Document:

Second Agreement to Loan and Security Agreement

 Exhibit 10.37 
  
 SECOND AMENDMENT 
 TO 
 LOAN AND SECURITY AGREEMENT 
  
 This Second Amendment to Loan and Security Agreement is entered into as of February 15, 2005 (the “Amendment”), by and between COMERICA BANK
(“Bank”) and BIOMARIN PHARMACEUTICAL INC. (“Borrower”). 
  
 RECITALS 
  
 Borrower and
Bank are parties to that certain Loan and Security Agreement dated as of May 14, 2004, as amended by a First Amendment to Loan and Security Agreement dated as of November 3, 2004 (collectively, the “Agreement”). The parties desire to amend
the Agreement in accordance with the terms of this Amendment. 
  
 NOW, THEREFORE, the parties agree as follows: 
  
 1.
Certain defined terms in Section 1.1 of the Agreement are hereby added or amended to read as follows: 
  
 “Advance” or “Advances” means a cash advance under the Revolving Facility. 
  
 “Credit Extension” means each Advance, Equipment
Advance, or any other extension of credit by Bank for the benefit of Borrower hereunder. 
  
 “Pledged Collateral” means account number BG5-001198 at Comerica Securities, Inc. and any succeeding or replacement
account(s), together with the investment property, financial assets, securities, securities entitlements, and all replacements and proceeds thereof, now existing or hereafter arising, contained in, credited to, or held in connection with any such
account. 
  
 “Revolving Facility” means
the facility under which Borrower may request Advances, as specified in Section 2.1(b). 
  
 “Revolving Line” means a credit extension of up to Twelve Million Five Hundred Thousand Dollars ($12,500,000). 
  
 “Revolving Maturity Date” means March 31, 2005.

  
 2. Section 2.1(b) is added to the Agreement, as follows:

  
 (b) Revolving Advances. 
  
 (i) Subject to and upon the terms and conditions of this
Agreement, Borrower may request Advances in an aggregate outstanding amount not to exceed the lesser of (i) the Revolving Line or (ii) ninety percent (90%) of the face value of the Pledged Collateral. If the aggregate outstanding balance of the
Advances ever exceeds such lesser amount, Borrower shall promptly repay Bank the amount of such excess. Subject to the terms and conditions of this Agreement, amounts borrowed pursuant to this Section 2.1(b) may be repaid and reborrowed at any time
prior to the Revolving Maturity Date, at which time all Advances under this Section 2.1(b) shall be immediately due and payable. Borrower may prepay any Advances without penalty or premium. 
  

 1 

 (ii) Whenever Borrower desires an Advance, Borrower will notify Bank by facsimile
transmission or telephone no later than 3:00 p.m. Pacific time, on the Business Day that the Advance is to be made. Each such notification shall be promptly confirmed by a Payment/Advance Form in substantially the form of Exhibit B hereto.
Bank is authorized to make Advances under this Agreement, based upon instructions received from a Responsible Officer. Bank shall be entitled to rely on any telephonic notice given by a person who Bank reasonably believes to be a Responsible
Officer, and Borrower shall indemnify and hold Bank harmless for any damages or loss suffered by Bank as a result of such reliance. Bank will credit the amount of Advances made under this Section 2.1(b) to Borrower’s deposit account.

  
 3. Section 2.2(a) of the Agreement is amended to read as
follows: 
  
 (a) Interest Rates. Each
Equipment Advance shall bear interest on the outstanding Daily Balance thereof at the applicable rate set forth in the LIBOR Addendum to Loan and Security Agreement executed in connection with this Agreement. Each Advance shall bear interest on the
outstanding Daily Balance thereof at a rate equal to the Prime Rate minus one half of one percent. 
  
 4. Without limiting the generality of “Collateral”, as defined in Section 1.1 of the Agreement, Collateral shall include the Pledged Collateral.
To secure prompt repayment of the Obligations, including the Advances, and in order to secure prompt performance by Borrower of each of its covenants and duties under the Loan Documents, Borrower grants to Bank a continuing security interest in the
Pledged Collateral. Without Bank’s prior written consent, Borrower shall not transfer or permit to be transferred any interest in or portion of the Pledged Collateral from one account to another or to any other Person or seek to release or
permit to be released any interest in or portion of the Pledged Collateral from Bank’s Lien. Borrower authorizes Comerica Securities, Inc. to provide Bank reasonable notice before permitting the transfer of any interest in or portion of the
Pledged Collateral from one account to another or the release to Borrower or any other Person any interest in or portion of the Pledged Collateral. Upon repayment of the Advances and termination of the Revolving Facility, whether on the Revolving
Maturity Date or at Borrower’s earlier election, Bank’s security interest in the Pledged Collateral shall terminate and the Pledged Collateral shall no longer be part of the Collateral. 
  
