Document:

Exhibit 10.3

Exhibit 10.3

EXECUTION COPY

AMENDMENT NO. 1

TO

DEVELOPMENT AND COMMERCIALIZATION AGREEMENT

THIS AMENDMENT NO. 1 (the “Amendment”), dated January 16, 2009, amends that certain
Development and Commercialization Agreement, dated as of January 4, 2009 (the “Development
Agreement”), by and between La Jolla Pharmaceutical Company, a Delaware corporation (the
“Company”), and BioMarin CF Limited, an Irish corporation (“BioMarin CF”).

RECITALS

A. WHEREAS, in connection with the Development Agreement, the Company and BioMarin
Pharmaceutical Inc., a Delaware corporation (“BioMarin Pharmaceutical”), entered into that
certain Securities Purchase Agreement, dated as of January 4, 2009 (the “Securities Purchase
Agreement”);

B. WHEREAS, the Company and BioMarin Pharmaceutical have made certain changes to the
Securities Purchase Agreement in the form of an amendment to the Securities Purchase Agreement (the
“Amendment to the Securities Purchase Agreement”); and

C. WHEREAS, the Company and BioMarin CF desire to make conforming changes to the Development
Agreement to reflect certain changes made by the Amendment to the Securities Purchase Agreement.

NOW, THEREFORE, in consideration of the foregoing, the parties agree as follows:

1. Section 1.41 of the Development Agreement is amended and restated in its entirety as
follows:

“1.41 “Securities Purchase Agreement” shall mean the form of securities purchase
agreement, as amended by the Amendment to the Securities Purchase Agreement, each as attached
to this Agreement as Exhibit 1.41.”

2. Exhibit 1.41 to the Development Agreement shall be amended and restated in its
entirety in the form of Exhibit 1.41 attached hereto.

3. Undefined capitalized terms used in this Amendment shall have the meanings ascribed to them
in the Development Agreement.

4. Except as expressly set forth in this Amendment, all other terms of the Development
Agreement shall remain in full force and effect and once this Amendment is executed by the parties
hereto, all references in the Development Agreement to “the Agreement” or “this Agreement,” as
applicable, shall refer to the Development Agreement, as modified by this Amendment.

5. This Amendment shall be governed by and construed in accordance with the laws of the State
of California applicable to contracts made and to be performed entirely within such State without
regard to conflict of laws principles thereof.

6. This Amendment may be executed in any number of counterparts and each of such counterparts
shall for all purposes be deemed to be an original, and all such counterparts shall together
constitute but one and the same instrument.

* * *

 

 

 

IN WITNESS WHEREOF, the Parties have caused this Amendment to be executed as of the date first
set forth above.

 

	 	 	 	 	 	 	 	 	 
	LA JOLLA PHARMACEUTICAL COMPANY	 	 	BIOMARIN CF LIMITED
	 
	 	 	 	 	 	 	 	 
	By: 

	/s/ Deirdre Y. Gillespie
	 	 	By: 
	/s/ G. Eric Davis
	 

	 
	 	 	 	 
	 

	Name: 	Deirdre Y. Gillespie, M.D.
	 	 	 	Name:
	 	G. Eric Davis
	 

	Title: 	President and Chief Executive Officer
	 	 	 	Title:
	 	Managing Director

 

 

 

Exhibit 1.41 

Securities Purchase Agreement

[Filed as Exhibit 10.30 to BioMarin’s Annual Report on Form 10-K

for the year ended December 31, 2008]Exhibit 10.4

Exhibit 10.4

EXECUTION COPY 

AMENDMENT NO. 1

TO

SECURITIES PURCHASE AGREEMENT

THIS AMENDMENT NO. 1 (the “Amendment”), dated January 16, 2009, amends that certain
Securities Purchase Agreement, dated as of January 4, 2009 (the “Agreement”), by and
between La Jolla Pharmaceutical Company, a Delaware corporation (the “Company”), and
BioMarin Pharmaceutical Inc., a Delaware corporation (the “Purchaser”).

