Exhibit 10.1
 EXECUTION VERSION
 CONFIDENTIAL TREATMENT REQUESTED
 Redacted Portions are indicated by [****]
 DEVELOPMENT AND COMMERCIALIZATION AGREEMENT
 Agreement Effective Date La Jolla BioMarin CF
 BACKGROUND
 Product SLE
    Securities Purchase Agreement




---



##### 



ARTICLE I
DEFINITIONS
 Adverse Event AE
 Affiliate control Controlled Affiliate
 Annual Net Sales
 Asia-Pacific Territory Exhibit 1.4
 ASPEN Study Exhibit 1.5
 BioMarin CF
 BioMarin CF Know-How
 Commercial Life
  - 2 -




---



##### 



Compound Exhibit 1.9
 Control Controlled Controlling
 CTA
 Data
 Dosing Study
 EMEA
 Existing In-License Exhibit 1.15
 FDA
 Financial Appendix Appendix A
 FTE
  - 3 -




---



##### 



Full Participation Point
       IND
 Interim Efficacy Analysis Interim Efficacy Analyses
 First Interim Efficacy Analysis
 Second Interim Efficacy Analysis
  - 4 -




---



##### 



Joint Patent
 Know-How
 La Jolla Know-How
 La Jolla Patents Exhibit 1.25
 La Jollas Knowledge Exhibit 1.26
 Major Market
 Marketing Approval
 Marketing Application
 MAA
  - 5 -




---



##### 



NDA
 Party Parties
 Patent(s)
 Positive Dosing Study
 Product
 Product Trademarks Exhibit 1.36
 PV Procedures
 Regulatory Authority
 Sales Representative
 SOP
 Securities Purchase Agreement Exhibit 1.41
 Sublicensee Sublicense
  - 6 -