 5. The Loan Payment/Advance Request Form to be delivered after the date of
this Amendment shall be in substantially the form of Exhibit B hereto. 
  
 6. The Compliance Certificate to be delivered after the date of this Agreement shall be in substantially the form of Exhibit C hereto. 
  
 7. Unless otherwise defined, all initially capitalized terms in this Amendment shall be as defined in the Agreement. The
Agreement, as amended hereby, shall be and remain in full force and effect in accordance with its respective terms and hereby is ratified and confirmed in all respects. Except as expressly set forth herein, the execution, delivery, and performance
of this Amendment shall not operate as a waiver of, or as an amendment of, any right, power, or remedy of Bank under the Agreement, as in effect prior to the date hereof. Borrower ratifies and reaffirms the continuing effectiveness of all
applications, instruments, documents and agreements entered into in connection with the Agreement. 
  
 8. Borrower represents and warrants that the representations and warranties contained in the Agreement are true and correct as of the date of this
Amendment, and that no Event of Default has occurred and is continuing. 
  
 9. This 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 instrument. 
  
 10. As a condition to the effectiveness of this Amendment, Bank shall have received, in form and substance satisfactory to
Bank, the following: 
  
 (a) this Amendment, duly executed by
Borrower; 
  

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 (b) an administrative fee of $12,500, provided Bank shall refund $5,000 of such fee upon Borrower’s
maintaining at least $12,500,000 in one or more accounts with Bank for 30 consecutive days after the date hereof, plus all Bank Expenses incurred through the date of this Amendment; 
  
 (c) Corporate Resolutions to Borrow; 
  
 (d) Control agreements with Comerica Securities, Inc.; and 
  
 (e) such other documents, and completion of such other matters, as Bank may reasonably deem necessary or appropriate.

  
 IN WITNESS WHEREOF, the undersigned have executed this
Amendment as of the first date above written. 
  

			
	 BIOMARIN PHARMACEUTICAL INC.

		
	 By:
	 	  

	 Title:
	 	  

	
	 COMERICA BANK

		
	 By:
	 	  

	 Title:
	 	  

  
  

 3 

 EXHIBIT B 
  

TECHNOLOGY & LIFE SCIENCES DIVISION 
 LOAN ANALYSIS 
 LOAN ADVANCE/PAYDOWN REQUEST FORM 
  
 DEADLINE FOR SAME DAY PROCESSING IS 3:00 P.M. PACIFIC TIME 
 DEADLINE FOR EQUIPMENT ADVANCES IS 3:00 P.M. PACIFIC TIME ** 
 DEADLINE FOR WIRE
TRANSFERS IS 3:30 P.M. PACIFIC TIME 

	**	Subject to 3 day advance notice. 

  

							
	 TO: Loan Analysis
	  	 	  	DATE:                                     
    TIME:                             
	 FAX #: (650) 846-6840
	  	 	  	 
	 FROM:
	  	BIOMARIN PHARMACEUTICAL INC.	  	TELEPHONE REQUEST (For Bank Use Only):
	 	  	Borrower’s Name	  	 	  	 
	 	  	 	  	The following person is authorized to request the loan payment transfer/loan advance on the designated account and is known to me.
	 FROM:
	  	  

	  	 	  	 
	 	  	Authorized Signer’s Name	  	 	  	 
	 FROM:
	  	  

	  	 	  	  

	 	  	Authorized Signature (Borrower)	  	 	  	Authorized Request & Phone #
	 PHONE #:
	  	  

	  	 	  	  

	 	  	 	  	 	  	Received by (Bank) & Phone #
	 FROM ACCOUNT#:
	  	  

	  	 	  	 
	 (please include Note number, if applicable)
	  	 	  	  

	 TO ACCOUNT #:
	  	  

	  	 	  	Authorized Signature (Bank)
	 (please include Note number, if applicable)
	  	 	  	 

  

									
	 REQUESTED TRANSACTION TYPE
	  	REQUESTED DOLLAR AMOUNT	  	    For Bank Use Only
					
	 PRINCIPAL INCREASE* (ADVANCE)
	  	$__________________________________________	  	Date Rec’d:	  	 	  	 
	 PRINCIPAL PAYMENT (ONLY)
	  	$__________________________________________	  	Time:	  	 	  	 
	 	  	 	  	Comp. Status:	  	YES	  	NO
	 OTHER INSTRUCTIONS:
	  	Status Date:	  	 	  	 
	  

	  	Time:	  	 	  	 
	  

	  	Approval:	  	 	  	 

  
 All representations and warranties of
Borrower stated in the Loan Agreement are true, correct and complete in all material respects as of the date of the telephone request for and advance confirmed by this Borrowing Certificate, including without limitation the representation that
Borrower has paid for and owns the equipment financed by Bank; provided, however, that those representations and warranties the date expressly referring to another date shall be true, correct and complete in all material respects as of such date.

