Document:

exhibit10_1.htm

Exhibit 10.1

 

FIFTH AMENDMENT

to the LowCal Agreements (as defined herein)

THIS FIFTH AMENDMENT TO THE LOWCAL AGREEMENTS (this “Fifth Amendment”), dated as of January 13, 2015, is by and among Eos Global Petro, Inc. (“Eos”), Eos Petro, Inc. (the “Company”), LowCal Industries, LLC (“LowCal”), Sail Property Management Group LLC (“Sail”) and LowCo [EOS/Petro], LLC (“LowCo, and collectively referred to with Eos, Company, LowCal and Sail as the “Parties”). Capitalized terms used but not otherwise defined herein shall have the meanings given to them in the LowCal Agreements.

 

WHEREAS, the Parties have previously entered into the following agreements (collectively referred to herein as the “LowCal Agreements”):

	
  

	
a.

	
Second Amended and Restated Loan Agreement and Secured Promissory Note, dated November 6, 2013, between Eos and LowCal (the “First Note”);

	
  

	
b.

	
Amended and Restated Leasehold Mortgage, Assignment, Security Agreement and Fixture Filing, dated April 23, 2013, between Eos and LowCal (the “Mortgage”);

	
  

	
c.

	
Guaranty, dated February 8, 2013, made by Company in favor of LowCal;

	
  

	
d.

	
Lock-Up/Leak-Out Agreement, dated February 8, 2013, between Company and LowCal;

	
  

	
e.

	
Lock-Up/Leak-Out Agreement, dated November 6, 2013, between Company and LowCal;

	
  

	
f.

	
Series B Convertible Preferred Stock Purchase Agreement, dated February 8, 2013, between Company and LowCal;

	
  

	
g.

	
Series B Convertible Preferred Stock Purchase Agreement, dated April 23, 2013, between Company and LowCal;

	
  

	
h.

	
Common Stock Purchase Agreement, dated November 6, 2013, between Company and LowCal;

	
  

	
i.

	
Extension Agreement, dated November 6, 2013, between the Parties;

	
  

	
j.

	
Warrant to Purchase Common Stock of the Company, dated August 14, 2013, with LowCo as the holder (“Warrant”),

	
  

	
k.

	
Compliance/Oversight Agreement, dated February 8, 2013, between Eos and Sail (the “Oversight Agreement”);

  

  

  

	
  

	
l.

	
First Amendment to the LowCal Agreements, dated April 23, 2013, between the Parties;

	
  

	
m.

	
Second Amendment to the LowCal Agreements, dated November 6, 2013, between the Parties;

	
  

	
n.

	
Third Amendment to the LowCal Agreements, effective January 9, 2014, between the Parties; and

	
  

	
o.

	
Third Amendment to the LowCal Agreements, dated August 14, 2014, between the Parties (which the Parties acknowledge should have been titled the Fourth Amendment).

 

WHEREAS, LowCal and Affiliates have caused the following amounts, which aggregate $750,000.00, to be loaned to the Company on the following dates (collectively referred to herein as the “2014 Deposits”):

	
  

	
a.

	
October 10, 2014 - $150,000.00;

	
  

	
b.

	
November 11, 2014 - $200,000.00;

	
  

	
c.

	
December 12, 2014 - $325,000.00; and

	
  

	
d.

	
December 17, 2014 - $75,000.00.

WHEREAS, the parties desire to memorialize the terms upon which the 2014 Deposits will be repaid in a new note, amend certain terms of the First Note and terminate the Oversight Agreement.

 

NOW, THEREFORE, in consideration of the mutual covenants and agreements hereinafter set forth and for other good and valuable consideration, the receipt and sufficiency of which are hereby acknowledged, the Parties have agreed to the following terms:

	
  

	
1.

	
Amended and Restated First Note. Simultaneously with the execution of this Fifth Amendment, the First Note shall be amended and restated in its entirety in the form attached hereto as Exhibit A (the “Amended First Note). As is more fully set forth in the Amended First Note, the amendments include: (i) forgiving all accrued and unpaid interest on the First Note, as well as all interest which otherwise would have accrued on the First Note from the date first written above into the future; (ii) removing the “Exit Fee,” as defined in the First Note; and (iii) extending the maturity date to June 30, 2015.  Upon conversion, in whole or in part, of the Amended First Note pursuant to Section 4 thereof, the Company covenants and agrees to issue to LowCal shares of common stock of the Company, in accordance with the provisions of Section 4 of the Amended First Note.   Upon payment in full of the Amended First Note, the Company covenants and agrees to issue shares of common stock of the Company to LowCal, in accordance with the provisions of Section 5 of the Amended First Note.

  

  

  

 

	
  

	
2.

	
Issuance of New Second Note. Simultaneously with the execution of this Fifth Amendment, the Company shall execute and deliver to LowCal the unsecured promissory note in the amount of $3,250,000.00 attached hereto as Exhibit B (the “Second Note”). The Parties agree that the principal amount of the Second Note reflects and equals: (i) the cash portion of the “Exit Fee” removed from the First Note; (ii) all consulting fees and expenses which otherwise would have been payable to Sail pursuant to the Oversight Agreement, which is being terminated pursuant to Section 3 herein; (iii) all accrued but unpaid interest being removed and forgiven from the First Note as of the date first written above; (iv) the 2014 Deposits, and (v) the value of the release by LowCal and LowCo of substantial rights and claims under the First Note and the LowCal Agreements and security and sources of funding for the repayment of the First Note.

	
  

	
3.

	
Termination of Oversight Agreement. The Parties acknowledge and agree that the Oversight Agreement shall be terminated and be of no further force and effect effective as of the day first written above. Any and all consulting fees and expenses payable to Sail pursuant to the terms thereof shall have been satisfied by issuance of the Second Note.

	
  

	
4.

	
Acknowledgement of Good Standing. The Parties agree and acknowledge that any and all Events of Default (as defined in the LowCal Agreements) of the Company or Eos and any events of default of LowCal, LowCo or Sail which may have occurred under the LowCal Agreements on or prior to the date hereof are hereby waived, and the Parties further acknowledge that the LowCal Agreements, other than the Oversight Agreement terminated pursuant to Section 3 above, are in good standing and full force and effect as of the date hereof.

	
  

	
5.

	
Future Release of Mortgage; Amendment of Mortgage. Each of LowCo, LowCal and Sail covenants and agrees that, notwithstanding anything to the contrary in the Mortgage or other LowCal Agreements, upon repayment in full of the Second Note, they shall each forever release, cancel and terminate in all respects the Mortgage and any other Liens otherwise in effect as a result of the Mortgage or Amended First Note, and they shall each execute, file or cause to be filed any and all notices, consents and agreements to effectuate such release, cancellation and termination.  Within ten (10) days after the date of this Agreement Eos and the Company will cause an amendment to the Mortgage, in form and substance reasonably satisfactory to LowCal and LowCo, to be filed to reflect the current $5,000,000 principal balance of the Amended First Note, so that the public filing of the Mortgage reflects that such current principal balance is secured by such Mortgage and the liens created thereby.  Such amendment shall not relinquish any exiting priority of the Mortgage.  Any filing, recording or other fees or taxes payable in connection therewith shall be paid by Eos.

	
  

	
6.

	
Piggyback Registration Rights. The Company agrees that all shares of common stock of the Company issued or issuable to LowCal or LowCo pursuant to any of the LowCal Agreements, the Second Note or the Amended First Note shall have the piggyback registration rights set forth in the registration rights appendix attached hereto as Exhibit C, the terms of which are incorporated herein by this reference.

  

  

  

 

	
  

	
7.

	
Notice. All notices or other communications hereunder shall be in writing and shall be deemed to have been duly given if delivered personally, or sent by a nationally recognized overnight delivery service, such as Federal Express, or mailed by certified or registered mail, return receipt requested, postage prepaid, to the following addresses:

 

If to the Company/Eos:

Eos Petro, Inc.

Attention: Nikolas Konstant

1999 Avenue of the Stars, Suite 2520

Los Angeles, CA 90067

Fax: (310) 277-0591

If to LowCal, LowCo or Sail:

LowCal Industries, LLC

6119 Greenville Ave

Suite 340

Dallas, TX 75206-1910

Fax: (323) 443-3868

 

Notices sent by mail shall be deemed received on the earlier to occur of actual delivery to the recipient, or the 3rd day after deposit in the mail.

 

	
  

	
8.

	
General Provisions.

	
  

	
a)

	
      General Interpretation.  The terms and conditions of this Fifth Amendment have been negotiated by the Parties and the language used in this Fifth Amendment shall be deemed to be the language chosen by the Parties hereto to express their mutual intent.  This Fifth Amendment shall be construed without regard to any presumption or rule requiring construction against the Party causing such instrument or any portion thereof to be drafted, or in favor of the Party receiving a particular benefit under the Fifth Amendment.  No rule of strict construction will be applied against any Party.

 

	
  

	
b)

	
     Signatures – Counterparts.  This Fifth Amendment may be executed in two or more counterparts, each of which shall be deemed an original, but all of which together shall constitute one and the same instrument.  This Fifth Amendment shall not be effective until the execution and delivery among all of the Parties of at least one set of counterparts.  The Parties authorize each other to detach and combine original signature pages and consolidate them into a single identical original.  Any of such completely executed counterparts shall be sufficient proof of this Fifth Amendment.  Facsimile copies or PDF copies via e-mail of signature pages shall be effective and enforceable as originals.

 

	
  

	
c)

	
     Captions, Headings and Exhibits.  The captions and headings of this Fifth Amendment are for convenience only and have no force and effect in the interpretation or construction of this Fifth Amendment.  All exhibits attached hereto are by this reference incorporated herein as though fully set forth in this Fifth Amendment.

 

  

  

  

 

	
  

	
d)

	
     Severability.  If any term, provision, covenant or condition of this Fifth Amendment shall be or become illegal, null, void or against public policy, or shall be held by any court of competent jurisdiction to be illegal, null or void or against public policy, the remaining provisions of this Fifth Amendment shall remain in full force and effect and shall not be affected, impaired or invalidated thereby.  The term, provision, covenant or condition that is so invalidated, voided or held to be unenforceable shall be modified or changed by the Parties to the extent possible to carry out the intentions and directives set forth in this Fifth Amendment; provided, however, that this Fifth Amendment shall be void, in its entirety, if the consideration therefore fails, is rescinded, cancelled, or declared void or unenforceable for any reason.

 

	
  

	
e)

	
     Assignment of this Fifth Amendment.  No Party shall have the right to assign its rights or delegate any of its obligations or duties under this Fifth Amendment without the express written consent of all of the other Parties.

 

	
  

	
f)

	
     Successors and Assigns.  Except as restricted herein, this Fifth Amendment shall be binding on and shall inure to the benefit of the Parties and their respective legal representatives, successors and assigns.

 

	
  

	
g)

	
     Sophistication. The Parties by their acceptance of this Fifth Amendment hereby acknowledge that they are sophisticated business persons who were represented by counsel during the negotiations regarding the provisions of this Fifth Amendment and are fully informed regarding said provisions.

 

	
  

	
h)

	
     Waiver.  The waiver of any breach of any provision hereunder by any Party shall not be deemed to be a waiver of any preceding or subsequent breach hereunder, nor shall any waiver constitute a continuing waiver.  No waiver shall be binding unless executed in writing by the Party making the waiver.

