Document:

Exhibit 4.6

 

Cooperatieve AAC LS U.A.

Forbion Co-Investment Coöperatief U.A.

Forbion Co-Investment Ii Coöperatief U.A.

Coöperatieve Gilde Healthcare II U.A.

S.J.H. VAN DEVENTER C.V.

 

CREDIT Lyonnais Innovation 6

LCL Innovation 1

CLVC

CLV1

 

ADVENT PRIVATE EQUITY FUND IV

ADVENT MANAGEMENT IV LP

 

TIVERINA INVERSIONES S.L.

ABADAI INVERSIONES S.L.

SURRIC INVERSIONES S.L.

HILOS Y POLO S.L.,

RAMON MOR

A-FIGUEROA MORA-FIGUEROA

ACTIVOS Y TENENCIAS 85 S.L.

FUNDACION PARA EL

DESARROLLO Y LA COOPERACION INTERNACIONAL

FUNDACION UNIVERSITARIA DE NAVARRA

 

JOSE LUIS PASCUAL PLAZA

LUPUS ALPHA MICRO CHAMPIONSs

LUPUS ALPHA ALL OPPERTUNITIES FUND

 

AND

 

STICHTING ADMINISTRATIEKANTOOR uniQure B.V.

 

AND

 

CHIESI FARMACEUTICI S.p.A.

 

AND

 

UNIQURE B.V.

 

 

CLASS C SHAREHOLDERS AGREEMENT

 

relating to uniQure B.V. 

 

 

 

TABLE OF CONTENTS

 

	
Clause
    	
 
    	
Headings
    	
 
    	
Page
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
1.
    	
 
    	
INTERPRETATION
    	
 
    	
5
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
2.
    	
 
    	
DIVIDEND POLICY
    	
 
    	
7
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
3.
    	
 
    	
TRANSFERS OF SHARES
    	
 
    	
7
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
 
    	
 
    	
3.1.
    	
General conditions Transfer
    	
 
    	
7
    
	
 
    	
 
    	
3.2.
    	
Tag Along
    	
 
    	
7
    
	
 
    	
 
    	
3.3.
    	
Drag Along
    	
 
    	
8
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
4.
    	
 
    	
ASSET SALE OR EXIT
    	
 
    	
8
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
5.
    	
 
    	
CONFIDENTIALITY
    	
 
    	
9
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
6.
    	
 
    	
TERM AND TERMINATION
    	
 
    	
10
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
 
    	
 
    	
6.1.
    	
Term
    	
 
    	
10
    
	
 
    	
 
    	
6.2.
    	
Termination
    	
 
    	
10
    
	
 
    	
 
    	
6.3.
    	
Surviving Clauses
    	
 
    	
10
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
7.
    	
 
    	
MISCELLANEOUS
    	
 
    	
10
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
 
    	
 
    	
7.1.
    	
 
    	
 
    
	
 
    	
 
    	
7.2.
    	
Conflict
    	
 
    	
10
    
	
 
    	
 
    	
7.3.
    	
Additional Investors
    	
 
    	
10
    
	
 
    	
 
    	
7.4.
    	
Notices
    	
 
    	
11
    
	
 
    	
 
    	
7.5.
    	
Amendment
    	
 
    	
11
    
	
 
    	
 
    	
7.6.
    	
Assignment
    	
 
    	
11
    
	
 
    	
 
    	
7.7.
    	
Entire Agreement
    	
 
    	
11
    
	
 
    	
 
    	
7.8.
    	
Partial Invalidity
    	
 
    	
11
    
	
 
    	
 
    	
7.9.
    	
Compensation of costs
    	
 
    	
11
    
	
 
    	
 
    	
7.10.
    	
No Waiver
    	
 
    	
11
    
	
 
    	
 
    	
7.11.
    	
No Rescission
    	
 
    	
12
    
	
 
    	
 
    	
7.12.
    	
Counterparts
    	
 
    	
12
    
	
 
    	
 
    	
7.13.
    	
Governing Law
    	
 
    	
12
    
	
 
    	
 
    	
7.14.
    	
Jurisdiction
    	
 
    	
12
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
SCHEDULE A (PARTIES)
    	
 
    
	
 
    	
 
    
	
SCHEDULE B (ACCESSION AGREEMENT)
    	
 
    

 

1

 

THIS CLASS C SHAREHOLDERS AGREEMENT (the “Agreement”) is made on July 8, 2013.

 

BETWEEN:

 

(1)                                Coöperatieve AAC LS U.A., a coöperative (coöperatie) incorporated under the laws of the Netherlands with its corporate seat in Amsterdam, and its business address at Gooimeer 2 35, 1411 DC Naarden, the Netherlands and registered with the Commercial Register of Gooi-, Eem- en Flevoland under number 34256402 (the “Existing Investor I”);

 

(2)                                Forbion Co-Investment Coöperatief U.A., a coöperative (coöperatie) incorporated under the laws of the Netherlands with its registered office in Naarden, and its business address at Gooimeer 2 35, 1411 DC Naarden, the Netherlands and registered with the Commercial Register of Gooi-, Eem- en Flevoland under number 32142360 (the “Existing Investor II”);

 

(3)                                Forbion Co-Investment II Coöperatief U.A., a coöperative (coöperatie) incorporated under the laws of the Netherlands with its registered office in Naarden, and its business address at Gooimeer 2 35, 1411 DC Naarden, the Netherlands and registered with the Commercial Register of Gooi-, Eem- en Flevoland under number 53958713 (the “Existing Investor III”);

 

(4)                                Coöperatieve Gilde Healthcare II U.A., a coöperative (coöperatie) incorporated under the laws of the Netherlands with its corporate seat in Utrecht, and its business address at Newtonlaan 91 (3584 BP), the Netherlands and registered with the Commercial Register of Midden-Nederland under number 30216414 (the “ Existing Investor IV “);

 

(5)                                S.J.H. VAN DEVENTER C.V., a Dutch limited partnership (commanditaire vennootschap), having its registered address at 1411 DC Naarden, Gooimeer 2 35, registered in the trade register under number 32158843 (the “Existing Investor V”);

 

(6)                                CREDIT LYONNAIS INNOVATION 6, a French venture capital fund (Fonds Commun de Placement dans l’Innovation), represented herein by its management company (société de gestion), Omnes Capital (formerly named Crédit Agricole Private Equity), a French corporation (société par actions simplifiée) with a share capital of 8,000,000 euros, the registered office of which is located at 37-41 rue du Rocher — 75008 Paris, France, registered with the French Registry of Commerce and Companies under number 428 711 196 RCS Paris (the “Existing Investor VI”);

 

(7)                                LCL INNOVATION 1, a French venture capital fund (Fonds Commun de Placement dans l’Innovation), represented herein by its management company (société de gestion), Omnes Capital (formerly named Crédit Agricole Private Equity), a French corporation (société par actions simplifiée) with a share capital of 8,000,000 euros, the registered office of which is located at 37-41 rue du Rocher — 75008 Paris, France, registered with the French Registry of Commerce and Companies under number 428 711 196 RCS Paris (the “Existing Investor VII”);

 

(8)                                CLVC (formerly named Crédit Lyonnais Venture Capital), a French corporation (Société anonyme) with a share capital of 14,786,948 euros, with its registered office located at 37-41 rue du Rocher — 75008 Paris, registered with the French Registry of Commerce and 

 

2

 

Companies under number 434 465 514 RCS Paris, represented herein by Omnes Capital (formerly named Crédit Agricole Private Equity), a French Corporation (société par actions simplifiée) with a share capital of 8,000,000 euros, the registered office of which is located at 37-41 rue du Rocher — 75008 Paris, France, registered with the French Registry of Commerce and Companies under number 428 711 196 RCS Paris (the “Existing Investor VIII”);

 

(9)                                CLV1 (formerly named Crédit Lyonnais Venture 1), a French venture capital fund (Fonds Commun de Placement à Risques), represented herein by its managing partner, Omnes Capital (formerly named Crédit Agricole Private Equity), a French corporation (société par actions simplifiée) with a share capital of 8,000,000 euros, the registered office of which is located at 37-41 rue du Rocher — 75008 Paris, France, registered with the French Registry of Commerce and Companies under number 428 711 196 RCS Paris (the “Existing Investor IX”);

 

(10)                         ADVENT PRIVATE EQUITY FUND IV, an English partnership, incorporated and existing under the laws of the United Kingdom, having its registered address at 25 Buckingham Gate, London, SW1E 6LD, United Kingdom and registered in England and Wales under number LP10002 (the “Existing Investor X”);

 

(11)                         ADVENT MANAGEMENT IV LP, a Scottish limited partnership, incorporated and existing under the laws of Scotland, having its registered address at 50 Lothian Road, Festival Square, Edinburgh, EH3 9WJ, United Kingdom and registered in Scotland under number SL005366 (the “Existing Investor XI”);

 

(12)                         TIVERINA INVERSIONES S.L., a Spanish private company with limited liability, having its place of business at Calle Zurbano no76 6a, Madrid, Spain, with Tax Identification Number B-85381960 (the “Existing Investor XII”);

 

(13)                         ABADAI INVERSIONES S.L., a Spanish private company with limited liability, having its place of business at Calle Zurbano no76 6a, Madrid, Spain, with Tax Identification Number B-85383511 (the “Existing Investor XIII”);

 

(14)                         SURRIC INVERSIONES S.L., a Spanish private company with limited liability, having its place of business at Calle Zurbano no76 6a, Madrid, Spain, with Tax Identification Number B-85381507 (the “Existing Investor XIV”);

 

