Document:

Manufacturing, Marketing and Sales Agreement

 EXHIBIT 10.30 
  
 CONFIDENTIAL TREATMENT REQUESTED 
 Redacted Portions are indicated by [****] 
  
 MANUFACTURING, MARKETING AND SALES AGREEMENT 
  
 THIS MANUFACTURING, MARKETING AND SALES AGREEMENT (this “Agreement”) is
made effective as of the 1st day of January, 2008 (the “Effective Date”), by and among BioMarin Pharmaceutical Inc., a Delaware
corporation having its principal place of business at 105 Digital Drive, Novato, California 94949 (“BioMarin”); Genzyme Corporation, a Massachusetts corporation having its principal place of business at 500 Kendall Square,
Cambridge, Massachusetts 02142 (“Genzyme”) and BioMarin/Genzyme LLC, a Delaware limited liability company having its principal place of business at 500 Kendall Street, Cambridge, Massachusetts 02142 (“BioMarin/Genzyme
LLC”). 
  
 RECITALS 
  
 A. BioMarin, Genzyme and BioMarin/Genzyme LLC are parties to a Collaboration
Agreement dated as of September 4, 1998 (the “Original Collaboration Agreement”) pursuant to which BioMarin and Genzyme through BioMarin/Genzyme LLC develop, manufacture, market and sell Aldurazyme (as defined herein).

  
 B. The Parties no longer desire to develop, manufacture,
market and sell Aldurazyme through a joint venture and instead have agreed that: (1) BioMarin will manufacture Aldurazyme and sell finished product to Genzyme; (2) Genzyme will label and commercially distribute, market and sell Aldurazyme
globally; (3) each of Genzyme and BioMarin may conduct its own research and development of Aldurazyme and other Collaboration Products (as defined herein) in accordance with the terms of this Agreement and the Amended and Restated Collaboration
Agreement (as defined herein); and (4) BioMarin/Genzyme LLC will maintain and provide intellectual property licenses and sublicenses to BioMarin and Genzyme so that they may fulfill their respective obligations under this Agreement, the Amended
and Restated Collaboration Agreement and the Fill Agreement (as defined herein). 
  
 C. BioMarin and Genzyme have amended and restated the Original Collaboration Agreement so that hereafter BioMarin/Genzyme LLC will no longer engage in commercial activities and will solely (1) hold the
intellectual property relating to Aldurazyme and license all such intellectual property on the terms set forth herein to BioMarin and Genzyme on the terms set forth in the Amended and Restated Collaboration Agreement (as defined below) and
(2) and engage in research and development activities that are mutually selected and funded by BioMarin and Genzyme. 

 D. To effect the foregoing, the Parties are also entering into (1) a separate Fill Agreement of even
date herewith pursuant to which Genzyme will continue to provide fill services for Aldurazyme to BioMarin (the “Fill Agreement”); (2) the aforementioned amendment and restatement of the Original Collaboration Agreement of even
date herewith (the “Amended and Restated Collaboration Agreement”); and (3) a Members Agreement of even date herewith pursuant to which certain current assets and liabilities of BioMarin/Genzyme LLC and its subsidiaries will be
distributed to its Members (the “Members Agreement”). 
  
 AGREEMENT 
  
 NOW, THEREFORE, in
consideration of the mutual promises set forth in this Agreement and other good and valuable consideration, the receipt and sufficiency of which are hereby acknowledged, the Parties hereto hereby agree as follows: 
  

	1.	DEFINITIONS. 

  

	1.1	“Adverse Experience” shall mean any undesirable physical, psychological or behavioral effect experienced by a human Patient or subject that is associated
with the use of Aldurazyme, whether or not considered product-related, including an adverse experience occurring: in the course of the use of Aldurazyme in professional practice; from an overdose of Aldurazyme (whether accidental or intentional);
from the abuse of Aldurazyme; from the withdrawal of Aldurazyme; or from any failure of Aldurazyme’s expected pharmacological action. 

  

	1.2	“Affiliate” shall mean any corporation, limited liability company, firm, partnership, limited liability partnership or other entity, whether de jure or de
facto, which, at the time in question, is directly or indirectly owned by or controlled by, or under common control with, BioMarin or Genzyme, as the case may be. For the purposes of this definition, a Party shall be deemed to have
“control” if such Party: (a) owns, directly or indirectly, fifty percent (50%) or more (or such lesser percentage which is the maximum allowed to be owned by a foreign corporation in a particular jurisdiction) of
(i) the voting stock or shareholders’ equity of a corporation, (ii) the partnership interests in a partnership, (iii) the membership interests in a limited liability company, or (iv) in the case of any other entity, the
right to receive fifty percent (50%) or more of either the profits or the assets upon dissolution; or (b) possesses, directly or indirectly, the power to direct or cause the direction of the management and policies of the corporation or
other entity or the power to elect more than fifty percent (50%) of the members of the governing body of the corporation, limited liability company or other entity. 

  

	1.3	“Agreement” shall have the meaning set forth in the introductory paragraph hereof. 

  

	1.4	“Aldurazyme” shall mean alpha-L-iduronidase meeting the Specifications. 

  

	1.5	“alpha-L-iduronidase” shall mean recombinant human alpha-L-iduronidase. 

  

	1.6	“Amended and Restated Collaboration Agreement” shall have the meaning set forth in the recitals. 

  

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	1.7	“Annual Net Sales” shall have the meaning set forth in Section 6.1(a). 

  

	1.8	“Batch” or “Lot” shall mean each separate and distinct quantity of Aldurazyme processed under continuous conditions and designated by
BioMarin with a batch or lot number. 

  

	1.9	“BioMarin Companies” shall mean BioMarin and BioMarin Genetics. 

  

	1.10	“BioMarin Genetics” shall mean BioMarin Genetics, Inc., a Delaware corporation and wholly-owned subsidiary of BioMarin. 

  

	1.11	“BLA” shall mean a Biologics License Application or similar application as approved by the FDA on April 30, 2003, that provides for marketing approval
for Aldurazyme in the United States, as the same may be updated or amended from time to time. 

  

	1.12	“cGMP Regulations” means the applicable current Good Manufacturing Practices as promulgated under ICH Q7A-Good Manufacturing Practice Guidance for Active
Pharmaceutical Ingredients, U.S. Federal Food, Drug and Cosmetic Act at 21 CFR and the EEC Guide to Good Manufacturing Practices for Medical Products (Vol. IV — rules governing medical products in the European Community 1989) in the most recent
version. 

  

	1.13	“Certificate of Analysis” or “COA” shall mean a document in the form attached as Exhibit B executed by BioMarin to certify that a
Batch or Lot of Aldurazyme meets the Specifications. 

  

	1.14	“Certificate of Compliance” or “COC” shall mean a document certifying a Batch or Lot of Aldurazyme meets Genzyme’s product release
criteria and was produced in compliance with cGMP requirements. 

  

	1.15	“Collaboration Product” shall mean Aldurazyme and any other pharmaceutical compositions of alpha-L-iduronidase, including without limitation any and all
improvements, derivatives, analogs, combination products, delivery systems and dosage forms related thereto. 

  

	1.16	“commercially reasonable and diligent efforts” will mean that level of effort which, consistent with the exercise of prudent scientific and business
judgment, is applied by the Party in question to its other therapeutic products at a similar stage of development and with similar commercial potential. 

  

	1.17	“Control” shall mean possession of the ability to grant a license or sublicense as provided for herein without violating the terms of an agreement with a
Third Party. 

  

	1.18	“Effective Date” shall mean January 1, 2008. 

  

	1.19	“EMEA” shall mean the European Agency for the Evaluation of Medical Products or any successor agency. 

  

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	1.20	“European Commission” shall mean that body of the European Union that grants Marketing Application Approvals. 

  

	1.21	“FDA” shall mean the United States Food and Drug Administration, or any successor agency. 

  

	1.22	“Field” shall mean any and all therapeutic applications of alpha-L-iduronidase for MPS I and other alpha-L-iduronidase deficiencies. Notwithstanding the
foregoing, the Field shall not include Gene Therapy for MPS I or other alpha-L-iduronidase deficiencies. For purposes of this Agreement, “Gene Therapy” shall mean treatment or prevention of MPS I or other alpha-L-iduronidase
deficiencies by means of ex vivo or in vivo introduction (via viral or nonviral gene transfer systems) of nucleotide sequences (including without limitation, DNA, RNA and complementary and reverse complementary nucleotide sequences
thereto, whether coding or non-coding). 

  

	1.23	“Fill Agreement” shall have the meaning set forth in the recitals. 

  

	1.24	“GAAP” shall mean the then-current United States generally accepted accounting principles, consistently applied. 

  

	1.25	“Insolvency Event” shall mean, with respect to a Party, that the Party (i) applies for or consents to the appointment of a receiver, trustee, liquidator
or custodian for itself or of all or a substantial part of its property; (ii) becomes unable, or admits in writing its inability, to pay its debts generally as they mature; (iii) makes a general assignment for the benefit of its or any of
its creditors; (iv) is dissolved or liquidated in full or in part; (v) commences 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 consents 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 (vi) takes any
action for the purpose of effecting any of the foregoing; or (vii) the Party has commenced against it proceedings for the appointment of a receiver, trustee, liquidator or custodian, or an involuntary case or other proceedings seeking
liquidation, reorganization or other relief under any bankruptcy, insolvency or other similar law now or hereafter in effect and such proceeding is not dismissed or discharged within sixty (60) calendar days of commencement.

  

	1.26	“Labeling Materials” shall have the meaning set forth in Section 3.1. 

  

	1.27	“MAA” shall mean the Marketing Application as approved by the EMEA on June 10, 2003 that provides for marketing approval for Aldurazyme in the European
Union, as the same may be updated or amended from time to time. 

  

	1.28	 “Manufacturing Know-How” shall mean all information, techniques, inventions, discoveries, improvements, practices, methods, knowledge,
skill, experience and other technology, whether or not patentable or copyrightable, and any copyrights based thereon, relating to or necessary or useful for the production, purification, packaging, 

  

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storage and transportation of Collaboration Products, including without limitation specifications, acceptance criteria, manufacturing batch records, standard
operating procedures, engineering plans, installation, operation and process qualification protocols for equipment, validation records, master files submitted to the FDA, process validation reports, environmental monitoring processes, test data
including pharmacological, toxicological and clinical test data, cost data and employee training materials. 

  

	1.29	“Marketing Application” shall mean a marketing authorization application filed by or under authority of Genzyme with the requisite health regulatory
authority of any country requesting approval for commercialization of Aldurazyme for a particular indication in such country, including as applicable, the MAA. 

  

	1.30	“Marketing Application Approval” shall mean, with respect to each country approval of the Marketing Application filed in such country by the health
regulatory authority in such country, including as applicable, the MAA. 

  

	1.31	“Marketing Costs” with respect to Aldurazyme shall mean the sales and marketing costs and expenses incurred by Genzyme with respect to work performed by
Genzyme and its Affiliates and subcontractors in connection with the performance of and in accordance with the marketing plan for Aldurazyme, including without limitation, sales and marketing costs related to performing market research, advertising,
producing promotional literature, sponsoring seminars and symposia, sales training meetings and seminars, originating sales, providing reimbursement and other patient support services, recruitment costs and salaries and associated expenses for sales
and marketing personnel and support staff, and distribution fees. 