 

	
  

	
i)

	
     Governing Law. This Fifth Amendment shall be governed by and construed and interpreted in accordance with the substantive laws of the State of California, without giving effect to any conflicts of law rule or principle that might require the application of the laws of another jurisdiction.  Each Party hereby irrevocably submits to the exclusive jurisdiction of the state and federal courts of the State of California in any such action, suit or proceeding, and agrees that any such action, suit or proceeding shall be brought only in such courts with venue for all purposes to be proper only in the state and federal courts located in Los Angeles County, California (and waives any objection based on forum non conveniens or any other objection to venue therein); provided, however, that such consent to jurisdiction shall not be deemed to be a general submission to the jurisdiction of said courts or in the State of California other than for such purpose.

 

	
  

	
j)

	
     Attorney’s Fees. Each of the Parties shall bear its own attorney’s fees and costs relating to the preparation of this Fifth Amendment and related documents.

  

  

  

	
  

	
k)

	
      No Third Party Rights. Nothing in this Fifth Amendment, whether express or implied, is intended to confer any rights or remedies under or by reason of this Fifth Amendment on any persons other than the Parties and their respective successors and assigns, nor is anything in this Fifth Amendment intended to relieve or discharge the obligations or liability of any third persons to any Party, nor shall any provision give any third persons any right of subrogation or action over or against any Party.

 

	
  

	
l)

	
     Entire Agreement and Amendment.  In conjunction with the matters considered herein, this Fifth Amendment, the First Amended Note and Second Note contain the entire understanding and agreement of the Parties and there have been no promises, representations, agreements, warranties or undertakings by any of the Parties, either oral or written, of any character or nature, hereafter binding except as set forth herein.  In the event of a conflict between this Fifth Amendment, the First Amended Note and Second Note, this Fifth Amendment shall govern. This Fifth Amendment may be altered, amended or modified only by an instrument in writing, executed by the Parties and by no other means.  Each Party waives its right to claim, contest or assert that this Agreement was modified, canceled, superseded or changed by any oral agreement, course of conduct, waiver or estoppel.

 

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IN WITNESS WHEREOF, the undersigned Parties hereby acknowledge that they have read, understand and consent to the modifications made to the LowCal Agreements by this Fifth Amendment.

	
 

EOS PETRO, INC.

 

 

By: /s/ Nikolas Konstant

Name: Nikolas Konstant

Title: Chairman

	
 

LOWCAL INDUSTRIES, LLC

 

 

By: /s/ Shlomo  Lowy

Name: Kinderlach Ltd Co

Its: Managing Member

	
EOS GLOBAL PETRO, INC.

 

 

By: /s/ Nikolas Konstant

 

Name: Nikolas  Konstant

Title: Chairman

	
SAIL PROPERTY MANAGEMENT GROUP, LLC

 

 

By: /s/ Shlomo  Lowy

Name: Sail Investments LLC

Title: Managing Member

 

 

LOWCO [EOS/PETRO], LLC

 

 

By: /s/ Shlomo  Lowy

Name: LowCo LLC

Its: Managing Member

 

 

 

 

 

Exhibit A

 

THE ISSUANCE AND SALE OF THE SECURITIES REPRESENTED BY THIS INSTRUMENT HAVE NOT BEEN REGISTERED UNDER THE SECURITIES ACT OF 1933, AS AMENDED (THE “SECURITIES ACT), OR APPLICABLE STATE SECURITIES LAWS. THE SECURITIES MAY NOT BE OFFERED FOR SALE, SOLD, TRANSFERRED OR ASSIGNED (I) IN THE ABSENCE OF (A) AN EFFECTIVE REGISTRATION STATEMENT FOR THE SECURITIES UNDER THE SECURITIES ACT, OR (B) AN OPINION OF COUNSEL, IN A FORM REASONABLY ACCEPTABLE TO THE COMPANY, THAT REGISTRATION IS NOT REQUIRED UNDER SAID ACT OR (II) UNLESS SOLD PURSUANT TO RULE 144 UNDER THE ACT.

 

EOS GLOBAL PETRO, INC.

 

AMENDED AND RESTATED

 

SECURED CONVERTIBLE PROMISSORY NOTE

 

Principal Amount:  $5,000,000.00

Note Purchase Price:  $4,980,000.00

 

Originally Issued:  February 8, 2013

Third Amendment and Restatement On:  January 13, 2015

 

FOR VALUE RECEIVED, EOS GLOBAL PETRO, INC., a Delaware corporation (the “Company”), hereby promises to pay to the order of LowCal Industries, LLC, a Wyoming liability company (“Holder”), or its registered assigns, the principal sum of Five Million Dollars ($5,000,000.00) (the “Aggregate Principal Amount”), with no interest (0%) per annum on all unpaid principal, subject to the provisions of this Note. All unpaid principal, together with any other amounts payable hereunder, shall be due and payable on the earliest to occur (the “Maturity Date”) of (i) June 30, 2015, (ii) when, upon or after the occurrence of an Event of Default (as defined below), such amounts are declared due and payable by Holder or made automatically due and payable in accordance with the terms hereof, or (iii) such other date as may otherwise be provided for in accordance with the provisions of this Note. All references to Dollars herein are to lawful currency of the United States of America.

 

This Note amends, and restates in its entirety a $5,000,000.00 secured promissory note originally issued by the Company to Holder on February 8, 2013, as subsequently amended (the “Original Note”). The Original Note first become convertible into shares of common stock of the Company at a conversion price of $2.50 per share pursuant to an amendment to the Original Note dated January 9, 2014. This Note became effective on January 13, 2015 in connection with a “Fifth Amendment to the LowCal Agreements” dated as of the same date between the Holder, the Company and certain affiliates thereof.

 

The following is a statement of the rights of Holder and the conditions to which this Note is subject, and to which Holder, by the acceptance of this Note, agrees:

 

1.           Interest. This Note shall not accrue any interest.

  

  

  

 

2.           Prepayments. The Company may prepay the outstanding principal amount of this Note in whole, but not in part, at any time (the “Prepayment Date”). If the Company intends to prepay this Note, it shall provide ten (10) Business Days written notice of such intention to Holder. For purposes of this Note, “Business Day” means any day other than a Saturday, Sunday or a day on which banking institutions in Los Angeles, California are authorized or obligated by law or executive order to close.

 

3.           Security. The obligations due under this Note will be secured pursuant to the existing filed mortgage in favor of Holder on the Company’s oil and gas leases constituting the Works Property in Edwards County, Illinois that secures obligations of the Company under the Original Note.

 

4.           Option of Holder to Convert Principal.

 

(a)           The principal amount of the Note may be converted in whole or in part at any time at the election of Holder into restricted shares of common stock of Eos Petro, Inc., a Nevada corporation (“Parent”) at a conversion price of $2.50 per share (“Conversion Shares”); provided, however, that under no circumstances may Holder elect to effect a conversion if, after giving effect to such conversion, LowCal Industries, LLC and its Affiliates (as defined below) would beneficially own in excess of 9.99% of the outstanding shares of the Parent’s common stock immediately after giving effect to such conversion. For purposes of the foregoing sentence, the number of shares beneficially owned by the Holder and its Affiliates shall include the number of shares issuable upon the conversion with respect to which the determination of such sentence is being made, but shall exclude the number of shares which would be issuable upon (A) conversion of the remaining, un-converted portion of this Note beneficially owned by Holder or any of its Affiliates and (B) exercise or conversion of the unexercised or non-converted portion of any other securities of the Parent (including, without limitation, any other notes or warrants) subject to a limitation on conversion or exercise analogous to the limitation contained herein beneficially owned by Holder or any of its Affiliates. Except as set forth in the preceding sentence, for purposes of this Section 4, beneficial ownership shall be calculated in accordance with Section 13(d) of the Securities Exchange Act of 1934, as amended, and the rules and regulations promulgated thereunder and the rules and regulations promulgated thereunder and the rules and regulations promulgated thereunder (the “Exchange Act”). To the extent that the limitation contained in this Section 4 applies, the determination of whether this Note is convertible (in relation to other securities owned by Holder) and of which a portion of this Note is convertible shall be in the sole discretion of Holder. To ensure compliance with this restriction, Holder will be deemed to represent to the Parent each time it elects to convert that such conversion has not violated the restrictions set forth in this Section 4, and the Parent shall have no obligation to verify or confirm the accuracy of such determination. In addition, a determination as to any group status as contemplated above shall be determined in accordance with Section 13(d) of the Exchange Act. For purposes of this Section 4, in determining the number of outstanding shares, Holder may rely on the number of outstanding shares as reflected in (x) the Parent’s most recent quarterly or annual report on Form 10-Q or Form 10-K or equivalent similar filing on such public disclosure service as the Parent may then be utilizing, as the case may be, (y) a more recent public announcement by the Parent including on the OTC Markets website, or (z) any other notice by the Parent or the Parent’s transfer agent approved by the Parent setting forth the number of shares outstanding. Upon the written or oral request of

  

  

  

 

Holder, the Parent shall within two (2) Business Days confirm orally and in writing to Holder the number of shares then outstanding on a fully diluted basis. The provisions of this Section 4 may be waived by the Holder, at the election of the Holder, upon not less than 61 days’ prior notice to the Parent, and the provisions of this Section 4 shall continue to apply until such 61st day (or such later date, as determined by Holder, as may be specified in such notice of waiver). The provisions of this Section 4 shall be implemented in a manner necessary to preserve the intended 9.99% beneficial ownership limitation herein contained and shall not be modified in a manner otherwise than in strict conformity with the terms of this Section 4 (or any portion hereof) which may be defective or inconsistent with the intended 9.99% beneficial ownership limitation herein contained or to make changes or supplements necessary or desirable to properly give effect to such 9.99% limitation. The limitations contained in this Section 4 shall apply to a successor holder of this Note. For purposes of this Note, “Affiliate” means any person that, directly or indirectly through one or more intermediaries, controls or is controlled by or is under common control with a Person, as such terms are used in and construed under Rule 144 under the Securities Act.

 

(b)           Upon delivery to the Parent of a notice of intent to convert, subject to compliance with Section 4(a), the Parent shall issue and deliver to Holder within ten (10) days after the date of such notice the number of shares of common stock of the Parent for the portion of the principal converted in accordance with the foregoing.

 

5.           Exit Stock and Exit Fee. On the date this Note has been paid in full, Holder shall be entitled to receive from the Parent 50,000 restricted shares of Parent’s common stock (the “Exit Stock”).  The Exit Fee previously set forth in the Original Note has been intentionally cancelled and is no longer issuable pursuant to the terms of this Note.

 

6.           Representations and Warranties of the Company.

 

(a)           Organization and Standing.  The Company is a corporation duly organized and validly existing under, and by virtue of, the laws of the State of Delaware and is in good standing under such laws.  The Company is qualified to do business as a foreign corporation in every jurisdiction in which the failure to so qualify would have a material adverse effect on the business, assets, liabilities, condition (financial or otherwise), results of operation or prospects of the Company.  The Company has the requisite corporate power and authority to own and operate its properties and assets and to carry on its business as presently conducted.

 

(b)           Corporate Power.  The Company has all requisite legal and corporate power and authority to enter into this Note and all other documents contemplated hereby, to sell this Note and to carry out and perform its other obligations under the terms of this Note and all other documents contemplated hereby.

 

(c)           Authorization.  All corporate action on the part of the Company, and its directors, officers and shareholders necessary for the authorization, execution, delivery and performance of all obligations of the Company under this Note and all other documents contemplated hereby and for the authorization, issuance and delivery by the Company of this Note has been taken.  This Note and all other documents contemplated hereby have been duly executed and delivered by the Company and constitute the valid and binding obligations of the

  

  

  

 

Company and are enforceable against the Company in accordance with their terms, except as enforceability may be limited by bankruptcy, insolvency or other laws affecting the enforcement of creditors’ rights generally, and except that the availability of the remedy of specific performance or other equitable relief is subject to the discretion of the court before which any proceeding therefor may be brought.