(15)                         HILOS Y POLO S.L., a Spanish private company with limited liability, having its place of business at Calle Zurbano no76 6a, Madrid, Spain, with Tax Identification Number B-83914481(the “Existing Investor XV”);

 

(16)                         RAMON MORA-FIGUEROA MORA-FIGUEROA, with professional domicile at Madrid, Calle Zurbano no76 6a and holder of a National Identity Card with number  31.241.827-F(the “Existing Investor XVI”);

 

(17)                         ACTIVOS Y TENENCIAS 85 S.L., a Spanish private company with limited liability, having its place of business at Calle Ana Teresa 85 B, Madrid Spain, with Tax identification number B-84366111(the “Existing Investor XVII”);

 

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(18)                         FUNDACION PARA EL DESARROLLO Y LA COOPERACION INTERNACIONAL, having its place of business at Calle Jose Abascal no44 2o, Madrid, Spain, with Tax identification number G-80787161(the “Existing Investor XVIII”);

 

(19)                         FUNDACION UNIVERSITARIA DE NAVARRA, having its place of business at Avd. Pio XII 53 1o, Pamplona, Spain, with Tax Identification Number G-31469125(the “Existing Investor XIX”);

 

(20)                         JOSE LUIS PASCUAL PLAZA, with professional domicile at Madrid, Calle del Pastor 4 and holder of a National Identity Card with number 02.153.208-V (the “Existing Investor XX”);

 

(21)                         LUPUS ALPHA MICRO CHAMPIONS, a Sub Fund of Lupus alpha Fonds, a Fonds Commun de placement under Luxembourg law, for which and on behalf wherof Lupus alpha Investment S.A., 69, route d’Esch, L-1470 Luxembourg, R.C.S. Luxembourg B-79272 (Management Company) acts (the “Existing Investor XXI”);

 

(22)                         LUPUS ALPHA ALL OPPORTUNITIES FUND, a Sub Fund of Lupus alpha Fonds, a Fonds Commun de placement under Luxembourg law, for which and on behalf wherof Lupus alpha Investment S.A., 69, route d’Esch, L-1470 Luxembourg, R.C.S. Luxembourg B-79272 (Management Company) acts (the “Existing Investor XXII”);

 

and

 

(23)                         Stichting Administratiekantoor uniQure B.V., a foundation organised under the laws of the Netherlands, having its registered office in Amsterdam, at Meibergdreef 61, 1105 BA Amsterdam Zuidoost, the Netherlands and registered with the Commercial Register of Amsterdam under number 55055036 (the “Trust Foundation”);

 

(24)                         Chiesi Farmaceutici S.p.A an Italian corporation, with its offices at Via Palermo, 26/A, 43122 Parma, Italy,(the “New Investor” or “Chiesi”)

 

and

 

(25)                         uniQure B.V., a private company with limited liability (besloten vennootschap met beperkte aansprakelijkheid), incorporated under the laws of the Netherlands with its registered office in Amsterdam, and its business address at Meibergdreef 61, 1105 BA Amsterdam Zuidoost, the Netherlands and registered with the Commercial Register of Amsterdam under number 54385229 (the “Company”);

 

The parties to this Agreement are hereinafter collectively referred to as the “Parties” and individually as a “Party”. Further details of the Parties are set out in SCHEDULE A (Parties). The Existing Investor I up to and including XXII and IV and the Trust Foundation are hereinafter jointly referred to as the “Existing Investors”. The Existing Investors and the New Investor, together with any other holder from time to time of Ordinary Shares Class A, Class B and Class C in the capital of the Company, are hereinafter collectively referred to as the “Investors” and individually as an “Investor”.

 

4

 

RECITALS:

 

(A)                              The New Investor and the Company have entered into that certain “Subscription Agreement” on 29 April 2013, pursuant to which the Company has agreed to issue Ordinary Shares Class C to Chiesi and Chiesi agreed to take new Class C Shares (the “Transaction”).

 

(B)                              The Company is engaged in the development of human gene based therapies.

 

(C)                              In this Agreement the Parties wish to set out the terms and conditions on which they have agreed to regulate the rights and obligations of the New Investor with respect to the ordinary shares Class C it holds from time to time.

 

IT IS AGREED as follows:

 

1.                                     INTERPRETATION

 

1.1.                           In this Agreement, the following definitions are used:

 

“Agreement” means this shareholders agreement including schedules and appendices thereto as amended in accordance with its terms.

 

“Articles of Association” means the articles of association of the Company, as amended from time to time.

 

“Asset Sale” has the meaning given in Clause 4.2.

 

“Business Day” means a day (other than a Saturday or a Sunday) on which banks in the Netherlands are open for normal business.

 

“Business Acquisition Agreement” has the meaning given in the recitals of this Agreement.

 

“Called Shareholders” has the meaning given in Clause 3.3.1.

 

“Called Shares” has the meaning given in Clause 3.3.1.

 

“Company” has the meaning given in the opening of this Agreement.

 

“DCC” means the Dutch Civil Code.

 

“Drag Along Notice” has the meaning given in Clause 3.3.2.

 

“Drag Along Option” has the meaning given in Clause 3.3.1.

 

“Existing Investor I up to and including XXII “ has the meaning given in the opening of this Agreement.

 

“Existing Investors” has the meaning given in the opening of this Agreement.

 

“Exit” has the meaning given in Clause 4.2.

 

“Group” means the Company together with its Subsidiaries.

 

5

 

“Investor” and “Investors” has the meaning given in the opening of this Agreement.

 

“Listing” has the meaning given in Clause 4.2.

 

“Liquidation” means a liquidation, dissolution or winding-up (liquidatie of ontbinding) of the Company.

 

“New Investor “ has the meaning given in the opening of this Agreement.

 

“Ordinary Shares Class A” means the ordinary shares class A (gewone aandelen) in the capital of the Company with a nominal value of EUR 0.01 each.

 

“Ordinary Shares Class B” means the  ordinary shares class B (gewone aandelen) in the capital of the Company with a nominal value of EUR 0.01 each.

 

“Ordinary Shares Class C” means  ordinary shares class C (gewone aandelen) in the capital of the Company with a nominal value of EUR 0.01 each.

 

“Sale” has the meaning given in Clause 3.2.1.

 

“Selling Investor” and “Selling Investors” has the meaning given in Clause 3.2.1.

 

“Shareholder” means any holder of Shares.

 

“Shares” means the issued and outstanding shares from time to time in the capital of the Company, consisting of Ordinary Shares Class A, Ordinary Shares Class B and Ordinary Shares Class C .

 

“Subsidiary” has the meaning given in article 2:24a of the DCC to the term “dochtermaatschappij”.

 

“Tag Along Notice” has the meaning given in Clause 3.2.1.

 

“Tag Offer” has the meaning given in Clause 3.2.3.

 

“Third Party Purchaser” has the meaning given in Clause 3.2.1.

 

“Transaction” has the meaning given in the recitals of this Agreement.

 

“Trust Foundation” has the meaning given in the opening of this Agreement.

 

1.2.                           In this Agreement, unless otherwise specified:

 

1.2.1.                           the masculine gender shall include the feminine and the neuter and vice versa;

 

1.2.2.                           references to a person shall include a reference to any individual, company, association, partnership, trust or joint venture (in each case whether or not having separate legal personality);

 

1.2.3.                           references to “include” and “including” shall be treated as references to “include without limitation” or “including without limitation”;

 

6

 

1.2.4.                           unless the context requires otherwise, words in the singular shall include the plural and vice versa;

 

1.2.5.                           the headings are for identification only and shall not affect the interpretation of this Agreement.

 

2.                                     DIVIDEND POLICY

 

2.1.1.                           The Shareholders are entitled to the distribution of the profits of the Company for each Shareholder in proportion to the number of Shares that it holds.

 

2.1.2.                           The Company shall apply profits available for distribution for its further development and expansion prior to making any distributions to Shareholders.

 

3.                                     TRANSFERS OF SHARES

 

3.1.                           General conditions Transfer

 

3.1.1.                           The New Investor shall not be authorised to transfer any of the Shares it holds, unless provided otherwise in this Agreement.

 

3.2.                           Tag Along

 

3.2.1.                           If an Investor or group of Investors holding 51% or more of the issued and outstanding Ordinary Shares Class A (the “Selling Investor” or “Selling Investors” as the case may be), that wishes (or wish) to sell any or all of the Shares held by it (or by them) to a person or any other person acting in concert with that person (a “Third Party Purchaser”) (a “Sale”), the Selling Investor(s) shall give not less than 15 Business Days advance notice of the proposed Sale to the Company and the New Investor (a “Tag Along Notice”).

 

3.2.2.                           The Tag Along Notice shall:

 

(A)                              specify the number of Shares the Selling Investor(s) propose(s) to sell;

 

(B)                              specify the name of the Third Party Purchaser;

 

(C)                              specify the price (in cash or otherwise) per Share that the Third Party Purchaser is proposing to pay;

 

(D)                              specify the proposed date of transfer;

 

(E)                               be governed by the laws of the Netherlands;

 

(F)                                specify the address where the counter notice should be sent.

 

3.2.3.                           As a condition to such Sale, the New Investor shall be entitled within 15 Business Days after receipt of the Tag Along Notice to notify the Selling Investor(s) that it wishes to sell a pro rata parte portion of the Shares held by it at the proposed price by sending a counter notice to the address specified in the Tag Along Notice (the “Tag Offer”).