  

	1.32	“Master Batch Record” shall mean a written description of the procedure to be followed by BioMarin in processing of a Batch of Aldurazyme, which description
shall include, but not be limited to, a complete list of all active and inactive ingredients, components, weights and measures and procedures used in processing the Aldurazyme within the meaning of 21 CFR part 211.186, or its successor as
in effect from time to time. 

  

	1.33	“Members Agreement” shall have the meaning set forth in the recitals. 

  

	1.34	“MPS-I” shall mean mucopolysaccharidosis I. 

  

	1.35	“Multiple Products Sale” shall mean a sale of Aldurazyme by Genzyme or its Affiliates to an independent Third Party customer that is associated, by contract
or course of dealing, with the use or sale or one or more other products or services [ ****]. In determining the consideration received for Aldurazyme, the following shall be excluded: (i) Aldurazyme from the quantities provided to Genzyme
pursuant to Section 6.1(d)(i), and (ii) transfers of Aldurazyme that are excluded from the definition of Net Sales under Section 1.36 below. 

  

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	1.36	“Net Sales” with respect to Aldurazyme shall mean the gross invoiced sales price of Aldurazyme billed to independent Third Party customers, including without
limitation Third Party distributors, in fully legitimate, arms length transactions, less: (a) credits or allowances granted upon billing corrections, (b) credits or allowances granted upon returns or rejections, provided that
such Aldurazyme shall be deemed to be unsold and treated in accordance with Section 6.7; (c) freight, shipping and insurance costs (incurred in transporting Aldurazyme to such customers to the extent separately billed);
(d) quantity, cash and other trade discounts [****], credits or allowances actually allowed and taken; (e) customs duties, surcharges and taxes and other governmental charges incurred in connection with the production, sale,
transportation, delivery, use, exportation or importation of Aldurazyme in final form; (f) amounts incurred resulting from governmental mandated rebate or discount programs; and (g) Third Party rebates and charge backs, hospital buying
group/group purchasing organization administration fees or managed care organization rebates; all in accordance with standard allocation procedures, allowance methodologies and accounting requirements consistently applied, which procedures,
methodologies and requirements are in accordance with GAAP. “Net Sales” with respect to Aldurazyme sold in Multiple Products Sales shall be calculated by Genzyme in good faith and shall equal a fair and equitable allocation of all
consideration received by Genzyme in connection with a Multiple Products Sale, considering the nature and economic value of each component of the Multiple Products Sale. The transfer of Aldurazyme by Genzyme or one of its Affiliates to another
Affiliate of Genzyme in furtherance of a sale to a Third Party shall not be considered a sale; in such cases, Net Sales shall be determined based on the invoiced sales price by the Affiliate to its customer, less the deductions allowed under this
Section 1.36. “Net Sales” shall not include transfers of Aldurazyme for use for clinical purposes, in compassionate or expanded access programs, or in programs for patients paying a nominal price for Aldurazyme.

  

	1.37	“Operating Agreement” shall mean that certain Operating Agreement of BioMarin/Genzyme LLC dated as of September 4, 1998 entered into by and among the
BioMarin Companies and Genzyme. 

  

	1.38	“Order” shall mean a firm purchase order originated by Genzyme and sent to BioMarin which sets forth, at a minimum, the quantities of Aldurazyme ordered, the
delivery dates and other material information as set forth in Section 3.3. 

  

	1.39	“Original Collaboration Agreement” shall have the meaning set forth in the recitals. 

  

	1.40	“Party” shall mean BioMarin, Genzyme or BioMarin/Genzyme LLC, as applicable, and “Parties” shall mean BioMarin, Genzyme and BioMarin/Genzyme
LLC, collectively. 

  

	1.41	“Patent Rights” shall mean all US and foreign patents and patent applications (including continuations, continuations-in-part, divisionals, reissues,
re-examinations, renewals, supplemental protection certificates and extensions) which are or become owned or controlled by a Party or to which such Party has, now or in the future, the right to grant licenses and other rights, which generically or
specifically claim Collaboration Products, a process for manufacturing Collaboration Products, an intermediate used in such process or a use of a Collaboration Product. 

  

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	1.42	“Patient” shall mean any person who is the actual recipient of Aldurazyme for clinical or medical purposes. 

  

	1.43	“Percentage Interest” shall have the meaning set forth in the Operating Agreement. 

  

	1.44	“Post-Marketing Studies” shall mean any clinical trial using Aldurazyme performed by Genzyme or its Affiliates required by regulatory authorities as a
condition to the issuance, continuation, or maintenance of a Marketing Application Approval. Post-Marketing Studies shall not include registries. 

  

	1.45	“Regulatory Approvals” shall mean all approvals from regulatory authorities in any country required lawfully to manufacture and market Aldurazyme in any such
country, including without limitation approval of any BLA, any establishment license application filed with the FDA to obtain approval of the facilities and equipment to be used to manufacture Aldurazyme, any Marketing Application Approval and any
product pricing approvals where applicable. 

  

	1.46	“Regulatory Scheme” shall mean the U.S. Food, Drug and Cosmetics Act and the regulations, interpretations and guidelines promulgated thereunder by the FDA or
the regulatory scheme applicable to Aldurazyme in any country other than the United States, as such statutes, regulations, interpretations and guidelines or regulatory schemes may be amended from time to time. 

  

	1.47	“Related Agreements” shall mean the Amended and Restated Collaboration Agreement, the Members Agreement, the Fill Agreement and the Operating Agreement.

  

	1.48	“Serious Adverse Experience” shall mean any Adverse Experience associated with the use of any of Aldurazyme that results in one or more of the following outcomes:
death; a life-threatening experience; required or prolonged inpatient hospitalization; persistent or significant disability or incapacity; a congenital anomaly or defect; or the occurrence of an important medical event that may jeopardize the health
of a Patient or subject, and may require medical or surgical intervention to prevent one of the outcomes listed above. 

  

	1.49	“SOP” shall mean standard operating procedure. 

  

	1.50	“Specifications” shall mean the written specifications for Aldurazyme set forth in Exhibit A hereto; provided that such specifications shall at
all times comply with the relevant Regulatory Scheme in the country of sale and in the country of use. The Specifications with respect to any particular country shall automatically be amended upon receipt of required Regulatory Approvals from the
regulatory authorities in such country for any changes thereto. Copies of the then-current Specifications shall be maintained by both BioMarin and Genzyme and shall become a part of this Agreement as if incorporated herein. 

 

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	1.51	“Taxes” shall have the meaning set forth in Section 6.5. 

  

	1.52	“Technology” shall mean inventions, trade secrets, copyrights, know-how, data and other intellectual property of any kind (including without limitation any
proprietary biological or other materials, compounds or reagents, but not including Patent Rights). 

  

	1.53	“Technical Agreement” shall have the meaning set forth in Section 3.18. 

  

	1.54	“Territory” shall mean the world. 

  

	1.55	“Third Party” shall mean any entity other than BioMarin/Genzyme LLC, BioMarin or Genzyme and their respective Affiliates. 

  

	1.56	“Third Party Licenses” shall mean those licenses between BioMarin/Genzyme LLC and Third Parties set forth on Schedule 1.56. If after the Effective
Date any Party enters into an agreement to license or acquire rights from a Third Party with respect to subject matter to be utilized in connection with Aldurazyme in accordance with Section 3.1.4 of the Amended and Restated
Collaboration Agreement, such agreements shall also be included in the definition of Third Party Licenses for purposes of this Agreement. 

  

	2.	EXCLUSIVE RELATIONSHIP. 

  

	2.1	Non-Compete. Except as otherwise expressly provided herein or in the Amended and Restated Collaboration Agreement and the Fill Agreement, during the term of this Agreement,
neither BioMarin/Genzyme LLC, Genzyme nor BioMarin, nor any of their respective Affiliates, shall independently, or with or through a Third Party, conduct research or development activities regarding, or engage in the manufacture, marketing, sale or
distribution of, Collaboration Products in the Field and in the Territory. 

  

	3.	MANUFACTURING. 

  

	3.1	Manufacturing. Subject to the terms and conditions of this Article 3, during the term of this Agreement, BioMarin shall supply Genzyme with filled (but not
labeled) Aldurazyme, and Genzyme shall exclusively purchase its requirements from BioMarin except as provided herein. The Aldurazyme supplied to Genzyme (i) shall conform in all respects to the Specifications, (ii) will be manufactured
under cGMP using the process licensed or approved by regulatory authorities, (iii) shall have a remaining shelf life at the time of delivery to Genzyme of at least [****] of the total expiry of the product and (iv) shall not have
experienced any manufacturing process deviations that would require notice to any regulatory authorities unless, prior to delivery to Genzyme, such regulatory authorities have been notified and have approved the deviations. BioMarin shall ensure
that Genzyme has a complete and accurate copy of the Master Batch Record (as it may be modified from time to time pursuant to this Agreement). BioMarin and Genzyme shall be responsible for release testing as provided in the Technical Agreement;
provided, however, that BioMarin agrees to complete all release testing for which is responsible under the Technical Agreement and provide the testing and release documentation to Genzyme within [****] of delivery of each such Lot.
Genzyme shall prepare all Aldurazyme in final packaged form, including, without limitation, all product labeling and other package inserts and materials required by applicable regulatory agencies (collectively, “Labeling
Materials”). 

  

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	3.2	Forecasts. 

  

	 	3.2.1 	Initial Forecast and Initial Shipments. Prior to the Effective Date, Genzyme will provide to BioMarin a written initial forecast of sales and estimates of forthcoming orders
of Aldurazyme from BioMarin that will cover the fifteen (15) month period commencing on the Effective Date. On the Effective Date, BioMarin will deliver sufficient vials of Aldurazyme to Genzyme such that Genzyme will hold at least [****] of
filled Aldurazyme. Thereafter, BioMarin will promptly deliver all additional quantities of filled Aldurazyme as soon as each Lot is available for delivery in accordance with the delivery schedule specified in the initial forecast through
September 30, 2008. 