---



##### 



Territory
 Third Party
 United States
 Additional Definitions
 Term
    
    Section DefinedAgreement
    
    IntroductionAll Other Costs
    
    Exhibit 4.2EAsia-Pacific License
    
    2.4(a)Asia-Pacific Licensee
    
    2.4(b)Auditing Party
    
    7.17(a)BioMarin CF Improvements
    
    11.1(c)BioMarin CF Indemnitees
    
    16.2Capitalized Asset
    
    Financial AppendixCo-Chair
    
    3.3Collaboration Agreement
    
    Financial AppendixCompletion Notice
    
    7.7Confidential Information
    
    10.1Contingent Right
    
    2.4(f)Controlled Affiliate
    
    1.2Cost Effective Price
    
    9.1(a)Data Monitoring Board
    
    7.6(a)Definitive Agreement
    
    2.4(c)Development Costs
    
    Financial AppendixDevelopment Transition Period
    
    14.4(a)(i)Dispute
    
    17.2Distribution Costs
    
    Financial AppendixEffective Date
    
    IntroductionEliminated Party
    
    14.4(a)(i)Enforcement Action
    
    11.3(b)(i)Equity Election Notice
    
    7.5Fault of BioMarin CF
    
    16.4(b)(ii)Fault of La Jolla
    
    16.4(b)(i)Field-Based FTEs
    
    6.2(a)(i)First Commercial Sale
    
    Financial AppendixFTE Costs
    
    Financial AppendixForce Majeure Event
    
    18.1Forecast
    
    Exhibit 9.1AFutile Determination
    
    7.6(b)GAAP
    
    Financial AppendixGMP
    
    5.4Gross Sales
    
    Financial AppendixIFRS
    
    Financial AppendixIndemnitee
    
    16.3Indemnitor
    
    16.3Infringement
    
    11.3(a)Infringement Actions
    
    11.4Initial Operating Plan/Budget
    
    4.2(b)Initial Product
    
    4.3(a)Inspected Party
    
    5.3Interest Rate
    
    7.14(b)IP Management Costs
    
    Financial AppendixJAMS
    
    17.3(a)Joint Promotion Plan
    
    6.2(c)Joint Steering Committee or JSC
    
    3.1(a)JHU License
    
    Exhibit 1.15La Jolla Improvements
    
    11.1(c)
  - 7 -




---



##### 



Term
    
    Section DefinedLa Jolla Indemnitees
    
    16.1Launch-1st Year Period
    
    Exhibit 4.2ELiabilities
    
    16.1Manufacturing Costs
    
    Financial AppendixManufacturing Process Development
    
    4.2(e)(ii)Marketing Costs
    
    Financial AppendixMaximum Regional Spend
    
    Exhibit 4.2EMultiple Product Sales
    
    Financial AppendixNegotiation Period
    
    2.4(c)New Technology
    
    11.6(c)Net Sales
    
    Financial AppendixNon-Futile Determination
    
    7.6(b)Objecting Party
    
    17.2Ongoing Trials
    
    14.4(a)(i)Operating Forecast
    
    4.2(a)Operating Plan/Budget
    
    4.2(a)Other Operating Expense
    
    Financial AppendixOther Operating Income
    
    Financial AppendixOut-of-Pocket Expenses
    
    Financial AppendixPaying Party
    
    Financial AppendixPhase IV Studies
    
    Exhibit 4.2EPrior Agreement
    
    10.6Producing Party
    
    9.4(a)Product Liability Claim
    
    16.5(a)Product Promotional Materials
    
    12.1Profit/Loss
    
    Financial AppendixPromotional/Sales/Marketing or PSM
    
    Exhibit 4.2EProposed Territory
    
    2.4(a)Prosecution and Maintenance or Prosecute and Maintain
    
    11.2(c)Publication
    
    10.4Purchased Interests
    
    14.2.2(a)Purchase Notice
    
    14.2.2(b)Purchase Price
    
    14.2.2(a)Purchase Right
    
    14.2.2(a)P-Value Achievement
    
    7.6(c)Quarterly Measurement
    
    Exhibit 4.2EQ4, Q5 and Q6
    
    Exhibit 9.1ARecall Costs
    
    16.4(b)Region
    
    Exhibit 4.2ERemaining Party
    
    14.4(a)(i)Renal Events Committee
    
    7.6(d)Report Table
    
    Financial AppendixResponding Party
    
    7.17(a)ROT
    
    Exhibit 4.2ERoyalty Notice
    
    6.3R&D/LCM
    
    Exhibit 4.2ESale Price
    
    14.2.1Sale Right
    
    14.2.1Sales Costs
    
    Financial AppendixSales Force Deployment Option
    
    6.2(a)Shared Costs
    
    Financial AppendixSEC
    
    Financial AppendixSecured Note
    
    14.2.1SLE
    
    BackgroundSold Interests
    
    14.2.1Third Party Claim
    
    16.1Wind-down Period
    
    14.4(a)(ii)Working Group
    
    3.22nd Year of Sales
    
    Exhibit 4.2E128 Flare Topline Data
    
    7.6(e)
  - 8 -




---



##### 



ARTICLE II
GRANT OF LICENSE
 License
   Sublicensees
  - 9 -




---



##### 



Unauthorized Activities; Activities Outside the Territory.
 BioMarin CF Rights Limited
 Territorial Integrity
    Asia-Pacific Matching Right
 [****]
 [****]
 [****]
 [****]
 [****]
  - 10 -




---



##### 



No Implied Obligations Contingent Right
 Audit Rights
 Right to Share Information
 ARTICLE III
GOVERNANCE
 Joint Steering Committee
 Establishment Joint Steering Committee JSC
 Duties
    - 11 -




---



##### 



Establishment of Working Group Working Group
  - 12 -




---



##### 



JSC Membership Co-Chair
 JSC Meetings
 Decision-Making
 Scope of Governance
  - 13 -




---



##### 



ARTICLE IV
DEVELOPMENT; OPERATING PLAN BUDGET
 Overall Efforts in Development
    - 14 -