	*	IS THERE A WIRE REQUEST TIED TO THIS LOAN ADVANCE? (PLEASE CIRCLE ONE)        YES    NO 

 
 If YES, the Outgoing Wire Transfer Instructions must be completed below.

  

					
	OUTGOING WIRE TRANSFER INSTRUCTIONS	  	Fed Reference Number	  	Bank Transfer Number
	
	The items marked with an asterisk (*) are required to be completed.
		
	 *Beneficiary Name
	  	 
	 *Beneficiary Account Number
	  	 
	 *Beneficiary Address
	  	 
	 Currency Type
	  	US DOLLARS ONLY
	 *ABA Routing Number (9 Digits)
	  	 
	 *Receiving Institution Name
	  	 
	 *Receiving Institution Address
	  	 
	 *Wire Account
	  	$

  
  

 EXHIBIT C 
  

COMPLIANCE CERTIFICATE 
  

			
	 TO:
	  	COMERICA BANK
		
	 FROM:
	  	 BIOMARINPHARMACEUTICAL INC.

  
 The undersigned
Responsible Officer of BIOMARIN PHARMACEUTICAL INC. hereby certifies that in accordance with the terms and conditions of the Loan and Security Agreement between Borrower and Bank (the “Agreement”), (i) Borrower is in complete compliance
for the period ending                              with all required covenants except as noted below
and (ii) all representations and warranties of Borrower stated in the Agreement are true and correct in all material respects as of the date hereof, except for such representations and warranties which speak as to a specific date, which are true and
correct as of such date. Attached herewith are the required documents supporting the above certification. The Responsible Officer further certifies that these are prepared in accordance with Generally Accepted Accounting Principles (GAAP) and are
consistently applied from one period to the next except as explained in an accompanying letter or footnotes. 
  
 Please indicate compliance status by circling Yes/No under “Complies” column. 
  

										
	 Reporting Covenant

	 	 Required

	 	Complies

	 Form 10Q
	 	Filed Quarterly within 45 days	 	Yes	  	No
	 Form 10K
	 	Filed FYE within 90 days	 	Yes	  	No
				
	 Financial Covenant

	 	 Required

	  	Actual

	 	Complies

	 Unrestricted Cash
	 	Greater of 6x RML* or $45,000,000	  	$	            	 	Yes	  	No
	 Unrestricted Cash at Bank
	 	Greater of $10,000,000 and balance of outstanding principal obligations to Bank	  	$	            	 	Yes	  	No
	 Securities Account Balance
	 	Advances <90% of Securities Account Balance	  	$	            	 	Yes	  	No

	*	4x RML for the months ending 11/30/04 and 12/31/04 

  

									
	 Comments Regarding Exceptions: See Attached.
	  	BANK USE ONLY
			
	 	  	 Received by:
	  	  

	 Sincerely,
	  	 	  	AUTHORIZED SIGNER
	 	  	 	  	 	  	 	  	 
	 	  	 Date:
                    

			
	
	  	 Verified:
	  	  

	 SIGNATURE
	  	 	  	AUTHORIZED SIGNER
	  

	  	 Date:
                    

	 TITLE
	  	 	  	 	  	 	  	 
	  

	  	 Compliance Status
	  	Yes	  	No
	 DATE
	  	 	  	 	  	 

  
  

 5 

 CORPORATE RESOLUTIONS TO BORROW 
  

			
	 Borrower:
	  	BIOMARIN PHARMACEUTICAL INC.

  
 I, the undersigned
Secretary or Assistant Secretary of BIOMARIN PHARMACEUTICAL INC. (the “Corporation”), HEREBY CERTIFY that the Corporation is organized and existing under and by virtue of the laws of Delaware. 
  
 I FURTHER CERTIFY that at a meeting of the Directors of the Corporation duly
called and held, at which a quorum was present and voting, (or by other duly authorized corporate action in lieu of a meeting), the following resolutions were adopted. 
  