  

	 	 3.2.2 
	 Rolling Forecasts. Beginning on March 17, 2008, Genzyme will provide to BioMarin a rolling written
fifteen (15) month forecast of sales and estimates of forthcoming orders of Aldurazyme from BioMarin that will cover the period commencing at the beginning of the third (3rd) following calendar quarter. For example, assuming that this Agreement is effective as of January 1, 2008, the forecast delivered by March 17, 2008 would cover the
period of October 1, 2008 through December 31, 2009. Genzyme shall update such rolling fifteen-month forecast every calendar quarter (such that each forecast shall cover fifteen (15) months on a rolling basis), and such updates shall
be provided not later than fifteen (15) days prior to the commencement of each quarterly period during the term hereof. The first three (3) months of each such forecast (i.e. October 1, 2008 through December 31, 2008 in
the above example) shall constitute a binding commitment to order the quantity of Aldurazyme forecast for such period (the “Firm Period”). Projections for months four (4) through fifteen (15) (i.e., January 1, 2009
through December 31, 2009 in the above example) shall constitute Genzyme’s reasonable best estimates of future orders, but shall not be binding on Genzyme. To the extent that any new quarterly forecast calls for BioMarin to provide vials
of Aldurazyme in quantities that exceed the average quarterly volume sold by Genzyme for the previous [****] period by more than [****], BioMarin shall not be required to provide such increased quantity of Aldurazyme without its consent;
provided, however, that BioMarin will use its commercially reasonable and diligent efforts to supply the entire quantity of Aldurazyme ordered by Genzyme during such period. Additionally, BioMarin shall not be obligated to supply vials
of Aldurazyme in quantities that exceed [****] of Genzyme’s estimate for vials of Aldurazyme for the relevant twelve-month non-binding period (based on the lowest non-binding forecast for each respective calendar quarter); provided,
however, that BioMarin will use its commercially reasonable and diligent efforts to supply the entire quantity of Aldurazyme ordered by Genzyme during such period, provided further, that BioMarin shall be under no obligation to alter its
scheduled manufacturing of other products. 

  

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	3.3	Orders. Genzyme shall submit to BioMarin an Order with each rolling fifteen-month forecast covering the Firm Period reflected in each such forecast. Each Order shall specify
the quantity ordered, the required delivery date (provided that such delivery date is not earlier than [****] days after the date of such Order), and any special instructions or invoicing information. For example, the forecast delivered
by March 17, 2008 shall state a required delivery date no earlier than [****], 2008 for the Order covering the Firm Period (i.e., the quarterly period running from October 1, 2008 through December 31, 2008) reflected in such forecast.
With respect to the quantity ordered, Genzyme shall indicate in the Order the number of vials of Aldurazyme (specifying the number of vials of Aldurazyme meeting each different Specification) to be used for each of the following categories, based on
Genzyme’s good faith estimates at such time: (a) commercial sales, (b) compassionate use or expanded access programs, (c) Post-Marketing Studies (other than Post-Marketing Studies conducted under the Amended & Restated
Collaboration Agreement), and (d) acceptance and in-country testing, final Lot release and regulatory requirements. Each Order issued shall be governed by the terms of this Agreement. BioMarin shall accept and fill all properly placed Orders
that are consistent with the rolling forecasts and the limitations set forth in Section 3.2. BioMarin shall notify Genzyme (i) within [****] business days of the date of the Order with respect to all Orders that conform to this
Agreement of its acceptance of such Order and (ii) within [****] business days of the date of the Order with respect to all Orders that BioMarin questions, in good faith, the consistency of such Order with the rolling forecasts or the
limitations set forth in Section 3.2, whether or not it is accepting such Order. Any terms or conditions of any such Order that conflict or are inconsistent with the terms of the Agreement are hereby rejected. Genzyme agrees to use its
commercially reasonable and diligent efforts to manage the purchase and delivery cycle so that Genzyme or its Affiliates is at all times after October 1, 2008 holding at least [****] of filled inventory of Aldurazyme, with the exact amount
being held in inventory to be determined based on anticipated demand as determined by Genzyme in good faith. 

  

	3.4	 Safety Stock. Except as provided below, BioMarin agrees to use its commercially reasonable and diligent efforts to hold at least [****] inventory of
Aldurazyme at all times after October 1, 2008, with the exact amount being held in inventory to be determined based on anticipated demand as reflected in Genzyme’s rolling forecasts. In addition, in the event that there is a proposed
change in the facilities used to manufacture Aldurazyme pursuant to Section 3.10 below or a proposed modification of the Specifications or the processes used to manufacture Aldurazyme pursuant to Section 3.11, BioMarin shall
(i) hold ready for immediate transfer to Genzyme sufficient additional inventory of filled Aldurazyme (“Change Control Safety Stock”) to reasonably ensure uninterrupted supply of such Aldurazyme in each country in the Territory
in which Aldurazyme is distributed, marketed and sold until the necessary Regulatory Approvals for such change or modification are obtained in such country; and (ii) ensure that all such Change Control Safety Stock shall at all times be
manufactured and produced using the facilities, Specifications or processes approved by 

  

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the appropriate government or regulatory authorities in each country in the Territory as appropriate to enable sale of such Change Control Safety Stock in
such country until the necessary Regulatory Approvals for such change or modification are obtained in such country. 

  

	3.5	Shipping of Aldurazyme. BioMarin will deliver Aldurazyme sold to Genzyme [****] by such means of transportation as shall be reasonably determined by BioMarin from time to
time. BioMarin shall use its commercially reasonable and diligent efforts to ship Aldurazyme so that it will arrive at Genzyme’s facility on or within [****] business days of the date specified in the relevant Order. To the extent BioMarin
incurs any shipping charges, premiums for freight insurance, customs duties or other import or export fees, or other transportation costs in connection with shipments of Aldurazyme to Genzyme, BioMarin will be solely responsible for such amounts.
Included with each shipment or provided separately but concurrently with such shipment shall be the Certificate of Analysis for the drug product, Certificate(s) of Analysis for each Lot of drug substance or intermediate that went into the drug
product, and a certificate of cGMP compliance in a form reasonably agreed to by BioMarin and Genzyme. All other obligations of BioMarin relating to the documents to be provided in connection with each shipment of Aldurazyme shall be governed by the
Technical Agreement. 

  

	3.6	Compliance with Laws. BioMarin shall comply with all applicable laws, rules and regulations applicable to the manufacture, storage, handling and shipment of Aldurazyme.

  

	3.7	Direct Shipment to Customers. BioMarin shall not be required to ship Aldurazyme directly to a customer of Genzyme unless Genzyme first seeks and obtains BioMarin’s prior
written approval for such a shipment. BioMarin shall not ship Aldurazyme to any Third Party (other than bulk material to vendors for the sole purpose of filling Aldurazyme on behalf of BioMarin), without Genzyme’s prior written authorization.

  

	3.8	Risk of Loss. [****] 

  

	3.9	Testing; Rejection. 

  

	 	(a)	[****] 

  

	 	(b)	[****] 

  

	 	(c)	[****] 

  

	 	(d)	[****] 

  

	 	(e)	[****] 

  

	 	(f)	[****] 

  

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	 	(g)	Genzyme shall not be obligated under any circumstances to accept delivery of any Lot of Aldurazyme unless it meets the Specifications and Regulatory Approvals for the
relevant country in the Territory (as determined through the mechanism set forth in this Section 3.9) and the applicable warranties set forth herein. Genzyme shall be entitled to return to BioMarin any Aldurazyme which the Parties
reasonably agree cannot or should not be sold due to a change in the Specifications or Regulatory Approvals, and BioMarin shall promptly replace, at no additional expense to Genzyme, such Aldurazyme with new Aldurazyme that does conform with the
approved Specifications and Regulatory Approvals for the relevant country in the Territory. 

  

	3.10	 Suppliers. Without limiting BioMarin’s responsibility under this Agreement, BioMarin shall have the right at any time to satisfy its supply obligations to Genzyme
hereunder, either in whole or in part, through arrangements with Third Parties engaged to perform services or supply facilities or goods in connection with the manufacture, testing, and/or packaging of Aldurazyme. BioMarin shall ensure that all such
facilities comply with applicable regulations and will give Genzyme written notice at least [****] days in advance of any such arrangement to determine whether such arrangement would require changes to a Marketing Application Approval application
filed in any country and, if so, whether prior approval of any such changes is required by the regulatory authority in such country. To the extent that a change is required, after receipt of all necessary data from BioMarin, Genzyme shall, at
BioMarin’s expense, use its commercially reasonable and diligent efforts to prepare and make such filings and, if required, seek approval for such change in such countries that Genzyme has responsibility for pursuant to
Section 5.1.2(a); and BioMarin shall, at its own expense, use its commercially reasonable and diligent efforts to make such filings and, if required, seek approval for such change in the United States. BioMarin shall be required to
continue to supply drug product using materials or testing from previously approved suppliers for each country in the Territory until such Regulatory Approvals are received in such country and to clearly mark which Lots use the new suppliers or
testing laboratories at the time of shipment. 

  

	3.11	 Changes to Aldurazyme. 

  

	 	(a)	BioMarin may, at its election and in its sole discretion, modify the Specifications of or processes used to manufacture Aldurazyme at any time, subject to (i) BioMarin
generating such data, including without limitation development, comparability, stability and validation data, as may be necessary to obtain Regulatory Approvals for such changes and (ii) such Regulatory Approvals being obtained;
provided, however, that to the extent that any such modification would require clinical trials with MPS-I patients in order to obtain the necessary Regulatory Approvals for such modification, BioMarin may not implement such
modification without the prior written consent of Genzyme, which consent shall not be unreasonably withheld, delayed or conditioned. BioMarin will give Genzyme written notice of any such change at least [****] days prior to its implementation,
together with a proposed amendment to Exhibit A reflecting and identifying such change. Exhibit A shall not be amended to reflect any such change unless and until any necessary amendments to any Marketing Applications and
Regulatory Approvals to implement such changes are approved by the appropriate regulatory authorities. 

  

 -12- 

	 	(b)	[****] 

  

	 	(c)	Additionally, Genzyme may be required, as a result of negotiations with or directions from a regulatory authority of a country in the Territory, to propose changes in the
Specifications as a condition to marketing or continuing to market Aldurazyme in that country. BioMarin shall use its commercially reasonable and diligent efforts to accommodate such changes to the Specifications for such country; provided
that BioMarin may decide not accommodate any such change to the extent that it would require a change to the manufacturing process, if (i) historical data does not support such proposed Specification changes, (ii) such change would
increase BioMarin’s cost of goods sold, or (iii) such change would jeopardize BioMarin’s ability to manufacture Aldurazyme for global demand. BioMarin recognizes that any such failure to accommodate any changes required by a
regulatory authority in the Territory (or any delay in accommodating any such changes) may result in the prevention or suspension of distribution and sales of Aldurazyme in that country. 

  

	 	(d)	Subject to Sections 3.2 and 3.3, BioMarin shall continue to supply Genzyme with Aldurazyme for each country in the Territory conforming to the then-approved Specifications or
manufacturing process for such country as requested by Genzyme until such time as any change in the Specifications or manufacturing process, as applicable, is approved by the relevant regulatory authority(ies) for such country.