RECITALS

A. WHEREAS, the Purchaser and the Company are parties to the Agreement, which provides for the
issuance of shares of the Company’s Series B Convertible Preferred Stock on the terms set forth
therein;

B. WHEREAS, Exhibit A to the Agreement sets forth the form of Certificate of Designation to be
filed with the Delaware Secretary of State to designate the rights, preferences and privileges of
the Company’s Series B-1 Convertible Preferred Stock (the “Certificate”);

C. WHEREAS, the Certificates of Designation to be filed with respect to the Company’s Series
B-2 Convertible Preferred Stock and Series B-3 Convertible Preferred Stock are to be in
substantially the form of the Certificate, with any appropriate adjustments needed to reflect the
issuance price for such shares;

D. WHEREAS, under the terms of the Certificate, the Series B Convertible Preferred Stock is
convertible into Common Stock at an initial ratio of one-for-three (i.e., three shares of Common
Stock for every one share of Series B Convertible Preferred Stock); and

E. WHEREAS, the Company and the Purchaser wish to amend the form of Certificate and certain
related terms of the Agreement to change the initial conversion ratio for the Common Stock to
one-for-thirty (i.e., thirty shares of Common Stock for every one share of Series B Convertible
Preferred Stock).

 

 

 

Now therefore, in consideration of the foregoing, the parties agree as follows:

1. Section 1.2 of the Agreement is amended and restated in its entirety as follows:

“1.2 Purchase and Sale. Subject to the terms and conditions of this Agreement and
the Collaboration Agreement, on the date hereof, the Company agrees to sell to the Purchaser,
and the Purchaser agrees to purchase from the Company, 339,104 Shares (the “Initial
Investment”) at a purchase price per share of $22.1171, in the case of the Initial
Closing, or, in the case of a Subsequent Closing (as defined below) at a price per common
share equivalent (based on the conversion ratio provided for in the applicable Certificate of
Designation, as adjusted) equal to one hundred ten percent (110%) of the average closing price
of the Common Stock of the Company as reported on the NASDAQ stock market or such other
reporting service as the stock is then quoted if not then quoted on NASDAQ (and if not then
traded at the value determined by an investment bank selected consistent with the provisions
of Section 14.3 of the Collaboration Agreement), for the ten (10)
consecutive trading days commencing five (5) trading days immediately prior to the date the
Company has publicly announced the event that triggered such payment (i.e., the P-Value
Achievement, or in the case of such payment where there is no P-Value Achievement, the
Company’s first public announcement of the results of the Second Interim Efficacy Analysis or
the first public announcement of the approval of an NDA for the Product under Section 7.13 of
the Collaboration Agreement (the “Announcement of Results”)). Notwithstanding the
foregoing, in no event will the price per common equivalent for the Shares issued in a
Subsequent Closing (based on the conversion ratio provided for in the applicable Certificate
of Designation, as adjusted) be less than $0.73724.

2. Exhibit A, as attached to the Agreement, is hereby amended and restated in its entirety and
is replaced with Exhibit A attached to this Amendment. The defined term “Certificate of
Designation,” as used in the Agreement, shall hereafter be deemed to refer to the Certificate
of Designation for the Series B-1 Convertible Preferred Stock attached as Exhibit A to this
Amendment.

3. Section 3.3 is hereby amended to substitute 37,301,327 for 37,301,387.

4. Undefined capitalized terms used in this Amendment shall have the meanings ascribed to them
in the Agreement.

5. Except as expressly set forth in this Amendment, all other terms of the Agreement shall
remain in full force and effect and once this Amendment is executed by the parties hereto, all
references in the Agreement to “the Agreement” or “this Agreement,” as applicable, shall refer to
the Agreement, as modified by this Amendment.

6. This Amendment shall be governed by and construed in accordance with the laws of the State
of California applicable to contracts made and to be performed entirely within such State without
regard to conflict of laws principles thereof.

7. This Amendment may be executed in any number of counterparts and each of such counterparts
shall for all purposes be deemed to be an original, and all such counterparts shall together
constitute but one and the same instrument.

* * *

 

2

 

IN WITNESS WHEREOF, the parties hereto have executed and delivered this Amendment as of the
date first above written.

	 	 	 	 	 
	PURCHASER:	 	BIOMARIN PHARMACEUTICAL INC.
	 
	 	 	 	 
	 

	 	By: 
	/s/ G. Eric Davis
	 

	 	 	 
	 

	 	 	Name: 	G. Eric Davis
	 

	 	 	Title: 	VP, General Counsel
	 
	 	 	 	 
	COMPANY:	 	LA JOLLA PHARMACEUTICAL COMPANY
	 
	 	 	 	 
	 

	 	By: 
	/s/ Deirdre Y. Gillespie
	 

	 	 	 
	 

	 	 	Name: 	 Deirdre Y. Gillespie
	 

	 	 	Title: 	President and Chief Executive Officer

 

3

 

EXHIBIT A

Certificate of Designation

 

 

 

LA JOLLA PHARMACEUTICAL COMPANY

CERTIFICATE OF DESIGNATIONS

OF

SERIES B-1 CONVERTIBLE PREFERRED STOCK

(Pursuant to Section 151 of the Delaware General Corporation Law)