 

(d)           Compliance with Other Instruments.  The execution, delivery and performance of an compliance with this Note and all other documents contemplated hereby will not result in any such violation or be in conflict with or constitute a default under any of the terms or provisions of any mortgage, indenture, contract, agreement or instrument to which the Company is a party or by which it or its assets are bound, or result in the creation of any mortgages, pledges, liens, leases, encumbrances or charges upon any of the properties or assets of the Company pursuant to any such term or provision.

 

(e)           Consents.  No consent, order, approval or authorization of, or declaration, filing or registration with, any Governmental Authority or any person is required to be made or obtained by the Company in connection with the execution and delivery or the consummation of this Note and all other documents contemplated hereby.

 

7.           Events of Default. The occurrence of any of the following shall constitute an “Event of Default” under this Note:

 

(a)           Failure to Pay. The Company shall fail to pay (i) he principal on the due date hereunder and such payment shall not have been made within five (5) Business Days of Company’s receipt of Holder’s written notice to the Company of such failure to pay or (ii) any other payment required under the terms of this Note on the date due and such payment shall not have been made within five (5) Business Days of the Company’s receipt of Holder’s written notice to Company of such failure to pay;

 

(b)           Breaches. The Company shall fail to observe or perform any other material covenant, representation, or warranty, obligation, condition or agreement contained in this Note or any other LowCal Agreement and such failure shall continue uncured for thirty (30) days after written notice to Company of such failure;

 

(c)           Voluntary Bankruptcy or Insolvency Proceedings. The Company shall (i) apply for or consent to the appointment of a receiver, trustee, liquidator or custodian of itself or of all or a substantial part of its property, (ii) be unable, or admit in writing its inability, to pay its debts generally as they mature, (iii) make a general assignment for the benefit of it or any of its creditors, (iv) be dissolved or liquidated, (v) become insolvent (as such term may be defined or interpreted under any applicable statute), (vi) commence a voluntary case or other proceeding seeking liquidation, reorganization or other relief with respect to itself or its debts under any bankruptcy, insolvency or other similar law now or hereafter in effect or consent to any such relief or to the appointment of or taking possession of its property by any official in an involuntary case or other proceeding commenced against it, or (vii) take any action for the purpose of effecting any of the foregoing; or

 

 

 

 

 

(d)           Involuntary Bankruptcy or Insolvency Proceedings. Proceedings for the appointment of a receiver, trustee, liquidator or custodian of the Company or of all or a substantial part of the property thereof, or an involuntary case or other proceedings seeking liquidation, reorganization or other relief with respect to the Company or the debts thereof under any bankruptcy, insolvency or other similar law now or hereafter in effect shall be commenced and an order for relief entered or such proceeding shall not be dismissed or discharged within thirty (30) days of commencement.

 

8.           Rights of Holder upon Default. Upon the occurrence or existence of any Event of Default, and at any time thereafter during the continuance of such Event of Default, Holder may by written notice to the Company, declare all outstanding obligations payable by the Company hereunder to be immediately due and payable without presentment, demand, protest or any other notice of any kind, all of which are hereby expressly waived, anything contained herein to the contrary notwithstanding. In addition to the foregoing remedies, upon the occurrence or existence of any Event of Default, Holder may exercise any other right power or remedy granted to it by this Note or otherwise permitted to it by law, either by suit in equity or by action at law, or both.

 

9.           Transfer of this Note. With respect to any offer, sale or other disposition of this Note, Holder will give written notice to the Company prior thereto, describing briefly the manner thereof, together with a written opinion of Holder’s counsel (if requested by the Company), or other evidence reasonably satisfactory to the Company, to the effect that such offer, sale or other distribution may be effected without registration or qualification (under any federal or state law then in effect). Upon receiving such written notice and reasonably satisfactory opinion, if so requested, or other evidence, the Company, as promptly as practicable, shall notify Holder that Holder may sell or otherwise dispose of this Note in accordance with the terms of the notice delivered to Company. If a determination has been made pursuant to this Section 9 that the opinion of counsel for Holder, or other evidence, is not reasonably satisfactory to the Company, the Company shall so notify Holder promptly after such determination has been made. Subject to the foregoing, transfers of this Note shall be registered upon registration books maintained for such purpose by or on behalf of the Company. Prior to presentation of this Note for registration of transfer, the Company shall treat the registered holder hereof as the owner and holder of this Note for the purpose of receiving all payments of principal and for all other purposes whatsoever, whether or not this Note shall be overdue and the Company shall not be affected by notice to the contrary.

 

10.           Assignment by the Company. Neither this Note nor any of the rights, interests or obligations hereunder may be assigned, by operation of law or otherwise, in whole or in part, by the Company without the prior written consent of Holder.

 

11.           Waiver and Amendment. Any provision of this Note may be amended, waived or modified upon the written consent of the Company and the Holder.

 

12.           Notices. All notices or other communications hereunder shall be in writing and shall be deemed to have been duly given if delivered personally, or sent by a nationally recognized overnight delivery service, such as Federal Express, or mailed by certified or registered mail, return receipt requested, postage prepaid, to the following addresses:

 

 

 

 

 

If to the Company:

 

Eos Petro, Inc.

Attention: Nikolas Konstant

1999 Avenue of the Stars, Suite 2520

Los Angeles, CA 90067

Fax: (310 ) 277-0591

 

If to Holder:

 

LowCal Industries, LLC

6119 Greenville Ave.

Suite 340

Dallas, TX 75206-1910

Fax: (323) 443-3868

 

Notices sent by mail shall be deemed received on the earlier to occur of actual delivery to the recipient, or the 3rd day after deposit in the mail.

 

13.           Payment. Payment shall be made in lawful tender of the United States.

 

14.           Expenses; Waivers. If action is instituted to collect this Note, the Company promises to pay all costs and expenses, including, without limitation, reasonable attorneys’ fees and costs, incurred in connection with such action.

 

15.           Governing Law.  All questions concerning the construction, validity, enforcement and interpretation of this Note shall be governed by and construed and enforced in accordance with the internal laws of the State of Nevada, without regard to the principles of conflicts of law thereof.

 

16.           Trial by Jury. TO THE FULLEST EXTENT PERMITTED BY APPLICABLE LAW, EACH PARTY HERETO HEREBY IRREVOCABLY WAIVES ANY AND ALL RIGHT TO TRIAL BY JURY IN ANY LEGAL PROCEEDING ARISING OUT OF OR RELATING TO THIS NOTE OR THE TRANSACTIONS CONTEMPLATED HEREBY.

 

17.           Attorneys’ Fees. If either party shall commence an action or proceeding to enforce any provisions of this Note, then the prevailing party in such action or proceeding shall be reimbursed by the other party for its attorneys’ fees and other costs and expenses incurred with the investigation, preparation and prosecution of such action or proceeding.

 

18.           Saturdays, Sundays, Holidays, etc. If the last or appointed day for the taking of any action or the expiration of any right required or granted herein shall not be a Business Day, then such action may be taken or such right may be exercised on the next succeeding Business Day.

 

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IN WITNESS WHEREOF, the Company has caused this Note to be issued as of the date first written above.

 

	 	EOS GLOBAL PETRO INC., a Delaware corporation
	 	 
	 	By:	
/s/ Nikolas Konstant

	 	Name:	
Nikolas Konstant

	 	Title:	
Chairman

 

  

  

  

 

Exhibit B

 

EOS PETRO, INC.

UNSECURED PROMISSORY NOTE

 

	U.S. $3,250,000.00  	January 13, 2015

 

FOR VALUE RECEIVED, the undersigned, Eos Petro, Inc., a Nevada corporation (the “Issuer”), hereby promises to pay to the order of LowCal Industries, LLC, a Wyoming limited liability company or its permitted assigns (the “Payee”), at such place as the Payee shall direct in writing, the principal sum of Three Million Two Hundred Fifty Thousand Dollars ($3,250,000.00), with simple interest accruing at an annual rate at 10.00% on the unpaid balance.  All references to Dollars herein are to lawful currency of the United States of America.

All unpaid principal of this Note and accrued interest shall be due and payable on June 30, 2015. This Note may be prepaid at any time in whole or in part without penalty.  Notwithstanding the foregoing, Issuer hereby covenants, acknowledges and agrees that this Note carries the following prepayment obligation on the part of the Issuer: all unpaid principal and accrued interest on this Note shall be prepaid in full by the Issuer upon the earlier to occur of: (i) the Issuer closing a transaction to acquire the capital stock or all or substantially all of the assets of “Target A” or “Target B” (the identities of such targets have been made known to Payee prior to the date hereof); or (ii) the Issuer closing an offering of capital stock or other equity financing for a minimum of Twenty Million Dollars ($20,000,000.00).

In any action at law or in equity to enforce or construe any provisions or rights under this Note, the unsuccessful party or parties to such litigation, as determined by a court pursuant to a final offer, judgment or decree, shall pay to the successful party or parties all costs, expenses and reasonable attorneys' fees incurred by such successful party.

This Note and all actions arising out of or in connection with this Note shall be governed by and construed in accordance with the laws of the State of Nevada, without regard to the conflicts of law provisions of the State of Nevada, or of any other state.

Eos Petro, Inc.,

a Nevada corporation

By: /s/ Nikolas Konstant

       Nicholas Konstant

       Its: Chairman of the Board

  

  

  

 

Exhibit C

 

Piggyback Registration Rights Appendix

 

Capitalized terms used but not otherwise defined herein shall have the meanings ascribed to such terms in the Fifth Amendment.

 

a)           Definitions. For the purposes of this appendix:

 

 (i) the term “Selling Shareholder” shall mean the Holder, its executive officers and directors and each person, if any, who controls the Holder within the meaning of Section 15 of the Securities Act of 1933, as amended (the “Securities Act”), or Section 20 of the Exchange Act of 1934, as amended (the “Exchange Act”);

 

(ii) the term “Registration Statement” shall include any final prospectus, exhibit, supplement or amendment included in or relating to, and any document incorporated by reference in, the Registration Statement (or deemed to be a part thereof) referred to in this appendix;

 

(iii) the term “untrue statement” shall mean any untrue statement or alleged untrue statement of a material fact, or any omission or alleged omission to state in the Registration Statement a material fact required to be stated therein or necessary to make the statements therein, in the light of the circumstances under which they were made, not misleading;

 

(iv) the term “Company Subsidiaries” shall mean all subsidiaries of the Company, whether or not wholly or partially owned;

 

(v) the term “Holder” shall mean LowCal and LowCo; and

 

(vi) the term “Shares” shall mean all shares of common stock of the Company issued or issuable pursuant to any of the LowCal Agreements, the Second Note or the Amended First Note.

 

	
  

	
b)

	
Registration Requirements. If, at any time, the Company files a Registration Statement after the execution of the Fifth Amendment in which the Company proposes to register any of its securities under the Securities Act (other than pursuant to Form S-4, Form S-8 or any successor form of limited purpose), the Company will give notice at least 20 days prior to the filing of each such Registration Statement to the Holder of its intention to effect such a registration and will include in such registration all Shares with respect to which the Company has received requests for inclusion therein within 10 days after receipt of the Company’s notice.  In connection with any offering involving an underwriting of shares of the Company’s capital stock, the Company shall not be required to include any of the Holder’s Shares in such underwriting unless the Holder accepts the terms of the underwriting as agreed upon between the Company and its underwriters, and then only in such quantity as the underwriters determine in their 

 

  

  

  

 

	
  

	
 

	
sole discretion will not jeopardize the success of the offering by the Company.  If the total number of Shares requested by the Holder to be included in such offering exceeds the amount of securities to be sold other than by the Company that the underwriters determine in their reasonable discretion is compatible with the success of the offering, then the Company shall be required to include in the offering only that number of such securities, including the Shares, which the underwriters and the Company determine in their sole discretion will not jeopardize the success of the offering.