  

	3.12	 Responsibility for Costs. BioMarin shall be solely responsible for all costs resulting from decisions made by BioMarin pursuant to Sections 3.10 and
3.11 above, including without limitation, Genzyme’s fully burdened costs incurred in accordance with such Sections (including Genzyme’s FTE costs, filing fees, translation costs, and the costs of any changes to Labeling
Materials resulting from decisions or changes made pursuant to such Sections) unless the change is related to (a) updating filings for raw materials, reagents, replacements for obsolete equipment or other material used in the
manufacture or testing of Aldurazyme that are no longer available on commercially reasonable terms or (b) updating filings related to material used in the manufacture, release or testing of Aldurazyme based on the process and procedures
included with then current Regulatory Approvals (such as validation of quantities of reference material), in which cases Genzyme shall make such filings and secure such Regulatory Approvals outside of the United States at Genzyme’s sole expense
and BioMarin shall make such filings and secure such Regulatory Approvals in the United States at BioMarin’s sole expense. Genzyme shall provide BioMarin with quarterly, reasonably detailed invoices for all such costs, and BioMarin shall
reimburse Genzyme for such costs within forty-five (45) days after receipt thereof. Notwithstanding any of the above, (i) any changes resulting from the request or direction of the FDA, EMEA or any other regulatory authority other than as
a direct result of a change proposed or implemented by BioMarin pursuant to Section 3.10 or 3.11, (ii) those changes set forth on Schedule 3.12 and (iii) the 

  

 -13- 

	 	 
validation of a Third Party [****] pursuant to Section 3.8 of the Fill Agreement shall not be deemed to be decisions made by BioMarin pursuant to
Section 3.10 and 3.11 above and Genzyme shall bear all costs and expenses related to making all filings and seeking approval for such changes in all countries outside of the United States while BioMarin shall bear all costs and expenses
related to making all filings and seeking approval for such changes in the United States; provided, however, that with respect to the changes set forth in Schedule 3.12, priority shall be given to those changes that fall into
the categories described in clauses (a), (b) and (i) above; provided further that Genzyme’s responsibility under this Section 3.12 with respect to the cost of making regulatory filings and securing Regulatory
Approvals outside of the United States (A) for the changes set forth in Schedule 3.12 shall end upon the earlier of (x) completion of such regulatory activities or (y) such time as the costs incurred with respect to such
regulatory activities equal [****] and (B) related to the validation of a Third Party pursuant to clause (iii) above shall be for the first [****] of such costs (thereafter, BioMarin shall be responsible for the next [****], of such
costs and BioMarin and Genzyme shall share equally any such costs in excess of [****] (with BioMarin reimbursing Genzyme for BioMarin’s portion of all such costs within forty-five (45) days after receipt of reasonably detailed invoices
therefor)). 

  

	3.13	Failure to Supply. 

  

	 	(a)	In the event that technical, regulatory or other reasons or events beyond BioMarin’s reasonable control prevent BioMarin from producing Aldurazyme, BioMarin will use its
commercially reasonable and diligent efforts to secure a Third Party contract manufacturer in a timely manner. All such contract manufacturing activities will be at BioMarin’s sole cost and expense and BioMarin shall have full authority to
direct and control the contract manufacturer and Genzyme, except as provided below, shall continue to purchase Aldurazyme from BioMarin. Notwithstanding the foregoing, during the technology transfer to and validation of any Third Party contract
manufacturer, Genzyme may, at its election and expense, have technical representatives present for such activities and BioMarin will consider, but will have no obligation to implement, any suggestions or recommendations made by such representatives.

  

	 	(b)	 In the event that BioMarin is unable to secure a Third Party contract manufacturer within [****] days, then BioMarin shall offer Genzyme the opportunity to
produce Aldurazyme. If Genzyme agrees to produce Aldurazyme, then BioMarin and Genzyme shall promptly negotiate in good faith commercially reasonable and diligent terms and conditions for such arrangement; provided, however, BioMarin
and Genzyme shall negotiate in good faith for payment amounts and structures to compensate Genzyme for its out of pocket and internal expenses incurred in the technology transfer and regulatory process, cost of involvement to allow it to manufacture
Aldurazyme and variable costs associated with the actual production of Aldurazyme. If BioMarin and Genzyme enter into such arrangement, then BioMarin will promptly initiate a technology transfer process during which BioMarin and its 

  

 -14- 

	 	 
subcontractors, as appropriate, will provide Genzyme or its designee with all know-how, data and information necessary or desirable to enable Genzyme to
manufacture or have manufactured Aldurazyme for the Territory. Additionally, BioMarin will make such employees involved in the development, manufacture and release of Aldurazyme available to ensure a complete technology transfer is achieved in a
timely fashion. 

  

	 	(c)	In the event that BioMarin and Genzyme enter into an arrangement pursuant to clause (b) above and BioMarin is subsequently able to resume production of
Aldurazyme, BioMarin may elect, upon [****] days prior written notice to Genzyme to reacquire such production responsibilities from Genzyme. If BioMarin elects to reacquire production responsibilities from Genzyme, then BioMarin will promptly
reimburse Genzyme the total amount of costs incurred by Genzyme (including, without limitation any and all capital expenditures) in connection with the technology transfer process described in clause (b) above, building or acquiring
capacity and resources to manufacture Aldurazyme, filing for and obtaining all necessary Regulatory Approvals to produce Aldurazyme, and transferring such manufacturing responsibility back to BioMarin. Such amounts to be reimbursed by BioMarin to
Genzyme pursuant to the previous sentence shall be offset by the amount such costs have already been recovered by Genzyme pursuant to payments made by BioMarin pursuant to clause (b) above. BioMarin shall reimburse Genzyme for such
amounts within forty-five (45) days after receipt of reasonably detailed invoices therefor. Upon BioMarin resuming production responsibilities from Genzyme with respect to Aldurazyme, the royalty payable by Genzyme to BioMarin shall be
increased to those levels set forth in Section 6.1 for Aldurazyme produced by BioMarin. 

  

	 	(d)	BioMarin will use its commercially reasonable and diligent efforts to continue to produce Aldurazyme for supply to such countries in the Territory where Aldurazyme is used or
sold until such time as this Third Party manufacturer or Genzyme, as the case may be, has been approved in all countries in the Territory. If BioMarin is not able to do so, then Genzyme will not be held in breach of any of its obligations due to an
inability to supply Patients and commercialize Aldurazyme in such country resulting from BioMarin’s failure to supply product that meets the regulatory requirements in such country. 

  

	3.14	Certificates of Analysis. BioMarin shall perform, or cause its contract manufacturer(s) to perform, quality assurance, control tests, and release on each Lot of Aldurazyme,
to the extent specified in and in compliance with the Technical Agreement, before delivery and shall prepare, or cause its contract manufacturer(s) to prepare and deliver, a written report of the results of such tests (for purposes of Sections
3.14 through 3.17 (inclusive), such contract manufacturer(s) shall be included in the definition of the term “BioMarin”). Each test report shall set forth for each Lot delivered the items tested, specifications and results in a
Certificate of Analysis containing the types of information typical for such reports or required by the FDA or other applicable regulatory authority. BioMarin shall maintain such certificates for a period of not less than five (5) years from
the date of manufacture or for such longer period as required under applicable requirements of the FDA or other applicable regulatory authority. 

  

 -15- 

	3.15	Certificates of Manufacturing Compliance. BioMarin shall prepare, or cause to be prepared and delivered, and maintain for a period of not less than five (5) years or for
such longer period as required under applicable requirements of the FDA or other applicable regulatory authority for each Lot of Aldurazyme manufactured a certificate of manufacturing compliance containing the types of information typical for such
reports or required by the FDA or other applicable regulatory authority, which certificate will certify that the Lot of Aldurazyme was manufactured in accordance with the Specifications and the cGMP Regulations as the same may be amended from time
to time. 

  

	3.16	Regulatory Inspections; Reports. BioMarin shall advise Genzyme immediately if an authorized agent of the FDA or other regulatory authority visits any of manufacturing
facilities used to produce Aldurazyme, for an inspection with respect to the Aldurazyme, and of any written or oral inquiries by any such authority about such facilities or the procedures for the manufacture, storage or handling of Aldurazyme or the
packaging, labeling, marketing, distribution, promotion, commercialization for sale and sale of Aldurazyme. BioMarin shall furnish to Genzyme the report and any and all correspondence issued by or provided to such agency in connection with such
visit or inquiry, to the extent that such report or correspondence relates to Aldurazyme, within ten (10) business days after BioMarin’s receipt of such report or correspondence, as the case may be, and Genzyme shall have the right to
comment on any response by BioMarin to such inspecting agency. Notwithstanding the prior sentence, BioMarin shall be under no obligation to accept any comments provided by Genzyme on any response by BioMarin to such inspecting agency and all
submissions to such agency by BioMarin shall be at its sole and absolute discretion. 

  

	3.17	Access to BioMarin Facilities. Genzyme shall have the right to audit once annually each of those portions of the manufacturing, finish processing or storage facilities where
Aldurazyme is being manufactured, finished or stored, or any subcontractor who is manufacturing, finishing or storing Aldurazyme for BioMarin, at any time during regular business hours and upon reasonable advance notice to ascertain compliance with
the cGMP Regulations, as the same may be amended from time to time. Subject to the terms and conditions of Section 8.1 below, confidential information disclosed to or otherwise gathered by Genzyme during any such audit or provided by
BioMarin shall be maintained as confidential. 

  

	3.18	Technical Agreement. Within ninety (90) days after the Effective Date, the Parties shall execute and deliver a new Technical Agreement containing customary provisions
consistent with the allocation of responsibilities set forth in this Agreement. Until a new agreement is executed and delivered, the Parties shall operate under the existing Technical Agreement. 

  

	3.19	 Hollister-Stier Agreement. BioMarin shall have sole responsibility for managing the relationship with Hollister-Stier Laboratories, LLC pursuant to the
Supply Agreement 

  

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by and between Hollister-Stier Laboratories, LLC and BioMarin/Genzyme LLC dated as of November 6, 2006, as it may be amended from time to time (as
amended, the “Hollister-Stier Agreement”) and shall be solely responsible for all amounts payable to Hollister-Stier and liabilities arising under such Agreement. As soon as practicable after the Effective Date, BioMarin shall use
its commercially reasonable and diligent efforts to cause the Hollister-Stier Agreement to be assigned from BioMarin/Genzyme LLC to BioMarin. 

  

	4.	MARKETING AND SALES 

  

	4.1	Exclusive Right to Market, Sell and Distribute. Subject to the terms and conditions of this Article 4, Genzyme shall have the exclusive right to distribute,
market and sell Aldurazyme throughout the world, including without limitation all pricing and reimbursement activities. Genzyme agrees (by itself or through its Affiliates or Third Parties selected by Genzyme at its election and in its sole
discretion) to use its commercially reasonable and diligent efforts to establish Aldurazyme in the markets, fulfill market demand and meet the marketing and distribution goals for Aldurazyme determined by Genzyme in its good faith, subject to the
receipt of necessary Regulatory Approvals and subject to BioMarin’s performance of its obligations under Article 3. 

  

	4.2	Compliance With Laws. Genzyme shall comply with applicable laws, rules and regulations in each country in which Aldurazyme is licensed, marketed or sold relating to the
marketing, sale and distribution of Aldurazyme. Without limiting the foregoing, Genzyme shall comply with all U.S. federal and state laws pertaining to price reporting and, except to the extent such compliance is BioMarin’s responsibility as
the holder of the BLA and subject to BioMarin’s compliance with its obligations under Section 5.1.2, marketing disclosure. 