 

7

 

3.3.                           Drag Along

 

3.3.1.                           In case a Selling Investor or Selling Investors, as the case may be, wish(es) to sell all, and for the avoidance of doubt, not less than all of the Shares held by it (or by them), to a Third Party Purchaser, the Selling Investor(s) shall have the right (the “Drag Along Option”), upon agreement on the terms and conditions of a bona fide offer by a Third Party Purchaser, to require all other Shareholders (the “Called Shareholders”) to sell and transfer all, and for the avoidance of doubt, no less than all, of their Shares (the “Called Shares”) to the proposed Third Party Purchaser in accordance with the provisions of this Clause 3.3.

 

3.3.2.                           The Selling Investor(s) that wish(es) to exercise the Drag Along Option shall give notice thereof in writing (the “Drag Along Notice”) to the Company and the Called Shareholders.

 

3.3.3.                           The transfer of the Called Shares to the Third Party Purchaser shall be completed within sixty (60) Business Days after service of the Drag Along Notice. The Called Shareholders shall be released from their obligation to transfer the Called Shares to the Third Party Purchaser if the Third Party Purchaser has not acquired the Called Shares on the expiration of such sixty (60) Business Day period.

 

3.3.4.                           If a Called Shareholder fails to cooperate with the transfer of its Called Shares to the Third Party Purchaser within a period of ten (10) Business Days from the proposed date of transfer, the Selling Investor(s) shall have the power and the duty to fulfil the obligations for and on behalf of the defaulting Called Shareholder.

 

3.3.5.                           Each Shareholder hereby grants an unconditional and irrevocable power of attorney to the Company to act on its behalf and to transfer the relevant Called Shares to the proposed purchaser in accordance with the provisions of this Clause 3.3.

 

3.3.6.                           The provisions of Clause 3.2 (Tag Along) do not apply to a (proposed) transfer of Shares of which a Drag Along Notice has been duly served pursuant to this Clause 3.3.

 

4.                                     ASSET SALE OR EXIT

 

4.1.                           It is the intention of the Parties that an Asset Sale or an Exit in particular a Listing (as defined in 4.2) be achieved as soon as practically possible and commercially sensible.

 

4.2.                           An Exit (“Exit”) shall be:

 

·                                                   a transfer (or a series of related transfers) of all the Shares issued and outstanding;

 

·                                                   the listing and admission to trading on a market for listed securities of either (i) the Shares, (ii) an intermediate holding company’s shares or (iii) the shares of new holding company established for the purposes of the Listing (a “Listing”); or

 

·                                                   a distribution pursuant to a winding-up or dissolution of the Company or any holding company of the Company, including following an Asset Sale.

 

8

 

4.3.                           An Asset Sale (“Asset Sale”) shall be a sale by the Company and/or one or more of its Subsidiaries of all, or substantially all, of the Group’s business, assets and undertaking.

 

4.4.                           A decision to achieve an Asset Sale requires the approval of the general meeting of shareholders of the Company and the affirmative vote of the Investors representing at least 51% of Ordinary Shares Class A.

 

4.5.                           Each Party (taking into account its rights and obligations under this Agreement) shall take all steps that are required to ensure the success of any proposed Exit or Asset Sale, subject always to fiduciary duties and compliance with applicable law, including that:

 

(A)                                        each Shareholder shall dispose of (a pro rata parte portion of) its Shares on the same terms and conditions as the other Shareholders; and

 

(B)                                        each of the Shareholders shall on a Listing, retain such number of Shares held at the time of the Listing for such period after the Listing as is required by the relevant listing rules or is recommended by the Company’s financial advisors in such Listing.

 

5.                                     CONFIDENTIALITY

 

5.1.                            Subject to Clause 5.2, each Party shall treat as strictly confidential and not disclose or use any information relating to this Agreement or any ancillary matter and the negotiations leading up to this Agreement and including the disclosure or use of any information relating to the Group and its business operations, unless provided otherwise in this Agreement.

 

5.2.                            The restrictions contained in Clause 5.1 shall not apply if and to the extent:

 

(A)                                        disclosure is required by any law or by a court;

 

(B)                                        disclosure is required by any securities exchange or regulatory or governmental body;

 

(C)                                        disclosure is necessary to enforce this Agreement in court proceedings.

 

(D)                                        the other Parties have given their written consent to disclosure;

 

(E)                                         the information has come into the public domain through no fault of the relevant Party’s group;

 

(F)                                          disclosure is necessary to obtain the advice of any professional adviser;

 

(G)                                        disclosure is necessary within the relevant Party’s group.

 

In the event of a disclosure of information pursuant to Clause 5.2 (A) or (B), the disclosing Party shall consult with the other Parties (to the extent permitted by applicable laws or regulations) as to the contents, form and timing of the disclosure to be made.

 

5.3.                            The restrictions contained in this Clause 5.1 shall apply to each Party (as applicable) during the term of this Agreement and shall remain in full force and effect after a Shareholder ceases to be a Shareholder.

 

9

 

6.            TERM AND TERMINATION

 

6.1.         Term

 

This Agreement shall remain in full force and effect from the date of this Agreement until terminated in accordance with the terms of Clause 6.2.

 

6.2.         Termination

 

6.2.1.         This Agreement can be terminated by unanimous consent of all Parties in writing.

 

6.2.2.         This Agreement shall automatically terminate upon:

 

(A)          completion of an Exit or Asset Sale;

 

(B)          completion of a Liquidation;

 

(C)          acquisition by one Shareholder of all Shares;

 

(D)          disposal by the New Investor of all the Shares it holds without prejudice of article 11 of the Company’s articles of association.

 

6.2.3.         This Agreement shall terminate in respect of a Shareholder from the date it ceases to be a Shareholder.

 

6.3.         Surviving Clauses

 

Termination of this Agreement shall be without prejudice to:

 

6.3.1.         any right, liability or obligation accrued under this Agreement but not satisfied or discharged at the date of termination; and

 

6.3.2.         the provisions of the Clauses 5 (Confidentiality), 7.3 (Notices), 7.12 (Governing Law) and 7.13 (Jurisdiction), which will remain in full force and effect.

 

7.            MISCELLANEOUS

 

7.1.         Conflict

 

Subject to applicable law, in case of an ambiguity or a conflict between provisions of the Articles of Association and provisions of this Agreement, the provisions of this Agreement shall prevail.

 

7.2.         Additional Investors

 

7.2.1.         No issue or transfer of Ordinary Shares Class C to any person who is not a Party to this Agreement shall be effectuated without first obtaining from such person a duly signed Accession Agreement in the form of SCHEDULE B (Accession Agreement), provided that the Parties to this Agreement will procure and will cooperate to sign such Accession Agreement in respect of any person that is 

 

10

 

                                                         permitted to acquire Ordinary Shares Class C pursuant to this Agreement and to whom it is envisaged to transfer such Ordinary Shares Class C.

 

7.2.2.         SCHEDULE A (Parties) shall be updated upon an issue or transfer of Shares to properly reflect the relevant changes.

 

7.3.         Notices

 

All notices, consents, waivers and other communications under this Agreement must be in writing in Dutch or in English and delivered by hand or sent by registered mail, express courier, fax or e-mail to such addresses and fax numbers as a Party may notify to the other Parties from time to time. A notice shall be effective upon receipt and shall be deemed to have been received at the time of delivery, if delivered by hand, registered mail or express courier, or at the time of successful transmission, if delivered by fax or e-mail.

 

7.4.         Amendment

 

This Agreement may only be amended by unanimous consent of all Parties in writing.

 

7.5.         Assignment

 

None of the Parties may assign or procure the assumption of its rights and obligations under this Agreement, either in whole or in part, to any other person without the prior written consent of the other Parties.

 

7.6.         Entire Agreement

 

This Agreement constitutes the entire agreement between the Parties with respect to the subject matter of this Agreement. This Agreement supersedes any and all earlier agreements, either verbally or in writing, between the Parties in relation to the subject matter of this Agreement.

 

7.7.         Partial Invalidity

 

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 such invalid or unenforceable provision shall be replaced or be deemed to be replaced by a provision that is considered to be valid and enforceable. The interpretation of the replacing provisions shall be as close as possible to the intent of the invalid or unenforceable provision.

 

7.8.         Compensation of costs

 

Each of the Parties hereto shall pay its own expenses incurred or to be incurred in connection with this Agreement and matters incidental to this Agreement, except for the expenses incurred or to be incurred by the Trust Foundation, the Existing Investors and the New Investor I, which expenses shall be borne by the Company.

 

7.9.         No Waiver

 

No failure by any Party to exercise, and no delay in exercising, any right under this Agreement, in the event of breach of contract by any Party hereto, will operate as a waiver of such right or any other right under this Agreement.

 

11

 

7.10.       No Rescission

 

The Parties waive their right to rescind (ontbinden) this Agreement pursuant to article 2:265 DCC after Completion.

 

7.11.      Counterparts

 

This Agreement may be signed in any number of counterparts each of which, when executed by one or more of the Parties, shall constitute an original.

 

7.12.       Governing Law

 

This Agreement is governed by the laws of the Netherlands.

 

7.13.       Jurisdiction

 

All disputes arising in connection with this Agreement shall be finally settled in accordance with the rules of the Netherlands Arbitration Institute (Nederlands Arbitrage Instituut) and:

 

7.13.1.       the arbitral tribunal shall be composed of three arbitrators;

 

7.13.2.       the place of arbitration will be Amsterdam, the Netherlands;

 

7.13.3.       the language of the proceedings will be English (unless the Parties agree otherwise);

 

7.13.4.       the arbitrators will decide according to the rules of Dutch law;

 

7.13.5.       the arbitral award will be final and binding;

 

7.13.6.       to ensure that the arbitral award shall not be published, each Party shall notify the administrator of the NAI within one calendar month after receipt of the arbitral award that they object to publication of the arbitral award by the NAI;

 

7.13.7.       the proceedings shall not be consolidated with other arbitral proceedings pursuant to Article 1046 of the Dutch Code of Civil Procedure.