La Jolla Pharmaceutical Company, a Delaware corporation (the “Corporation”), in accordance
with the provisions of Section 103 of the Delaware General Corporation Law (the “DGCL”) does hereby
certify that, in accordance with Section 151(g) of the DGCL, the following resolution was duly
adopted by the Board of Directors of the Corporation as of December 18, 2008:

RESOLVED, that the Board of Directors of the Corporation pursuant to authority expressly
vested in it by the provisions of the Amended and Restated Certificate of Incorporation of the
Corporation, hereby authorizes the issuance of a series of preferred stock designated as the Series
B-1 Convertible Preferred Stock, par value $0.01 per share, of the Corporation and hereby fixes the
designation, number of shares, powers, preferences, rights, qualifications, limitations and
restrictions thereof (in addition to any provisions set forth in the Amended and Restated
Certificate of Incorporation of the Corporation which are applicable to the preferred stock of all
classes and series) as follows:

SERIES B-1 CONVERTIBLE PREFERRED STOCK

1. Designation, Amount and Par Value. The series of preferred stock shall be designated as
the Corporation’s Series B-1 Convertible Preferred Stock (the “Series B-1 Preferred Stock”), and
the number of shares so designated shall be 339,104. Each share of Series B-1 Preferred Stock shall
have a par value of $0.01 per share.

 

 

 

2. Liquidation; Dissolution or Winding Up. In the event of any liquidation (other than a
liquidation following an M&A Event (as defined below)), dissolution or winding up (either voluntary
or involuntary) of the Corporation, subject to the rights of any series of Preferred Stock or other
class of stock of the Corporation whose terms expressly provide that it ranks senior to the Series
B-1 Preferred Stock as to dividends and distributions, upon dissolution or winding up of the
Corporation (the “Senior Stock”), the holders of Series B-1 Preferred
Stock shall be entitled to receive, prior and in preference to any distribution of any of the
assets of the Corporation to the holders of the Corporation’s common stock, par value $0.01 per
share (the “Common Stock”) and pari passu with any distribution of any of the assets of the
Corporation to the holders of any other series of Preferred Stock or other class of stock of the
Corporation whose terms expressly provide that they rank pari passu with the Series B-1 Preferred
Stock as to dividends and distributions upon the liquidation, dissolution or winding up of the
Corporation (“Parity Stock”) by reason of their ownership thereof, an amount per share equal to the
sum of (i) $22.1171, plus (ii) an amount equal to accrued but unpaid dividends on such share. If
upon the occurrence of such event, the assets and funds to be distributed among the holders of the
Series B-1 Preferred Stock and the Parity Stock shall be insufficient to permit the payment to such
holders of the full aforesaid preferential amounts that such holders are entitled to, then, the
entire assets and funds of the Corporation remaining legally available for distribution shall be
distributed ratably among the holders of the Series B-1 Preferred Stock and the Parity Stock in
proportion to their respective liquidation preferences. The Corporation shall mail to each holder
of Series B-1 Preferred Stock, at least ten (10) days prior to any liquidation event, a notice
setting forth the date on which such event is expected to become effective and the type and amount
of anticipated proceeds per share of Common Stock to be distributed with respect thereto and shall
afford each such holder the opportunity to convert such shares of Series B-1 Preferred Stock into
Common Stock pursuant to Section 5 (conditional upon the consummation of such liquidation event)
prior to the consummation thereof.

3. Consolidation; Merger; Sale of Assets; Stock Splits; Consolidations, Etc. 

(a) Mandatory Conversion. In case the Corporation shall consummate any consolidation, merger
or sale of all or substantially all of the assets of the Corporation (other than a
recapitalization, subdivision, combination, reclassification, exchange or substitution of shares
provided for elsewhere in Sections 3(b) or 6 hereof) (each, an “M&A Event”), then immediately prior
to consummation of such consolidation, merger or asset sale, all shares of Series B-1 Preferred
shall automatically be converted into Common Stock in accordance with the ratio provided in
Section 5(a) and the procedures described in Section 5(b). The conversion of the shares shall take
place without regard to the delivery of certificates to the Corporation as provided for in
Section 5(b).

(b) In the event the Corporation shall (i) effect a subdivision of the outstanding shares of
Common Stock (by reclassification or otherwise than by payment of a dividend in shares of Common
Stock) into a greater number of shares of Common Stock (without a corresponding subdivision of the
Series B-1 Preferred Stock), then the Series B-1 Conversion Rate in effect immediately before that
subdivision shall be proportionately increased; or (ii) effect a combination or consolidation of
the outstanding shares of Common Stock (by reclassification or otherwise) into a lesser number of
shares of Common Stock (without a corresponding combination or consolidation of the Series B-1
Preferred Stock), then the Series B-1 Conversion Rate in effect immediately before that combination
or consolidation shall be proportionately decreased. Any adjustment under this Section shall become
effective at the close of business on the date the subdivision, combination or consolidation
becomes effective.