  

	4.3	Marketing and Distribution Expenses. 

  

	 	4.3.1	Genzyme shall be solely responsible for payment of all Marketing Costs. 

  

	 	4.3.2	Beginning on January 1, 2008 and continuing so long as this Agreement remains in force, Genzyme agrees to [****]. 

  

	 	4.3.3	Genzyme shall keep complete and accurate books and records of all Marketing Costs relating to Aldurazyme. BioMarin shall have the right, at BioMarin’s expense, through a
certified independent public accountant, to have reasonable access upon reasonable advance notice and during normal business hours to such books and records for the purpose of verifying the accuracy of such Marketing Costs; provided,
however, that BioMarin shall not have the right to perform any audit work pursuant to this Section 4.3.3 unless and until [****]. BioMarin and Genzyme shall agree in good faith upon reasonable procedures before BioMarin’s
auditors begin the audit work pursuant to this Section 4.3.3. The report issued by such auditor shall be final, binding and conclusive upon each of BioMarin and Genzyme. Such verifications may be conducted not more than once in each
calendar year. The books and records for any particular calendar quarter may not be examined under this Section 4.4.3 more than once. 

  

 -17- 

	 	4.3.4	BioMarin agrees that all information subject to review under this Section 4.3 (other than information directly related to Aldurazyme) is Genzyme’s
confidential information and that it shall cause its accounting firm to retain all such information in confidence (even as to BioMarin) subject to the confidentiality restrictions of Article 8 hereof. Notwithstanding the prior sentence,
Genzyme acknowledges and agrees that such accounting firm’s obligation to retain all such information in confidence from BioMarin shall not apply in the event that BioMarin and Genzyme are involved in a dispute under Section 13.8
related to this Section 4.3 and such information is necessary for BioMarin’s use in connection with such dispute; provided, however, that BioMarin shall only use such information in connection with such dispute and
such information shall remain subject to the confidentiality restrictions of Article 8. 

  

	4.4	Medical Information. Genzyme shall have sole responsibility for responding to all requests for medical information regarding Aldurazyme. If BioMarin receives any such
requests, it shall promptly direct such requests to Genzyme. Notwithstanding the foregoing, BioMarin may maintain a website or other informational resources where it may publish general medical information relating to MPS I, so long as such website
complies in all respects with applicable laws, rules and regulations. This Section 4.4 shall not prohibit BioMarin from publishing non-medical information in its investor relations communications, including on BioMarin’s website,
provided that such publication is consistent with the terms and conditions of Article 8. 

  

	4.5	Pharmacovigilance. 

  

	 	4.5.1	Genzyme and its Affiliates will be responsible for continuing all pharmacovigilance activities with respect to Aldurazyme, including without limitation maintaining Aldurazyme
Core Safety Information. BioMarin shall have the right to audit, at any time during regular business hours and upon reasonable advance notice to audit such pharmacovigiliance activities with respect to Aldurazyme. Such audit rights shall include the
right to review, inspect and/or audit any systems or processes involved or utilized in such pharmacovigilance activities as well as the right to review, inspect or audit all information or data necessary to be filed with any regulatory or
governmental authority. 

  

	 	4.5.2	 In the event that the Aldurazyme Labeling Materials must be updated per new Core Safety Information, after receipt of all necessary information and data from
Genzyme, BioMarin agrees to use its commercially reasonable and diligent efforts make all necessary regulatory filings to FDA and obtain approvals in order to ensure the content of Aldurazyme Labeling Materials in the United States is in-line with
the Core Safety Information. Genzyme shall be responsible for using its commercially reasonable and diligent efforts to 

  

 -18- 

	 	 
make such filings and obtain such approvals outside of the United States. Each of BioMarin and Genzyme shall be given the opportunity to attend and
participate in any meetings or discussions that the other Party may have with any regulatory authorities regarding any submissions or approvals and shall be provided with an opportunity to review and comment upon any correspondence, submission or
communication that the other Party may have with any such regulatory authority prior to such correspondence, submission or communication being made or submitted to such regulatory authority. 

  

	 	4.5.3	As of the Effective Date and throughout the term of this Agreement, Genzyme shall maintain one or more written SOP(s) to collect Adverse Experience information. The content
of any SOP(s) shall include, but not be limited to, specific instructions regarding the type of Adverse Experience information collected, the time frame for collection, provisions for secure transmissions, and inter-company processes to be used for
notification of said information. In addition to the foregoing, the Parties shall update the current guidelines and procedures to govern receipt, processing, assessment and submission of Aldurazyme Adverse Experience and Serious Adverse Experience
reports to the FDA, the European Commission and other regulatory authorities within sixty (60) days after the Effective Date. Procedures described therein shall ensure compliance with all applicable laws and regulations. The Parties acknowledge
that Genzyme’s SOPs as they exist as of the Effective Date satisfy the requirements of this Section 5.2.1 for reports to the FDA. 

  

	 	4.5.4	BioMarin shall have responsibility for filing all 15-day alert reports, 15-day alert follow-up reports and periodic adverse event reports to the FDA per 21 CFR §600.80.
Genzyme or its designee shall have responsibility for filing such reports outside of United States, including EMEA and other regulatory authorities according to the local regulations. 

  

	4.6	Complaints. Within sixty (60) days after the Effective Date, Genzyme and BioMarin shall develop a written protocol for exchanging information on product complaints
concerning Aldurazyme of which either Party becomes aware, so as to allow each of Genzyme and BioMarin to comply with its regulatory obligations. At a minimum, such protocol shall provide that the Party receiving such complaint or information shall
promptly, but in any event not later than twenty-four (24) hours after receipt, advise the other Party (excluding, for purposes of this Section, BioMarin/Genzyme LLC) in writing of the details of such complaint or information, after learning of
such information. Promptly thereafter, Genzyme shall report such incident to the appropriate regulatory authorities in the countries in which Aldurazyme is being commercialized, in accordance with the appropriate laws and regulations of the relevant
countries and authorities; provided, however, that with respect to reports that are to be made with the FDA, BioMarin shall make such report using the information prepared by Genzyme. Such reporting activities shall be coordinated
between Genzyme and BioMarin where time and law permit. The Party initially receiving the complaint or information shall provide the other Party with all follow-up information related to such incident generated by the receiving Party or required by
the other Party to satisfy its regulatory filing obligations. 

  

 -19- 

	4.7	Registries; Post-Marketing Studies. 

  

	 	4.7.1	Registries. Genzyme shall have the responsibility for conducting the MPS-I registry program as it exists on the Effective Date at Genzyme’s sole expense. In the event
that the scope of the registry program is expanded to implement any Post-Marketing Studies required by regulatory authorities to be conducted through or as part of the registry program, such costs above the general cost of maintaining the registry
program as currently conducted will be funded by BioMarin/Genzyme LLC pursuant to the terms of the Amended and Restated Collaboration Agreement. 

  

	 	4.7.2	Post-Marketing Studies. With respect to any Post Marketing Studies required by a jurisdiction where Aldurazyme does not have Regulatory Approval as of the Effective Date,
Genzyme shall, at its sole expense and in its sole discretion, have the responsibility for conducting any such Post-Marketing Studies that are not funded by BioMarin/Genzyme LLC pursuant to the terms of the Amended and Restated Collaboration
Agreement. 

  

	4.8	Use of Trademarks. Aldurazyme shall be sold under trademarks determined by Genzyme and owned by or licensed to Genzyme or BioMarin/Genzyme LLC. 

  

	4.9	Records. Genzyme shall maintain complete and accurate records of all movements and transactions involving Aldurazyme by an appropriate identifier and by customer so that all
such movements and transactions can be traced quickly and effectively. Genzyme shall maintain such records for a minimum period of five (5) years after the relevant movement or transaction contained in such record. The records maintained by
Genzyme pursuant to this Section 4.9 shall be Genzyme’s confidential information under Article 8 and subject to BioMarin’s audit rights under Section 6.5. 

  

	4.10	Regulatory Inspections; Reports. Genzyme shall advise BioMarin immediately of any visit by an authorized agent of a regulatory authority to, or written or oral inquiries by
any such authority about, any Genzyme facilities (or facilities of any subcontractors engaged by Genzyme) used to package, label or store Aldurazyme or procedures for the filling, manufacture, storage, or handling of Aldurazyme, or the packaging,
labeling, marketing, distribution, promotion, commercialization for sale and sale of Aldurazyme. Genzyme shall furnish to BioMarin the report and any and all correspondence issued by or provided to such authority in connection with such visit or
inquiry to the extent such report or correspondence relates to Aldurazyme, within ten (10) business days after Genzyme’s receipt of such report or correspondence, as the case may be, and BioMarin shall have the right to comment on any
response by Genzyme to such inspecting agency. Notwithstanding the prior sentence, Genzyme shall be under no obligation to accept any comments provided by BioMarin on any response by Genzyme to such inspecting agency and all submissions to such
agency by Genzyme shall be at its sole and absolute discretion. 

  

 -20- 

	4.11	Access to Genzyme Facilities. BioMarin shall have the right to audit once annually each of those portions of the distribution or storage facilities of Genzyme or its
Affiliates where Aldurazyme is being stored, sold or distributed, or any subcontractor or other Third Party who is storing, selling or distributing Aldurazyme for Genzyme, at any time during regular business hours and upon reasonable advance notice
to ascertain compliance with cGMP Regulations, as the same may be amended from time to time. Such audit rights shall include the right to review, inspect and/or audit any systems or processes involved or utilized in the storage, selling or
distribution of Aldurazyme as well as the right to review, inspect or audit all information or data necessary to be filed with any regulatory authority (including the FDA) or related to the BLA. Genzyme shall furnish to BioMarin any report or
correspondence issued by or provided to any governmental authority in connection with any governmental visit or inquiry regarding the facilities at which Genzyme, its Affiliates or any Third Party stores, sells or distributes Aldurazyme or the
procedures related thereto. Subject to the terms and conditions of Section 8.1 below, confidential information disclosed to or otherwise gathered by BioMarin during any such audit or provided by Genzyme shall be maintained as
confidential. 

  

	4.12	Third Party Licenses. Genzyme hereby agrees to pay any and all royalties, sublicense fees and other costs owed by BioMarin/Genzyme LLC with respect to the manufacture or sale
of Aldurazyme to any Third Party pursuant to the terms and conditions of the Third Party Licenses. Genzyme further agrees to fulfill any and all of the reporting obligations BioMarin/Genzyme LLC may have to any Third Party related to Aldurazyme by
virtue of the Third Party Licenses. 

  

	4.13	Suspension of Distribution. Each Party shall notify the other promptly if it becomes aware of a problem with the quality of Aldurazyme distributed in the Territory, or a
directive from the FDA, the EMEA or any other applicable regulatory authority related to Aldurazyme distributed in the Territory. Upon receipt of any such notice from BioMarin, Genzyme may suspend sales and distribution of Aldurazyme in the
Territory, at its reasonable discretion. After any such suspension, BioMarin and Genzyme shall consult in good faith to determine whether and when to resume sales and distribution of Aldurazyme; provided, however, that no such
suspension shall be deemed to be a default by Genzyme hereunder except to the extent that Genzyme does not resume sales and distribution of Aldurazyme within fifteen (15) business days after the Parties agree that such quality problem has been
resolved. 