 

[signature pages to follow]

 

12

 

This Agreement has been entered into on the date first above written.

 

	
Coöperatieve   AAC LS U.A.  
    	
 
    	
Coöperatieve   AAC LS U.A.    
    
	
 
    	
 
    	
 
    
	
/s/H.A. Slootweg
    	
 
    	
/s/M.A. van Osch  
    
	
By: Forbion 1   Management B.V.  
    	
 
    	
By: Forbion 1   Management B.V.                
    
	
 
    	
 
    	
 
    
	
Title: 
    	
Director
    	
 
    	
Title: 
    	
Director  
    
	
By: 
    	
H.A. Slootweg
    	
 
    	
By: 
    	
M.A. van Osch
    
	
Title: 
    	
Director
    	
 
    	
Title: 
    	
Director
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
Forbion   Co-Investment Coöperatief U.A.
    	
 
    	
Forbion   Co-Investment Coöperatief U.A.
    
	
 
    	
 
    	
 
    
	
/s/H.A. Slootweg
    	
 
    	
/s/M.A. van Osch
    
	
By:  Forbion   1 Management B.V.
    	
 
    	
By: Forbion 1 Management B.V.
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
Title: 
    	
Director
    	
 
    	
Title: 
    	
Director
    
	
By:  
    	
H.A. Slootweg
    	
 
    	
By: 
    	
M.A. van   Osch  
    
	
Title: 
    	
Director
    	
 
    	
Title: 
    	
Director
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
Forbion   Co-Investment II Coöperatief U.A.
    	
 
    	
Forbion   Co-Investment II Coöperatief U.A.
    
	
 
    	
 
    	
 
    
	
/s/H.A. Slootweg
    	
 
    	
/s/M.A. van Osch
    
	
By: Forbion 1 Co II   Management B.V.
    	
 
    	
By: Forbion 1 Co II   Management B.V.
    
	
 
    	
 
    	
 
    
	
Title: 
    	
Director
    	
 
    	
Title: 
    	
Director
    
	
By:  
    	
H.A. Slootweg
    	
 
    	
By:  
    	
M.A. van Osch
    
	
Title: 
    	
Director
    	
 
    	
Title: 
    	
Director
    
	
 
    	
 
    
	
 
    	
 
    
	
S.J.H. VAN DEVENTER C.V.
    	
 
    
	
 
    	
 
    
	
/s/H.A. Slootweg
    	
 
    
	
By:  S.J.H. van Deventer C.V.
    	
 
    
	
Forbion Capital Partners Management Services   B.V. (text illegible) 
    
	
Title: 
    	
Director
    	
 
    
	
By:  
    	
H.A. Slootweg
    	
 
    
	
Title: 
    	
Proxyholder
    	
 
    
						

 

13

 

	
Coöperatieve   Gilde Healthcare II U.A.
    	
 
    	
Coöperatieve Gilde Healthcare II U.A.
    
	
 
    	
 
    	
 
    
	
/s/Edwin de   Graaf
    	
 
    	
/s/ Pieter van der Meer
    
	
By: 
    	
Gilde Healthcare II Management B.V.
    	
 
    	
By:  
    	
Gilde Healthcare II Management B.V.
    
	
 
    	
 
    	
 
    
	
Title:
    	
 Director
    	
 
    	
Title:  
    	
Director
    
	
By:
    	
Edwin de Graaf
    	
 
    	
By: 
    	
Pieter van der Meer
    
	
Title: 
    	
 
    	
 
    	
Title:
    	
 
    

 

14

 

	
Credit   Lyonnais Innovation 6
    	
 
    	
Credit Lyonnais Innovation 6
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
/s/Fabien Prévost
    
	
By: 
    	
Omnes Capital
    	
 
    	
By: 
    	
 Omnes Capital
    
	
 
    	
 
    	
 
    
	
Title:
    	
Managing Company
    	
 
    	
Title:  
    	
Managing Company
    
	
By:
    	
 
    	
 
    	
By:  
    	
Fabien Prévost
    
	
Title:
    	
 
    	
 
    	
Title: 
    	
CFO
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
CLVC
    	
 
    	
CLVC
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
/s/Fabien Prévost
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By:
    	
 
    	
 
    	
By:  
    	
Fabien Prévost
    
	
Title:
    	
 
    	
 
    	
Title: 
    	
CFO
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
LCL   INNOVATION 1
    	
 
    	
LCL INNOVATION 1
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
/s/Fabien Prévost
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title: 
    	
Managing Company
    	
 
    	
Title:  
    	
Managing Company
    
	
By:
    	
 
    	
 
    	
By:  
    	
Fabien Prévost
    
	
Title:
    	
 
    	
 
    	
Title: 
    	
CFO
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
CLV1
    	
 
    	
CLV1
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
/s/Fabien Prévost
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title: 
    	
Managing Company
    	
 
    	
Title:  
    	
Managing Company
    
	
By:
    	
 
    	
 
    	
By:  
    	
Fabien Prévost
    
	
Title:
    	
 
    	
 
    	
Title:
    	
CFO
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
LCL   INNOVATION 1
    	
 
    	
LCL INNOVATION 1
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
/s/Fabien Prévost
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By:
    	
 
    	
 
    	
By:  
    	
Fabien Prévost
    
	
Title:
    	
 
    	
 
    	
Title: 
    	
CFO
    

 

15

 

	
ADVENT   PRIVATE EQUITY FUND IV
    	
 
    	
ADVENT PRIVATE EQUITY FUND IV
    
	
 
    	
 
    	
 
    
	
/s/R.B. Parekh
    	
 
    	
/s/Shahzad Malik
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By: 
    	
R.B. Parekh
    	
 
    	
By: 
    	
Shahzad Malik
    
	
Title: 
    	
General Partner
    	
 
    	
Title: 
    	
General Partner
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
Partner,   Advent Venture Partners LLP
    	
 
    	
 
    
	
Acting   in its capacity as Manager of
    	
 
    	
 
    
	
Advent   Private Equity Fund IV
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
ADVENT   MANAGEMENT IV LP
    	
 
    	
ADVENT MANAGEMENT IV LP
    
	
 
    	
 
    	
 
    
	
/s/R.B. Parekh
    	
 
    	
/s/Shahzad Malik
    
	
By:
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By:
    	
 
    	
 
    	
By:
    	
Shahzad Malik
    
	
Title:
    	
General Partner
    	
 
    	
Title: 
    	
General Partner
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
Partner,   Advent Venture Partners LLP
    	
 
    	
 
    
	
Acting   in its capacity as Manager of
    	
 
    	
 
    
	
Advent   Management IV Limited Partnership
    	
 
    	
 
    

 

16

 

	
TIVERINA INVERSIONES S.L.
    	
 
    	
TIVERINA   INVERSIONES S.L.
    
	
 
    	
 
    	
 
    
	
/s/Ramon Mora-Figueroa Mora-Figueroa
    	
 
    	
 
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By: 
    	
Ramon Mora-Figueroa Mora-Figueroa
    	
 
    	
By:
    	
 
    
	
Title: 
    	
Managing Director
    	
 
    	
Title:
    	
 
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
ABADAI INVERSIONES S.L.
    	
 
    	
ABADAI   INVERSIONES S.L.
    
	
 
    	
 
    	
 
    
	
/s/Pablo Mora-Figueroa
    	
 
    	
 
    	
 
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By: 
    	
Pablo Mora-Figueroa
    	
 
    	
By:
    	
 
    
	
Title: 
    	
Managing Director
    	
 
    	
Title:
    	
 
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
SURRIC INVERSIONES S.L.
    	
 
    	
SURRIC   INVERSIONES S.L.
    
	
 
    	
 
    	
 
    
	
/s/Ramon Mora-Figueroa Mora-Figueroa
    	
 
    	
 
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By: 
    	
Ramon Mora-Figueroa Mora-Figueroa
    	
 
    	
By:
    	
 
    
	
Title: 
    	
Managing Director
    	
 
    	
Title:
    	
 
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
HILOS Y POLO S.L.
    	
 
    	
HILOS   Y POLO S.L.
    
	
 
    	
 
    	
 
    
	
/s/Ramon Mora-Figueroa Mora-Figueroa
    	
 
    	
 
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By: 
    	
Ramon Mora-Figueroa Mora-Figueroa
    	
 
    	
By:
    	
 
    
	
Title: 
    	
Managing Director
    	
 
    	
Title:
    	
 
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
RAMON MORA-FIGUEROA   MORA-FIGUEROA
    	
 
    	
RAMON   MORA-FIGUEROA MORA-FIGUEROA
    
	
 
    	
 
    	
 
    
	
/s/Ramon Mora-Figueroa Mora-Figueroa
    	
 
    	
 
    	
 
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    

 

17

 

	
ACTIVOS Y TENENCIAS 85 S.L.
    	
