pursuant to the Fee Letter, solely with respect to expenses to the extent invoiced at least three
(3) business days prior to the Effective Date, shall, on the Effective Date, have been paid. “Fee Letter” means that certain fee letter, dated as of the Effective Date, by and between the Borrower and the New Lender. 

4. Miscellaneous. 
 (a)
This Agreement may be executed in any number of counterparts and by different parties hereto in separate counterparts, each of which when so executed shall be deemed to be an original and all of which taken together shall constitute but one and the
same agreement. Delivery of an executed counterpart of a signature page to this Agreement by telecopier or other electronic transmission shall be effective as delivery of a manually executed counterpart of this Agreement. 

(b) THIS AGREEMENT AND THE OTHER AMENDMENT DOCUMENTS AND ANY CLAIMS, CONTROVERSY, DISPUTE OR CAUSE OF ACTION (WHETHER IN CONTRACT OR TORT
OR OTHERWISE) BASED UPON, ARISING OUT OF OR RELATING TO THIS AGREEMENT OR ANY OTHER AMENDMENT DOCUMENT AND THE TRANSACTIONS 

  
 6 

 
CONTEMPLATED HEREBY AND THEREBY SHALL (EXCEPT, AS TO ANY OTHER AMENDMENT DOCUMENT, AS EXPRESSLY SET FORTH THEREIN) BE CONSTRUED IN ACCORDANCE WITH AND GOVERNED BY THE LAW OF THE STATE
OF NEW YORK. 
 (c) Section and Subsection headings in this Agreement are included herein for convenience of reference only and shall
not constitute a part of this Agreement for any other purpose or be given any substantive effect. 
 (d) As of the Effective Date, the
Revolving Commitments section of Schedule 2.01 to the Credit Agreement will be deemed to be amended and restated in its entirety in the form of Exhibit B attached hereto. 

(e) Each of the parties hereto hereby irrevocably and unconditionally: 

(i) submits, for itself and its property, to the exclusive jurisdiction of the Supreme Court of the State of New York sitting
in New York County and of the United States District Court of the Southern District of New York sitting in New York County, and any appellate court from any thereof, in any action or proceeding arising out of or relating to this Agreement or any
other Loan Document, or for recognition or enforcement of any judgment, and each of the parties hereto hereby irrevocably and unconditionally agrees that all claims in respect of any such action or proceeding may be heard and determined in such New
York State or, to the extent permitted by law, in such Federal court; 
 (ii) 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; 

(iii) agrees that nothing herein shall affect any right that the Administrative Agent or any Lender may otherwise have to bring
any action or proceeding relating to this Agreement or any other Loan Document against any Loan Party or its properties in the courts of any competent jurisdiction; 

(iv) irrevocably and unconditionally waives, to the fullest extent they may legally and effectively do so, any objection which
it may now or hereafter have to the laying of venue of any suit, action or proceeding arising out of or relating to this Agreement or any other Loan Document in any court referred to in paragraph (e)(i) of this Section 4. Each of the parties
hereto hereby irrevocably 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 court; 

(v) consents to service of process in the manner provided for notices in Section 9.01 of the Credit Agreement; and 

(vi) agrees that nothing herein will affect the right of any party to this Agreement to serve process in any other manner
permitted by law. 
 [remainder of page intentionally left blank] 

  
 7 

 IN WITNESS WHEREOF, the parties hereto have caused this Incremental Amendment and Lender
Joinder Agreement to be executed by their respective officers, as of the first date written above. 
  

			
	 JPMORGAN CHASE BANK, N.A., as New Lender

		
	By:	 	 /s/ Erik Barragan

	 Name:
	 	 Erik Barragan

	 Title:
	 	 Authorized Officer

  
 [Incremental
Amendment and Lender Joinder Agreement (Horizon)] 

 
					
	 HORIZON THERAPEUTICS USA, INC., as Borrower

		
	By:	 	 /s/ Paul W. Hoelscher

	Name:	 	Paul W. Hoelscher
	Title:	 	Chief Financial Officer
	
	HZNP USA LLC
	HORIZON ORPHAN HOLDINGS LLC
	HORIZON ORPHAN LLC
	CURZION PHARMACEUTICALS, INC.
	 HORIZON PROPERTIES HOLDING LLC

HORIZON OPHTHALMOLOGY, INC.

	 HORIZON THERAPEUTICS SERVICES LLC

HORIZON MEDICINES LLC

	HORIZON THERAPEUTICS, LLC
		
	By:	 	 /s/ Paul W. Hoelscher

		 	Name:	 	Paul W. Hoelscher
		 	Title:	 	Chief Financial Officer

  
 [Incremental
Amendment and Lender Joinder Agreement (Horizon)] 

 
					
	HORIZON THERAPEUTICS PUBLIC LIMITED COMPANY
		
	By:	 	 /s/ Patrick McIlvenny

		 	Name:	 	Patrick McIlvenny
		 	Title:	 	as lawfully appointed attorney
	
	HORIZON THERAPEUTICS HOLDINGS LIMITED
		
	By:	 	 /s/ Patrick McIlvenny

		 	Name:	 	Patrick McIlvenny
		 	Title:	 	as lawfully appointed attorney
	
	HZNP LIMITED
		
	By:	 	  

		 	Name:	 	Kevin Insley
		 	Title:	 	as lawfully appointed attorney
	
	HORIZON THERAPEUTICS TREASURY DAC
		
	By:	 	 /s/ Patrick McIlvenny

		 	Name:	 	Patrick McIlvenny
		 	Title:	 	as lawfully appointed attorney

  
 [Incremental
Amendment and Lender Joinder Agreement (Horizon)] 

 
					
	HORIZON THERAPEUTICS PUBLIC LIMITED COMPANY
		
	By:	 	  

		 	Name:	 	Patrick McIlvenny
		 	Title:	 	as lawfully appointed attorney
	
	HORIZON THERAPEUTICS HOLDINGS LIMITED
		
	By:	 	  

		 	Name:	 	Patrick McIlvenny
		 	Title:	 	as lawfully appointed attorney
	
	HZNP LIMITED
		
	By:	 	 /s/ Kevin Insley

		 	Name:	 	Kevin Insley
		 	Title:	 	as lawfully appointed attorney
	
	HORIZON THERAPEUTICS TREASURY DAC
		
	By:	 	  

		 	Name:	 	Patrick McIlvenny
		 	Title:	 	as lawfully appointed attorney

  
 [Incremental
Amendment and Lender Joinder Agreement (Horizon)] 

 
					