  

	5.	REGULATORY MATTERS. 

  

	5.1	Marketing and Regulatory Approvals. 

  

	 	5.1.1	 Marketing Applications. Genzyme shall be responsible, in its sole discretion and at its sole expense, for filing Marketing Applications for Aldurazyme in
each country, other than as expressly provided in Sections 3.10, 3.11, 3.12 and 5.1.2, in which Aldurazyme is licensed, marketed or sold, up to and including Marketing Application Approval, and for thereafter maintaining such Marketing
Application Approvals. In connection with Genzyme’s filing 

  

 -21- 

	 	 
of Marketing Applications, BioMarin shall provide Genzyme with a copy, in English, of all necessary supporting information and data when required, to prepare
appropriate regulatory submissions affecting approval and maintenance of market or of product pricing. Nothing in this Article 5 shall be deemed to require Genzyme to file Marketing Applications and obtain Marketing Application Approvals in
each country in the Territory; Genzyme shall determine in good faith where and when to file and maintain such applications and approvals in a manner consistent with its obligation to use its commercially reasonable and diligent efforts to
commercialize Aldurazyme. 

  

	 	5.1.2	Regulatory Approvals. 

  

	 	(a)	Genzyme shall be responsible at its sole expense: (i) for the filing of all reports, documents or other information regarding Aldurazyme required by the EMEA or any
other governmental agency or body outside of the United States having jurisdiction related to Aldurazyme and for securing all necessary Regulatory Approvals and for ensuring that Aldurazyme remains in good standing with all such governmental
agencies or bodies and all other applicable jurisdictions (other than the United States) for as long as Genzyme determines to market and sell Aldurazyme in such jurisdictions; and (ii) for obtaining and maintaining all permits and licenses
necessary to enable Genzyme to distribute Aldurazyme under this Agreement. Genzyme agrees to provide BioMarin with copies of all filings, reports, documents or other information provided by it to any regulatory authority outside of the United States
related to Aldurazyme within thirty (30) days of making such filing with such regulatory authority. 

  

	 	(b)	BioMarin shall be responsible at its sole expense: (i) for the filing of all reports, documents or other information regarding Aldurazyme required by the FDA or any
other governmental agency in the United States having jurisdiction related to Aldurazyme and for securing all necessary Regulatory Approvals and for ensuring that Aldurazyme remains in good standing with the FDA and such other governmental agencies;
and (ii) for obtaining and maintaining all permits and licenses to enable BioMarin to produce Aldurazyme for the global market under this Agreement. BioMarin agrees to provide Genzyme with copies of all filings, reports, documents or other
information provided by it to any regulatory authority in the United States related to Aldurazyme within thirty (30) days of making such filing with such regulatory authority. 

  

	 	(c)	 All regulatory filings for Aldurazyme in the United States will be filed by and in the name of BioMarin. BioMarin agrees that: (i) it shall hold the
licenses issued in respect of such Regulatory Approval filings, and maintain control over the manufacturing facilities, equipment and personnel, to the extent required by the Regulatory Scheme; (ii) it shall maintain compliance with the
applicable Regulatory Scheme; (iii) each of the Parties shall have an irrevocable right of access and reference to such Regulatory Approval filings, licenses and facilities in connection with its exercise of its rights and performance of its
obligations under this Agreement, the Amended and Restated Collaboration Agreement and 

  

 -22- 

	 	 
the Fill Agreement; and (iv) it shall comply with the provisions of Article 12 hereof with respect to the ownership and/or disposition of
such Regulatory Approvals in the event this Agreement is terminated and that it will provide the level of cooperation described in this Agreement in connection therewith. 

  

	 	(d)	All regulatory filings for Aldurazyme outside of the United States will be filed by and in the name of Genzyme or its designee. Genzyme agrees that: (i) it shall hold
the licenses issued in respect of such Regulatory Approval filings, maintain control over the distribution facilities, equipment and personnel, and engage in pharmacovigilence to the extent required by the Regulatory Scheme; (ii) it shall
maintain compliance with the applicable Regulatory Scheme; (iii) each of the Parties shall have an irrevocable right of access and reference to such Regulatory Approval filings, licenses and facilities in connection with its exercise of its
rights and performance of its obligations under this Agreement, the Amended and Restated Collaboration Agreement and the Fill Agreement; and (iv) it shall comply with the provisions of Article 12 hereof with respect to the ownership
and/or disposition of such Regulatory Approvals in the event this Agreement is terminated and that it will provide the level of cooperation described in this Agreement in connection therewith. 

  

	 	(e)	No Party may assign or otherwise transfer a Regulatory Approval for Aldurazyme without the prior written consent of the other Parties; provided, however, that
either BioMarin or Genzyme may, without such consent, assign such Regulatory Approvals (i) in connection with a corporate reorganization, to any member of an affiliated group, all or substantially all of the equity interest of which is owned
and controlled by such Party or its direct or indirect parent corporation or (ii) in connection with a merger, consolidation or sale of substantially all of such Party’s assets to an unrelated Third Party; provided, however,
that such Regulatory Approvals shall not be transferred separate from this Agreement and the Related Agreements and all or substantially all of such Party’s other business assets, including without limitation those business assets that are the
subject of this Agreement. Any purported assignment in violation of this Section 5.1.2(e) shall be void. 

  

	5.2	Recalls. 

  

	 	5.2.1	 BioMarin may, in its sole discretion, determine whether or when to initiate any recalls of Aldurazyme in the United States and Genzyme may, in its sole
discretion, determine whether or when to initiate any recalls of Aldurazyme anywhere else in the world. As of the Effective Date and throughout the term of this Agreement, BioMarin and Genzyme shall prepare and maintain a written SOP, to handle any
recalls of Aldurazyme. Such SOP shall include, without limitation, prior notice to and consultation with the other Party of any recall; provided, however, that in no event shall such consultation be deemed to limit or supersede the
Parties’ ability to make recall decisions in their sole discretion pursuant to the first sentence hereof. Genzyme shall transmit recall decision information to BioMarin to Vice President, 

  

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Regulatory Affairs, by phone at (415) 506-6708 or facsimile at (415) 506-6306. BioMarin shall transmit recall decision information to Genzyme to
Regulatory Affairs/QA, attention: Vice President, Regulatory Affairs and Corporate Quality Compliance, by phone at (508) 661-6172 or facsimile at (508) 661-8851. In the event that (i) any regulatory authority or other governmental
agency or authority issues a request or directive or orders that Aldurazyme be recalled or retrieved, (ii) a court of competent jurisdiction orders that Aldurazyme be recalled or retrieved or (iii) a Party determines that Aldurazyme should
be recalled, retrieved or a “Dear Doctor” letter is required relating to restrictions on use of Aldurazyme in a country for which it is responsible, the Party responsible for the relevant country pursuant to the first sentence hereof shall
conduct such activity and the Parties shall take all appropriate corrective actions and shall execute the steps detailed in the SOP. To the extent practicable, the Parties shall coordinate the notices of any such recall to be delivered to regulatory
authorities (including the timing thereof). Genzyme and BioMarin shall cooperate fully with one another in conducting such action. Genzyme shall destroy units of Aldurazyme lawfully recalled only upon BioMarin’s (or any regulatory
authority’s) written instruction to destroy such units of Aldurazyme, and only then in accordance with BioMarin’s procedures and instructions. Otherwise, Genzyme may return the recalled units of Aldurazyme to BioMarin in accordance with
BioMarin’s instructions within thirty (30) days after completion of the action. In the event that either BioMarin or Genzyme becomes aware of circumstances that may necessitate a recall of Aldurazyme in any country for which it is not
responsible pursuant to this Section 5.2, it will promptly notify the other Party of such circumstances. 

  

	 	5.2.2	In the event a recall results from the manufacture of Aldurazyme (including without limitation changes made pursuant Sections 3.11 and 3.12 hereof) or BioMarin’s
negligence or willful misconduct, BioMarin shall be responsible for the expenses thereof. In the event a recall results from Genzyme’s filling, packaging, labeling or storage of Aldurazyme, or Genzyme’s negligence or willful misconduct
(including without limitation any negligence or willful misconduct resulting in adulteration of Aldurazyme), Genzyme shall be responsible for the expenses thereof. Otherwise, the Parties shall share equally the expenses of the action. For purposes
of this Agreement, the expenses of the action shall be the expenses of notification and return or destruction (if such destruction is authorized by BioMarin or the applicable regulatory authority) of units of the recalled Aldurazyme, the cost of
replacement of the recalled Aldurazyme, and any costs directly associated with the distribution of replacement Aldurazyme. For the avoidance of doubt, amounts payable under Article 6 hereof shall only be payable on Aldurazyme used to replace
the recalled Aldurazyme (not on the returned Aldurazyme itself). 

  

 -24- 

	6.	PAYMENTS. 

  

	6.1	Transfer Royalties. 

  

	 	(a)	For the Aldurazyme supplied by BioMarin to Genzyme pursuant to Article 3, Genzyme shall pay to BioMarin the following royalties based upon the Annual Net Sales of
Aldurazyme: 

  

				
	 Annual Net Sales

	  	Royalty Payment %

	 
	 of between $0 and $130,000,000
	  	39.5	%
	 of between $130,000,001 and $140,000,000
	  	40	%
	 of between $140,000,001 and $155,000,000
	  	42	%
	 of between $155,000,001 and $170,000,000
	  	44	%
	 of between $170,000,001 and $185,000,000
	  	46	%
	 of between $185,000,001 and $200,000,000
	  	48	%
	 of $200,000,001 and above
	  	50	%

  
 As used herein,
“Annual Net Sales” shall mean total aggregate Net Sales of all Aldurazyme sold in the particular calendar year. 
  

	 	(b)	It is expressly understood that the royalty payment to be made by Genzyme to BioMarin pursuant to Section 6.1(a) shall be based on a tiered structure. For
instance, if total Annual Net Sales are $145,000,000, the royalty payable from Genzyme to BioMarin pursuant to the methodology outlined in Section 6.1(a) would be equal to $57,450,000, calculated as follows: ($130,000,000 x 39.5%) +
($10,000,000 x 40%) + ($5,000,000 x 42%). 

  

	 	(c)	In the event that the [****] Aldurazyme in any country in which Aldurazyme has received all necessary Regulatory Approvals and, if applicable, pricing approvals as of the
Effective Date (“Existing Markets”) is [****] over any calendar quarter, then [****]. The term [****] for purposes of this clause (c) shall mean a [****] of Aldurazyme. 

  

	 	(d)	Notwithstanding the foregoing, 

  

 -25- 

 (i) BioMarin agrees to supply Genzyme’s requirements of Aldurazyme for compassionate use and
expanded access programs free of charge, concurrent with delivery of commercial vials, in an amount equal to [****]. Thereafter, BioMarin agrees to supply additional vials to Genzyme during the relevant calendar year for such compassionate use and
expanded access programs for [****] until it has provided an additional [****] of Aldurazyme. Thereafter, any additional vials required by Genzyme in the relevant calendar year for such compassionate use and expanded access programs shall be
provided by BioMarin at [****]. The term “vial” for purposes of this clause (d)(i) shall mean a 2.9 mg vial of Aldurazyme; and 
  
 (ii) BioMarin agrees that Genzyme may use up to [****] vials per Lot of Aldurazyme delivered to Genzyme to supply Genzyme’s requirements of
Aldurazyme for appropriate acceptance testing and required in-country release testing and final Lot testing free of charge. Genzyme shall account for any Aldurazyme so used in the quarterly report described in Section 6.4. 
  