---



##### 



ASPEN Study
 Development Costs
 Dosing Study
 Operating Plan/Budget
 General Operating Plan/Budget
 Initial Operating Plan/Budget Exhibit 4.2B Initial Operating
Plan/Budget
 Amendments
  - 15 -




---



##### 



Timing and Process for Annual Amendments
   Operating Plan/Budget Content
   Exhibit 4.2E
  - 16 -




---



##### 



Exchange of Data and Know-How
 By Either Party i.e. Initial Product
 Provision of Data to JSC
 Term of Ongoing Obligations
  - 17 -




---



##### 



ARTICLE V
REGULATORY MATTERS
 Regulatory Responsibilities
 ASPEN Study and Dosing Study
 NDA Regulatory Responsibilities
 Other Regulatory Responsibilities
 Costs
  - 18 -




---



##### 



Filings and Meetings with Regulatory Authorities
 Regulatory Filings and Correspondence
 Regulatory Interactions
 Role of JSC
 Other Regulatory Matters
 Regulatory Inspections
 Audit Rights GMP
  - 19 -




---



##### 



Adverse Event Management
     ARTICLE VI
COMMERCIALIZATION AND PROMOTION
 Commercialization
 General
 Territorial Allocation
 United States Exhibit 6.1B
  - 20 -




---



##### 



Countries of the Territory Outside the Unites States
 Promotion of the Products
 Activities Prior to Full Participation Point
 La Jollas Sales Force Deployment Option
 Exercise of Option Sales Force
Deployment Option
 La Jolla FTEs
  - 21 -




---



##### 



Sales Activities
 Role of the Joint Steering Committee
 Joint Promotion Plan Joint Promotion Plan
 Performance Standards
  - 22 -




---



##### 



BioMarin CF Practices and Procedures
 Sales Efforts; Costs
 Sales Efforts of the Parties
 Costs
 Sales Representative Compensation Weighting
 Timing i.e.
 Right to Seek to Change Structure to Royalties on Net Sales
 Booking Sales
 Commercialization Activities Outside the Territory
  - 23 -




---



##### 



ARTICLE VII
PAYMENTS
 Commencement Payments
    Payments in Connection with First Interim Efficacy Analysis
     - 24 -




---



##### 



Payments in Connection with Second Interim Efficacy Analysis
     Payment in Connection with 128 Flare Topline Data
     - 25 -




---



##### 



Partial Payments in Equity i.e. Equity Election Notice
 Certain Terms
 Data Monitoring Board
 Non-Futile Determination Futile Determination
 P-Value Achievement
  - 26 -




---



##### 



Renal Events Committee
 128 Flare Topline Data
 Notice by La Jolla Completion Notice
 Termination
 i.e.
   - 27 -




---



##### 



Regulatory Milestones
 One Time MilestoneRegulatory Milestone
    
    Payment Amount1. First receipt of an approval of
an NDA for the Product in the ASPEN
Study:a. if by such receipt La
Jolla has completed a
Positive Dosing Study
    
    $
    45,000,000b. if by such receipt La
Jolla has not completed a
Positive Dosing Study
    
    $
    30,000,000c. only in the circumstance
that the P-Value Achievement
occurs at the First Interim
Efficacy Analysis or the
Second Interim Efficacy
Analysis and the Dosing Study
is not complete at the time
of approval of the NDA, if
within one (1) year of such
approval La Jolla completes a
Positive Dosing Study
    
    $
    15,000,0002. First receipt of an approval of
an MAA (or Marketing Approval in all
of the Major Markets other than the
United States)
    
    $
    10,000,000
  - 28 -




---



##### 



Net Sales Milestones
 One Time MilestoneNet Sales Milestone
    
    Payment Amount1. First time that Annual Net Sales
of Products within the Territory
equal or exceed Two Hundred Fifty
Million Dollars ($250,000,000)
    