	HORIZON THERAPEUTICS FINANCE LIMITED
		
	By:	 	 /s/ Patrick McIlvenny

		 	Name:	 	Patrick McIlvenny
		 	Title:	 	as lawfully appointed attorney
	
	HORIZON THERAPEUTICS IRELAND DAC
		
	By:	 	 /s/ Patrick McIlvenny

		 	Name:	 	Patrick McIlvenny
		 	Title:	 	as lawfully appointed attorney
	
	HORIZON THERAPEUTICS CAPITAL LIMITED
		
	By:	 	 /s/ Patrick McIlvenny

		 	Name:	 	Patrick McIlvenny
		 	Title:	 	as lawfully appointed attorney

  
 [Incremental
Amendment and Lender Joinder Agreement (Horizon)] 

 
					
	HORIZON THERAPEUTICS FINANCE S.À R.L
	Société à responsabilité limitée
	Registered office : 19, rue de Bitbourg, L-1273
	Luxembourg
	R.C.S. Luxembourg : B 186.460
		
	By:	 	 /s/ David Caraher

		 	Name:	 	David Caraher
		 	Title:	 	Manager A

  
 [Incremental
Amendment and Lender Joinder Agreement (Horizon)] 

 
					
	HORIZON THERAPEUTICS INVESTMENT LIMITED
		
	By:	 	 /s/ Patrick McIlvenny

		 	Name:	 	Patrick McIlvenny
		 	Title:	 	Director
	
	HZNP FINANCE LIMITED
		
	By:	 	  

		 	Name:	 	Kevin Insley
		 	Title:	 	Director

  
 [Incremental
Amendment and Lender Joinder Agreement (Horizon)] 

 
					
	HORIZON THERAPEUTICS INVESTMENT LIMITED
		
	By:	 	  

		 	Name:	 	Patrick McIlvenny
		 	Title:	 	Director
	
	HZNP FINANCE LIMITED
		
	By:	 	 /s/ Kevin Insley

		 	Name:	 	Kevin Insley
		 	Title:	 	Director

  
 [Incremental
Amendment and Lender Joinder Agreement (Horizon)] 

 
			
	 CITIBANK, N.A.,
 as Administrative
Agent

		
	By:	 	 /s/ Michael Tortora

	Name:	 	 Michael Tortora

	Title:	 	 Vice President

  
 [Incremental
Amendment and Lender Joinder Agreement (Horizon)] 

 Execution Version 

Exhibit A 
  

			
	 NEW LENDER
	  	 COMMITMENT

	 JPMorgan Chase Bank, N.A.
	  	$75,000,000

 Execution Version 

Exhibit B 
 Schedule
2.01 Commitments 
 Revolving Commitments 
  

					
	 Revolving Lender
	  	Revolving Commitment	  	Applicable Percentage
	 Citibank, N.A.
	  	$75,000,000.00	  	27.27%
	 Morgan Stanley Senior Funding, Inc.
	  	$75,000,000.00	  	27.27%
	 Goldman Sachs Bank USA
	  	$50,000,000.00	  	18.19%
	 JPMorgan Chase Bank, N.A.
	  	$75,000,000.00	  	27.27%
	 Total
	  	$275,000,000.00	  	100%EX-10.4

 Exhibit 10.4 

CERTAIN CONFIDENTIAL INFORMATION CONTAINED IN THIS DOCUMENT, MARKED BY [***], HAS BEEN OMITTED BECAUSE HORIZON THERAPEUTICS PLC HAS
DETERMINED THE INFORMATION (I) IS NOT MATERIAL AND (II) WOULD LIKELY CAUSE COMPETITIVE HARM TO HORIZON THERAPEUTICS PLC IF PUBLICLY DISCLOSED. 

EXECUTION COPY 
 API
SUPPLY AGREEMENT 
 This API Supply Agreement (“Agreement”) is made as of the 3rd day of November, 2010
(“Effective Date”), by and between Raptor Therapeutics, Inc., a Delaware Corporation, with a place of business at 9 Commercial Boulevard, Suite 200, Novato, California 94949, U.S.A. (“RAPTOR”), and Cambrex
Profarmaco Milano, Via Cucchiari 17, 20155 Milan, Italy (“CAMBREX”). RAPTOR and CAMBREX may be referred to individually as a “Party” or collectively as the “Parties.” 

Background 
 RAPTOR
is engaged in the business of developing and commercializing therapeutic products through the application of specialized drug targeting platforms and formulation expertise for under-served patient populations; 

CAMBREX is engaged in the manufacture and supply of active pharmaceutical ingredients for research and development purposes and/or commercial
use; 
 RAPTOR desires to purchase from CAMBREX, and CAMBREX desires to supply to RAPTOR, the active pharmaceutical ingredient known as
cysteamine bitartrate (as further defined below, the “API”) for use by RAPTOR in manufacturing finished products incorporating such active pharmaceutical ingredient, all in accordance with the terms and conditions of this Agreement.

 NOW, THEREFORE, in consideration of the mutual covenants and premises herein contained, the Parties hereto agree as follows: 

ARTICLE 1 

DEFINITIONS 
 1.1
“Affiliate” or “Affiliates” shall mean, with respect to a Party, any corporation, limited liability company or other business entity controlling, controlled by or under common control with such Party, for so long as
such relationship exists. For the purposes of this definition, control means: (a) to possess, directly or indirectly, the power to direct affirmatively the management and policies of such corporation, limited liability company or other business
entity, whether through ownership of voting securities or by contract relating to voting rights or corporate governance; or (b) ownership of more than fifty percent (50%) of the voting stock in such corporation, limited liability company or
other business entity (or such lesser percent as may be the maximum that may be owned pursuant to Applicable Laws of the country of incorporation or domicile, as applicable). 

1.2 “API” shall mean cysteamine bitartrate, with the chemical structure set forth in Exhibit 1.2
attached hereto. 

 EXECUTION COPY 

 

 1.3 “Applicable Laws” shall mean: (a) all relevant federal, state and
local laws, statutes, rules, regulations, and ordinances in the United States, Europe and/or any other jurisdiction, as well as industry standards and guidelines applicable to the manufacture and supply of API, including, the United States Federal
Food, Drug and Cosmetic Act; (b) cGMPs; and (c) all applicable regulations and guidelines of any Regulatory Authority; in each case, together, with any and all amendments thereto. 