 
    	
ACTIVOS Y TENENCIAS 85 S.L.
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
[SIGNATURE ILLEGIBLE]
    	
 
    	
 
    	
 
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By: 
    	
[NAME ILLEGIBLE]
    	
 
    	
By:
    	
 
    
	
Title: 
    	
Managing Director
    	
 
    	
Title:
    	
 
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
FUNDACION UNIVERSITARIA DE   NAVARRA
    	
 
    	
FUNDACION   UNIVERSITARIA DE NAVARRA
    
	
 
    	
 
    	
 
    
	
[SIGNATURE ILLEGIBLE]
    	
 
    	
 
    	
 
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By: 
    	
Director
    	
 
    	
By:
    	
 
    
	
Title: 
    	
[ILLEGIBLE]
    	
 
    	
Title:
    	
 
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
JOSE LUIS PASCUAL PLAZA
    	
 
    	
JOSE   LUIS PASCUAL PLAZA
    
	
 
    	
 
    	
 
    
	
[SIGNATURE ILLEGIBLE]
    	
 
    	
[SIGNATURE   ILLEGIBLE]
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
FUNDACION   PARA EL DESSARROLLO Y COOPERACION INTERNACIONAL
    	
 
    	
FUNDACION   PARA EL DESSARROLLO Y COOPERACION INTERNACIONAL
    
	
 
    	
 
    	
 
    
	
/s/Ramon   Mora-Figueroa Mora-Figueroa
    	
 
    	
[SIGNATURE   ILLEGIBLE]
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
Title:
    	
 
    	
 
    	
Title:
    	
 
    
	
By: 
    	
Ramon Mora-Figueroa Mora-Figueroa
    	
 
    	
By:   
    	
[NAME   ILLEGIBLE]
    
	
Title: 
    	
Managing Director
    	
 
    	
Title:
    	
Managing   Director
    

 

18

 

	
LUPUS ALPHA MICRO CHAMPIONS
    	
 
    	
LUPUS   ALPHA MICRO CHAMPIONS
    
	
 
    	
 
    	
 
    
	
/s/Michael Frick
    	
 
    	
/s/G.   Albert
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title: 
    	
Verwaltungsrat
    	
 
    	
Title:
    	
 
    
	
By: 
    	
Michael Frick
    	
 
    	
By:   
    	
Göte   Albert
    
	
Title:
    	
 
    	
 
    	
Title:   
    	
Verwaltungsrat
    
	
 
    	
 
    	
 
    
	
 
    	
 
    	
 
    
	
LUPUS ALPHA ALL OPPERTUNITIES   FUND
    	
 
    	
LUPUS   ALPHA ALL OPPERTUNITIES FUND
    
	
 
    	
 
    	
 
    	
 
    
	
/s/Michael Frick
    	
 
    	
/s/G.   Albert
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title: 
    	
Verwaltungsrat
    	
 
    	
Title:
    	
 
    
	
By: 
    	
Michael Frick
    	
 
    	
By:   
    	
Göte   Albert
    
	
Title:
    	
 
    	
 
    	
Title:   
    	
Verwaltungsrat
    

 

19

 

	
Stichting   Administratiekantoor uniQure B.V.
    	
 
    	
Stichting Administratiekantoor uniQure B.V.
    
	
 
    	
 
    	
 
    
	
[SIGNATURE ILLEGIBLE]
    	
 
    	
[SIGNATURE ILLEGIBLE]
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    
	
Title: 
    	
Director
    	
 
    	
Title: 
    	
Director
    
	
By: 
    	
[NAME ILLEGIBLE]
    	
 
    	
By: 
    	
[NAME ILLEGIBLE]
    
	
Title: 
    	
Director
    	
 
    	
Title: 
    	
Director
    

 

20

 

	
Chiesi   Farmaceutici S.p.A.
    	
 
    	
Chiesi Farmaceutici S.p.A.
    
	
 
    	
 
    	
 
    
	
/s/Alberto Chiesi
    	
 
    	
/s/Ugo DiFrancesco
    
	
By: 
    	
Mr. Alberto Chiesi
    	
 
    	
By: 
    	
Mr. Ugo DiFrancesco
    
	
Title: 
    	
President
    	
 
    	
Title: 
    	
CEO
    

 

21

 

	
uniQure   B.V.
    	
 
    	
uniQure B.V.
    
	
 
    	
 
    	
 
    
	
/s/Aldag
    	
 
    	
/s/PJ Morgan
    
	
By:
    	
 
    	
 
    	
By:
    	
 
    
	
 
    	
 
    	
 
    	
 
    	
 
    
	
Title: 
    	
CEO
    	
 
    	
Title: 
    	
CFO
    
	
By: 
    	
Aldag
    	
 
    	
By: 
    	
PJ Morgan
    
	
Title:
    	
CEO
    	
 
    	
Title: 
    	
CFO
    

 

22

 

SCHEDULE A (PARTIES)

 

(1)                                Coöperatieve AAC LS U.A., a coöperative (coöperatie) incorporated under the laws of the Netherlands with its corporate seat in Amsterdam, and its business address at Gooimeer 2 35, 1411 DC Naarden, the Netherlands and registered with the Commercial Register of Gooi-, Eem- en Flevoland under number 34256402, represented by its managing director Forbion 1 Management B.V., a private company with limited liability (besloten vennootschap met beperkte aansprakelijkheid), incorporated under the laws of the Netherlands with its registered office (zetel) in Amsterdam, and its business address at Gooimeer 2 35, 1411 DC Naarden, the Netherlands and registered with the Commercial Register of Gooi-, Eem- en Flevoland under number 34249898); 

 

(2)                                Forbion Co-Investment Coöperatief U.A., a coöperative (coöperatie) incorporated under the laws of the Netherlands with its registered office in Naarden, and its business address at Gooimeer 2 35, 1411 DC Naarden, the Netherlands and registered with the Commercial Register of Gooi-, Eem- en Flevoland under number 32142360, represented by its managing director Forbion 1 Management B.V., a private company with limited liability (besloten vennootschap met beperkte aansprakelijkheid), incorporated under the laws of the Netherlands with its registered office (zetel) in Amsterdam, and its business address at Gooimeer 2 35, 1411 DC Naarden, the Netherlands and registered with the Commercial Register of Gooi-, Eem- en Flevoland under number 34249898);

 

(3)                                Forbion Co-Investment II Coöperatief U.A., a coöperative (coöperatie) incorporated under the laws of the Netherlands with its registered office in Naarden, and its business address at Gooimeer 2 35, 1411 DC Naarden, the Netherlands and registered with the Commercial Register of Gooi-, Eem- en Flevoland under number 53958713, represented by its managing director Forbion 1 CO II Management B.V., a private company with limited liability (besloten vennootschap met beperkte aansprakelijkheid), incorporated under the laws of the Netherlands with its registered office (zetel) in Amsterdam, and its business address at Gooimeer 2 35, 1411 DC Naarden, the Netherlands and registered with the Commercial Register of Gooi-, Eem- en Flevoland under number 53951956);

 

(4)                                Coöperatieve Gilde Healthcare II U.A., a coöperative (coöperatie) incorporated under the laws of the Netherlands with its corporate seat in Utrecht, and its business address at Newtonlaan 91 (3584 BP), the Netherlands and registered with the Commercial Register of Midden-Nederland under number 30216414, represented by its managing director Gilde Healthcare II Management B.V., a private company with limited liability (besloten vennootschap met beperkte aansprakelijkheid), incorporated under the laws of the Netherlands with its registered office (zetel) seat in Utrecht, and its business address at Newtonlaan 91 (3584 BP), the Netherlands and registered with the Commercial Register of Midden-Nederland under number 30215056

 

(5)                                S.J.H. VAN DEVENTER C.V., a Dutch limited partnership (commanditaire vennootschap), having its registered address at 1411 DC Naarden, Gooimeer 2 35, registered in the trade register under number 32158843

 

(6)                                CREDIT LYONNAIS INNOVATION 6, a French venture capital fund (Fonds Commun de Placement dans l’Innovation), represented herein by its management company (société de gestion), Omnes Capital (formerly named Crédit Agricole Private Equity), a French corporation (société par actions simplifiée) with a share capital of 8,000,000 euros, the registered office of which is located at 37-41 rue du Rocher — 75008 Paris, France, registered with the French Registry of 

 

23

 

Commerce and Companies under number 428 711 196 RCS Paris

 

(7)                                LCL INNOVATION 1, a French venture capital fund (Fonds Commun de Placement dans l’Innovation), represented herein by its management company (société de gestion), Omnes Capital (formerly named Crédit Agricole Private Equity), a French corporation (société par actions simplifiée) with a share capital of 8,000,000 euros, the registered office of which is located at 37-41 rue du Rocher — 75008 Paris, France, registered with the French Registry of Commerce and Companies under number 428 711 196 RCS Paris

 

(8)                                CLVC (formerly named Crédit Lyonnais Venture Capital), a French corporation (Société anonyme) with a share capital of 14,786,948 euros, with its registered office located at 37-41 rue du Rocher — 75008 Paris, registered with the French Registry of Commerce and Companies under number 434 465 514 RCS Paris, represented herein by Omnes Capital (formerly named Crédit Agricole Private Equity), a French Corporation (société par actions simplifiée) with a share capital of 8,000,000 euros, the registered office of which is located at 37-41 rue du Rocher — 75008 Paris, France, registered with the French Registry of Commerce and Companies under number 428 711 196 RCS Paris

 

(9)                                CLV1 (formerly named Crédit Lyonnais Venture 1), a French venture capital fund (Fonds Commun de Placement à Risques), represented herein by its managing partner, Omnes Capital (formerly named Crédit Agricole Private Equity), a French corporation (société par actions simplifiée) with a share capital of 8,000,000 euros, the registered office of which is located at 37-41 rue du Rocher — 75008 Paris, France, registered with the French Registry of Commerce and Companies under number 428 711 196 RCS Paris

 

(10)                         ADVENT PRIVATE EQUITY FUND IV, an English partnership, incorporated and existing under the laws of the United Kingdom, having its registered address at 25 Buckingham Gate, London, SW1E 6LD, United Kingdom and registered in England and Wales under number LP10002

 

(11)                         ADVENT MANAGEMENT IV LP, a Scottish limited partnership, incorporated and existing under the laws of Scotland, having its registered address at 50 Lothian Road, Festival Square, Edinburgh, EH3 9WJ, United Kingdom and registered in Scotland under number SL005366