 I FURTHER CERTIFY that the officers, employees, and agents named below are duly elected, appointed, or employed by or for
the Corporation, as the case may be, and occupy the positions set forth opposite their respective names: 
  

					
	 NAMES

	 	 POSITIONS

	 	 ACTUAL SIGNATURES

	 __________________________________
  
	 	 __________________________________
  
	 	 __________________________________
  

	 __________________________________
  
	 	 __________________________________
  
	 	 __________________________________
  

	 __________________________________
  
	 	 __________________________________
  
	 	 __________________________________
  

	 __________________________________
  
	 	 __________________________________
  
	 	 __________________________________
  

	 __________________________________
  
	 	 __________________________________
  
	 	 __________________________________
  

  
 Borrow Money.
To borrow from time to time from COMERICA BANK (“Bank”), on such terms as may be agreed upon between the officers, employees, or agents of the Corporation and Bank, such sum or sums of money as in their judgment should be borrowed, without
limitation. 
  
 Execute Loan Documents. To execute and
deliver to Bank that certain Second Amendment to Loan and Security Agreement dated as of February 15, 2005 (the “Amendment”) and any documents related to the Amendment or to that certain Loan and Security Agreement dated as of May 14,
2004, as amended from time to time (collectively with the Amendment, the “Loan Documents”), and also to execute and deliver to Bank one or more amendments, renewals, extensions, modifications, consolidations, or substitutions for the Loan
Documents. 
  
 Grant Security. To grant a security interest
to Bank in the Collateral described in the Loan Documents, which security interest shall secure all of the Corporation’s Obligations, as described in the Loan Documents, and to enter into one or more securities account control agreements
related to the Collateral. 
  
 Negotiate Items. To draw,
endorse, and discount with Bank all drafts, trade acceptances, promissory notes, or other evidences of indebtedness payable to or belonging to the Corporation or in which the Corporation may have an interest, and either to receive cash for the same
or to cause such proceeds to be credited to the account of the Corporation with Bank, or to cause such other disposition of the proceeds derived therefrom as they may deem advisable. 
  
 Further Acts. In the case of lines of credit, to designate additional or alternate individuals as being authorized to
request advances thereunder, and in all cases, to do and perform such other acts and things, to pay any and all fees and costs, and to execute and deliver such other documents and agreements as they may in their discretion deem reasonably necessary
or proper in order to carry into effect the provisions of these Resolutions. 
  

 6 

 BE IT FURTHER RESOLVED, that any and all acts authorized pursuant to these resolutions and performed
prior to the passage of these resolutions are hereby ratified and approved, that these Resolutions shall remain in full force and effect and Bank may rely on these Resolutions until written notice of their revocation shall have been delivered to and
received by Bank. Any such notice shall not affect any of the Corporation’s agreements or commitments in effect at the time notice is given. 
  
 I FURTHER CERTIFY that the officers, employees, and agents named above are duly elected, appointed, or employed by or for the Corporation, as the case may
be, and occupy the positions set forth opposite their respective names; that the foregoing Resolutions now stand of record on the books of the Corporation; and that the Resolutions are in full force and effect and have not been modified or revoked
in any manner whatsoever. 
  
 I FURTHER CERTIFY that true and
correct copies of the Certificate of Incorporation of the Corporation have been delivered to Bank as in full force and effect on the date hereof. 
  
 IN WITNESS WHEREOF, I have hereunto set my hand on February 15, 2005 and attest that the signatures set opposite the names listed above are their genuine
signatures. 
  

			
	 CERTIFIED TO AND ATTESTED BY:

		
	 X
	 	  

  

 7 

 ITEMIZATION OF AMOUNT FINANCED 
  
 DISBURSEMENT INSTRUCTIONS 
  

			
	 Name: BIOMARIN PHARMACEUTICAL INC.
	  	Date: February 16, 2005
		
	 $
	  	credited to deposit account No.                      when Advances are requested by
Borrower
	
	 Amounts paid to others on your behalf:

		
	 $12,500
	  	to Comerica Bank for Loan Fee
		
	 $
	  	to Bank counsel fees and expenses
		
	 $
	  	to                     
		
	 $
	  	to                     
		
	 $             
	  	TOTAL (AMOUNT FINANCED)

  
 Upon consummation of this transaction,
this document will also serve as the authorization for Comerica Bank to disburse the loan proceeds as stated above. 
  