1.4 “cGMPs” shall mean current good manufacturing practices, as provided for (and as amended from time to time) in the
Current Good Manufacturing Practice regulations promulgated by the FDA under the United States Food, Drug and Cosmetic Act and in the European Community Directive 91/356/EEC (Principles and guidelines of good manufacturing practice for medicinal
products), as well as applicable documents developed by the International Conference on Harmonization (ICH), and similar requirements of other Regulatory Authorities, and subject to any arrangements, additions or clarifications, and the respective
roles and responsibilities, agreed from time to time between the Parties. 
 1.5 “Drug Master File” or
“DMF” shall mean a drug master file filed with the FDA or the EMEA which includes information relating to the facilities, processes, or articles used in manufacturing, processing, packaging, and storing of the API, or any equivalent
filing in any jurisdiction outside the United States or Europe. 
 1.6 “EMEA” shall mean the European Medicines Evaluation
Agency, or any successor entity thereto performing substantially similar functions. 
 1.7 “Facility” shall mean
CAMBREX’s cGMP-compliant facilities located at Via Cucchiari 17, 20155 Milan, Italy. 
 1.8 “FDA” shall mean the
United States Food and Drug Administration, or any successor entity thereto performing substantially similar functions. 
 1.9
“Inflation Index” shall mean the annual average rate of change in the Harmonized Indices of Consumer Prices for the European Union, as published by Eurostat (or, to the extent that such index ceases to exist, any alternative
inflation index mutually agreed by the Parties). 
 1.10 “Price” shall mean the price for the API set forth in Exhibit
3.1. 
 1.11 “Product” shall mean a finished pharmaceutical product incorporating the API. 

1.12 “Regulatory Authority” shall mean the FDA, EMEA or a regulatory body with similar regulatory authority in a jurisdiction
other than the United States or Europe. 
 1.13 “Specifications” shall mean those specifications and release requirements
and/or procedures and/or other similar requirements for the manufacture of API, as the same are set forth in Exhibit 1.13. 

  
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 1.14 Additional Defined Terms. Each of the following terms shall have the meaning described
in the corresponding section of this Agreement indicated below: 
  

									
	Term	 	Section Defined	 	Term	 	Section Defined	 	 
	Agreement	 	Introduction	 	[***]	 	2.1	 	
	CAMBREX	 	Introduction	 	Party / Parties	 	Introductions	 	
	Confidential Information	 	8.1	 	Purchase Order	 	2.3.2	 	
	Effective Date	 	Introduction	 	Q1, Q2, Q3, Q4	 	2.2	 	
	Force Majeure Event	 	11.6	 	Quality Agreement	 	4.2	 	
	Indemnitee	 	10.3	 	RAPTOR	 	Introduction	 	
	Indemnitor	 	10.3	 	Renewal Term	 	7.1	 	
	Initial Term	 	7.1	 	Required Changes	 	4.4	 	
	JAMS	 	11.4	 	Rolling Forecast	 	2.2	 	
	Laboratory	 	4.3.2	 	Shortage of Supply	 	2.5	 	
	Late Shipment	 	7.3	 	Term	 	7.1	 	

 ARTICLE 2 

SUPPLY 
 2.1 API
Supply. Subject to the terms and conditions of this Agreement, CAMBREX shall supply to RAPTOR, such quantities of the API as may be specified in purchase orders submitted by RAPTOR pursuant to Section 2.3 below from time to time during the
Term. All API to be supplied under this Agreement shall be manufactured by CAMBREX at the Facility, in conformance with Applicable Laws, the Specifications and the Quality Agreement. Subject to Section 2.5, RAPTOR agrees that during the Term,
RAPTOR will purchase: [***]. 
 2.2 Forecasts. Beginning no later than [***], RAPTOR shall provide CAMBREX with an initial forecast
of the quantities of the API estimated to be required during [***] (each, a “Rolling Forecast”). Subject to Section 2.3 below, such Rolling Forecasts are non-binding and serve only to
facilitate CAMBREX’s production scheduling. 
 2.3 Orders. 

2.3.1 Orders. Together with each Rolling Forecast provided under Section 2.2 above, RAPTOR shall place a firm order with CAMBREX
for supplies of API for delivery in [***]. The total quantity of API ordered by RAPTOR for delivery in [***] shall equal at least the 

  
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quantity of API forecasted for [***] in such Rolling Forecast. For the avoidance of doubt, RAPTOR may order quantities of API in addition to those specified in the then-current Rolling Forecast
for delivery hereunder in accordance with the lead times therefor and subject to CAMBREX’s total capacity constraints; provided that CAMBREX shall use commercially reasonable efforts to accept and fulfill all orders for API provided by RAPTOR
under this Agreement. 
 2.3.2 Form of Orders. RAPTOR’s orders shall be made pursuant to a written purchase order (each, a
“Purchase Order”) that specifies [***]; provided that the maximum lead time shall not [***] unless otherwise mutually agreed and CAMBREX shall use commercially reasonable efforts to achieve a maximum lead time of no more than [***].
RAPTOR is informed and understands that production of the API is scheduled based upon demand, and no campaign for production of the API will be scheduled until a firm Purchase Order is placed. To the extent a particular Purchase Order issued by
RAPTOR pursuant to this Section 2.3.2 is for less than a full lot of API (or a multiple thereof), upon CAMBREX’s reasonable written request, RAPTOR will confirm its acceptance to increase the applicable Purchase Order to a full lot of API
(or the nearest whole multiple thereof). Subject to the preceding sentence, CAMBREX shall accept all orders RAPTOR submits to CAMBREX in accordance with this Article 2. CAMBREX shall provide to RAPTOR written notice of CAMBREX’s acceptance
(each, an “Acceptance Notice”) of each Purchase Order within [***] of CAMBREX’s receipt of such Purchase Order and each such notice shall include confirmation of the delivery date of the applicable quantity of
API; provided that to the extent no delivery date is included in an Acceptance Notice issued by CAMBREX or CAMBREX fails to issue an Acceptance Notice within the applicable time period, the applicable delivery date shall be deemed to be
the delivery date specified by RAPTOR in the corresponding Purchase Order. Except as to the quantity of API, delivery date and delivery location specified in a Purchase Order which shall be binding on the Parties, NO TERMS OR CONDITIONS CONTAINED IN
ANY PURCHASE ORDER, ORDER ACKNOWLEDGMENT OR SIMILAR STANDARDIZED FORM SHALL BE CONSTRUED TO AMEND OR MODIFY THE TERMS OF THIS AGREEMENT, AND ALL SUCH TERMS AND CONDITIONS ARE HEREBY EXCLUDED. 

2.4 Shipping. CAMBREX shall deliver quantities of API ordered by RAPTOR in accordance with Section 2.3 above, to the location
specified in the applicable Purchase Order. All shipments shall be [***]. CAMBREX shall ship API, together with all relevant documentation relating to the API, in accordance with any agreed-upon shipment specifications or as otherwise reasonably
directed by RAPTOR in writing and in accordance with this Agreement. RAPTOR shall only be obligated to pay for quantities of API actually delivered in compliance with the applicable Purchase Order and the terms of this Agreement. 