 

c)           Company Efforts. The Company shall use reasonable best efforts to:

 

(i) furnish to the Holder with respect to the Shares registered under the Registration Statement such number of copies of the Registration Statement and the prospectus (including supplemental prospectuses) filed with the Securities and Exchange Commission (“SEC”) in conformance with the requirements of the Securities Act and such other such documents as the Holder may reasonably request, in order to facilitate the public sale or other disposition of all or any of the Shares by the Holder;

 

(ii) make any necessary blue sky fillings to permit the Shares to be sold in any state requested by the Holder;

 

(iii) pay the expenses incurred by the Company and the Holder in complying with this appendix, including, all registration and filing fees, FINRA fees, exchange listing fees, printing expenses, fees and disbursements of counsel for the Holder, blue sky fees and expenses and the expense of any special audits incident to or required by any such registration (and including reasonable attorneys’ fees of one counsel to the Holder, but excluding any and all underwriting discounts and selling commissions applicable to the sale of Shares by the Holder);

 

(iv) advise the Holder, promptly after it shall receive notice or obtain knowledge of the issuance of any stop order by the SEC delaying or suspending the effectiveness of the Registration Statement or of the initiation of any proceeding for that purpose;

 

(v) promptly use its commercially reasonable best efforts to prevent the issuance of any stop order or to obtain its withdrawal at the earliest possible moment if such stop order should be issued; and

 

(vi) with a view to making available to the Holder the benefits of Rule 144 and any other rule or regulation of the SEC that may at any time permit the Holder to sell Shares to the public without registration, (i) make and keep public information available, as those terms are understood and defined in Rule 144, until the earlier of (A) such date as all of the Shares qualify to be resold pursuant to Rule 144 within any 90 day period without restriction or any other rule of similar effect or (B) such date as all of the 

 

  

  

  

 

Shares shall have been resold; (ii) file with the SEC in a timely manner all reports and other documents required of the Company under the Securities Act and under the Exchange Act; and (iii) furnish to the Holder upon request, as long as the Holder owns any Shares, (A) a written statement by the Company as to whether it has complied with the reporting requirements of the Securities Act and the Exchange Act, (B) a copy of the Company’s most recent Annual Report on Form 10-K or Quarterly Report on Form 10-Q, and (C) such other information as may be reasonably requested in order to avail the Holder of any rule or regulation of the SEC that permits the selling of any such Shares without registration.

 

	
  

	
d)

	
Underwriting Status. The Company understands that the Holder disclaims being an underwriter, but acknowledges that a determination by the SEC that the Holder is deemed an underwriter shall not relieve the Company of any obligations it has pursuant to this appendix.

 

e)           Registration Indemnification. For the purposes of this appendix:

 

(i) The Company agrees to indemnify and hold harmless each Selling Shareholder from and against any losses, claims, damages or liabilities to which such Selling Shareholder may become subject (under the Securities Act or otherwise) insofar as such losses, claims, damages or liabilities (or actions or proceedings in respect thereof) arise out of, or are based upon (i) any untrue statement contained in the Registration Statement, (ii) any inaccuracy in the representations and warranties of the Company contained in this appendix or the failure of the Company to perform its obligations hereunder or (iii) any failure by the Company to fulfill any undertaking included in the Registration Statement, and the Company will reimburse such Selling Shareholder for any reasonable legal expense or other actual accountable out-of-pocket expenses reasonably incurred in investigating, defending or preparing to defend any such action, proceeding or claim; provided, however, that the Company shall not be liable in any such case to the extent that such loss, claim, damage or liability arises out of, or is based upon, an untrue statement made in such Registration Statement in reliance upon and in conformity with written information furnished to the Company by or on behalf of such Selling Shareholder specifically for use in preparation of the Registration Statement or the failure of such Selling Shareholder to comply with its covenants and agreements contained herein or any statement or omission in any prospectus that is corrected in any subsequent prospectus that was delivered to the Selling Shareholder prior to the pertinent sale or sales by the Selling Shareholder.

 

(ii) The Holder agrees to indemnify and hold harmless the Company and Company Subsidiaries (and each person, if any, who controls the Company or Company Subsidiaries within the meaning of Section 15 of the Securities Act, each officer of the Company who signs the Registration

 

  

  

  

 

Statement and each director of the Company and Company Subsidiaries) from and against any losses, claims, damages or liabilities to which the Company or Company Subsidiaries (or any such officer, director or controlling person) may become subject (under the Securities Act or otherwise), insofar as such losses, claims, damages or liabilities (or actions or proceedings in respect thereof) arise out of, or are based upon, (i) any inaccuracy in the representations and warranties of the Holder contained in this appendix or the failure of the Holder to perform its obligations hereunder or (ii) any untrue statement contained in the Registration Statement if, and only if, such untrue statement was made in reliance upon and in conformity with written information furnished by or on behalf of the Holder specifically for use (and identified as such) in preparation of the Registration Statement, and Holder will reimburse the Company or Company Subsidiary (or such officer, director or controlling person, as the case may be), for any reasonable legal expense or other reasonable actual accountable out-of-pocket expenses reasonably incurred in investigating, defending or preparing to defend any such action, proceeding or claim.  The obligation to indemnify shall be limited to the net amount of the proceeds received by the Selling Shareholder from the sale of the Shares pursuant to the Registration Statement.

 

(iii) Promptly after receipt by any indemnified person of a notice of a claim or the beginning of any action in respect of which indemnity is to be sought against an indemnifying person pursuant to this appendix, such indemnified person shall notify the indemnifying person in writing of such claim or of the commencement of such action, but the omission to so notify the indemnifying party will not relieve it from any liability which it may have to any indemnified party under this appendix (except to the extent that such omission materially and adversely affects the indemnifying party’s ability to defend such action) or from any liability otherwise than under this appendix.  Subject to the provisions hereinafter stated, in case any such action shall be brought against an indemnified person, the indemnifying person shall be entitled to participate therein, and, to the extent that it shall elect by written notice delivered to the indemnified party promptly after receiving the aforesaid notice from such indemnified party, shall be entitled to assume the defense thereof, with counsel reasonably satisfactory to such indemnified person.  After notice from the indemnifying person to such indemnified person of its election to assume the defense thereof (unless it has failed to assume the defense thereof and appoint counsel reasonably satisfactory to the indemnified party), such indemnifying person shall not be liable to such indemnified person for any legal expenses subsequently incurred by such indemnified person in connection with the defense thereof; provided, however, that if there exists or shall exist a conflict of interest that would make it inappropriate, in the reasonable opinion of counsel to the indemnified person, for the same counsel to represent both the indemnified person and such indemnifying person or any affiliate or associate thereof, the

 

  

  

  

 

indemnified person shall be entitled to retain its own counsel (who shall not be the same as the opining counsel) at the expense of such indemnifying person; provided, however, that no indemnifying person shall be responsible for the fees and expenses of more than one separate counsel (together with appropriate local counsel) for all indemnified parties.  In no event shall any indemnifying person be liable in respect of any amounts paid in settlement of any action unless the indemnifying person shall have approved the terms of such settlement; provided, however, that such consent shall not be unreasonably withheld.  No indemnifying person shall, without the prior written consent of the indemnified person, effect any settlement of any pending or threatened proceeding in respect of which any indemnified person is or could reasonably have been a party and indemnification could have been sought hereunder by such indemnified person, unless such settlement includes an unconditional release of such indemnified person from all liability on claims that are the subject matter of such proceeding.

 

(iv) If the indemnification provided for in this appendix is unavailable to or insufficient to hold harmless an indemnified party in respect of any losses, claims, damages or liabilities (or actions or proceedings in respect thereof) referred to therein, then each indemnifying party shall contribute to the amount paid or payable by such indemnified party as a result of such losses, claims, damages or liabilities (or actions in respect thereof) in such proportion as is appropriate to reflect the relative fault of the Company and Company Subsidiaries, on the one hand, and the Holder, on the other, in connection with the statements or omissions or other matters which resulted in such losses, claims, damages or liabilities (or actions in respect thereof), as well as any other relevant equitable considerations.  The relative fault shall be determined by reference to, among other things, in the case of an untrue statement, whether the untrue statement relates to information supplied by the Company and Company Subsidiaries, on the one hand, or Holder, on the other, and the parties’ relative intent, knowledge, access to information and opportunity to correct or prevent such untrue statement.  The Company and the Holder agree that it would not be just and equitable if contribution pursuant to this subsection were determined by pro rata allocation or by any other method of allocation which does not take into account the equitable considerations referred to above in this subsection.  The amount paid or payable by an indemnified party as a result of the losses, claims, damages or liabilities (or actions in respect thereof) referred to above in this subsection shall be deemed to include any legal or other expenses reasonably incurred by such indemnified party in connection with investigating or defending any such action or claim.  Notwithstanding the provisions of this subsection, the Holder shall not be required to contribute any amount in excess of the amount by which the net amount received by the Holder from the sale of the Shares to which such loss relates exceeds the amount of any damages which the Holder has otherwise been required to pay to the Company or

 

  

  

  

 

Company Subsidiaries by reason of such untrue statement.  No person guilty of fraudulent misrepresentation (within the meaning of Section 11(f) of the Securities Act) shall be entitled to contribution from any person who was not guilty of such fraudulent misrepresentation.

 

	
  

	
f)

	
Allocation. Each of the Company and Holder acknowledges that the provisions of this appendix fairly allocate the risks in light of the ability of the parties to investigate the Company and Company Subsidiaries and their business in order to assure that adequate disclosure is made in the Registration Statement as required by the Securities Act and the Exchange Act.

 

	
  

	
g)

	
Additional Information. The Company may request the Holder to furnish the Company with such information with respect to the Holder and the Holder’s proposed distribution of any Shares pursuant to the Company’s Registration Statement, as applicable, as the Company may from time to time reasonably request in writing or as shall be required by law or by the SEC in connection therewith, and the Holder agrees to furnish the Company with such information as a condition to the inclusion of any of the Holder’s Shares in such Registration Statement.