 

 

 

4. Mandatory Conversion Upon Termination of Development Agreement. Immediately upon
termination of that certain Development and Commercialization Agreement dated as of January 4, 2009
by and between La Jolla Pharmaceutical Company and BioMarin CF Limited for any reason in accordance
with the terms thereof, each share of Series B-1 Preferred Stock shall automatically be converted
into that number of fully paid and nonassessable shares of Common Stock equal to the Series B-1
Conversion Rate. The “Series B-1 Conversion Rate” shall initially be one for thirty (i.e., thirty
shares of Common Stock for every one share of Series B-1 Preferred Stock). The “Series B-1
Conversion Rate” shall be subject to adjustment from time to time in accordance with Sections 3 and
6. All references herein to the Series B-1 Conversion Rate herein shall mean the Series B-1
Conversion Rate as so adjusted.

5. Optional Conversion. The holders of the Series B-1 Preferred Stock shall have optional
conversion rights as follows, which must be exercised as to all shares of Series B-1 Preferred
Stock outstanding:

(a) Conversion Ratio. Subject to Section 5(b) below, each share of Series B-1
Preferred Stock shall be convertible, at the option of the holder thereof, at any time after the
date of issuance of such share, at the office of the Corporation or any transfer agent for the
Series B-1 Preferred Stock, into that number of fully paid and non-assessable shares of Common
Stock determined by multiplying such share of Series B-1 Preferred Stock and the then-applicable
Series B-1 Conversion Rate.

(b) Mechanics of Conversion. Before any holder of Series B-1 Preferred Stock shall be
entitled to convert the same into shares of Common Stock pursuant to this Section 5, such holder
shall surrender the certificate or certificates therefor, duly endorsed in blank, at the office of
the Corporation or of any transfer agent for the Series B-1 Preferred Stock, and shall give written
notice by mail, postage prepaid, to the Corporation at its principal corporate office, of the
election to convert the same and shall state therein the name or names (so long as such certificate
is in the name of the holder or an affiliate of the holder) in which the certificate or
certificates for shares of Common Stock are to be issued. The Corporation shall, as soon as
practicable thereafter, issue and deliver at such office to such holder of Series B-1 Preferred
Stock, or to the nominee or nominees of such holder, a certificate or certificates for the number
of shares of Common Stock to which such holder shall be entitled as aforesaid. Such conversion
shall be deemed to have been made immediately prior to the close of business on the date of such
surrender of the shares of Series B-1 Preferred Stock to be converted, and the person or persons
entitled to receive the shares of Common Stock issuable upon such conversion shall be treated for
all purposes as the record holder or holders of such shares of Common Stock as of such date. If the
conversion is in connection with an underwritten offer of securities registered pursuant to the
Securities Act of 1933, as amended, the conversion may, at the option of any holder tendering
Series B-1 Preferred Stock for conversion, be conditioned upon the closing with the underwriter of
the sale of securities pursuant to such offering, in which event the person(s) entitled to receive
the Common Stock issuable upon such conversion of the Series B-1 Preferred Stock shall not be
deemed to have converted such Series B-1 Preferred Stock until immediately prior to the closing of
such sale of securities.

 

 

 

6. Adjustment for Common Stock Dividends, Distributions, Reclassification, Exchange and
Substitution.

(a) In the event the Corporation shall at any time declare or pay any dividend on the Common
Stock payable in shares of Common Stock or other distribution payable in additional shares of
Common Stock, then the holders of the outstanding shares of Series B-1 Preferred Stock shall be
entitled to receive such dividend or other distribution on an as-converted to Common Stock basis.

(b) If at any time the Common Stock issuable upon the conversion of the Series B-1 Preferred
is changed into the same or a different number of shares of any class or classes of stock, whether
by recapitalization, reclassification or otherwise (other than a subdivision or combination of
shares or stock dividend or a reorganization, merger, consolidation or sale of assets provided for
in Sections 3 or 6(b)), in any such event each holder of Series B-1 Preferred shall have the right
thereafter to convert such stock into the kind and amount of stock and other securities and
property receivable upon such recapitalization, reclassification or other change by holders of the
maximum number of shares of Common Stock into which such shares of Series B-1 Preferred could have
been converted immediately prior to such recapitalization, reclassification or change, all subject
to further adjustment as provided herein or with respect to such other securities or property by
the terms thereof.