2.5 Shortage of Supply. If CAMBREX is unable, or anticipates that it will not be able, to supply RAPTOR’s requirements for the API
in accordance with Section 2.3 above (a “Shortage  

  
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of Supply”), CAMBREX shall immediately notify RAPTOR in writing of the same, and shall include in such notice its best estimate of the duration of the delay. CAMBREX shall, at its own
cost, use commercially reasonable efforts to remedy any Shortage of Supply and resume supplying API meeting the requirements of this Agreement to RAPTOR as soon as possible. In addition to the foregoing measures, if CAMBREX is unable to supply
RAPTOR’s requirements of API, CAMBREX shall allocate the quantities of the API that CAMBREX has in inventory, and that CAMBREX is able to produce, on a reasonable worldwide basis (based upon sales history and realistic forecasted demand). [***]
In the event of a Shortage of Supply exceeding [***], in addition to any other rights or remedies that RAPTOR may have under this Agreement, or at law or in equity, RAPTOR shall be relieved from its obligations to purchase any quantities of API
identified in any outstanding Purchase Order. 
 ARTICLE 3 

PAYMENTS 
 3.1
Price. Except as otherwise provided herein, the Price for the API subject to this Agreement shall be listed on Exhibit 3.1. [***] 

3.2 Invoicing; Payment. CAMBREX (or CAMBREX’s agent, GYMA Laboratories) shall submit an invoice to RAPTOR upon shipment of API
ordered by RAPTOR hereunder. All invoices shall be sent to the address specified in the Purchase Order therefor, and each invoice shall state the Price for the API in a given shipment, plus any taxes and other costs incident to the purchase or
shipment initially paid by CAMBREX but to be borne by RAPTOR hereunder. All payments shall be made by direct bank transfer to an account designated in CAMBREX’ s invoice. Payment terms shall be [***] from invoice date. Payment by RAPTOR shall

  
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not constitute acceptance of any shipment of API or impair RAPTOR’s right of inspection and rejection under Article 4 below. 

ARTICLE 4 
 QUALITY

 4.1 Quality Assurance. All API supplied by CAMBREX shall meet the current Specifications and shall be manufactured in
accordance with all Applicable Laws and the Quality Agreement at the Facility. CAMBREX agrees that, prior to each shipment of API hereunder, it shall perform quality control procedures reasonably necessary to ensure that the API to be shipped
conforms fully with the Specifications. Each shipment of API shall be accompanied by a certificate of analysis [***] and such additional documents as may be specified in the Quality Agreement or as otherwise reasonably required by RAPTOR from time
to time. 
 4.2 Quality Agreement. Prior to RAPTOR issuing its first Purchase Order to CAMBREX pursuant to this Agreement (and in any
event, [***] after the Effective Date), the Parties shall enter into an agreement specifying the Parties’ respective responsibilities for storage, release, quality control and quality assurance with respect to the API (the “Quality
Agreement”). The Quality Agreement is not intended and shall not be construed to limit any of the rights and obligations of the Parties set forth in the body of this Agreement. Subject to the foregoing, to the extent possible, the Quality
Agreement will be interpreted with the terms set forth in the body of this Agreement. If there is any conflict or inconsistency between the terms of the Quality Agreement and the terms set forth in the body of this Agreement, however, the terms set
forth in the body of this Agreement shall control. 
 4.3 Rejection and Replacement of API. 

4.3.1 Inspection by RAPTOR. RAPTOR and/or its designee shall have [***] following its receipt of a shipment of API to reject such API
on the grounds that all or part of the shipment fails to conform to the applicable Specifications or otherwise fails to conform to the warranties given by CAMBREX in Section 9.2, which rejection shall be accomplished by giving written notice to
CAMBREX summarizing the manner in which all or part of such shipment fails to meet the foregoing requirements. 
 4.3.2 Resolution of
Disputes. CAMBREX shall respond in writing to a rejection notice from RAPTOR within [***] from the date of receipt of such rejection notice in accordance with Section 4.3.1 above. If CAMBREX does not agree with RAPTOR’s determination
that such API fails to conform to the Specifications or the warranties provided by CAMBREX in Section 9.2, then CAMBREX and RAPTOR shall use reasonable efforts to resolve such disagreement as promptly as possible. Without limiting the
foregoing, [***] 

  
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[***]. 
 4.3.3 Replacement of API. API accepted by CAMBREX as not meeting the
applicable requirements and/or the Specifications, or which is determined by the Laboratory not to meet such requirements and/or the Specifications, shall be returned by RAPTOR to CAMBREX, or disposed of, as directed by CAMBREX and at CAMBREX’s
expense. CAMBREX shall replace all such rejected API within the shortest possible time, but in any event, within [***] days after its receipt of notice of such rejection (or, if applicable, the Laboratory’s determination that such API was non-conforming), [***]. Without limiting any other provision in this Agreement, RAPTOR may withhold payment for such shipment or the portion thereof that has been rejected by RAPTOR pursuant to this
Section 4.3. The warranties given by CAMBREX in Section 9.2 below shall survive any failure to reject by RAPTOR under this Section 4.3. 

4.4 Changes. 
 4.4.1
CAMBREX shall maintain change control systems that ensure that all major changes are appropriately notified in a timely manner and in certain cases, as provided in Section 4.4.2 below or otherwise agreed by the Parties in the Quality Agreement,
are agreed with RAPTOR. 
 4.4.2 CAMBREX shall promptly inform RAPTOR in writing of any proposal of major change to the manufacturing
process, equipment, packaging, testing, specifications, or any item specially mentioned in the DMF. [***] Notwithstanding the foregoing, in no event will CAMBREX implement any major changes with respect to quantities of API to be supplied to RAPTOR,
before obtaining RAPTOR’s approval (if applicable) and prior to all necessary filings with and approvals by applicable Regulatory Authorities have been made or obtained by CAMBREX and/or RAPTOR, as applicable. 

  
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 ARTICLE 5 

RECORDS; INSPECTIONS 

5.1 Record Keeping. CAMBREX shall generate and maintain complete and accurate records and samples as necessary to evidence compliance
with this Agreement and all Applicable Laws and other requirements of applicable governmental authorities relating to the manufacture of APL All such records and samples shall be maintained by CAMBREX in accordance with the procedures set out in the
Quality Agreement for the applicable time period specified therein. 
 5.2 Inspection. During the term of this Agreement, and for
[***] thereafter, or as otherwise required by Applicable Laws, RAPTOR (and/or its designee) shall have the right to inspect and audit, during regular business hours: (a) any facility at which any of the manufacturing or processing activities
relating to the API are performed, including the Facility; (b) any of CAMBREX’ s manufacturing and quality control records and all other documentation relating to the manufacturing and processing activities with respect to the API
(including any internal quality control audits or reviews conducted by CAMBREX); and (c) accounts and records for the purpose of determining the amounts payable or owed under this Agreement. Such inspections and audits shall be conducted [***]
and in accordance with any procedures for audits specified in the Quality Agreement; provided however that RAPTOR shall have the right to conduct additional inspections and audits under this Section 5.2 [***]. 