 

	
  

	
h)

	
Survival. The obligations under this appendix shall survive completion of any offering of Shares in any Registration Statement for a period of two years from the effective date of such Registration Statement.EX-10.1

 Exhibit 10.1 

EXECUTION VERSION 
  

FORM OF 
 CONTINGENT VALUE RIGHTS
AGREEMENT 
 BETWEEN 
 BIOMARIN
PHARMACEUTICAL INC., 
 BIOMARIN FALCONS B.V. 

and 
 AMERICAN STOCK
TRANSFER & TRUST COMPANY, LLC, 
 AS RIGHTS AGENT 

Dated as of January 14, 2015 

 TABLE OF CONTENTS 

 

							
			
		  		  	 	Page	  
		
	
ARTICLE 1            DEFINITIONS
	  	 	1	  
			
	 Section 1.1
	  	 Definitions
	  	 	1	  
		
	
ARTICLE 2            CONTINGENT VALUE RIGHTS
	  	 	4	  
			
	 Section 2.1
	  	 Holders of CVRs; Appointment of Rights Agent
	  	 	4	  
			
	 Section 2.2 
	  	 Nontransferable
	  	 	5	  
			
	 Section 2.3
	  	 No Certificate; Registration; Registration of Transfer; Change of Address
	  	 	5	  
			
	 Section 2.4
	  	 Payment Procedures
	  	 	5	  
			
	 Section 2.5
	  	 No Voting, Dividends or Interest; No Equity or Ownership Interest
	  	 	7	  
			
	 Section 2.6 
	  	 Ability to Abandon CVR
	  	 	7	  
		
	 ARTICLE 3            
THE RIGHTS AGENT
	  	 	8	  
			
	 Section 3.1
	  	 Certain Duties and Responsibilities
	  	 	8	  
			
	 Section 3.2 
	  	 Certain Rights of Rights Agent
	  	 	8	  
			
	 Section 3.3
	  	 Resignation and Removal; Appointment of Successor
	  	 	10	  
			
	 Section 3.4
	  	 Acceptance of Appointment by Successor
	  	 	11	  
		
	 ARTICLE 4            
COVENANTS
	  	 	11	  
			
	 Section 4.1
	  	 List of Holders
	  	 	11	  
			
	 Section 4.2
	  	 Efforts
	  	 	11	  
			
	 Section 4.3
	  	 Guarantee
	  	 	11	  
			
	 Section 4.4
	  	 Fundamental Transactions
	  	 	12	  
		
	
ARTICLE 5            AMENDMENTS
	  	 	12	  
			
	 Section 5.1
	  	 Amendments Without Consent of Holders or Rights Agent
	  	 	12	  
			
	 Section 5.2
	  	 Amendments with Consent of Holders
	  	 	13	  
			
	 Section 5.3
	  	 Effect of Amendments
	  	 	13	  
		
	 ARTICLE 6            
MISCELLANEOUS
	  	 	13	  
			
	 Section 6.1
	  	 Notices to Rights Agent, Parent and Buyer
	  	 	13	  
			
	 Section 6.2
	  	 Notice to Holders
	  	 	14	  
			
	 Section 6.3
	  	 Entire Agreement
	  	 	14	  
			
	 Section 6.4
	  	 Successors and Assigns
	  	 	14	  
			
	 Section 6.5
	  	 Benefits of Agreement; Action by Majority of Holders
	  	 	15	  

  
 -i- 

 TABLE OF CONTENTS 

(continued) 

							
			
	 	  	 	  	Page	 
			
	 Section 6.6
	  	 Governing Law
	  	 	15	  
			
	 Section 6.7
	  	 Jurisdiction
	  	 	15	  
			
	 Section 6.8 
	  	 WAIVER OF JURY TRIAL
	  	 	16	  
			
	 Section 6.9
	  	 Severability Clause
	  	 	16	  
			
	 Section 6.10
	  	 Counterparts; Effectiveness
	  	 	16	  
			
	 Section 6.11
	  	 Termination
	  	 	16	  
			
	 Section 6.12
	  	 Force Majeure
	  	 	17	  
			
	 Section 6.13
	  	 Construction
	  	 	17	  

  
 -ii- 

 FORM OF 

CONTINGENT VALUE RIGHTS AGREEMENT 

THIS CONTINGENT VALUE RIGHTS AGREEMENT, dated as of January 14, 2015 (this “Agreement”), is entered into by and between
BioMarin Pharmaceutical Inc., a Delaware corporation (“Parent”), BioMarin Falcons B.V., a private company with limited liability organized under the laws of The Netherlands and a wholly owned indirect subsidiary of Parent
(“Buyer”), and American Stock Transfer & Trust Company, LLC, a New York limited liability trust company, as Rights Agent. 

PREAMBLE 
 WHEREAS, Prosensa
Holding N.V., a public limited liability company (naamloze vennootschap) organized under the laws of The Netherlands (the “Company”), Parent, and Buyer, have entered into a Purchase Agreement, dated as of November 23,
2014 (the “Purchase Agreement”), pursuant to which Buyer will commence a tender offer to purchase any and all of the outstanding ordinary shares, par value €0.01 per share of the Company (collectively, the
“Shares”); 
 WHEREAS, pursuant to the Purchase Agreement, and in accordance with the terms and conditions thereof, Buyer
has agreed to provide, or to designate one of its Affiliates to provide, Holders (as defined below) contingent value rights representing the right to receive the Milestone Payments (as defined below) contingent upon the achievement of the Milestones
(as defined below) during the Milestone Period (as defined below); and 
 WHEREAS, pursuant to this Agreement, the maximum potential amount
payable per CVR (as defined below) is $2.07, without interest. 
 NOW, THEREFORE, in consideration of the premises and the consummation of
the transactions referred to above, it is mutually covenanted and agreed, for the proportionate benefit of all Holders, as follows: 
 
ARTICLE 1 
 DEFINITIONS 

Section 1.1        Definitions.  

Capitalized terms used but not otherwise defined herein have the meanings ascribed thereto in the Purchase Agreement. The following terms have
the meanings ascribed to them as follows: 
 “Assignee” has the meaning set forth in Section 6.4. 

        “Carve-Out Transaction” means any transaction (including a sale of assets, merger, sale of
stock or other equity interests, or exclusive licensing transaction), other than a Change of Control, pursuant to which any Intellectual Property or Material Contracts necessary for the production, development or sale of the Product held or owned by
the Buyer or the Company, as applicable, immediately after the Closing are, directly or indirectly sold, exclusively licensed or otherwise transferred to or acquired by, directly or indirectly, a Person other than Parent or any of its Subsidiaries.

 “Change of Control” means (a) a sale or other disposition of all or
substantially all of the assets of Parent on a consolidated basis (other than to any Subsidiary (direct or indirect) of Parent and other than a transaction in which the holders of the outstanding voting securities of Parent immediately prior to such
transaction(s) (taken in the aggregate) receive as a distribution with respect to securities of Parent more than 50% of the total combined voting power of all outstanding voting securities of the acquiring entity or a parent or holding company of
the acquiring entity immediately after such transaction(s), or (b) a merger, consolidation, reorganization, recapitalization or similar transaction involving Parent except where the holders of the outstanding voting securities of Parent
immediately prior to such merger or consolidation (taken in the aggregate) possess beneficial ownership of 50% or more of the total combined voting power of all outstanding voting securities of Parent, the surviving entity, the acquiring entity or a
parent or holding company of the acquiring entity, immediately after such merger, consolidation, reorganization, recapitalization or similar transaction. 

“CVR” means the right of Holders to receive the Milestone Payments, pursuant to the Purchase Agreement and this Agreement.

 “CVR Register” has the meaning set forth in Section 2.3(b). 

“Diligent Efforts” means carrying out those obligations and tasks that comprise a level of effort and expenditure of
resources that is consistent with commercially reasonable practices normally and typically devoted by Parent in connection with the development and commercialization of a product owned or controlled by Parent which is, at the time of measurement of
the level of effort and expenditure of resources, similarly situated (including with respect to market potential and sales potential), and at a stage of development or commercialization similar, to the Product, based on conditions then prevailing
and reasonably expected to occur and reasonably taking into account, without limitation, issues of safety and efficacy, anticipated pricing and reimbursement rates, costs, labeling, pricing reimbursement, the competitiveness of alternative products
in the marketplace, the patent and other proprietary position of the Product and such other competitive products (including applicable intellectual property rights), the likelihood of and requirements for regulatory approval and other relevant
scientific, technical, legal and commercial factors. For the avoidance of doubt, a failure to achieve a Milestone in and of itself may be consistent with Diligent Efforts. 

“EU Milestone” will be deemed to occur upon Buyer’s or its Affiliates’ (or their respective successors or assigns)
receipt of approval (whether full, accelerated, conditional or otherwise) by the European Commission of a “marketing authorisation application” in the European Union, through the centralized procedure, for the Product, which authorization
grants Buyer or its Affiliates (or their respective successors or assigns) the right to market and sell the Product in the European Union in accordance with Applicable Law for the treatment of duchenne muscular dystrophy, including subject to any
applicable label restrictions. For the avoidance of doubt, the EU Milestone does not refer to approval in one or more individual countries of the European Union or to a positive opinion by the European Medicines Agency Committee for Medicinal
Products for human use. 

  
 2 

 “EU Milestone Achievement Certificate” has the meaning set forth in Section
2.4(b). 
 “EU Milestone Payment” means, per CVR, an amount in cash equal to $2.07, without interest. 

“EU Milestone Period” means the period commencing as of the date of this Agreement and ending at 11:59 p.m., New York City
time, on February 15, 2017. 
 “Guaranteed Obligations” has the meaning set forth in Section 4.3. 

“Holder” means, at the relevant time, a Person in whose name a CVR is registered in the CVR Register. 

“Loss” has the meaning set forth in Section 3.2(g). 

“Majority of Holders” means any record Holder or Holders of more than 50% of the outstanding CVRs, as set forth on the CVR
Register. 
 “Milestones” means the EU Milestone and the US Milestone. 

“Milestone Non-Achievement Certificate” has the meaning set forth in Section 2.4(c). 

“Milestone Payment” means each of the EU Milestone Payment and the US Milestone Payment. 

“Milestone Payment Date” means the date that is 15 Business Days following achievement of a Milestone. 

“Milestone Period” means the EU Milestone Period and the US Milestone Period, as applicable. 

“Notice” has the meaning set forth in Section 6.1. 

“Officer’s Certificate” means a certificate (i) signed by an authorized officer of Parent, in his or her capacity
as such, and (ii) delivered to the Rights Agent. 
 “Permitted Transfer” means a Transfer of one or more CVRs
(i) upon death by will or intestacy; (ii) by instrument to an inter vivos or testamentary trust in which the CVRs are to be passed to beneficiaries upon the death of the trustee; (iii) made pursuant to a court order;
(iv) made by operation of law (including a consolidation or merger) or without consideration in connection with the dissolution, liquidation or termination of any corporation, limited liability company, partnership or other entity; (v) in
the case of CVRs payable to a nominee, from a nominee to a beneficial owner (and, if applicable, through an intermediary) or from such nominee to another nominee for the same beneficial owner, in each case as allowable by The Depository Trust
Company (“DTC”); (vi) to Parent, Buyer or their Affiliates; or (vii) as provided in Section 2.6. 

  
 3 

 “Product” means drisapersen. 

“Rights Agent” means the Rights Agent named in the first paragraph of this Agreement, until a successor Rights Agent will
have become such pursuant to the applicable provisions of this Agreement, and thereafter “Rights Agent” will mean such successor Rights Agent. 

“Transfer” means transfer, pledge, hypothecation, encumbrance, assignment or other disposition (whether by sale, merger,
consolidation, liquidation, dissolution, dividend, distribution or otherwise), the offer to make such a transfer or other disposition, and each Contract, arrangement or understanding, whether or not in writing, to effect any of the foregoing. 

“US Milestone” will be deemed to occur upon Buyer’s or its Affiliates’ (or their respective successors or assigns)
receipt of approval (whether full, accelerated, conditional or otherwise) by the United States Food and Drug Administration of a “new drug application”, which approval grants Buyer or its Affiliates (or their respective successors or
assigns) the right to market and sell the Product in the United States in accordance with Applicable Law for the treatment of duchenne muscular dystrophy, including subject to any applicable label restrictions. 

“US Milestone Achievement Certificate” has the meaning set forth in Section 2.4(a). 

“US Milestone Payment” means, per CVR, an amount in cash equal to $2.07, without interest. 

“US Milestone Period” means the period commencing as of the date of this Agreement and ending at 11:59 p.m., New York City
time, on May 15, 2016. 
 ARTICLE 2 

CONTINGENT VALUE RIGHTS 
 
Section 2.1        Holders of CVRs; Appointment of Rights Agent. 