			
	  

	 	  

	 Signature
	 	Signature

  
  

 8 

			
	COMERICA BANK	  	 
	 	  	AUTOMATIC DEBIT AUTHORIZATION
		
	 To: Comerica Bank
	  	 
		
	 Re: Loan # _______________________
	  	 
	
	You are hereby authorized and instructed to charge account No.
                     in the name of BIOMARIN PHARMACEUTICAL INC.
	
	 for principal and interest payments due on above referenced loan as set forth below and credit the loan referenced
above.

	
	 x Debit each interest payment as it becomes due according to the terms of the note and any renewals or amendments
thereof.

	
	 x Debit each principal payment as it becomes due according to the terms of the note and any renewals or amendments
thereof.

	
	 This Authorization is to remain in full force and effect until revoked in writing.

		
	 Borrower Signature
	  	Date
	  

	  	February 15, 2005

  

 9Convertible Promissory Note dated Jan 12, 2005

 Exhibit 10.38 
  
 CONVERTIBLE PROMISSORY NOTE 
  

			
	 $25,000,000.00
	 	January 12, 2005 (the “Issue Date”)
	 	 	Novato, California

  
 SECTION 1.
Principal Repayment. BioMarin Pharmaceutical Inc., a Delaware corporation (the “Company”), having an address at 105 Digital Drive, Novato, California 94949, for value received, hereby promises to pay to Medicis Pharmaceutical
Corporation, a Delaware corporation (the “Holder”), having an address at 8125 N. Hayden Road, Scottsdale, Arizona 85258, on the earlier to occur of: (a) the Option Closing Date (as defined in the Securities Purchase Agreement dated
as of May 18, 2004, by and among the Company, BioMarin Pediatrics Inc., the Holder and Medicis Pediatrics, Inc. (formerly known as Ascent Pediatrics, Inc.), as amended, supplemented or otherwise modified from time to time in accordance therewith
(the “Securities Purchase Agreement”); and (b) August 17, 2009 (such date being referred to hereinafter as the “Maturity Date”), in lawful money of the United States of America, in immediately available funds, the
lesser of (1) the principal amount of TWENTY-FIVE MILLION DOLLARS ($25,000,000.00) or (2) an amount constituting the aggregate principal amount of all Advances (as defined below) outstanding hereunder on the Maturity Date, in each case, plus any
accrued and unpaid interest due thereon as hereinafter provided. Such Advances shall be endorsed from time to time by the Holder on the Schedule of Advances attached hereto, provided, however, that the failure of the Holder to make any
such recordation shall not affect the obligations of the Company to make a payment when due of any amount owing under this Note. For purposes of clarity, this Note is not a revolver and thus, no more than an aggregate principal amount of $25,000,000
may be borrowed under this note irregardless of whether any portion of the $25,000,000 amount is then-currently outstanding or has been repaid. This Convertible Promissory Note (this “Note”) has been issued pursuant to that certain
Settlement Agreement and Mutual Release dated as of January 12, 2005 by and among the Company, BioMarin Pediatrics Inc., a Delaware corporation and wholly-owned subsidiary of the Company, the Holder and Medicis Pediatrics, Inc., a Delaware
corporation (formerly known as Ascent Pediatrics, Inc.) and a wholly-owned subsidiary of the Holder. 
  
 SECTION 2. Advances. Subject to the terms hereof, the Holder shall, from time to time after July 1, 2005, make advances (collectively, the
“Advances” and each an “Advance”) of funds available hereunder to the Company. Holder shall not have any obligation to make any Advance to Company under this Note from and after the earliest of the following to
occur: 
  
 (a) the date on which the Company enters into a
contract or agreement to effectuate a Change in Control (as defined herein) of the Company; 
  
 (b) the date of consummation of a Change in Control of Company; 
  
 (c) the Company fails to pay any principal of or interest on any Advance (including scheduled interest payments, mandatory prepayments or the payment due
at maturity), when such principal or interest becomes due and such failure to pay has not been cured within five (5) days after the date such payment is due; 
  

 1 

 (d) a proceeding shall have been instituted in a court of competent jurisdiction in respect of the
Company seeking (i) an involuntary or voluntary case under any applicable bankruptcy, insolvency, reorganization or other similar law now or hereafter in effect, (ii) the appointment of a receiver, liquidator, assignee, custodian, trustee,
sequestrator, conservator (or similar official) of the Company for any substantial part of its property, or (iii) the winding-up or liquidation of the Company’s affairs; provided that with respect to any such proceeding, case or appointment
that is instituted without the application or consent of the Company, such proceeding, case or appointment shall have continued undismissed or unstayed and in effect for a period of 30 or more days; 
  
 (e) the Company admits in writing its inability to pay its debts as they
mature; or 
  
 (f) the Maturity Date. 
  