	6.2	Quarterly Payments. Genzyme shall pay all amounts owed to BioMarin for Aldurazyme pursuant to Section 6.1 on a quarterly basis within forty-five (45) days
after the end of each calendar quarter. In computing the royalty payable for any quarter, the Parties shall take into account the aggregate Net Sales to date from the beginning of the relevant calendar year. 

  

	6.3	Monthly Reports. Within [****] business days after the end of each calendar month, Genzyme shall provide BioMarin with a preliminary report of Net Sales of Aldurazyme for the
immediately proceeding month (a “Flash Report”). As soon as practicable, but in no event later than [****] business days after the end of each calendar month, Genzyme shall provide BioMarin with a final report of Net Sales of
Aldurazyme for the immediately proceeding month. Such monthly reports shall not include inventory data. 

  

	6.4	Quarterly Reports. Within forty-five (45) business days after the end of each calendar quarter, Genzyme shall provide BioMarin with a true accounting of all payment
obligations, if any, owed in accordance with this Article 6, together with a statement setting out all details necessary to calculate the amounts actually due hereunder with respect to Net Sales made in that calendar quarter, including vials
of Aldurazyme sold on a country-by-country basis, gross sales of Aldurazyme in that calendar quarter including vials of Aldurazyme sold on a country-by-country basis, Net Sales in that calendar quarter on a country-by-country basis, all relevant
deductions, and all relevant exchange rate conversions. Any additional payments owed by Genzyme to BioMarin shall accompany such statement. 

  

	6.5	Sales Records; Audit. 

  

	 	6.5.1	 Genzyme shall keep complete and accurate books and records of all Net Sales of Aldurazyme on a country-by-country basis. BioMarin shall have the right, at
BioMarin’s expense, through a certified independent public accountant, to have reasonable access upon reasonable advance notice and during normal business hours to such books and records for the sole purpose 

  

 -26- 

	 	 
of verifying the accuracy of the royalty report and payments under this Article 6 in respect of any calendar quarter ending not more than thirty-six
(36) months prior to the date of such notice. BioMarin and Genzyme shall agree in good faith upon reasonable procedures before BioMarin’s auditors begin the audit work pursuant to this Section 6.5.1. Such verifications may be
conducted not more than once in each calendar year during the term of this Agreement and once during the three (3) year period following the expiration or termination of this Agreement. The records and royalty reports for any particular
calendar quarter may not be examined under this Section 6.5.1 more than once. 

  

	 	6.5.2	In the event such audit concludes that adjustments should be made in BioMarin’s favor, then any appropriate payments (plus accrued interest as determined by
Section 6.8) shall be paid by Genzyme within [****] days after the date Genzyme receives BioMarin’s accounting firm’s written report so concluding, unless Genzyme shall have a good faith dispute as to the conclusions set forth
in such written report in which case Genzyme shall provide written notice to BioMarin within such [****] day period of the nature of its disagreement with such written report. The Parties shall thereafter, for a period of sixty (60) days,
attempt in good faith to resolve such dispute and if they are unable to do so then the matter will be submitted to dispute resolution in accordance with Section 13.8 hereof. Any overpayments by Genzyme will be promptly refunded by
BioMarin to Genzyme with interest calculated as provided in Section 6.8 from the date such overpayment was made. If any such examination reveals an underpayment to BioMarin in excess of [****] for the examined period, then Genzyme shall
promptly reimburse BioMarin for the costs of such examination within forty-five (45) days after receipt of a reasonably detailed invoice therefor unless Genzyme is disputing such conclusion as provided above. 

  

	 	6.5.3	BioMarin agrees that all information subject to review under this Section 6.5 (other than information directly related to Aldurazyme) is Genzyme’s
confidential information and that it shall cause its accounting firm to retain all such information in confidence (even as to BioMarin) subject to the confidentiality restrictions of Article 8 hereof. Notwithstanding the prior sentence,
Genzyme acknowledges and agrees that such accounting firm’s obligation to retain all such information in confidence from BioMarin shall not apply in the event that BioMarin and Genzyme are involved in a dispute under Section 13.8
related to this Section 6.5 and such information is necessary for BioMarin’s use in connection with such dispute; provided, however, that BioMarin shall only use such information in connection with such dispute and
such information shall remain subject to the confidentiality restrictions of Article 8. 

  

	6.6	 Taxes. Any taxes, levies or similar governmental charges, now in force or enacted in the future, however designated (“Taxes”) including
related penalties and interest, imposed by 

  

 -27- 

	 	 
any governmental authority on or measured or incurred by the sale or shipment of Aldurazyme by BioMarin to Genzyme or the sale of Aldurazyme by Genzyme shall
be paid by Genzyme. Notwithstanding anything to the contrary herein, each Party shall be solely responsible with respect to its own income Taxes. 

  

	6.7	Payment for Unsold Aldurazyme. [****] 

  

	6.8	Payment Terms. All sums due under this Agreement shall be payable in United States dollars. Monetary conversion from the currency of a foreign country in which Aldurazyme is
sold into United States currency shall be calculated in accordance with GAAP using the average of the relevant daily exchange rates for each month of the calendar quarter based on rates published by Bloomberg. All payments to be made under this
Agreement shall be paid by check or wire transfer as selected by paying Party, and shall be delivered to such address or wired to such account or accounts as the Party owned such payment shall designate from time to time in writing to the paying
Party. Any such payment shall be deemed to have been paid when received with respect to check payments or when recorded in the proper account with respect to wire transfer payments. 

  

	6.9	Late Fees; Collection Costs. If any amounts due under this Agreement are not made when due, the Party from whom such payment was due will be charged a late charge of
[****] per month determined on a daily basis from the date due until the date paid. Payment of such late charges alone (i.e., without the late payment itself) will not excuse or cure the breach or default for late payment. If either BioMarin or
Genzyme retains a collection agency, attorney or other person or entity to collect overdue payments, all collection costs, including but not limited to reasonable attorneys’ fees and expenses, will be paid by the Party from whom such payment
was due. 

  

	6.10	No Deductions. No part of any amount payable by a Party may be reduced due to any counterclaim, set-off, adjustment or other right that such Party may have against another
Party, except with respect to Aldurazyme that is returned to BioMarin in accordance with Section 3.9 or 5.2. 

  

	7.	RESEARCH AND DEVELOPMENT 

  

	7.1	Independent Research and Development Activities. Except as otherwise provided herein or in the Amended and Restated Collaboration Agreement, to the extent that the Steering
Committee (as defined in the Amended and Restated Collaboration Agreement) does not exercise the right of first refusal granted to BioMarin/Genzyme LLC under Section 3.1.5 or Article 5 of the Amended and Restated Collaboration Agreement,
each of BioMarin and Genzyme shall have the right to independently conduct, at its own expense, any research or development of Aldurazyme, including without limitation Post-Marketing Studies and through investigator-initiated studies.

  

	7.2	Access to Data. Each Party shall provide the other Party will full access to the data generated in any research and development activities conducted with Aldurazyme, and the
other Party shall be free to use such information to perform its obligations under this Agreement or any Related Agreement. 

  

 -28- 

	8.	CONFIDENTIALITY. 

  

	8.1	Nondisclosure Obligations. Except as otherwise provided in this Article 8, during the term of this Agreement and for a period of five (5) years
thereafter, the Parties shall maintain in confidence and use only for purposes specifically authorized under this Agreement any information furnished to it by the other Party hereto pursuant to this Agreement which if disclosed in tangible form is
marked “Confidential” or with other similar designation to indicate its confidential or proprietary nature or if disclosed orally or by inspection is indicated orally to be confidential or proprietary by the Party disclosing such
information at the time of such disclosure and is confirmed in writing as confidential or proprietary by the disclosing Party (describing in reasonable detail the information to be treated as confidential) within a reasonable time after such
disclosure (collectively, “Information”). 

  
 To the extent it is reasonably necessary or appropriate to fulfill its obligations or exercise its rights under this Agreement, the Amended and Restated Collaboration Agreement or the Fill Agreement, a Party may
disclose Information of the other Party it is otherwise obligated under this Section 8.1 not to disclose to its Affiliates, permitted sublicensees, consultants, outside contractors and clinical investigators, on a need-to-know basis and
on the condition that such entities or persons agree to keep the Information confidential for the same time periods and to substantially the same extent as such Party is required to keep such Information confidential; and a Party or its permitted
sublicensees may disclose such Information to government or other regulatory authorities to the extent that such disclosure is reasonably necessary to obtain patents or authorizations to conduct clinical trials or to file and maintain Regulatory
Approvals with and to market commercially Aldurazyme. The obligation not to disclose Information shall not apply to any part of such Information that: (i) is or becomes patented, published or otherwise becomes publicly known other than by acts
of the Party obligated not to disclose such Information or its Affiliates or sublicensees in contravention of this Agreement; (ii) can be shown by written documents to have been disclosed to the receiving Party or its Affiliates or sublicensees
by a Third Party, provided that such Information was not obtained by such Third Party directly or indirectly from the disclosing Party under this Agreement; (iii) prior to disclosure under this Agreement was already in the possession of
the receiving Party or its Affiliates or sublicensees, provided that such Information was not obtained directly or indirectly from the disclosing Party under this Agreement; (iv) can be shown by written documents to have been
independently developed by the receiving Party or its Affiliates without breach of any of the provisions of this Agreement; or (v) is required to be disclosed by the receiving Party to comply with applicable laws or regulations, or with a court
or administrative order, provided that the receiving Party notifies the disclosing Party in writing prior to any such disclosure and agrees to use reasonable efforts to secure confidential treatment thereof prior to its disclosure (whether by
protective order or otherwise). 
  

 -29- 

	8.2	Terms of this Agreement; Press Releases. The Parties agree to seek confidential treatment for any filing of this Agreement with the Securities and Exchange Commission and
shall agree upon the content of the request for confidential treatment made by each Party in respect of such filing. Except as permitted by the foregoing provisions or as otherwise required by law, each of the Parties agrees not to disclose any
terms or conditions of this Agreement to any Third Party without the prior consent of the other Parties; provided that each of BioMarin and Genzyme shall be entitled to disclose the terms of this Agreement without such consent to its advisors
and potential investors or other financing sources on the condition that such entities or persons agree to keep such terms confidential for the same time periods and to the same extent as such Party is required to keep such terms confidential. The
Parties agree that all press releases related to Aldurazyme shall be issued jointly by BioMarin and Genzyme and that the Party preparing any such press release shall provide the other Party with a draft thereof reasonably in advance of disclosure so
as to permit the other Party to review and comment on such press release. Notwithstanding the foregoing, the Parties shall agree upon a press release to announce the execution of this Agreement, together with a corresponding Question &
Answer outline for use in responding to inquiries about the Agreement; thereafter, BioMarin and Genzyme may each disclose to Third Parties the information contained in such press release and Question & Answer outline without the need for
further approval by the other. 