(a)    As provided in the Purchase Agreement, each Holder will be entitled to one CVR for (i) each Share that is
validly accepted for payment, and paid for, pursuant to Section 2.01(d) of the Purchase Agreement or pursuant to any Subsequent Offering Period, (ii) each Share underlying a Company Option that is cancelled pursuant to Section 2.03(a)
or Section 2.03(b) of the Purchase Agreement, (iii) each Company Restricted Share that is validly tendered and accepted for payment and paid for pursuant to Section 2.03(c) of the Purchase Agreement and (iv) each Share (including
Company Restricted Share) converted, cancelled or otherwise entitled to receive a CVR pursuant to Section 2.04(f) or 2.07 of the Purchase Agreement. 

(b)    Buyer hereby appoints the Rights Agent to act as rights agent for Buyer in accordance with the terms and conditions
set forth in this Agreement, and the Rights Agent hereby accepts such appointment. 

  
 4 

Section 2.2        Nontransferable. 

A Holder may not at any time Transfer a CVR, other than pursuant to a Permitted Transfer. Any attempted Transfer that is not a Permitted
Transfer, in whole or in part, will be void ab initio and of no effect. 

Section 2.3        No Certificate; Registration; Registration
of Transfer; Change of Address. 
 (a)    CVRs will not be evidenced by a certificate or other instrument. 

(b)    The Rights Agent will maintain an up-to-date register (the “CVR Register”) for the purposes of
(i) identifying the Holders of CVRs and (ii) registering CVRs and Permitted Transfers thereof. The CVR Register will show one position for Cede & Co. representing all the Shares held by DTC on behalf of the beneficial street
holders of the Shares that were tendered by such holders in the Offer and accepted for payment or held by such holders as of immediately prior to the Second Step Distribution. The Rights Agent will have no responsibility whatsoever directly to the
street holders with respect to Transfers of CVRs. 
 (c)    Subject to the restriction on transferability set forth in
Section 2.2, every request made to Transfer a CVR must be in writing and accompanied by a written instrument of Transfer and other requested documentation in form reasonably satisfactory to the Rights Agent, duly executed by the Holder or Holders
thereof, or by the duly appointed legal representative, personal representative or survivor of such Holder or Holders, setting forth in reasonable detail the circumstances relating to the Transfer. Upon receipt of such written notice, the Rights
Agent will, subject to its reasonable determination that the Transfer instrument is in proper form and the Transfer is a Permitted Transfer and otherwise complies with the other terms and conditions of this Agreement, register the Transfer of the
applicable CVRs in the CVR Register. All Transfers of CVRs registered in the CVR Register will be the valid obligations of Buyer, evidencing the same right, and entitling the transferee to the same benefits and rights under this Agreement, as those
held by the transferor. Any Transfer of CVRs will be without charge (other than the cost of any transfer tax) to the applicable Holder. 

(d)    A Holder may make a written request to the Rights Agent to change such Holder’s address of record in the CVR
Register. Such written request must be duly executed by such Holder. Upon receipt of such written notice, the Rights Agent will promptly record the change of address in the CVR Register. 

Section 2.4        Payment Procedures. 

(a)    If the US Milestone occurs at any time prior to the expiration of the U.S. Milestone Period, then, on or prior to
the Milestone Payment Date relating thereto, Buyer will deliver to the Rights Agent (i) a certificate (the “US Milestone Achievement Certificate”) certifying the date of the satisfaction of the US Milestone and that the Holders
are entitled to receive the US Milestone Payment and (ii) a wire transfer of immediately available funds to an account designated by the Rights Agent, in the aggregate amount equal to the number of CVRs (as reflected in the CVR Register) then
outstanding multiplied by the amount of the US Milestone Payment. After receipt of the wire transfer described in the foregoing sentence, the Rights Agent will promptly (and in any event, within 10 Business Days) pay, by check mailed,

  
 5 

 
first-class postage prepaid, to the address of each holder set forth in the CVR Register or by other method of delivery as specified by the applicable Holder in writing to the Rights Agent, an
amount in cash equal to the number of CVRs registered to such Holder in the CVR Register multiplied by the US Milestone Payment. For the avoidance of doubt, each of the requirements of the US Milestone must be fully satisfied for the US Milestone to
be considered to be attained, and the Holders will not be entitled to, and Parent and Buyer will not be liable for, any US Milestone Payments in the event of any partial satisfaction of the US Milestone. 

(b)    If the EU Milestone occurs at any time prior to the expiration of the EU Milestone Period, then, on or prior to the
Milestone Payment Date relating thereto, Buyer will deliver to the Rights Agent (i) a certificate (the “EU Milestone Achievement Certificate”) certifying the date of the satisfaction of the EU Milestone and that the Holders are
entitled to receive the EU Milestone Payment and (ii) a wire transfer of immediately available funds to an account designated by the Rights Agent, in the aggregate amount equal to the number of CVRs then outstanding (as reflected on the CVR
Register) multiplied by the amount of the EU Milestone Payment. After receipt of the wire transfer described in the foregoing sentence, the Rights Agent will promptly (and in any event, within 10 Business Days) pay, by check mailed, first-class
postage prepaid, to the address of each Holder set forth in the CVR Register or by other method of delivery as specified by the applicable Holder in writing to the Rights Agent, an amount in cash equal to the number of CVRs registered to such Holder
in the CVR Register multiplied by the EU Milestone Payment. For the avoidance of doubt, each of the requirements of the EU Milestone must be fully satisfied for the EU Milestone to be considered to be attained, and the Holders will not be entitled
to, and Parent and Buyer will not be liable for, any EU Milestone Payments in the event of any partial satisfaction of the EU Milestone. 

(c)    If a Milestone is not attained at any time prior to the expiration of the applicable Milestone Period, then on or
before the date that is 10 Business Days after the end of such Milestone Period, Buyer will deliver to the Rights Agent an Officer’s Certificate (the “Milestone Non-Achievement Certificate”) certifying that the applicable
Milestone has not occurred and that Buyer has complied in all material respects with its obligations under this Agreement. The Rights Agent will promptly (and in any event, within 10 Business Days after receipt) deliver a copy of such Milestone
Non-Achievement Certificate to the Holders. The Rights Agent will deliver to Buyer a certificate certifying the date of delivery of such certificate to the Holders. 

(d)    If the Rights Agent does not receive from the Majority of Holders a written objection to a Milestone
Non-Achievement Certificate within 30 Business Days after the date of delivery of such Milestone Non-Achievement Certificate by the Rights Agent to the Holders, the Holders will be deemed to have accepted such Milestone Non-Achievement Certificate,
and Parent, Buyer and their Affiliates will have no further obligation with respect to the applicable Milestone Payment. 

(e)    Except to the extent any portion of any Milestone Payment is required to be treated as imputed interest pursuant to
Applicable Law, the Holders and the parties hereto agree to treat the CVRs and all Milestone Payments for all Tax purposes as additional consideration for the Shares, Company Options and Company Restricted Shares pursuant to the Purchase Agreement,
and none of the Holders and the parties hereto will take any position to the contrary on any Tax Return or for other Tax purposes except as required by Applicable Law. 

  
 6 

 (f)    Parent, Buyer and the Rights Agent will be entitled to deduct and
withhold, or cause to be deducted and withheld, from any Milestone Payment otherwise payable pursuant to this Agreement, such amounts as it is required to deduct and withhold with respect to the making of such payment under any provision of
Applicable Law relating to Taxes, including with respect to Milestone Payments that relate to Company Options cancelled pursuant to Section 2.03(a) of the Purchase Agreement or a Reorganization. To the extent that amounts are so deducted and
withheld, such deducted and withheld amounts will be treated for all purposes of this Agreement as having been paid to the Holder in respect of which such deduction and withholding was made. Prior to making any such Tax deductions or withholdings or
causing any such Tax deductions or withholdings to be made with respect to any Holder, the Rights Agent will, to the extent practicable, provide notice to the Holder of such potential Tax deduction or withholding and a reasonable opportunity for the
Holder to provide any necessary Tax forms (including an IRS Form W-9 or an applicable IRS Form W-8) in order to avoid or reduce such withholding amounts; provided that the time period for payment of a Milestone Payment by the Rights
Agent set forth in Section 2.4(a) or Section 2.4(b) will be extended by a period equal to any delay caused by the Holder providing such forms, provided, further, that in no event shall such period be extended for more
than 10 days, unless otherwise requested by the Holder for the purpose of delivering such forms and agreed to by the Rights Agent. 

(g)    Any portion of a Milestone Payment that remains undistributed to the Holders six months after the Milestone Payment
Date will be delivered by the Rights Agent to Buyer, and any Holder will thereafter look only to Buyer for payment of such Milestone Payment. 

(h)    If any Milestone Payment (or portion thereof) remains unclaimed by a Holder two years after the Milestone Payment
Date (or immediately prior to such earlier date on which such Milestone Payment would otherwise escheat to or become the property of any Governmental Entity), such Milestone Payment (or portion thereof) will, to the extent permitted by Applicable
Law, become the property of Buyer, free and clear of all claims or interest of any Person previously entitled thereto. Neither Buyer nor the Rights Agent will be liable to any Person in respect of a Milestone Payment delivered to a public official
pursuant to any applicable abandoned property, escheat or similar legal requirement under Applicable Law. 

Section 2.5        No Voting, Dividends or Interest; No Equity
or Ownership Interest. 
 (a)    CVRs will not have any voting or dividend rights, and interest will not accrue on
any amounts payable in respect of CVRs. 
 (b)    CVRs will not represent any equity or ownership interest in Parent,
Buyer, any constituent company to the Reorganization or any of their respective Affiliates. 

Section 2.6        Ability to Abandon CVR. 

A Holder may at any time, at such Holder’s option, abandon all of such Holder’s remaining rights in a CVR by transferring such CVR
to Buyer without consideration therefor, and such rights will be cancelled. Nothing in this Agreement is intended to prohibit Parent, Buyer or its Affiliates from offering to acquire or acquiring CVRs, in private transactions or otherwise, for
consideration in its sole discretion. 

  
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 ARTICLE 3 

THE RIGHTS AGENT 
 
Section 3.1        Certain Duties and Responsibilities. 

(a)    The Rights Agent will not have any liability for any actions taken or not taken in connection with this Agreement,
except to the extent such liability arises as a result of the willful misconduct, bad faith or gross negligence of the Rights Agent or breach by the Rights Agent of this Agreement. 

(b)    Only the Majority of Holders may direct the Rights Agent to act on behalf of the Holders in enforcing any of their
rights hereunder. The Rights Agent will be under no obligation to institute any claim, action, suit, audit, investigation or proceeding, or to take any other action likely to result in the incurrence of material expenses by the Rights Agent, unless
the Majority of Holders (on behalf of the Holders) will furnish the Rights Agent with reasonable security and indemnity for any costs and expenses that may be incurred. All rights of action under this Agreement may be enforced by the Rights Agent,
any claim, action, suit, audit, investigation or proceeding instituted by the Rights Agent will be brought in its name as the Rights Agent and any recovery in connection therewith will be for the proportionate benefit of all the Holders, as their
respective rights or interests may appear on the CVR Register. 

Section 3.2        Certain Rights of Rights Agent. 

(a)    The Rights Agent undertakes to perform such duties and only such duties as are specifically set forth in this
Agreement, and no implied covenants or obligations will be read into this Agreement against the Rights Agent. 

(b)    The Rights Agent may rely and will be protected by Buyer in acting or refraining from acting upon any resolution,
certificate, statement, instrument, opinion, report, notice, request, direction, consent, order or other paper or document believed by it in good faith to be genuine and to have been signed or presented by the proper party or parties. 