 The Company shall give the Holder written notice (each, an “Advance Notice”)
requesting an Advance, which shall specify the amount of such Advance and the date that the Company desires to receive such Advance, which date shall be not less than four (4) business days after the date of delivery of such Advance Notice to the
Holder. Upon receipt of any Advance Notice, the Holder shall make the Advance to the Company as described therein on the date set forth therein in accordance herewith. Notwithstanding any term or provision of this Note to the contrary, the Holder
shall not be required to make an Advance hereunder if the amount of such Advance together with the aggregate principal amount of all Advances then outstanding pursuant to this Note exceeds $25,000,000.00. 
  
 SECTION 3. Payments. The Company promises to pay interest on the
outstanding principal amount of each Advance from the date such Advance is made until payment in full of the principal amount thereof in accordance herewith, which interest shall accrue at the per annum rate equal to the sum of (a) the Three-Month
LIBOR plus (b) 1%. “Three -Month LIBOR” shall mean, as of the date of any determination thereof, the interest rate then most recently published in the “Money Rates” section of The Wall Street Journal as the
three-month London Interbank Offered Rate. The initial Three-Month LIBOR shall be determined on the date the first Advance is made and thereafter the Three-Month LIBOR shall be adjusted on the first day of the calendar quarter with respect to which
such interest accrues in accordance with the immediately preceding sentence. Interest shall be due and payable quarterly in arrears not later than the tenth day (each such date, an “Interest Payment Date”) of the calendar quarter
immediately following the calendar quarter during which such interest has accrued and shall be calculated on the basis of a 365 day year for the actual number of days elapsed. The initial Interest Payment Date shall be the tenth day of the calendar
quarter immediately following the calendar quarter in which the first Advance is made. On the Maturity Date, all outstanding principal and accrued and unpaid interest are due and payable. 
  
 Notwithstanding any other provision of this Note, interest on the indebtedness contemplated by this Note is expressly
limited so that in no contingency or event whatsoever, whether by acceleration of the maturity of such indebtedness or otherwise, shall the interest contracted for, charged or received by the Holder exceed the maximum amount permissible under
applicable law. If from any circumstances whatsoever fulfillment of any provisions of this Note or of any other document evidencing or pertaining to the indebtedness contemplated 

  

 2 

 
hereby, at the time performance of such provision shall be due, shall involve transcending the limit of validity prescribed by law, then, ipso facto, the
obligation to be fulfilled shall be reduced to the limit of such validity, and if from any such circumstances the Holder shall ever receive anything of value as interest or deemed interest by applicable law under this Note or any other document
evidencing or pertaining to the indebtedness contemplated hereby or otherwise an amount that would exceed the highest lawful rate, such amount that would be excessive interest shall be applied to the reduction of the principal amount owing under
this Note or on account of any other indebtedness of the Company to the Holder, and not to the payment of interest, or if such excessive interest exceeds the unpaid balance of principal of this Note and such other indebtedness, such excess shall be
refunded to the Company. In determining whether or not the interest paid or payable with respect to any indebtedness of the Company to the Holder, under any specific contingency, exceeds the highest lawful rate, the Company and the Holder shall, to
the maximum extent permitted by applicable law: (a) characterize any non–principal payment as an expense, fee or premium rather than as interest; (b) exclude voluntary prepayments and the effects thereof; (c) amortize, prorate, allocate and
spread the total amount of interest throughout the term of such indebtedness so that the actual rate of interest on account of such indebtedness does not exceed the maximum amount permitted by applicable law; and/or (d) allocate interest between
portions of such indebtedness, to the end that no such portion shall bear interest at a rate greater than that permitted by applicable law. 
  