ARTICLE 6 
 REGULATORY
MATTERS 
 6.1 Regulatory Actions. CAMBREX shall permit the FDA and other Regulatory Authorities, as applicable, to conduct
such inspections of the Facility, and/or any other facility at which any of the manufacturing or processing activities relating to the API are performed, as such Regulatory Authorities may request, including
pre-approval inspections, and shall cooperate with such Regulatory Authorities with respect to such inspections and any related matters, in each case that is related to the manufacture and supply of APL
CAMBREX shall (a) give RAPTOR prior written notice of any such inspections related to the API; and (b) keep RAPTOR informed about the results and conclusions of each such regulatory inspection, including any actions taken by CAMBREX to
remedy any conditions cited in such inspections related to the API; all as further described, and in accordance with the procedures specified, in the Quality Agreement. 

6.2 Regulatory Cooperation. CAMBREX agrees to provide to RAPTOR, as requested, with all information and data in CAMBREX’s
possession or control necessary or reasonably useful for RAPTOR (and/or its designees) to apply for, obtain and maintain regulatory approvals for any Product in any country, including information relating to the Facility, or the methodology, raw
materials and intermediates used in the manufacture, processing or packaging of API, or any other matters required or requested to be provided to the FDA or any other Regulatory Authority. In addition, CAMBREX agrees to cooperate with RAPTOR (and/or
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obligations to submit or report information relevant to API pursuant to FDA regulations and other Applicable Laws. 

6.3 Drug Master Files. CAMBREX shall provide, or cooperate with RAPTOR to provide, the appropriate authorizations to each applicable
Regulatory Authority allowing RAPTOR (and/or its designee) the right to reference all Drug Master Files to support any regulatory filing for any Product developed, manufactured and/or commercialized by RAPTOR, its Affiliates and/or licensees. If the
[***] filed with the FDA as of the Effective Date is not sufficient to support the applicable regulatory filing for a Product, CAMBREX shall supplement such Drug Master File or file a separate Drug Master File(s) with the applicable Regulatory
Authority(ies) (including, if applicable, the EMEA,) as necessary to support such regulatory filing(s); provided that [***]. Any such new DMF filing will also require process validation and pilot work, and [***]. In addition, RAPTOR agrees to
purchase [***] of API manufactured for the purposes of performing such validation/pilot work. CAMBREX shall use commercially reasonable efforts to correct any deficiencies of such Drug Master File(s) identified by any Regulatory Authority in a
prompt and efficient manner so as to prevent any delay in RAPTOR (or any of its Affiliates or licensees) obtaining regulatory approval for a Product based on such Drug Master File(s). In addition, CAMBREX shall be responsible for maintaining such
Drug Master File(s) in accordance with Applicable Laws and ensuring that all data and information incorporated therein is accurate and current as necessary to support obtaining and maintaining the applicable regulatory filing(s) and regulatory
approval(s) by RAPTOR (and/or its designees) provided that [***]. 
 6.4 Recall. Any recalls of any of RAPTOR’s Products shall,
as between the Parties, be controlled solely by RAPTOR; provided, however, that if CAMBREX reasonably believes a recall may be necessary with respect to any API provided under this Agreement, CAMBREX shall immediately notify RAPTOR in writing.
CAMBREX shall provide assistance to RAPTOR (and/or its designee), as reasonably requested, in conducting such recall, including providing all pertinent records that may assist RAPTOR in effecting such recall. 

ARTICLE 7 
 TERM AND
TERMINATION 
 7.1 Term. The term of this Agreement shall commence on the Effective Date and shall continue for an initial
term of ten (10) years (“Initial Term”). Thereafter, this Agreement shall automatically be renewed for successive two (2) year periods (each, a “Renewal Term;” and all such Renewal Terms together with the
Initial Term, collectively, the “Term”), unless either Party notifies the other Party in writing at least one (1) year prior to the expiration of the then-current Term that such Party does not wish to renew this Agreement for
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 7.2 Termination for Material Breach. If either Party materially breaches this
Agreement or the Quality Agreement at any time, the non-breaching Party shall have the right to terminate this Agreement by written notice to the breaching Party, if such breach is not cured [***] after
written notice is given by the non-breaching Party to the breaching Party specifying the breach. 

7.3 Termination for Failure to Supply. Without limiting any other provision of this Agreement, including Sections 2.5 and 7.2 above, if
[***] Late Shipments of API occur in any [***], then RAPTOR shall have the right to terminate this Agreement immediately by written notice to CAMBREX. For purposes of this Section 7.3, a “Late Shipment” shall mean [***]. 

7.4 Termination by RAPTOR. RAPTOR may terminate this Agreement immediately upon written notice to CAMBREX if: (a) RAPTOR, in its
sole discretion, determines that Products will not be marketed by RAPTOR (or its designee); or (b) the FDA or EMEA withdraws approval of, or fails to approve, the manufacturing or marketing by RAPTOR (or its designee) of all Products then in
development. 
 7.5 Effects of Termination. It is understood that termination or expiration of this Agreement shall not relieve a
Party from any liability that, at the time of such termination or expiration, has already accrued to the other Party, except as specified in this Section 7.5. Upon expiration or termination of this Agreement for any reason (other than by RAPTOR
pursuant to Section 7.2 above), to the extent CAMBREX so notifies RAPTOR, RAPTOR shall have the obligation to purchase all API ordered under any outstanding Purchase Orders. [***]. 

7.6 Survival. The provisions of Sections 4.l, 5.1, 5.2, 7.5, 11.2, 11.3 and 11.4 and Articles 6, 8, 9 and 10 shall survive the
expiration or termination of this Agreement for any reason. In addition, the provisions of the Quality Agreement shall survive expiration or termination of this Agreement until the date of expiration of the last-to-expire batch of API delivered by CAMBREX to RAPTOR hereunder. All other rights and obligations of the Parties shall cease upon termination of this Agreement. Except as otherwise expressly provided in
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 ARTICLE 8 

CONFIDENTIALITY 

8.1 Confidential Information. Except as otherwise provided in this Article 8, during the Term and for a period of [***] thereafter,
each Party shall maintain in confidence and only use for the purposes of this Agreement any confidential information, data and/or materials supplied to such Party by the other Party (“Confidential Information”). A receiving
Party’s obligations under this Article 8 shall not apply to any information, data or material that, in each case as demonstrated by written documentation: (a) was already known to the receiving Party, other than under an obligation of
confidentiality, at the time of disclosure; (b) was generally available to the public or otherwise part of the public domain at the time of its disclosure to the receiving Party; (c) became generally available to the public or otherwise
part of the public domain after its disclosure and other than through any act or omission of the receiving Party in breach of this Agreement; (d) was subsequently lawfully disclosed to the receiving Party by a person other than the disclosing
Party; or (e) was independently developed by the receiving Party without reference to any Confidential Information of the disclosing Party. 