(c)    Whenever the Rights Agent deems it desirable that a matter be proved or established prior to taking or omitting any
action hereunder, the Rights Agent may, in the absence of bad faith, gross negligence or willful misconduct on its part, (i) rely upon an Officer’s Certificate, which Officer’s Certificate shall be full authorization and protection to
the Rights Agent and (ii) incur no liability and be held harmless by Buyer for or in respect of any action taken or omitted to be taken by it under the provisions of this Agreement in reliance upon such Officer’s Certificate. 

(d)    The Rights Agent may engage and consult with counsel of its selection, and the written advice or opinion of such
counsel will, in the absence of bad faith, gross negligence or willful misconduct on the part of the Rights Agent, be full and complete authorization and protection in respect of any action taken or not taken by the Rights Agent hereunder in good
faith and in reliance thereon. 

  
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 (e)    Any permissive rights of the Rights Agent hereunder will not be
construed as a duty. 
 (f)    The Rights Agent will not be required to give any note or surety in respect of the
execution of its powers or otherwise under this Agreement. 
 (g)    Buyer agrees to indemnify the Rights Agent for, and
to hold the Rights Agent harmless from and against, any loss, liability, damage or expense (each, a “Loss”) suffered or incurred by the Rights Agent and arising out of or in connection with Rights Agent’s performance of its
obligations under this Agreement, including the reasonable costs and expenses of defending the Rights Agent against any claims, charges, demands, actions or suits arising out of or in connection with such performance, except to the extent such Loss
has been determined by a court of competent jurisdiction to have resulted from the Rights Agent’s gross negligence, bad faith or willful misconduct or breach of this Agreement. Buyer’s obligations under this Section 3.2(g) to
indemnify the Rights Agent will survive the resignation or removal of any Rights Agent and the termination of this Agreement. 

(h)    In addition to the indemnification provided under Section 3.2(g), Buyer agrees (i) to pay the fees of the
Rights Agent in connection with the Rights Agent’s performance of its obligations hereunder, as agreed upon in writing by the Rights Agent and Buyer on or prior to the date of this Agreement, and (ii) to reimburse the Rights Agent promptly
upon demand for all reasonable and documented out-of-pocket expenses, including all Taxes (other than income, receipt, franchise or similar Taxes) and governmental charges, incurred by the Rights Agent in the performance of its obligations under
this Agreement, except that Buyer will have no obligation to pay the fees of the Rights Agent or reimburse the Rights Agent in connection with any lawsuit initiated by the Rights Agent on behalf of itself or the Holders, except in the case of any
suit enforcing the provisions of Section 2.4(a) and/or Section 2.4(b), if Buyer is found by a court of competent jurisdiction to be liable to the Rights Agent or the Holders, as applicable in such suit (and for the avoidance
of doubt, to the extent that the Majority of Holders have furnished the Rights Agent reasonable security and indemnity related to such suit, Buyer shall reimburse such Holders for the cost of such security and indemnity). 

(i)    No provision of this Agreement will require the Rights Agent to expend or risk its own funds or otherwise incur any
financial liability in the performance of any of its duties hereunder or in the exercise of its rights if there are reasonable grounds for believing that repayment of such funds or adequate indemnification against such risk or liability is not
reasonably assured to it. 
 (j)    The Rights Agent will not be deemed to have knowledge of any event of which it was
supposed to receive notice thereof hereunder, and the Rights Agent shall be fully protected and will not incur any liability for failing to take action in connection therewith, in each case, unless and until it has received such notice in writing.

  
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 (k)    The Rights Agent and any shareholder, affiliate, director, officer or
employee of the Rights Agent may buy, sell or deal in any securities of Parent, Buyer or the Company or become peculiarly interested in any transaction in which Parent, Buyer or the Company may be interested, or contract with or lend money to
Parent, Buyer or the Company or otherwise act as fully and freely as though it were not the Rights Agent under this Agreement. Nothing herein will preclude the Rights Agent from acting in any other capacity for Parent, Buyer or the Company or for
any other Person. 
 (l)    The Rights Agent may execute and exercise any of the rights or powers hereby vested in it or
perform any duty hereunder either itself or by or through its attorney or agents. 

Section 3.3        Resignation and Removal; Appointment of
Successor. 
 (a)    The Rights Agent may resign at any time by giving written notice to Buyer, specifying a date
when such resignation will take effect, which notice will be given at least 60 days prior to the date so specified (or, if earlier, the appointment of the successor Rights Agent). 

(b)    Buyer will have the right to remove the Rights Agent at any time by a written notice to the Rights Agent,
specifying a date when such removal will take effect. Notice of such removal will be given by Buyer to the Rights Agent, which notice will be given at least 60 days prior to the date so specified (or, if earlier, the appointment of the successor
Rights Agent). 
 (c)    If the Rights Agent resigns, is removed or become incapable of acting, Buyer will promptly
appoint a qualified successor Rights Agent. Notwithstanding the foregoing, if Buyer fails to make such appointment within a period of 60 days after giving notice of such removal or after it has been notified in writing of such resignation or
incapacity by the resigning or incapacitated Rights Agent, then the incumbent Rights Agent may apply to any court of competent jurisdiction for the appointment of a new Rights Agent. The successor Rights Agent so appointed will, upon its acceptance
of such appointment in accordance with this Section 3.3(c) and Section 3.4, become the Rights Agent for all purposes hereunder. 

(d)    Buyer will give notice of each resignation or removal of the Rights Agent and each appointment of a successor
Rights Agent in accordance with Section 6.2. Each notice will include the name and address of the successor Rights Agent. If Buyer fails to send such notice within 10 Business Days after acceptance of appointment by a successor Rights Agent, the
successor Rights Agent will cause the notice to be mailed at the expense of Buyer. 
 (e)    Notwithstanding anything to
the contrary in this Section 3.3, unless consented to in writing by the Majority of Holders, Buyer will not appoint as a successor Rights Agent any Person that is not a stock transfer agent of national reputation or the corporate trust
department of a commercial bank. 
 (f)    The Rights Agent will cooperate with Buyer and any successor Rights Agent in
connection with the transition of the duties and responsibilities of the Rights Agent to the successor Rights Agent, including transferring the CVR Register to the successor Rights Agent. 

  
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Section 3.4        Acceptance of Appointment by Successor.

 Every successor Rights Agent appointed hereunder will, at or prior to such appointment, execute, acknowledge and deliver to Buyer and to
the retiring Rights Agent an instrument accepting such appointment and a counterpart of this Agreement, and such successor Rights Agent, without any further act, deed or conveyance, will become vested with all the rights, powers, trusts and duties
of the Rights Agent; provided that upon the request of Buyer or the successor Rights Agent, such resigning or removed Rights Agent will execute and deliver an instrument transferring to such successor Rights Agent all the rights,
powers and trusts of such resigning or removed Rights Agent. 
 ARTICLE 4 

COVENANTS 
 
Section 4.1        List of Holders. 
 Buyer will furnish or cause to be furnished
to the Rights Agent, in such form as Buyer receives from the Company’s transfer agent (or other agent performing similar services for the Company), the names and addresses of the Holders within 15 Business Days following the Closing Date. 

Section 4.2        Efforts. 

During the US Milestone Period, Buyer (and its successors and assigns) will, and will cause its (and their) Affiliates to, use Diligent
Efforts to achieve the US Milestone prior to the end of the US Milestone Period, and during the EU Milestone Period, Buyer (and its successors and assigns) will, and will cause its (and their) Affiliates to, use Diligent Efforts to achieve the EU
Milestone prior to the end of the EU Milestone Period. 

Section 4.3        Guarantee. 

Parent, intending to be legally bound, hereby absolutely, irrevocably and unconditionally guarantees the due and punctual payment and
performance of (a) Buyer’s obligations under this Agreement, including to pay the US Milestone Payment and EU Milestone Payment when as and if such payments become due, and (b) Buyer’s liability and obligations (including for
breach and for the aggregate amounts of the US Milestone Payment and EU Milestone Payment when, as and if due to Holders) under this Agreement (collectively, the “Guaranteed Obligations”). Parent acknowledges and agrees that such
guarantee shall be a guarantee of performance and not of collection and shall not be conditioned or contingent upon pursuit of any remedies against Buyer. If Buyer shall default in the due and punctual performance of the Guaranteed Obligations,
Parent will forthwith perform or cause to be performed such Guaranteed Obligations at its sole cost and expense. This guarantee may not be revoked or terminated and shall remain in full force and effect without interruption and shall be binding on
Parent and its successors and assigns until the Guaranteed Obligations have been satisfied in full. 

  
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Section 4.4        Fundamental Transactions. 

In the event that Parent or Buyer desires to consummate a Change of Control or Carve-Out Transaction while either of the US Milestone and/or
the EU Milestone has not been attained but remains eligible to be attained, Parent will cause the Person acquiring Parent (or acquiring substantially all of its assets ) with respect to a Change of Control or the Person acquiring the subject
Intellectual Property or Material Contracts with respect to a Carve-Out Transaction to assume Parent’s and Buyer’s (as applicable, depending upon the structure of the Change of Control or Carve-Out Transaction) obligations, duties and
covenants under this Agreement. No later than the consummation of any Change of Control or Carve-Out Transaction, Parent shall deliver to the Rights Agent an Officer’s Certificate, stating that such Change of Control or Carve-Out Transaction
complies with this Section 4.4 and that all conditions precedent herein provided for relating to such transaction have been complied with. 

ARTICLE 5 

AMENDMENTS 
 
Section 5.1        Amendments Without Consent of Holders or Rights Agent. 

(a)    Parent and Buyer, at any time or from time to time, may unilaterally enter into one or more amendments to this
Agreement for any of the following purposes, without the consent of any of the Holders or the Rights Agent, so long as, in the cases of clauses (ii) through (iv), such amendments do not, individually or in the aggregate, adversely affect the
interests of the Holders: 
 (i)    to evidence the appointment of another Person as a successor Rights
Agent and the assumption by any successor Rights Agent of the covenants and obligations of the Rights Agent herein in accordance with the provisions hereof; 

(ii)    to add to the covenants of Parent or Buyer such further covenants, restrictions, conditions or
provisions for the protection and benefit of the Holders; 
 (iii)    to cure any ambiguity, to correct
or supplement any provision in this Agreement that may be defective or inconsistent with any other provision in this Agreement, or to make any other provisions with respect to matters or questions arising under this Agreement; 

(iv)    as may be necessary or appropriate to ensure that CVRs are not subject to registration under the
1933 Act or the 1934 Act; or 
 (v)    any other amendment to this Agreement that would provide any
additional rights or benefits to the Holders or that does not adversely affect the legal rights under this Agreement of any such Holder. 
 Notwithstanding
anything to the contrary contained herein, Parent, Buyer and the Rights Agent may, but will not be obligated to, enter into any amendment that adversely affects, in any material respect, the Rights Agent’s own rights, duties, responsibilities
or protections. 

  
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 (b)    Promptly after the execution by Parent and Buyer of any amendment
pursuant to this Section 5.1, Buyer will (or will cause the Rights Agent to) notify the Holders in general terms of the substance of such amendment in accordance with Section 6.2. 

Section 5.2        Amendments with Consent of Holders.

 (a)    In addition to any amendments to this Agreement that may be made by Parent and Buyer without the consent of
any Holder or the Rights Agent pursuant to Section 5.1, with the consent of the Majority of Holders, Parent, Buyer and the Rights Agent may enter into one or more amendments to this Agreement for the purpose of adding, eliminating or changing
any provisions of this Agreement, even if such addition, elimination or change is adverse to the interests of the Holders. 