7. Reservation of Stock Issuable Upon Conversion. The Corporation shall at all times
reserve and keep available out of its authorized but unissued shares of Common Stock solely for the
purpose of effecting the conversion of the shares of the Series B-1 Preferred Stock such number of
its shares of Common Stock or other securities into which the Series B-1 Preferred Stock is then
convertible as shall from time to time be sufficient to effect the conversion of all outstanding
shares of the Series B-1 Preferred Stock; and if at any time the number of authorized but unissued
shares of Common Stock shall not be sufficient to effect the conversion of all then outstanding
shares of the Series B-1 Preferred Stock, in addition to such other remedies as shall be available
to the holder of such Series B-1 Preferred Stock, the Corporation will take such corporate action
as may, in the opinion of its counsel, be necessary to increase its authorized but unissued shares
of Common Stock to such number of shares as shall be sufficient for such purposes.

8. Cash and Property Dividends. In the event dividends are paid in cash or property of the
Corporation on shares of Common Stock, an additional dividend in cash or property of the
Corporation shall be paid with respect to all outstanding shares of Series B-1 Preferred in an
amount per share (on an as-if-converted to Common Stock basis) equal to the amount paid or set
aside for each share of Common Stock. So long as any shares of Series B-1 Preferred shall be
outstanding, no dividend, whether in cash or property, shall be paid or declared, nor shall any
other distribution be made, on any Parity Stock or Common Stock until all dividends (set forth
herein) on the Series B-1 Preferred shall have been paid or declared and set apart. The provisions
of this Section shall not, however, apply to (i) a dividend payable in Common Stock, (ii) the
acquisition of shares of any Common Stock in exchange for shares of any other Common Stock, or
(iii) any repurchase of any outstanding securities of the Corporation that is approved by the
Corporation’s Board of Directors.

 

 

 

9. No Voting Rights. Except as provided herein, the holders of shares of Series B-1
Preferred Stock shall have no voting rights.

10. No Redemption. The shares of Series B-1 Preferred Stock shall not be redeemable.

11. Reacquired Shares. Any shares of Series B-1 Preferred Stock converted, purchased or
otherwise acquired by the Corporation in any manner whatsoever shall be retired and cancelled
promptly after the acquisition thereof. All such shares shall upon their cancellation become
authorized but unissued shares of Preferred Stock and may be reissued as part of a new series of
Preferred Stock subject to the conditions and restrictions on issuance set forth herein, in the
Amended and Restated Certificate of Incorporation, or in any other Certificate of Designations
creating a series of Preferred Stock or any similar stock or as otherwise required by law.

12. Notices. Any notice required to be given to the holders of shares of Series B-1
Preferred Stock shall be deemed given if deposited in the United States mail, postage prepaid, and
addressed to the holder of record at its address appearing on the books of the Corporation.

13. Headings. The headings herein are for convenience only, do not constitute a part of
this Certificate of Designations and shall not be deemed to limit or affect any of the provisions
hereof.

14. Ranking. The Series B-1 Preferred Stock shall, with respect to distributions of assets
in the event of any liquidation (other than a liquidation following an M&A Event), dissolution or
winding up of the Corporation rank senior to the Common Stock and the Series A Junior Participating
Cumulative Preferred Stock of the Corporation.

15. Amendments. No provision of this Certificate of Designations may be amended, altered or
repealed (including by merger, consolidation or otherwise), without the affirmative vote of the
holders of a majority of the shares of Series B-1 Preferred Stock then outstanding. Any of the
rights of the holders of Series B-1 Preferred Stock set forth herein may be waived by the
affirmative vote of the holders of a majority of the shares of Series B-1 Preferred Stock then
outstanding. No waiver of any default with respect to any provision, condition or requirement of
this Certificate of Designations shall be deemed to be a continuing waiver in the future or a
waiver of any subsequent default or a waiver of any other provision, condition or requirement
hereof, nor shall any delay or omission of either party to exercise any right hereunder in any
manner impair the exercise of any such right.

[End of text. Signature page follows.]

 

 

 

IN WITNESS WHEREOF, the Corporation has caused this Certificate of Designations to be duly
executed as of this 16th day of January, 2009.

	 	 	 	 	 
	 	LA JOLLA PHARMACEUTICAL COMPANY

 	 
	 	By:  	 	 
	 	 	Name:  	Deirdre Y. Gillespie, M.D. 	 
	 	 	Title:  	President and Chief Executive Officer

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