8.2 Confidentiality; Non-Disclosure. Each Party agrees not to disclose any Confidential
Information of the other Party except to those employees and consultants who have a need to know and provided that each person to whom Confidential Information is disclosed agrees to be bound by the same terms regarding the disclosure and use of
Confidential Information as set forth in this Article 8. Each Party further agrees not to use or disclose the Confidential Information of the other Party except as otherwise permitted by this Agreement, or as may be necessary to exercise its rights
or perform its obligations under this Agreement. Nothing contained in this Article 8 shall prevent either Party from disclosing any Confidential Information of the other Party to: (a) regulatory agencies for the purpose of obtaining approval to
distribute and market Products; provided, however, that all reasonable steps are taken to maintain the confidentiality of such Confidential Information to be disclosed; (b) to accountants, lawyers or other professional advisors or in connection
with a merger, acquisition, securities offering or other strategic transaction, subject in each case, to the recipient entering into an agreement to protect such Confidential Information from disclosure; or (c) is required by law or regulation
to be disclosed; provided, however, that the Party subject to such disclosure requirement has provided written notice to the other Party promptly upon receiving notice of such requirement in order to enable the other Party to seek a protective order
or otherwise prevent disclosure of such Confidential Information. 
 ARTICLE 9 

REPRESENTATIONS AND WARRANTIES 

9.1 Mutual Warranties. Each Party represents and warrants to the other Party that: (a) it has the power and authority to enter
into this Agreement and to perform its obligations hereunder and to grant to the other Party the rights granted to such other Party under this Agreement; (b) it has obtained all necessary corporate approvals to enter into and execute this
Agreement and to perform its obligations hereunder; and (c) the execution, delivery and performance of this Agreement does not conflict with any agreement, instrument or understanding, oral or written, to which it is a party

  
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or by which it may be bound, nor will it enter into or assume during the Term, any contract or other obligation with a third party that would in any way limit the performance of its obligations
under this Agreement. 
 9.2 CAMBREX Warranties. CAMBREX represents and warrants that: 

9.2.1.1 API. All API supplied hereunder shall comply with all Applicable Laws and the Quality Agreement and meet all Specifications,
and CAMBREX shall perform and document all manufacturing and supply activities contemplated herein in compliance with all Applicable Laws. 

9.2.1.2 Shelf Life. The API has a shelf life of [***]. All API supplied by CAMBREX under this Agreement shall have a shelf life of no
less than [***] at the time of delivery of such API to RAPTOR (or its designee). 
 9.2.1.3 Facilities and Equipment. The Facility,
all equipment used for the manufacture of API within the Facility and the activities contemplated herein will comply with all Applicable Laws and CAMBREX shall obtain and maintain all governmental registrations, permits, licenses and approvals
necessary for CAMBREX to manufacture and supply API to RAPTOR, and otherwise to perform its obligations, under this Agreement 
 9.2.1.4
No Encumbrance. Title to all API provided to RAPTOR under this Agreement shall pass as provided in this Agreement, free and clear of any security interest, lien, or other encumbrance. 

9.2.1.5 Personnel. Neither CAMBREX, nor any of its Affiliates, nor, to the best of CAMBREX’s knowledge, any of their respective
employees have been “debarred” by the FDA, or subject to a similar sanction from any Regulatory Authority in any jurisdiction outside the United States, nor have debarment proceedings against CAMBREX, any of its Affiliates, or any of their
respective employees been commenced. CAMBREX will promptly notify RAPTOR in writing if any such proceedings have commenced or if CAMBREX, any of its Affiliates, or any of their respective employees are debarred by the FDA or any other Regulatory
Authority. 
 9.3 RAPTOR Warranties. RAPTOR represents and warrants that it shall comply in all materials respects with all
Applicable Laws pertaining to the distribution, sale, and/or marketing of Product. 
 9.4 DISCLAIMER. EXCEPT AS PROVIDED IN THIS
ARTICLE 9, NEITHER PARTY MAKES ANY REPRESENTATIONS OR WARRANTIES (EXPRESS, IMPLIED, STATUTORY OR OTHERWISE) WITH RESPECT TO THE SUBJECT MATTER HEREOF AND EACH PARTY EXPRESSLY DISCLAIMS ANY SUCH ADDITIONAL REPRESENTATIONS AND WARRANTIES, INCLUDING
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 OF FITNESS FOR A PARTICULAR PURPOSE, MERCHANTABILITY OR
NON-INFRINGEMENT OF THIRD PARTY INTELLECTUAL PROPERTY RIGHTS. 
 ARTICLE 10 

INDEMNIFICATION AND LIMITATION OF LIABILITY 

10.1 RAPTOR. It is understood that CAMBREX has no control over the ultimate use of the API or Products. RAPTOR shall indemnify, defend
and hold harmless CAMBREX, its directors, officers, employees, agents, successors and assigns from and against any liabilities, expenses or costs (including reasonable attorneys’ fees and court costs) arising out of [***]. 

10.2 CAMBREX. CAMBREX shall indemnify, defend and hold harmless RAPTOR, its directors, officers, employees, agents, successors and
assigns from and against all liabilities, expenses, and costs (including reasonable attorneys’ fees and court costs) arising out of any [***]. 

10.3 Indemnification Procedure. Any Party seeking indemnification under this Article 10 (the “Indemnitee”) shall:
(a) promptly notify the indemnifying Party (the “Indemnitor”) of such claim; (b) provide the Indemnitor sole control over the defense and/or settlement thereof; and (c) at the Indemnitor’s request and expense,
provide full information and reasonable assistance to Indemnitor with respect to such claims. Without limiting the foregoing, with respect to claims brought under Section 10.1 or 10.2 above, the Indemnitee, at its own expense, shall have the
right to participate with counsel of its own choosing in the defense and/or settlement of any such claim. The indemnification under this Article 10 shall not apply to amounts paid in settlement of any claim if such settlement is effected without the
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 10.4 Insurance. During the Term and for a period of [***] thereafter, CAMBREX shall
maintain, with financially sound and reputable insurers, insurance reasonably sufficient to cover CAMBREX’s activities and obligations under this Agreement. Without limiting the foregoing, CAMBREX shall maintain: [***]. At the reasonable
request of RAPTOR, CAMBREX shall provide to RAPTOR copies of certificates of insurance evidencing coverage in accordance with this Section 10.4. 