(b)    Promptly after the execution by Parent, Buyer and the Rights Agent of any amendment pursuant to the provisions of
this Section 5.2, Buyer will (or will cause the Rights Agent to) notify the Holders in general terms of the substance of such amendment in accordance with Section 6.2. 

Section 5.3        Effect of Amendments. 

Upon the execution of any amendment under this Article 5, this Agreement will be modified in accordance therewith, such amendment will form a
part of this Agreement for all purposes and every Holder will be bound thereby. 
 ARTICLE 6 

MISCELLANEOUS 
 
Section 6.1        Notices to Rights Agent, Parent and Buyer. 
 All notices,
requests and other communications (each, a “Notice”) to any party hereunder (shall be in writing. Such Notice shall be deemed given (a) on the date of delivery, if delivered in person or by facsimile or e-mail (upon
confirmation of receipt) prior to 5:00 p.m. in the time zone of the receiving party or on the next Business Day, if delivered after 5:00 p.m. in the time zone of the receiving party or (b) on the first Business Day following the date of
dispatch, if delivered by FedEx or by other internationally recognized overnight courier service (upon proof of delivery), addressed as follows: 

if to the Rights Agent, to: 

American Stock Transfer & Trust Company, LLC 

6201 15th Avenue 

Brooklyn, NY 11219 

Attention: Relationship Management 

with a copy, which shall not constitute notice, to: 

American Stock Transfer & Trust Company, LLC 

6201 15th Avenue 

Brooklyn, NY 11219 

Attention: General Counsel 

  
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 if to Parent or Buyer, to: 

BioMarin Pharmaceutical Inc. 

105 Digital Drive 

Novato, CA 94949 

Attention: Chief Executive Officer 

with a copy, which shall not constitute notice, to: 

BioMarin Pharmaceutical Inc. 

105 Digital Drive 

Novato, CA 94949 

Attention: General Counsel 
 or
to such other address or facsimile number as such party may hereafter specify for the purpose by notice to the other parties hereto. 
 
Section 6.2        Notice to Holders. 
 All Notices required to be given to the
Holders will be given (unless otherwise herein expressly provided) in writing and mailed, first-class postage prepaid, to each Holder affected by such event, at his, her or its address set forth in the CVR Register, not later than the latest date,
and not earlier than the earliest date, prescribed for the sending of such Notice, if any, and will be deemed given on the date of mailing. In any case where notice to the Holders is given by mail, neither the failure to mail such Notice, nor any
defect in any Notice so mailed, to any particular Holder will affect the sufficiency of such Notice with respect to other Holders. 
 
Section 6.3        Entire Agreement. 
 This Agreement and the Purchase Agreement
constitute the entire agreement between the parties with respect to the subject matter of this Agreement and supersede all prior agreements and understandings, both written and oral, among or between any of the parties with respect to the subject
matter of this Agreement. 
 Section 6.4        Successors
and Assigns. 
 This Agreement will be binding upon, and will be enforceable by and inure solely to the benefit of, the Holders, Parent,
Buyer and the Rights Agent and their respective successors and assigns. The Rights Agent may not assign this Agreement without Buyer’s consent. Subject to Section 4.4 hereof, each of Parent and Buyer may assign, in its sole
discretion and without the consent of any other party, any or all of its rights, interests and obligations hereunder to one or more its Affiliates or a party with whom Parent is merged or consolidated, or any entity resulting from any merger or
consolidation to which Parent shall be a party (each, an “Assignee”) and any such Assignee may thereafter assign, in its sole discretion and without the consent of any other 

  
 14 

 
party, any or all of its rights, interests and obligations set forth hereunder to one or more additional Assignees; provided, however, that in connection with any
assignment to an Assignee, Parent and Buyer shall agree to remain liable for the performance by Parent and Buyer of their obligations hereunder (to the extent Parent or Buyer exists following such assignment). Parent, Buyer or an Assignee may not
otherwise assign this Agreement without the prior consent of the Majority of Holders, provided that Parent or Buyer may assign this Agreement to an Affiliate of Parent without the prior consent of the Majority of Holders so long as
Parent and Buyer remain fully obligated for performance under this Agreement. Any attempted assignment of this Agreement or any of such rights in violation of this Section 6.4 will be void ab initio and of no effect. 

Section 6.5        Benefits of Agreement; Action by Majority
of Holders. 
 Nothing in this Agreement, express or implied, will give to any Person (other than Parent, Buyer, the Rights Agent, the
Holders and their permitted successors and assigns hereunder) any benefit or any legal or equitable right, remedy or claim under this Agreement or under any covenant or provision herein contained, all such covenants and provisions being for the sole
benefit of Parent, Buyer, the Rights Agent, the Holders and their permitted successors and assigns. The Holders will have no rights hereunder except as are expressly set forth herein. Except for the right of the Rights Agent set forth herein, the
Majority of Holders will have the sole right, on behalf of all Holders, by virtue of or under any provision of this Agreement, to institute any action or proceeding at law or in equity or in bankruptcy or otherwise upon or under or with respect to
this Agreement, and no individual Holder or other group of Holders will be entitled to exercise such rights. 
 
Section 6.6        Governing Law. 
 This Agreement and CVRs will be governed by and
construed in accordance with the laws of the State of New York, without regard to the conflicts of law rules of such state. 
 
Section 6.7        Jurisdiction. 
 The parties hereto agree that any Action seeking
to enforce any provision of, or based on any matter arising out of or in connection with, this Agreement or the transactions contemplated hereby (whether brought by any party or any of its Affiliates or against any party or any of its Affiliates)
must be brought solely in any state court or United States federal court located in the County of New York, New York, United States, and each of the parties hereby irrevocably consents to the jurisdiction of such courts (and of the appropriate
appellate courts therefrom) in any such Action and irrevocably waives, to the fullest extent permitted by Applicable Law, any objection that it may now or hereafter have to the laying of the venue of any such Action in any such court or that any
such Action brought in any such court has been brought in an inconvenient forum. Process in any such Action may be served on any party anywhere in the world, whether within or without the jurisdiction of any such court. Without limiting the
foregoing, each party agrees that service of process on such party as provided in Section 6.1 or Section 6.2 shall be deemed effective service of process on such party. 

  
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Section 6.8        WAIVER OF JURY TRIAL. 

EACH OF THE PARTIES HERETO HEREBY IRREVOCABLY WAIVES ANY AND ALL RIGHT TO TRIAL BY JURY IN ANY LEGAL PROCEEDING ARISING OUT OF OR RELATED
TO THIS AGREEMENT OR THE TRANSACTIONS CONTEMPLATED HEREBY. EACH PARTY CERTIFIES AND ACKNOWLEDGES THAT (I) NO REPRESENTATIVE, AGENT OR ATTORNEY OF ANY OTHER PARTY HAS REPRESENTED, EXPRESSLY OR OTHERWISE, THAT SUCH OTHER PARTY WOULD NOT, IN THE
EVENT OF LITIGATION, SEEK TO ENFORCE THE FOREGOING WAIVER, (II) EACH PARTY UNDERSTANDS AND HAS CONSIDERED THE IMPLICATION OF THIS WAIVER, (III) EACH PARTY MAKES THIS WAIVER VOLUNTARILY, AND (IV) EACH PARTY HAS BEEN INDUCED TO ENTER INTO THIS
AGREEMENT BY, AMONG OTHER THINGS, THE MUTUAL WAIVERS AND CERTIFICATIONS IN THIS SECTION. 

Section 6.9        Severability Clause. 

In the event that any provision of this Agreement, or the application of any such provision to any Person or set of circumstances, is for any
reason be determined to be invalid, unlawful, void or unenforceable to any extent, the remainder of this Agreement, and the application of such provision to Persons or circumstances other than those as to which it is determined to be invalid,
unlawful, void or unenforceable, will not be impaired or otherwise affected and will continue to be valid and enforceable to the fullest extent permitted by Applicable Law. Upon such a determination, the parties will negotiate in good faith to
modify this Agreement so as to effect the original intent of the parties as closely as possible in a mutually acceptable manner in order that the transactions contemplated hereby be consummated as originally contemplated to the fullest extent
possible. 
 Section 6.10        Counterparts;
Effectiveness. 
 This Agreement may be signed in any number of counterparts, each of which will be deemed an original, with the same
effect as if the signatures thereto and hereto were upon the same instrument. This Agreement or any counterpart may be executed and delivered by facsimile copies or delivered by electronic communications by portable document format (.pdf), each of
which shall be deemed an original. This Agreement will become effective when each party hereto will have received a counterpart hereof signed by the other party hereto. Until and unless each party has received a counterpart hereof signed by the
other party hereto, this Agreement will have no effect and no party will have any right or obligation hereunder (whether by virtue of any other oral or written agreement or other communication). 

Section 6.11        Termination. 

This Agreement will terminate and be of no force or effect, and the parties hereto will have no liability hereunder, upon the earlier to occur
of (a) the payment of both of the US Milestone Payment and the EU Milestone Payment and (b) the expiration of both the US Milestone Period and the EU Milestone Period. The termination of this Agreement will not affect or limit the right to
receive the Milestone Payments under Section 2.4 to the extent earned prior to termination of this Agreement and the provisions applicable thereto will survive the expiration or termination of this Agreement. 

  
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Section 6.12        Force Majeure. 

Notwithstanding anything to the contrary contained herein, the Rights Agent will not be liable for any delays or failures in performance
resulting from acts beyond its reasonable control including, acts of God, terrorist acts, shortage of supply, breakdowns or malfunctions, interruptions or malfunctions of computer facilities, or loss of data due to power failures or mechanical
difficulties with information storage or retrieval systems, labor difficulties, war or civil unrest. 

Section 6.13        Construction. 

(a)    For purposes of this Agreement, whenever the context requires: singular terms will include the plural, and vice
versa; the masculine gender will include the feminine and neuter genders; the feminine gender will include the masculine and neuter genders; and the neuter gender will include masculine and feminine genders. 

(b)    As used in this Agreement, the words “include” and “including,” and variations thereof, will
not be deemed to be terms of limitation, but rather will be deemed to be followed by the words “without limitation.” 

(c)    The headings contained in this Agreement are for convenience of reference only, will not be deemed to be a part of
this Agreement and will not be referred to in connection with the construction or interpretation of this Agreement. 

(d)    Any reference in this Agreement to a date or time shall be deemed to be such date or time in New York City, United
States, unless otherwise specified. The parties and the Company have participated jointly in the negotiation and drafting of this Agreement. In the event an ambiguity or question of intent or interpretation arises, this Agreement shall be construed
as if drafted jointly by the parties and the Company and no presumption or burden of proof shall arise favoring or disfavoring any Person by virtue of the authorship of any provision of this Agreement. 

(e)    All financial references herein are in United States Dollars. 

[Remainder of page intentionally left blank] 

  
 17 

 Each of the parties has caused this Agreement to be executed on its behalf by a duly authorized
officer of it as of the day and year first above written. 
  

			
	BIOMARIN PHARMACEUTICAL INC.
		
	By:	 	/s/ George Eric Davis
		 	     Name: George Eric Davis

    Title: SVP, General Counsel

  

			
	BIOMARIN FALCONS B.V.
		
	By:	 	/s/ George Eric Davis
		 	     Name: George Eric Davis

    Title: Managing Director

  

			
	 AMERICAN STOCK TRANSFER &

TRUST COMPANY, LLC

		
	By:	 	/s/ Paula Caroppoli
		 	     Name: Paula Caroppoli

    Title: Senior Vice President

  
 18

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