 

(12)                         TIVERINA INVERSIONES S.L., a Spanish private company with limited liability, having its place of business at Calle Zurbano no76 6a, Madrid, Spain, with Tax Identification Number B-85381960

 

(13)                         ABADAI INVERSIONES S.L., a Spanish private company with limited liability, having its place of business at Calle Zurbano no76 6a, Madrid, Spain, with Tax Identification Number B-85383511

 

(14)                         SURRIC INVERSIONES S.L., a Spanish private company with limited liability, having its place of business at Calle Zurbano no76 6a, Madrid, Spain, with Tax Identification Number B-85381507

 

(15)                         HILOS Y POLO S.L., a Spanish private company with limited liability, having its place of business at Calle Zurbano no76 6a, Madrid, Spain, with Tax 

 

24

 

Identification Number B-83914481

 

(16)                         RAMON MORA-FIGUEROA MORA-FIGUEROA, with professional domicile at Madrid, Calle Zurbano no76 6a and holder of a National Identity Card with number 31.241.827-F

 

(17)                         ACTIVOS Y TENENCIAS 85 S.L., a Spanish private company with limited liability, having its place of business at Calle Ana Teresa 85 B, Madrid Spain, with Tax identification number B-84366111

 

(18)                         FUNDACION PARA EL DESARROLLO Y LA COOPERACION INTERNACIONAL, having its place of business at Calle Jose Abascal no44 2o, Madrid, Spain, with Tax identification number G-80787161

 

(19)                         FUNDACION UNIVERSITARIA DE NAVARRA, having its place of business at Avd. Pio XII 53 1o, Pamplona, Spain, with Tax Identification Number G-31469125

 

(20)                         JOSE LUIS PASCUAL PLAZA, with professional domicile at Madrid, Calle del Pastor 4 and holder of a National Identity Card with number 02.153.208-V

 

(21)                         LUPUS ALPHA MICRO CHAMPIONS, a Sub Fund of Lupus alpha Fonds, a Fonds Commun de placement under Luxembourg law, for which and on behalf wherof Lupus alpha Investment S.A., 69, route d’Esch, L-1470 Luxembourg, R.C.S. Luxembourg B-79272 (Management Company) acts

 

(22)                         LUPUS ALPHA ALL OPPORTUNITIES FUND, a Sub Fund of Lupus alpha Fonds, a Fonds Commun de placement under Luxembourg law, for which and on behalf wherof Lupus alpha Investment S.A., 69, route d’Esch, L-1470 Luxembourg, R.C.S. Luxembourg B-79272 (Management Company) acts

 

And 

 

(23)                         Sichting Administratiekantoor uniQure B.V., a foundation organised under the laws of the Netherlands, having its registered office in Amsterdam, at Meibergdreef 61, 1105 BA Amsterdam Zuidoost, the Netherlands and registered with the Commercial Register of Amsterdam under number 55055036, duly represented by its board members Mr. W. Stevens, W. Swarte and R. Jongejan , a private company with limited liability (besloten vennootschap met beperkte aansprakelijkheid), incorporated under the laws of the Netherlands with its registered office (zetel) in Amsterdam, and its business address at Gooimeer 2 35, 1411 DC Naarden, the Netherlands and registered with the Commercial Register of Gooi-, Eem- en Flevoland under number 53951956);

 

And 

 

(24)                         uniQure B.V., a private company with limited liability (besloten vennootschap met beperkte aansprakelijkheid), incorporated under the laws of the Netherlands with its registered office in Amsterdam, and its business address at Meibergdreef 61, 1105 BA Amsterdam Zuidoost, the Netherlands and registered with the Commercial Register of Amsterdam under number 54385229, duly represented by its managing directors Mr. J. Aldag and Mr. P. Morgan

 

25

 

and

 

(25)                         Chiesi Farmaceutici S.p.A. an Italian corporation, with its offices at Via Palermo, 26/A, 43122 Parma, Italy and registered with the Commercial Register of [     ]under number [       ], duly represented by [        ].

 

26

 

SCHEDULE B (ACCESSION AGREEMENT)

 

This Accession Agreement (the “Agreement”) is made on [·]

 

RECITALS:

 

(A)                              [Note: insert name] (the “New Shareholder”) on [Note: insert date] acquired [Note: insert number] Ordinary Shares Class C by [an issue of new Shares] [a transfer of Shares by [Note: insert name of transferor] the “Transferor”)].

 

(B)                             This Agreement is entered into in compliance with the terms of the Class C shareholders agreement dated [Note: insert date] between the Existing Investors, the New Investor, the Company and the Trust Foundation (all as defined therein) (which agreement is referred to in this Agreement as the “Class C Shareholders Agreement”).

 

IT IS AGREED as follows:

 

(1)                                Definitions used in this Agreement have the same meaning as given to them in the Class C Shareholders Agreement unless stated otherwise and the provisions of Clause 1 (Interpretation) of the Class C Shareholders Agreement shall apply to this Agreement.

 

(2)                                The New Shareholder agrees to become a Party to the Class C Shareholders Agreement and to be bound by the terms of the Class C Shareholders Agreement in all respects as a Shareholder. [The New Shareholder assumes all the obligations of the Transferor in that capacity under the Class C Shareholders Agreement.]

 

(3)                                SCHEDULE A (Parties) to the Class C Shareholders Agreement shall be updated to properly reflect the relevant changes in the ownership of the Shares.

 

(4)                                The contact details of the new Shareholder are as set out in the updated version of SCHEDULE A (Parties), to be attached to the Class C Shareholders Agreement in replacement of the existing version.

 

(5)                                [Note: insert other relevant details]

 

(6)                                The provisions of the Clauses 7.3, 7.4, 7.5, 7.6, 7.7, 7.11, 7.12 and 7.13 shall apply mutatis mutandis to this Agreement.

 

THUS AGREED AND SIGNED ON [·],

 

[Note: to be signed by New Shareholder and Parties to the Class C Shareholders Agreement]

 

27Exhibit 10.3

 

Confidential Materials omitted and filed separately with the

Securities and Exchange Commission. Double asterisks denote omissions.

 

License Agreement- Non-Exclusive

 

(expresSF+® cells)

 

This License Agreement (the “Agreement”) is entered into and made effective the 22nd day of March, 2007, (the “Effective Date”) between PROTEIN SCIENCES CORPORATION, whose principal place of business is at 1000 Research Parkway, Meriden, CT 06512 USA (hereinafter referred to as “LICENSOR”) and AMSTERDAM MOLECULAR THERAPEUTICS whose principal place of business is at Meibergdreef 61, P.O. Box 22506, 1100 DA Amsterdam, The Netherlands, (hereinafter referred to as “LICENSEE”).

 

WITNESSETH

 

WHEREAS, LICENSOR is the assignee of U.S. Patent No. [**];

 

WHEREAS, LICENSEE desires to acquire a non-exclusive worldwide license under the Licensed Patent and to use the Technology for Product research, development and commercialization purposes; and

 

WHEREAS, LICENSOR warrants that it possesses the right to license the Licensed Patent (as herein defined);

 

NOW THEREFOR, for these and other valuable considerations, the receipt of which is hereby acknowledged, the parties agree as follows:

 

1. DEFINITIONS:

 

1.1  “Affiliate” shall mean any corporation or other business entity controlled by, controlling or under common control with LICENSOR or LICENSEE.  For this purpose, “control” shall mean direct or indirect beneficial ownership of at least fifty percent (50%) of the voting stock of, or at least a fifty percent (50%) interest in the income of, such corporation or other business entity, or such other relationship as, in fact, constitutes actual control.

 

1.2  “Product(s)” shall mean any recombinant Adeno-associated Virus (rAAV) vector product (or part thereof) that is developed as a direct result of LICENSEE’s use of the Technology and which product (or part thereof) is listed in Appendix I to this Agreement, it being understood that Appendix I may be amended during the term of this Agreement to incorporate new Product(s) developed by LICENSEE.

 

1.3  “Technology” shall mean the cells marketed by LICENSOR as expresSF+® cells, or the cells deposited by LICENSOR at the ATCC as CRL-12579, and any progeny or derivatives thereof.

 

1.4  “Territory” shall mean the world.

 

1.5  “Licensed Patent” shall mean claims 1-8 of U.S. Patent No. [**] and any counterparts thereto in the Territory.

 

 

1.6  “Intellectual Property Rights” shall mean the Licensed Patent, the Technology and the know how associated therewith.

 

2. GRANT:

 

2.1  In consideration for payment of fees, LICENSOR hereby (i) grants to LICENSEE a non-exclusive license under the Licensed Patent and the Technology and know how involving the Technology in the Territory, without the right to sublicense, and (ii) transfers cells, both for the following uses: to research and have researched, to develop and have developed, to manufacture and have manufactured, to import and have imported, to market and have marketed and to offer for sale and sell Products anywhere in the Territory (“Permitted Acts”).  By this Agreement, LICENSOR is not granting — either expressly or by implication — any licenses under any other patents or technology owned or licensed by LICENSOR, including U.S. Patents Nos. [**].

 

2.2  LICENSEE agrees that the Technology is covered by the Licensed Patent and that if LICENSEE challenges the validity of the Licensed Patent or uses any allegation of invalidity — either by LICENSEE or a third party — as a basis for non-payment of fees hereunder, then LICENSOR shall have the right to terminate this Agreement pursuant to Section 12.1.