10.5 LIMITATION OF LIABILITY. EACH PARTY’S LIABILITY SHALL BE LIMITED AS SET FORTH HEREIN AND IN NO EVENT SHALL EITHER PARTY BE
LIABLE FOR ANY SPECIAL, INCIDENTAL, INDIRECT, PUNITIVE, CONSEQUENTIAL OR EXEMPLARY OR PUNITIVE DAMAGES; INCLUDING LOST PROFITS, OR OPPORTUNITY OR GOODWILL, HOWEVER CAUSED, UNDER ANY THEORY OF LIABILITY AND EVEN IF SUCH PARTY HAS BEEN ADVISED OF THE
POSSIBILITY OF SUCH DAMAGES; PROVIDED HOWEVER THAT THE FOREGOING SHALL NOT BE DEEMED TO LIMIT THE INDEMNIFICATION OBLIGATIONS OF EITHER PARTY UNDER THIS ARTICLE 10 TO THE EXTENT A THIRD PARTY RECOVERS ANY PUNITIVE, EXEMPLARY, SPECIAL, CONSEQUENTIAL,
INCIDENTAL OR INDIRECT DAMAGES. CAMBREX’S MAXIMUM LIABILITY TO RAPTOR FOR EACH EVENT GIVING RISE TO ANY INJURIES, CLAIMS, LOSSES, EXPENSES, OR DAMAGES, INCLUDING, BUT NOT LIMITED TO EVENTS RESULTING FROM NEGLIGENCE, ERRORS, OMISSIONS OR STRICT
LIABILITY, SHALL NOT EXCEED [***]. To the extent that this clause conflicts with any other clause of this Agreement, this clause shall take precedence over such conflicting clause. If applicable law prevents enforcement of this Section 10.5,
then this Section shall be deemed modified to provide the maximum protection to each Party as is allowable under applicable law. 

ARTICLE 11 
 GENERAL
PROVISIONS 
 11.1 Assignment. The Parties agree that their rights and obligations under this Agreement may not be assigned
or otherwise transferred to a third party without the prior written consent of the other Party hereto. Notwithstanding the foregoing, either Party may transfer or assign its rights and obligations under this Agreement to a successor to all or
substantially all of its business or assets relating to this Agreement whether by sale, merger, operation of law or otherwise; provided that such assignee or transferee has agreed to be bound by the terms and 

  
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 conditions of this Agreement. Subject to the foregoing, this Agreement shall be binding upon and inure to the
benefit of the Parties hereto, their successors and assigns. 
 11.2 Governing Law. This Agreement shall be governed by, and
construed and interpreted in accordance with, the laws of the State of New York, as if entered into by New York residents and executed and wholly performed within the State of New York. 

11.3 Disputes. Except for any disputes with respect to non-conforming API, which shall be
resolved in accordance with Section 4.3 above, if CAMBREX and RAPTOR are unable to resolve any dispute between them, either CAMBREX or RAPTOR may, by written notice to the other, have such dispute referred to the senior management of CAMBREX
and RAPTOR for attempted resolution by good faith negotiations within [***] after such notice is received. If the Parties are unable to resolve such dispute in accordance with the aforementioned procedure or within such [***] period, subject to
Section 11.4 below, either Party shall have the right to pursue any and all other remedies available to such Party. 
 11.4
Arbitration. Except for any disputes with respect to non-conforming API, which shall be resolved in accordance with Section 4.3 above, any dispute or claim arising out of or in connection with this
Agreement or the performance, breach or termination thereof which is unable to be resolved pursuant to discussions between the Parties in accordance with Section 11.3 above, shall, upon notice by either Party to the other, be submitted to
binding arbitration in New York City, New York under the Rules of the Judicial Arbitration and Mediation Services, Inc. (or any successor entity thereto, collectively, “JAMS”) by one (1) arbitrator appointed in accordance with
said rules. The arbitrator may engage an independent expert with experience in the subject matter of the dispute to advise the arbitrator. The decision and/or award rendered by the arbitrator shall be written, final and non-appealable and may be entered in any court of competent jurisdiction. The Parties agree that, any provision of applicable law notwithstanding, they will not request, and the arbitrator shall have no authority to
award, punitive or exemplary damages against any Party. The costs of any arbitration, including administrative fees and fees of the arbitrator, shall be shared equally by the Parties, unless otherwise determined by the arbitrator. Each Party shall
bear the cost of its own attorneys’ and expert fees. Notwithstanding the foregoing, either Party may apply to any court of competent jurisdiction for injunctive relief without breach of this arbitration provision. 

11.5 Notices. Any notice or report required or permitted to be given or made under this Agreement by either Party shall be in writing
and in English and delivered to the other Party at its address indicated below (or to such other address as a Party may specify by like notice) by courier or by registered or certified airmail, postage prepaid, or by facsimile; provided, however,
that all facsimile notices shall be promptly confirmed, in writing, by courier or by registered or certified airmail, postage prepaid. All notices shall be effective as of the date received by the addressee. 

  
 [***] = CERTAIN
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 -15- 

 EXECUTION COPY 

 

					
		 	If to RAPTOR:	 	Raptor Therapeutics, Inc.
		 		 	 9 Commercial Boulevard
 Suite 200

Novato, CA 94949
 Attn: President

Fax: (415) 382-1368

			
		 	If to CAMBREX:	 	Cambrex Profarmaco Milano
		 		 	 Via Cucchiari 19
 20129 Milan, Italy

Attn: Aldo Magnini
 Fax: +39 02 33105730

			
		 		 	 Cambrex Corporation
 One Meadowlands Plaza

East Rutherford NJ 07073
 Attn: General Counsel

Fax: (201) 804-9852

 11.6 Force Majeure. Neither Party will be liable for its failure to perform any of its obligations
hereunder during any period in which such performance is delayed by acts of God, fire, war, embargo, riots, or other similar cause outside the reasonable control of such Party (“Force Majeure Event”). A Party affected by a Force
Majeure Event will promptly notify the other Party, explaining the nature and expected duration thereof and such Party shall use all reasonable efforts to remedy or mitigate such Force Majeure Event and the effects thereof. Notwithstanding the
foregoing, if a Party is unable to perform any of its obligations under this Agreement for a period of more than [***] as a result of a Force Majeure Event, the other Party may terminate this Agreement upon written notice to the affected Party. 

11.7 Interpretation. The headings to the several Articles and Sections of this Agreement are not a part of this Agreement, but are
included for convenience of reference only and shall not affect its meaning or interpretation. In this Agreement: (a) the word “including” shall be deemed to be followed by the phrase “without limitation” or like expression;
(b) the singular shall include the plural and vice versa; and (c) masculine, feminine and neuter pronouns and expressions shall be interchangeable. 