    $
    13,500,0002. First time that Annual Net Sales
of Products within the Territory
equal or exceed Five Hundred Million
Dollars ($500,000,000)
    
    $
    25,000,0003. First time that Annual Net Sales
of Products within the Territory
equal or exceed Seven Hundred Fifty
Million Dollars ($750,000,000)
    
    $
    37,500,0004. First time that Annual Net Sales
of Products within the Territory
equal or exceed One Billion Dollars
($1,000,000,000)
    
    $
    50,000,000
 Milestone Reporting and Payments
 Cost-Profit Sharing
  - 29 -




---



##### 



Futile Determination or Failed P-Value Achievement
 Adverse Outcome
 Rights Relating to Approval of the NDA without P-Value Achievement
 Other Payment Terms
 Payments Non-Refundable
 Payment Method The Wall Street Journal Interest Rate
 Currency Conversion The Wall Street Journal
 Withholding Taxes
  - 30 -




---



##### 



Books and Records
 Audit Rights
 Request Auditing Party Responding Party
 Discrepancies
 Restriction on Certain Equity Investments
  - 31 -




---



##### 



ARTICLE VIII
CERTAIN COVENANTS
 General Communications
 Conduct of Activities
 ARTICLE IX
MANUFACTURING
 Manufacturing
   Funding for La Jolla Facility
  - 32 -




---



##### 



Cooperation
 Materials Produced by Third Party Contractors
 Materials Produced by BioMarin CF
 Shortage of Supply
 Procedures Producing
Party
 Allocation
 BioMarin CF Manufacturing
 Restrictions on Manufacturing Locations
  - 33 -




---



##### 



ARTICLE X
CONFIDENTIALITY
 Confidential Information Confidential Information
      Permitted Disclosures ,
  - 34 -




---



##### 



Confidential Terms
 Publication of Product Information Publication
 Press Releases and Announcements
 Initial Release
 Further Publicity
 Certain Events
  - 35 -




---



##### 



Prior Non-Disclosure Agreements Prior Agreement
 ARTICLE XI
INTELLECTUAL PROPERTY
 Inventions and Other Intellectual Property
 General Principles
 Joint Ownership
 Grant-Back License BioMarin CF Improvements La Jolla Improvements
  - 36 -




---



##### 



Prosecution and Maintenance of Patents
 Control of Prosecution Prior to Full Participation
 Control of Prosecution After Full Participation
  - 37 -




---



##### 



Scope of Activities Prosecution and
Maintenance Prosecute and Maintain
 Cooperation
 Prosecution and Maintenance Costs
  - 38 -




---



##### 



Enforcement
 Notice Infringement
 Enforcement Actions
 Enforcement Action
  Cooperation
 Recoveries
  - 39 -




---



##### 



Third Party Infringement Claims Infringement Actions
 Patent Marking
 Third Party Technologies
 Existing Third Party Technology Exhibit 11.6
 Provisions of Existing In-License Exhibit 11.6 Exhibit 11.6 Exhibit 11.6
 New Technology
  - 40 -




---



##### 



ARTICLE XII
TRADEMARKS
 Display Product Promotional Materials
 Title
 Grant of License
 Registration of Trade Marks
 Certain Covenants
  - 41 -




---



##### 



Recordation of Licenses
 Approval of Packaging and Promotional Materials
 Enforcement
 Termination of Trade Mark License
 Trademark Costs
 ARTICLE XIII
TERM AND TERMINATION
 Term
  - 42 -




---



##### 



Breach
 Convenience.
 Election Not to Pay Under Sections 7.2, 7.3, 7.4 and 7.13
 ARTICLE XIV
EFFECT OF TERMINATION
 Accrued Obligations
  - 43 -




---



##### 



Right of Sale/Right of Purchase
 Right of Sale
 Right of Purchase
       Procedure
 Termination under Sections 13.2 or 13.3
 Wind-down Period
 Development Eliminated Party Ongoing Trials Remaining Party Development Transition Period
  - 44 -