 

2.3  LICENSEE agrees that if either party makes commercially useful derivatives, developments or improvements to, from, of or utilizing Intellectual Property Rights, such derivatives, developments and improvements and all intellectual property rights therein, including patent rights, shall belong to LICENSOR. LICENSEE shall promptly notify LICENSOR of such derivatives, developments and improvements, and LICENSOR in its sole discretion shall determine whether or not to pursue intellectual property protection therefor.  At LICENSOR’s request, LICENSEE and its employees and agents shall assign to LICENSOR the intellectual property rights in, to and under the derivatives, developments and improvements to, from, of or utilizing the Intellectual Property Rights, without any additional consideration from LICENSOR, and execute all necessary documents so that LICENSOR may pursue intellectual property protection for the derivatives, developments and improvements.  As compensation for assigning the intellectual property rights in, to and under the derivatives, developments and improvements, LICENSOR hereby grants to LICENSEE a non-exclusive royalty-free license under those intellectual property rights, under the terms of this Agreement; said intellectual property rights shall thus be included in the term “Licensed Patent” as used herein.  For clarity, all conceptions, inventions, discoveries, data, information, or any results whatsoever and wheresoever generated by or upon behalf of LICENSEE in carrying out the Permitted Acts that relate solely and exclusively to only the Product and all intellectual property (including secret know how) or property solely and exclusively covering or relating thereto shall be solely owned by the LICENSEE, provided that, effective from and after termination of this Agreement, LICENSOR is hereby granted a royalty-free, perpetual, irrevocable, sublicensable non-exclusive license therein in the Territory.

 

2.4  LICENSOR agrees to provide regulatory and technical support in the manufacturing of the LICENSEE’s master (working) cell bank (MCB/WCB) and the LICENSEE’s master (working) virus bank (MVB/WVB) using the Technology.  The regulatory support will include a documentation package regarding history and characterization of the Technology for cGMP manufacturing, including the signed certificate of analysis for WCB #040704 or any other MCB

 

 

owned by PSC of which a vial will be send to AMT.  The fees payable by the LICENSEE hereunder shall include ten hours of technical and FDA or EMEA/CHMP regulatory assistance from the LICENSOR.  Any hours exceeding this will be billed at $[**] USD/hour.

 

2.5  LICENSEE agrees to provide LICENSOR with EMEA/CHMP filing reference numbers so that LICENSOR can refer to such filings solely in relation to the LICENSOR’s corresponding regulatory filings covering the Technology.

 

3. TERM:

 

This Agreement and the license granted by this Agreement shall be non-exclusive for a term commencing as of the Effective Date of this Agreement and continuing thenceforth subject to payment of the fees set out in Section 8, unless terminated pursuant to Section 12.

 

4. UNITED STATES LAWS:

 

4.1  It is understood that LICENSOR is subject to United States laws and regulations controlling the export of technical data, computer software, laboratory prototypes and other commodities (including the Arms Export Control Act, as amended and the Export Administration Act of 1979), and that its obligations hereunder are contingent on compliance with applicable United States export laws and regulations.  The transfer of certain technical data and commodities may require a license from the cognizant agency of the United States Government and/or written assurances by LICENSEE that LICENSEE shall not export data or commodities to certain foreign countries without prior approval of such agency.  LICENSOR neither represents that a license shall not be required nor that, if required, it shall be issued.

 

5. USE OF TRADEMARK:

 

5.1  LICENSOR shall have no responsibility with respect to LICENSEE’S own trademarks and tradename, and LICENSEE in respect to the use thereof will defend, indemnify and hold harmless LICENSOR against any and all third party claims.  LICENSEE agrees that LICENSOR may include LICENSEE’s name in any listings by LICENSOR of LICENSOR’s licensees. LICENSEE shall not use LICENSOR’s names or marks without the express written permission of LICENSOR.

 

6. INDEMNIFICATION:

 

6.1  LICENSEE agrees to release, indemnify and hold harmless the LICENSOR, its directors, officers and employees against any and all losses, expenses, claims, actions, lawsuits and judgments thereon (including attorney’s fees through the appellate levels) which may be brought against LICENSOR, its directors, officers and employees as a result of or arising out of use, production, manufacture, sale, lease, consumption or advertisement by, or on behalf of, LICENSEE of any Product or Technology licensed under this Agreement.

 

7. WARRANTY:

 

7.1  LICENSOR MAKES NO WARRANTIES, EXPRESS OR IMPLIED, AND HEREBY DISCLAIMS ALL SUCH WARRANTIES, AS TO ANY MATTER WHATSOEVER,

 

 

INCLUDING, WITHOUT LIMITATION, THE CONDITION, INCLUDING PURITY, OF ANY INVENTION(S), TECHNOLOGY OR PRODUCT, WHETHER TANGIBLE OR INTANGIBLE, LICENSED UNDER THIS AGREEMENT; OR OF MERCHANTABILITY, OR FITNESS FOR A PARTICULAR PURPOSE OF THE INVENTION, TECHNOLOGY OR PRODUCT; OR OWNERSHIP; OR THAT THE USE OF THE LICENSED PATENT, TECHNOLOGY OR PRODUCT WILL NOT INFRINGE ANY PATENT, COPYRIGHTS, TRADEMARKS, OR OTHER RIGHTS.  LICENSOR SHALL NOT BE LIABLE FOR ANY DIRECT, CONSEQUENTIAL, OR OTHER DAMAGES SUFFERED BY ANY LICENSEE OR ANY THIRD PARTIES RESULTING FROM THE USE, PRODUCTION, MANUFACTURE, SALE, LEASE, CONSUMPTION, OR ADVERTISEMENT OF THE TECHNOLOGY OR PRODUCT.

 

7.2  LICENSOR represents and warrants that is has good title to the Technology and Licensed Patent and has the right to license the Licensed Patent as provided herein.  LICENSEE agrees to indemnify, hold harmless, and defend LICENSOR from and against any and all claims, loss, damages, or expenses arising out of any third party claims of infringement resulting from the use by LICENSEE of the Technology or out of the use, sale or other disposition of Products.

 

8. FEES AND CELLS:

 

8.1  In consideration of the license herein granted, LICENSEE shall pay fees to LICENSOR as follows:

 

(a) License Issue Fee of $50,000 that shall be deemed earned and due immediately upon the execution of this Agreement, at which time the LICENSEE shall be provided with a supply of [**].

 

(b) A license fee of $50,000 for every additional product added to Appendix I upon the addition of such Product to Appendix I; and

 

(c) License Maintenance Fees of $[**] on each one-year anniversary thereafter, upon receipt of which by LICENSOR, the LICENSEE be provided with a supply of up to [**] if the LICENSEE so requests.

 

8.2  All payments shall be made hereunder in U.S. dollars.

 

9. MARKING AND STANDARDS:

 

9.1  LICENSEE agrees to maintain satisfactory standards in respect to the nature of the Product manufactured and/or sold by LICENSEE. LICENSEE agrees that all Products manufactured and/or sold by it shall be of a quality that is appropriate to products of the type here involved.  LICENSEE shall use appropriate patent marking on Products with respect to the Licensed Patent.

 

10. ASSIGNMENT:

 

10.1  This Agreement is not assignable by LICENSEE or by operation of law without the prior written consent of LICENSOR at its sole discretion.

 

 

10.2  This Agreement shall extend to and be binding upon the successors and legal representatives and permitted assigns of LICENSEE. This Agreement shall extend to and be binding upon the successors and legal representatives and assigns of LICENSOR.

 

11. NOTICE:

 

Any notice, payment, or other correspondence (hereinafter collectively referred to as “correspondence”) required or permitted to be given hereunder shall be mailed by certified mail or delivered by hand to the party to whom such correspondence is required or permitted to be given hereunder.  If mailed, any such notice shall be deemed to have been given when mailed as evidenced by the postmark at point of mailing.  If delivered by hand, any such correspondence shall be deemed to have been given when received by the party to whom such correspondence is given, as evidenced by written and dated receipt of the receiving party.

 

All correspondence to LICENSEE shall be addressed as follows:

 

Sander J.H. van Deventer
 Chief Scientific Officer

Amsterdam Molecular Therapeutics

Meibergdreef 61

1105 BA Amsterdam

The Netherlands

 

All correspondence to LICENSOR shall be addressed as follows:

 

Manon M.J. Cox
 Chief Operating Officer

Protein Sciences Corporation

1000 research Parkway

Meriden, CT 06512 USA

 

Either party may change the address to which correspondence to it is to be addressed by notification as provided herein.

 

12. TERMINATION:

 

12.1  LICENSOR and LICENSEE shall have the right to terminate this Agreement if the other party commits a material breach of an obligation under this Agreement and continues in default for more than [**] months after receiving written notice of such default.  Such termination shall be effective upon further written notice to the breaching party after failure by the breaching party to cure such default.

 

12.2  LICENSEE agrees that breach of terms of this Agreement would immediately and irreparably damage LICENSOR in a way not capable of being fully compensated by monetary damages and accordingly, the LICENSOR is entitled to injunctive relief in addition to such other relief to which it may be entitled at law or in equity.

 

 

12.3  The license and rights granted in this Agreement have been granted on the basis of the special capability of LICENSEE to perform research and development work leading to the manufacture and marketing of the Product. Accordingly, LICENSEE covenants and agrees that in the event any proceedings under the Dutch Bankruptcy Act or any amendment thereto, be commenced by or against LICENSEE, and, if against LICENSEE, said proceedings shall not be dismissed with prejudice before either an adjudication in bankruptcy or the confirmation of a composition, arrangement, or plan of reorganization, or in the event LICENSEE shall be adjudged insolvent or make an assignment for the benefit of its creditors, or if a writ of attachment or execution be levied upon the license hereby created and not be released or satisfied within ten (10) days thereafter, or if a receiver be appointed in any proceeding or action to which LICENSEE is a party with authority to exercise any of the rights or privileges granted hereunder and such receiver be so discharged within a period of forty-five (45) days after his appointment, any such event shall be deemed to constitute a breach of this Agreement by LICENSEE and, LICENSOR, at the election of LICENSOR, but not otherwise, ipso facto, and without notice or other action by LICENSOR, shall terminate this Agreement and all rights of LICENSEE hereunder and all rights of any and all persons claiming under LICENSEE.