11.8 Waiver. Any waiver of the terms and conditions hereof must be explicitly in writing and executed by a duly authorized officer of
the Party waiving compliance. The waiver by either of the Parties of any breach of any provision hereof by the other shall not be construed to be a waiver of any succeeding breach of such provision or a waiver of the provision itself. The delay or
failure of any Party at any time to require performance of any provision of this Agreement shall in no manner affect such Party’s rights at a later time to enforce the same. 

  
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 EXECUTION COPY 

 

 11.9 Severability. Should any section, or portion thereof, of this Agreement be held
invalid or unenforceable in any jurisdiction by any court of competent authority or by a legally enforceable directive of any governmental body, such section or portion thereof shall be validly reformed so as to approximate the intent of the Parties
as nearly as possible and, if unreformable, shall be deemed divisible and deleted with respect to such jurisdiction, but the Agreement shall not otherwise be affected. 

11.10 Independent Contractors. The relationship of RAPTOR and CAMBREX established by this Agreement is that of independent contractors.
Nothing in this Agreement shall be construed to create a partnership, joint venture, agency or other fiduciary relationship between RAPTOR and CAMBREX. Neither Party shall have any right, power or authority to assume, create or incur any expense,
liability or obligation, express or implied, on behalf of the other. 
 11.11 Entire Agreement; Amendment. The terms and provisions
contained in the Agreement (including the Exhibits hereto and any Purchase Orders issued pursuant hereto) and the Quality Agreement constitute the entire agreement between the Parties and shall supersede all previous communications, representations,
agreements or understandings, either oral or written, between the Parties with respect to the subject matter hereof. No agreement or understanding varying or extending this Agreement shall be binding upon either Party hereto, unless set forth in a
writing which specifically refers to the Agreement signed by duly authorized officers or representatives of the respective Parties, and the provisions hereof not specifically amended thereby shall remain in full force and effect. 

11.12 Counterparts. This Agreement may be executed in counterparts, each of which shall be deemed an original, but which together shall
constitute one and the same instrument. 
 [Remainder of page intentionally left blank; signature page follows] 

  
 -17- 

 EXECUTION COPY 

 

 IN WITNESS WHEREOF, the Parties hereto have caused their duly authorized representatives to
execute this API Supply Agreement as of the Effective Date. 
  

									
	 RAPTOR THERAPEUTICS, INC.
	 		 	 CAMBREX PROFARMACO MILANO

					
	 By:
	 	 /s/ Thomas E. Daley
	 		 	By:	 	 /s/ Paolo Russolo

	 Name:
	 	 Thomas E. Daley
	 		 	 Name:
	 	 Paolo Russolo

	 Title:
	 	 President
	 		 	 Title:
	 	 President

 EXECUTION COPY 

 

 Exhibit 1.2 

[***] 

  
 [***] = CERTAIN
CONFIDENTIAL INFORMATION OMITTED 

 EXECUTION COPY 

 

 Exhibit 1.13 

Specifications 

 EXECUTION COPY 

 

 Exhibit 3.1 

Price 
 [***] 

  
 [***] = CERTAIN
CONFIDENTIAL INFORMATION OMITTED 

 Amendment to API Supply Agreement 

between Cambrex Profarmaco Milano, and Raptor Pharmaceuticals Inc. 

Background: This Amendment is made by and between Cambrex Profarmaco Milano, (“Cambrex”) and Raptor Pharmaceuticals Inc. (formerly
named Raptor Therapeutics, Inc.) (“Raptor”) pursuant to Section 11.11 of that certain API Supply Agreement dated November 3, 2010 by and between the parties (the “Agreement”). Cambrex and Raptor wish to amend the
Agreement to add Raptor’s wholly owned subsidiary, Raptor Pharmaceuticals Europe B.V., as an additional party to the Agreement. 
 NOW
THEREFORE in consideration of the premises hereof and other good and valuable consideration, the receipt and sufficiency of which are hereby acknowledged by the parties, the parties agree to amend the Agreement as follows: 

 

	1.	 Amendment to Agreement: 

Raptor Pharmaceuticals Europe B.V. (“Raptor BV”), a wholly owned subsidiary of Raptor, located at Naritaweg 165,
Telestone-Teleport, 1043 BW Amsterdam, the Netherlands, is hereby added as a party to the Agreement and will thereupon have all the rights and obligations of “RAPTOR” thereunder. All references to “RAPTOR” in the Agreement shall
refer to Raptor and/or Raptor BV, as applicable. Raptor BV may procure its API supply from Cambrex separately from Raptor. 
  

	2.	 API Supply. For the avoidance of doubt, the purchases made by both Raptor and Raptor BV in [***] shall
be included in determining whether RAPTOR has satisfied the [***] requirement set forth in Section 2.1 of the Agreement. 

  

	3.	 Forecasts. For the further avoidance of doubt, the Rolling Forecasts set forth in Section 2.2 of
the Agreement may be provided by either Raptor or Raptor BV. 

  

	4.	 No Other Modifications. The “Background” section of this document is incorporated into
the Amendment. Except as expressly amended by this Amendment, the terms and conditions of the Agreement shall remain in full force and effect. 

  

	5.	 Counterparts. This Amendment may be executed in two or more counterparts, each of which shall be deemed
an original and all or which together shall constitute one instrument. 

  

	6.	 Entire Agreement. The Agreement, as amended hereby, together with this Amendment, constitute the full,
complete, final and integrated agreement between the parties related to the subject matter hereof and thereof and supersede all previous written or oral negotiations, commitments, agreements, transactions or understandings concerning the subject
matter hereof. 

  
 [***] = CERTAIN
CONFIDENTIAL INFORMATION OMITTED 

 IN WITNESS WHEREOF, the parties hereto have caused this Amendment to be duly executed by
their authorized representatives, effective as of April 9, 2013. 
  

									
	 RAPTOR PHARMACEUTICALS INC.
	 		 	 CAMBREX PROFARMACO MILANO

					
	 By:
	 	 /s/ Georgia Erbez
	 		 	By:	 	 /s/ Paolo Russolo

	 Name:
	 	 Georgia Erbez
	 		 	 Name:
	 	 Paolo Russolo

	 Title:
	 	 CFO
	 		 	 Title:
	 	 President

		
	 RAPTOR PHARMACEUTICALS EUROPE B.V.
	 	
					
	 By:
	 	 /s/ Henk Doude von Troostwijk
	 		 		 	
	 Name:
	 	 Henk Doude von Troostwijk
	 		 		 	
	 Title:
	 	 Director B
	 		 		 	
					
	 By:
	 	 /s/ Kim R. Tsuchimoto
	 		 		 	
	 Name:
	 	 Kim R. Tsuchimoto
	 		 		 	
	 Title:
	 	 Director A

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