 SECTION 4. Acceleration Upon a Change in Control. Notwithstanding Section 1 hereof, if the Company fails to pay any principal of or interest on any
Advance (including scheduled interest payments, mandatory prepayments or the payment due at maturity), when such principal or interest becomes due and such failure to pay has not been cured within five (5) days after the date such payment is due,
the aggregate principal amount of all outstanding Advances plus accrued but unpaid interest thereon shall become due and payable at the option of Holder, in its sole discretion. Notwithstanding Section 1 hereof, the aggregate principal amount of all
outstanding Advances plus accrued but unpaid interest thereon shall be immediately due and payable upon any of the following to occur: 
  
 (a) a Change in Control of the Company. As used herein: 
  
 “Change in Control” of the Company shall be deemed to have occurred at such time as: 
  
 (i) any “person” or “group” (as such terms are used for
purposes of Sections 13(d) and 14(d) of the Securities Exchange Act of 1934, as amended (the “Exchange Act”), is or becomes the “beneficial owner” (as such term is used in Rule 13d-3 under the Exchange Act), directly or
indirectly, of fifty percent (50%) or more of the total voting power of all classes of the Company’s capital stock entitled to vote generally in the election of directors (the “Voting Stock”); or 
  
 (ii) the Company consolidates with, or merges with or into, another person
or entity (a “Person”) or any Person consolidates with, or merges with or into, the Company, in any such event other than pursuant to a transaction in which the Persons that “beneficially owned,” directly or indirectly,
the shares of the Company’s Voting Stock 

  

 3 

 
immediately prior to such transaction, “beneficially own,” immediately after such transaction directly or indirectly, shares of the voting stock
representing not less than a majority of the total voting power of all outstanding classes of Voting Stock of the continuing or surviving corporation; or 
  
 (iii) the sale of all or substantially all of the assets of the Company to any “person” or “group” (as such terms are used in
Sections 13(d) and 14(d) of the Exchange Act, including Rule 13d-5(b)(1) under the Exchange Act); or 
  
 (b) a proceeding shall have been instituted in a court of competent jurisdiction in respect of the Company seeking (i) an involuntary or voluntary case
under any applicable bankruptcy, insolvency, reorganization or other similar law now or hereafter in effect, (ii) the appointment of a receiver, liquidator, assignee, custodian, trustee, sequestrator, conservator (or similar official) of the Company
for any substantial part of its property, or (iii) the winding-up or liquidation of the Company’s affairs; provided that with respect to any such proceeding, case or appointment that is instituted without the application or consent of the
Company, such proceeding, case or appointment shall have continued undismissed or unstayed and in effect for a period of 30 or more days. 
  
 SECTION 5. Prepayment. The principal amount of any Advance or Advances may be prepaid, in whole or in part, at any time without penalty,
together with the interest thereon accrued through the date of such prepayment; provided, however, no prepayment shall be made without at least fifteen (15) days’ prior written notice of such prepayment to the Holder (a
“Prepayment Notice”). Partial prepayments shall be credited first against the interest accrued, but unpaid to the date of prepayment on all Advances and thereafter against the principal balance of any Advance or Advances as the
Company shall select. 
  
 SECTION 6. Conversion.

  
 (a) The Holder shall have the right on any Conversion Date
(as defined below) to convert the unpaid principal amount of each Advance then outstanding into fully paid and nonassessable shares of the Company’s Common Stock, $.001 par value per share (the “Common Stock”) at the Conversion
Price (as defined below) per share applicable to such Advance. As used herein, “Conversion Date” shall mean any of the following: (i) the Maturity Date, (ii) the date of consummation of any Change in Control of the Company (and
immediately preceding the consummation of such Change in Control), or (iii) the date of prepayment of any Advance on the date set forth therefor in the Prepayment Notice with respect thereto. As used herein, the “Conversion Price”
per share of Common Stock applicable to any Advance shall mean an amount equal to the average closing sales price per share of the Common Stock over the twenty (20) trading days immediately preceding the date such Advance is made. It is anticipated
that each Advance will have a different Conversion Price. 
  
 (b)
To exercise the conversion right described in this Section 6, the Holder must deliver written notice of its intention to such effect, specifying the amount of the Advance or Advances to be so converted, delivered to the Company not later than ten
(10) days prior to the Conversion Date giving rise to such right of conversion. The conversion of the unpaid principal amount or any portion thereof of any outstanding Advance may only be effected in increments of 

  

 4 

 
not less than One Hundred Thousand Dollars ($100,000) or the total unpaid principal amount of any outstanding Advance, and thereupon the amount of principal
owed under such Advance shall be reduced by an amount equal to the Conversion Price applicable to such Advance, multiplied by the number of shares of Common Stock issued upon such conversion. The Company shall not be obligated to issue any
fractional share in connection with the conversion of any Advance hereunder. If in connection with the conversion of any Advance hereunder a fraction of a share of Common Stock would be issuable but for the immediately preceding sentence, then the
Company will pay the Holder the cash value of such fractional share, based upon the Conversion Price per share applicable to such Advance. 
  
