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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 Pharmaceutic


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