 

12.4  LICENSEE shall have the right to terminate this Agreement upon ninety (90) days notice prior to each anniversary date hereof.

 

12.5  Any termination of this Agreement shall be without prejudice to LICENSOR’s right to recover all amounts accruing to LICENSOR prior to such termination and cancellation. Except as otherwise provided, should this Agreement be terminated for any reason, LICENSEE shall have no rights, express or implied, under any Intellectual Property Right that is the subject matter of this Agreement. Upon termination, LICENSEE shall have the right to dispose of Products then in its possession and to complete existing contracts for such products, so long as contracts are completed within [**] months from the date of termination. LICENSEE agrees to return to LICENSOR any Intellectual Property Rights and progeny, derivatives, developments or improvements thereof remaining in LICENSEE’s possession after [**] months from the date of termination.

 

13. CERTIFICATE OF INSURANCE:

 

13.1  LICENSEE agrees to carry and keep in force, at its expense, general and product liability insurance of not less than $[**] to cover liability for damages on account of bodily or personal injury or death to any person, or damage to property of any person; such insurance shall not be canceled for any cause without at least [**] days prior written notice to Protein Sciences Corporation.  At the time of execution of this Agreement, LICENSEE shall provide a certificate of insurance to LICENSOR.

 

14. GOVERNING LAW:

 

Any disputes, controversies or claims which arise under, out of, in connection with, or relating to this Agreement shall be governed by and interpreted in accordance with the laws of the State of Connecticut, without regard to choice of law, and the parties agree that all disputes, controversies or claims which arise under, out of, in connection with, or relating to this Agreement shall be brought in the Courts situated in the State of Connecticut for resolution thereof, and the parties

 

 

therefore submit to the exclusive jurisdiction of the State and Federal Courts situated in the State of Connecticut for resolution of all disputes, controversies or claims which arise under, out of, in connection with, or relating to this Agreement.

 

15. CAPTIONS:

 

The captions and Section headings of this Agreement are solely for the convenience of reference and shall not affect its interpretation.

 

16. SEVERABILITY:

 

Should any part or provision of this Agreement be held unenforceable or in conflict with the applicable laws or regulations of any jurisdiction, the invalid or unenforceable part or provision shall be replaced with a provision which accomplishes, to the extent possible, the original business purpose of such part or provision in valid and enforceable manner, and the remainder of the Agreement shall remain binding upon the parties hereto.

 

17. SURVIVAL:

 

17.1  The provisions of Sections 2.3, 4, 5, 6, 7, 8 and 14 shall survive the termination or expiration of this Agreement and shall remain in full force and effect.

 

17.2  The provisions of this Agreement which do not survive termination or expiration hereof (as the case may be) shall, nonetheless, be controlling on, and shall be used in construing and interpreting, the rights and obligations of the parties hereto with regard to any dispute, controversy or claim which may arise under, out of, in connection with, or relating to this Agreement.

 

18. AMENDMENT:

 

No amendment or modification of the terms of this Agreement, including any modification or amendment of this Section, shall be binding on either party unless reduced to writing and signed by an authorized officer of the party to be bound.

 

19. WAIVER:

 

No failure or delay on the part of a party in exercising any right hereunder will operate as a waiver of, or impair, any such right.  No single or partial exercise of any such right will preclude any other or further exercise thereof or the exercise of any other right.  No waiver of any such right will be deemed a waiver of any other right hereunder.

 

20. ENTIRE AGREEMENT:

 

This Agreement constitutes the entire agreement between the parties hereto respecting the subject matter hereof, and supersedes and terminates all prior agreements respecting the subject matter hereof, whether written or oral, and may be amended only by an instrument in writing executed by both parties hereto.

 

 

IN WITNESS WHEREOF, the parties hereto have caused this Agreement to be executed by their respective officers thereunto duly authorized to be effective as of the Effective Date.

 

	
Protein Sciences   Corporation
    	
Amsterdam Molecular Therapeutics
    
	
 
    	
 
    
	
 
    	
 
    
	
By:  
    	
/s/ Manon M.J. Cox
    	
 
    	
By: 
    	
/s/ Sander J.H. van Deventer 
    
	
Manon M.J. Cox
    	
 
    	
Sander J.H. van Deventer
    
	
Chief Operating   Officer
    	
 
    	
Chief Scientific Officer
    

 

 

APPENDIX I

 

1.                                      Any rAAV Vector for the treatment of lipoprotein lipase deficiency disease in humans.

 

 

License Agreement - Non-Exclusive

 

(express+® cells)

 

AMENDMENT

 

Reference is made to a certain License Agreement dated as of March 22, 2007 by and between PROTEIN SCIENCES CORPORATION, whose principal place of business is at 1000 Research Parkway, Meriden, CT 06512 USA (hereinafter referred to as “LICENSOR”) and Amsterdam Molecular Therapeutics (AMT) B.V. whose principal place of business is at Meibergdreef 61, P.O. Box 22506,1100 DA Amsterdam, The Netherlands, (hereinafter referred to as “LICENSEE”), as amended (the “Agreement”).

 

Whereas:

 

On 10 May 2012, Amsterdam Molecular Therapeutics (AMT) B.V. changed its name to uniQure biopharma B.V.

 

LICENSOR and LICENSEE agree to amend the Agreement by adding to Appendix I thereof the following:  “any rAAV Vector for the treatment of Acute Intermittent Porphyria disease in humans; any rAAV Vector for the treatment of Hemophilia B disease in humans”.  The revised Appendix I is attached to this Agreement.

 

The Agreement is further amended as follows:

 

1.              In Section 2.1 of the Agreement, the words “without the right to sublicense” shall be changed to “with the right to sublicense”, subject to the provision of Clause 4 of this Amendment.

 

2.              Any Product LICENSEE proposes to add to Appendix I shall be automatically granted to LICENSEE as an exclusive license in the Territory subject to LICENSEE certifying to LICENSOR that it has acquired intellectual property covering such Product or it is actively developing such Product and subject further to a [**]-day period during which LICENSOR shall notify LICENSEE if LICENSOR has, prior to LICENSEE’S proposed addition, (i) already granted a third party an exclusive license to such Product or competitive product or (ii) LICENSOR has an ongoing internal development program itself for such Product or a competitive product, in which case such Product will not be added to Appendix I.  In the event that LICENSOR has granted a non-exclusive license to a third party for such Product or a competitive product, such Product will be added to Appendix I but designated as non-exclusive.  After the lapse of the aforementioned [**]-day period without notification to LICENSEE by LICENSOR, the Product concerned shall be automatically granted to LICENSEE as an exclusive license in the Territory.

 

3.              Section 2.3 of the Agreement is amended as follows:

 

a.              The portion of the last sentence shall be deleted starting with phrase “,provided that,”; and

 

b.              Each time the phrase “Intellectual Property Rights” is used in such Section it is amended to read “Intellectual Property Rights or Technology”.

 

4.              Section 8 of the Agreement is amended as follows:

 

a.              The $[**] annual fee in respect of each Product in Appendix I shall not be payable after a total of $[**] has been paid in respect of such Product or such Product is no longer being developed;

 

 

b.              The maximum amount payable per annum in respect of Appendix I is $[**] per year regardless of the number of Products listed therein, and

 

c.               In addition to the fees payable in respect of Products listed in Appendix I, in the event any Product that is or has been listed in Appendix I is being sold by LICENSEE in any market or is partnered with, licensed to or funded by another entity, LICENSOR shall be entitled to a fee of $[**] per year payable from the date such Product is first sold or such partnership, license or funding occurs and ending on the earlier of ten (10) years after the first commercial sale of the Product or the date such Product is no longer being sold in any market or such partnership, license or funding is terminated.

 

5.              uniQure biopharma B.V.  may sublicense under the Licensed Patent and the Technology and know how involving the Technology to the extent and only to the extent that such sublicense is reasonably necessary for a sublicensee to research, have researched, develop, have developed, manufacture, have manufactured, import, have imported, market, have marketed, offer for sale, sell and have sold a Product listed in Appendix 1 in the Territory.  No general license to use the Technology by such sublicensee is implied or granted by the foregoing.  Section 10 is amended to provide that LICENSEE may assign the Agreement in the event of a reorganization, merger, transfer, share exchange, consolidation, or sale or disposition of all or substantially all of the assets of LICENSEE.

 

In all other respects the Agreement is unchanged.

 

	
PROTEIN SCIENCES CORPORATION
    	
uniQure biopharma B.V.
    
	
 
    	
 
    
	
 
    	
 
    
	
By: 
    	
/s/Manon M.J. Cox
    	
 
    	
By: 
    	
/s/ Piers Morgan
    
	
Manon M.J. Cox 
    	
 
    	
Name: Piers Morgan 
    
	
President and CEO 
    	
 
    	
Title: CFO 
    
	
Date: June 13,   2012
    	
 
    	
Date: June 13, 2012
    

 

 

APPENDIX I

 

1.              Any rAAV Vector for the treatment of lipoprotein lipase deficiency disease in humans.   Exclusive.

2.              Any rAAV Vector for the treatment of Acute Intermittent Porphyria disease in humans.   Exclusive.

3.              Any rAAV Vector for the treatment of Hemophilia B disease in humans.   Exclusive.

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