 (c) The Company shall at all times reserve and keep available for issuance upon the conversion of the principal amount of Advances under this Note such
number of its authorized but unissued shares of Common Stock as will be sufficient to permit the conversion in full of the aggregate principal amount of the Advances outstanding from time to time. 
  
 (d) If the Company at any time or from time to time after the date the first
Advance is made effects a subdivision of the outstanding Common Stock, the Conversion Price per Advance then in effect immediately before that subdivision shall be proportionately decreased and the number of shares of Common Stock heretofore
receivable upon the conversion of the unpaid principal balance of such Advance shall be proportionately increased. If the Company at any time or from time to time after the date the first Advance is made combines the outstanding shares of Common
Stock into a smaller number of shares, the Conversion Price per Advance then in effect immediately before that combination shall be proportionately increased and the number of shares of Common Stock heretofore receivable upon the conversion of the
unpaid principal balance of such Advance shall be proportionately decreased. Each adjustment under this Section 6(d) shall become effective at the close of business on the day the subdivision or combination, as the case may be, becomes effective.

  
 SECTION 7. Miscellaneous. 
  
 (a) This Note shall be construed in accordance with, and governed in all
respects by, the internal laws of the State of New York (without giving effect to principles of conflicts of laws). 
  
 (b) Any legal action or other legal proceeding relating to this Agreement or the enforcement of any provision of this Agreement may be brought or
otherwise commenced in any state or federal court located in New York, New York in the Borough of Manhattan. Each party to this Agreement: 
  
 (i) expressly and irrevocably consents and submits to the jurisdiction of each state and federal court located in New York, New York in
the Borough of Manhattan (and each appellate court located in the State of New York) in connection with any such legal proceeding; 
  
 (ii) agrees that each state and federal court located in New York, New York in the Borough of Manhattan shall be deemed to be a convenient
forum; and 
  

 5 

 (iii) agrees not to assert (by way of motion, as a defense or otherwise), in any such
legal proceeding commenced in any state or federal court located in New York, New York in the Borough of Manhattan, any claim that such party is not subject personally to the jurisdiction of such court, that such legal proceeding has been brought in
an inconvenient forum, that the venue of such proceeding is improper or that this Agreement or the subject matter of this Agreement may not be enforced in or by such court. 
  
 (c) Neither the Company nor the Holder may assign its rights or delegate any of its obligations under this Note (or any part
thereof); provided, however, that if the Company or the Holder or any of their respective successors (i) consolidates with or merges into any other entity and shall not be the continuing or surviving entity of such consolidation or
merger or (ii) transfers all or substantially all of its properties and assets to any entity, then, and in each such case, proper provision shall be made so that the successors and assigns of the Company or the Holder, as the case may be, shall
assume the obligations set forth in this Note. 
  
 (d) All notices
and other communications provided for hereunder shall be in writing and shall be sent to the Company’s or the Holder’s respective addresses as specified in the first paragraph hereof, or to such other address as the Company or the Holder,
respectively, may designate. All such notices and other communications shall be deemed properly delivered, given and received (a) if delivered personally, upon delivery, (b) if delivered by registered or certified mail (return receipt requested)
from the United States, upon the earlier of actual delivery or three business days after being mailed, (c) if sent by overnight delivery by a recognized overnight delivery service for overnight delivery, upon the earlier of actual delivery or one
business day after being sent, or (d) if given by facsimile, upon confirmation of transmission by facsimile (or, if such confirmation does not occur during normal business hours on a business day, then on the next business day). 
  
 (e) All amendments to this Note, and any waiver or consent of the Holder,
must be in writing and signed by the Holder and the Company. 
  
 (f) The Company hereby waives diligence, presentment, protest, demand, notice of intent to accelerate, notice of acceleration and notice of every kind other than notices expressly provided herein or by the Settlement Agreement, the
Securities Purchase Agreement or required by applicable law. 
  
 [signature page follows] 
  

 6 

 IN WITNESS WHEREOF, the undersigned has executed this Note effective as of the date first
above written. 
  

			
	 BIOMARIN PHARMACEUTICAL INC., a
 Delaware corporation

		
	 By:
	 	 /s/ Jeffrey H. Cooper

	 Name:
	 	 Jeffrey H. Cooper

	 Title:
	 	 Vice President Controller/
 Chief Financial Officer

  

 7 

 Schedule of Advances 
  

											
	 Date

	  	 Amount of
 Advance

	  	 Conversion
 Price

	  	 Amount of
Principal
 Paid or Repaid

	  	 Unpaid
 Principal
 Balance

	  	 Notation
 Made
by

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