---



##### 



Commercialization Wind-down Period
 Manufacturing
 Assignment of Regulatory Filings and Marketing Approvals
 Data and Know-How Disclosure
  - 45 -




---



##### 



Transition
 Licenses.
 Sublicensees
 Return of Materials
 Expiration of Term
  - 46 -




---



##### 



Surviving Sections and Articles
 ARTICLE XV
REPRESENTATIONS, WARRANTIES AND COVENANTS
 General Representations
 Duly Organized
 Due Execution; Binding Agreement
  - 47 -




---



##### 



Authorizations
 No Other Affiliates
 Representations and Warranties of La Jolla
 Exhibit 15.2
      Exhibit 15.2
  - 48 -




---



##### 



Exhibit 1.25
 Exhibit 1.25 Exhibit 15.2I
    Representations and Warranties of BioMarin CF
       - 49 -




---



##### 



DISCLAIMER
 ARTICLE XVI
INDEMNIFICATION
 Indemnification of La Jolla La Jolla
Indemnitees Liabilities Third Party Claim
 Indemnification of BioMarin CF BioMarin CF Indemnitees
 Procedure Indemnitee Indemnitor
  - 50 -




---



##### 



Recalls
 Voluntary and Mandatory Recalls; Decision-Making
 Costs of Recall Recall Costs
   Products Liability Claims
 Product Liability Claim
  - 51 -




---



##### 



WAIVER OF CERTAIN CLAIMS
 ARTICLE XVII
DISPUTE RESOLUTION
 Determination by CEOS
 Arbitration Proceedings Dispute Objecting Party
  - 52 -




---



##### 



Conduct of Arbitration
 General Provisions JAMS
 Decision of Arbitrator
 Location; Costs.
 Timetable for Completion in Thirty (30) Days
  - 53 -




---



##### 



ARTICLE XVIII
GENERAL PROVISIONS
 Force Majeure Force Majeure Event
 Governing Law; Venue
 Waiver of Breach
 Modification
 Severability
 Entire Agreement
  - 54 -




---



##### 



Notices
 To La Jolla:
    
    With a copy to:La Jolla Pharmaceutical Company
    
    Wilson, Sonsini, Goodrich & Rosati6455 Nancy Ridge Drive
    
    650 Page Mill RoadSan Diego, CA 92121
    
    Palo Alto, CA 94304Telephone: (858) 452-6600
    
    Telephone: (650) 493-9300Facsimile: (858) 626-2851
    
    Facsimile: (650) 493-6811Attention: Chief Executive Officer
    
    Attention: Kenneth A. ClarkGoodwin Procter, LLP53 State StreetBoston, MA 02109Fax: (617) 523-1231Attention: Mitchell Bloom and Ryan A. MurrTo BioMarin CF:
    
    With a copy to:BioMarin CF Limited
    
    BioMarin Pharmaceutical Inc.Dominion House
    
    105 Digital Drive60 Montrose Avenue
    
    Novato, CA 94949Nassau, New Providence, The Bahamas
    
    Telephone: (415) 506-6307Telephone: (415) 506-6307
    
    Facsimile: (415) 506-6425Facsimile: (415) 382-7889
    
    Attention: General CounselAttention: Managing Director
   - 55 -




---



##### 



Assignment
 No Partnership or Joint Venture
 Interpretation
  - 56 -




---



##### 



Export Laws
 Counterparts; Facsimile Signatures
 [Remainder of page intentionally left blank; signature page follows.]
  - 57 -




---



##### 



LA JOLLA PHARMACEUTICAL COMPANY
    
    
    
    BIOMARIN CF LIMITEDBy:
    
    /s/ Deirdre Y. Gillespie
    
    
    
    By:
    
    /s/ G. Eric DavisName:
    
    Deirdre Y. Gillespie, M.D.
    
    
    
    
    
    Name:
    
    G. Eric DavisTitle:
    
    President and Chief Executive Officer
    
    
    
    
    
    Title:
    
    Managing Director




---



##### 



Exhibit List
                Appendix List




---



##### 



Exhibit 1.4
 Asia-Pacific Territory




---



##### 



Exhibit 1.5
 ASPEN Study




---



##### 



Exhibit 1.9
 Compound




---



##### 



Exhibit 1.15
 Existing In-License
 JHU License




---



##### 



Exhibit 1.25
 La Jolla Patents
 ISSUED
PATENTS
AS OF DECEMBER 8, 2008
 Patent No.
          1
  2
    
 1
    
    Validated in AT, BE, DK, FR, DE, GR, IT, LU, MC, NL,
PT, ES, SE, CH and GB.2
    
    Validated in AT, BE, CH, DE, DK, ES, FR, GB, GR, IE,
IT, LU, NL, PT and SE. IT patent status being confirmed. See Exhibit 15.2
below.




---



##### 



ISSUED PATENTS
AS OF DECEMBER 8, 2008
 Patent No.
  3
             4
     
 3
    
    Patent abandoned for failure to pay annuity. Petition
to reinstate in preparation. See Exhibit 15.2 below.4
    
    Validated in AT, BE, CH, DE, DK, FR, GB, GR, IT, LU,
NL, ES and SE.




---



##### 



ISSUED PATENTS
AS OF DECEMBER 8, 2008
 Patent No.
   Licensed Patents
      PENDING PATENT APPLICATIONS
AS OF DECEMBER 8, 2008
 Patent Application No.




---



##### 



PENDING PATENT APPLICATIONS
AS OF DECEMBER 8, 2008
 Patent Application No.




---



##### 



Exhibit 1.26
 La Jollas Senior Management Team




---



##### 



Exhibit 1.36
 Product Trademarks
 Mark/Serial Number/Registration Number
    
    CountryRIQUENT
Serial No: 78/102,996
Reg. No: 2,787,557
    
    United StatesRIQUENT
Serial No: 78/947,249
Reg. No: 3,318,222
    
    United StatesRIQUENT
Serial No: 1144359
Reg. No: TMA624679
    
    CanadaRIQUENT
Serial No: 002675601
Reg. No: 002675601
    
    Europe *RIQUENT
Serial No: IR954406
Reg. No: IR954406
    
    International (designating Europe)*
 *
    
    European Community Trademark Countries: Austria, Benelux (Belgium, The Netherlands &
Luxembourg), Bulgaria, Denmark, Finland, France, Germany, Greece, Ireland, Italy, Portugal,
Romania, Spain, Sweden, United Kingdom, Cyprus, The Czech Republic, Estonia, Hungary, Latvia,
Lithuania, Malta, Poland, Slovakia, and Slovenia.




---



##### 



Exhibit 1.41
 Securities Purchase Agreement




---



##### 



Exhibit 4.2B
 Initial Development Plan/Budget




---



##### 



Exhibit 4.2E
 Operating Plan/Budget Requirements




---



##### 



Exhibit 6.1B
 Joint US Marketing Activities




---



##### 



Exhibit 9.1A
 Manufacturing




---



##### 



Exhibit 11.6
 Existing In-License Provisions
   Provisions extracted from Existing In-License




---



##### 








---



##### 



Exhibit 15.2
 Exceptions to La Jollas Representations and Warranties
 th




---



##### 



Exhibit 15.2I
 Form of Consent of Existing In-License Licensor




---



##### 



[LJP LETTERHEAD]
  VIA EMAIL AND FEDERAL EXPRESS
 
 Re:
    
    Exclusive License Agreement between The Johns Hopkins
University (JHU) and La Jolla Pharmaceutical Company
(LJP) effective as of November 25, 2002, as amended
(License Agreement)
  ®
 ®




---



##### 



CONSENTED AND AGREED BY THE JOHNS HOPKINS UNIVERSITY
 By:Name:Title:Date:




---



##### 



Appendix A
 Financial Appendix