  

	9.	REPRESENTATIONS AND WARRANTIES. 

  

	9.1	General Representations and Warranties. Each Party hereby represents and warrants: 

  

	 	9.1.1	Duly Organized. It is a corporation (or limited liability company as the case may be) duly organized, validly existing and in good standing under the laws of the jurisdiction
of its incorporation (or organization as the case may be), is qualified to do business and is in good standing as a foreign corporation (or limited liability company as the case may be) in each jurisdiction in which the conduct of its business or
the ownership of its properties requires such qualification and failure to have such would prevent it from performing its obligations under this Agreement and has all requisite corporate power (or limited liability power as the case may be) and
authority to conduct its business as now being conducted, to own, lease and operate its properties and to execute, deliver and perform this Agreement. 

  

	 	9.1.2	Authorization. It is duly authorized to execute and deliver this Agreement and to perform its obligations hereunder. The person executing this Agreement on its behalf has
been duly authorized to do so by all requisite corporate action. 

  

	 	9.1.3	No Third Party Approval. No authorization, consent, approval, license, exemption of, or filing or registration with, any court or governmental authority or regulatory body
(other than health regulatory authorities) is required for the due execution, delivery or performance by it of this Agreement, except as provided herein. 

  

 -30- 

	 	9.1.4	Enforceable Agreement. This Agreement has been duly and validly executed and delivered, and assuming it is a legally enforceable agreement of the other Parties hereto,
constitutes the legal, valid and binding obligation of such Party, enforceable against it in accordance with its terms, subject to the effect of any applicable bankruptcy, insolvency, reorganization, moratorium, or similar laws now or hereinafter in
effect relating to creditors’ rights generally or to general principles of equity. 

  

	 	9.1.5	No Violation. None of the execution, delivery or performance of this Agreement by such Party, nor the consummation by such Party of the transactions contemplated hereby will
(i) result in a violation of any provision of such Party’s charter documents or by-laws; or (ii) with or without the giving of notice or the lapse of time, or both, conflict with, or result in any violation or breach of, or constitute
a default under, or result in any right to accelerate or result in the creation of any lien, charge or encumbrance pursuant to, or right of termination under, any provision of any material agreement to which such Party is a party or by which such
Party or any of its assets or properties are bound or which is applicable to such Party or any of its assets or properties. 

  

	 	9.1.6	No Debarment. It is not currently debarred, suspended, or otherwise excluded by the FDA or any other regulatory authority from conducting business and shall not knowingly use
in connection with this Agreement the services of any person debarred by the FDA. 

  

	 	9.1.7	Accuracy of Data. All laboratory, scientific, technical and/or other data submitted by or on behalf of such Party, as the case may be, relating to Aldurazyme shall, to the
best of its knowledge, be true and correct and shall not contain any material falsification, misrepresentations or omissions. 

  

	9.2	Additional Representations and Warranties. 

  

	 	9.2.1	Genzyme Warranties. Genzyme warrants that the Aldurazyme sold pursuant to Article 4 hereof will be marketed and sold in all material respects in accordance with the
conditions of any applicable Marketing Application Approvals, Regulatory Approvals, any applicable labeling claims and any applicable requirements of the Regulatory Scheme regarding the promotion, sale and marketing of Aldurazyme (except to the
extent any of the foregoing is the responsibility of BioMarin as the holder of the BLA). 

  

	 	9.2.2	BioMarin Warranties. 

  

	 	(a)	 BioMarin warrants that the Aldurazyme delivered by BioMarin pursuant to Article 3 hereof will conform in all material respects to the Specifications,
the 

  

 -31- 

	 	 
conditions of any applicable Regulatory Approvals regarding the manufacturing process and any applicable requirements of the Regulatory Scheme regarding the
manufacturing process. 

  

	 	(b)	When each Lot of Aldurazyme leaves BioMarin’s facility, such Lot shall be of good, merchantable and usable quality, free of defects in material and workmanship, suitable
for the purposes for which Aldurazyme is to be used, and shall not be adulterated or misbranded due to any defect caused by BioMarin within the meaning of the U.S. Regulatory Scheme and other substantially similar laws and statutes in any country
outside the United States in which Aldurazyme is to be sold. 

  

	 	(c)	The manufacturing facilities for Aldurazyme shall conform in all material respects to the standards of those Regulatory Authorities with jurisdiction over such facilities,
including, but not limited to, those set forth in the cGMP Regulations. 

  

	 	9.2.3	No Representations or Warranties as to Safety of Aldurazyme or Specifications as of the Effective Date. 

  

	 	(a)	Each of the Parties acknowledges that none of the other Parties nor their respective personnel are obligated to engage in any refinement or development of Aldurazyme pursuant
to the terms of this Agreement other than with respect to BioMarin, the production of Aldurazyme pursuant to the Specifications or as approved by BioMarin and any refinement and development required as a result of any changes to Aldurazyme made
pursuant to Section 3. 

  

	 	(b)	Each of the Parties acknowledges that the Specifications as they exist as of the Effective Date have been jointly developed by the Parties pursuant to the terms of the
Original Collaboration Agreement. 

  

	 	(c)	BioMarin/Genzyme LLC and Genzyme acknowledge that other than [****] each Lot of Aldurazyme by BioMarin as required by the Specifications, the applicable Regulatory Approvals
and the applicable requirements of the Regulatory Scheme (including without limitation cGMP Regulations) and/or the Technical Agreement, BioMarin shall not be in any way responsible for [****]. 

  

	9.3	Disclaimer of Representations and Warranties. EXCEPT AS OTHERWISE EXPRESSLY SET FORTH IN THIS AGREEMENT OR IN A RELATED AGREEMENT, NONE OF BIOMARIN, GENZYME OR
BIOMARIN/GENZYME LLC MAKES ANY REPRESENTATIONS OR EXTENDS ANY WARRANTIES OF ANY KIND, EITHER EXPRESS OR IMPLIED, INCLUDING WITHOUT LIMITATION WARRANTIES OF MERCHANTABILITY, FITNESS FOR A PARTICULAR PURPOSE, AND THE NON-INFRINGEMENT OF ANY
THIRD-PARTY PATENTS OR PROPRIETARY RIGHTS. ALL UNIFORM COMMERCIAL CODE WARRANTIES ARE EXPRESSLY DISCLAIMED BY THE PARTIES. 

  

 -32- 

	9.4	Limitation of Liability. EXCEPT WITH RESPECT TO CLAIMS FOR INDEMNIFICATION UNDER ARTICLE 11 HEREOF AND AS OTHERWISE EXPRESSLY SET FORTH IN THIS AGREEMENT, IT IS AGREED
BY THE PARTIES THAT NO PARTY SHALL BE LIABLE TO ANOTHER PARTY FOR ANY SPECIAL, CONSEQUENTIAL, EXEMPLARY OR INCIDENTAL DAMAGES (INCLUDING LOST OR ANTICIPATED REVENUES OR PROFITS RELATING TO THE SAME), ARISING FROM ANY CLAIM RELATING TO THIS
AGREEMENT, WHETHER SUCH CLAIM IS BASED ON CONTRACT, TORT (INCLUDING NEGLIGENCE) OR OTHERWISE, EVEN IF AN AUTHORIZED REPRESENTATIVE OF SUCH PARTY IS ADVISED OF THE POSSIBILITY OR LIKELIHOOD OF SAME. 

  

	10.	LICENSE GRANTS: PATENT PROSECUTION AND LITIGATION. 

  

	10.1	License Grants. Pursuant to the terms and conditions of Article 3 of the Amended and Restated Collaboration Agreement, the Parties have granted each other licenses
with respect to all Patent Rights, Technology and Manufacturing Know-How owned or Controlled by the Parties related to Collaboration Products in the Field. 

  

	10.2	Rights and Obligations of the Parties. The rights and obligation of the Parties with respect to filing, prosecution and maintenance of all Patent Rights, defense of Third
Party infringement claims and enforcement of Patent Rights are set forth in Article 9 of the Amended and Restated Collaboration Agreement. 

  

	11.	INDEMNITY. 

  

	11.1	In Favor of BioMarin. Genzyme shall defend, indemnify and hold harmless each of BioMarin, its Affiliates and their respective officers, directors and employees (collectively,
the “BioMarin Indemnified Parties”) from and against any and all claims, demands, losses, damages, liabilities, settlement amounts, costs or expenses whatsoever (including reasonable attorneys’ fees and costs) (collectively,
“Losses”) (i) arising from or related to any claim, action or proceeding asserted by a Third Party (a “Third Party Claim”) against any BioMarin Indemnified Party arising from Genzyme’s breach of this
Agreement or negligence or willful misconduct in connection with its performance of its obligations under this Agreement or (ii) allocated to it in Section 11.3, except to the extent (which shall reduce Genzyme’s
indemnification obligation hereunder) in the case of (i) and (ii) such Losses also arise from BioMarin’s breach of any warranty or representation herein, BioMarin’s failure to perform any covenant herein, or any negligent or
willful act or omission of BioMarin in performing its obligations under this Agreement. 

  

	11.2	 In Favor of Genzyme. BioMarin shall defend, indemnify and hold harmless each of Genzyme, its Affiliates and their respective officers, directors and
employees (collectively, the “Genzyme Indemnified Parties”) from and against any and all Losses (i) arising from or related to any Third Party Claim against any Genzyme Indemnified Party arising from BioMarin’s breach of
this Agreement or negligence or willful misconduct in connection with its performance of its obligations hereunder, (ii)

  

 -33- 

	 	 
arising from or related to any Third Party Claim against any Genzyme Indemnified Party and lost profits of Genzyme, in each case, resulting from an
interruption in the supply of Aldurazyme due to changes implemented by BioMarin pursuant to Section 3.10 or 3.11 (other than any change made at the request or direction of any regulatory authority) or (iii) allocated to it in
Section 11.3, except to the extent (which shall reduce BioMarin’s indemnification obligation hereunder), in each of case (i), (ii) and (iii) such Losses also arise from Genzyme’s breach of any warranty or
representation herein, Genzyme’s failure to perform any covenant herein, or any negligent or willful act or omission of Genzyme in performing its obligations under this Agreement. 

  

	11.3	Product Liability. If any Party receives a Third Party Claim or suffers any Losses relating to product liability, the other Party shall contribute to such Losses to the
extent set forth in this Section 11.3. 

  

	 	11.3.1	Alleged Design Defect Regarding Aldurazyme. Each of the Parties acknowledge and agree that Aldurazyme produced using the Specifications in effect as of the Effective Date was
produced through their joint efforts. To the extent that a Third Party Claim is asserted against any of the Parties or any of their Affiliates related to a design defect in Aldurazyme meeting the Specifications in effect as of the Effective Date,
the Parties acknowledge and agree that they shall each be responsible for fifty percent (50%) of all Losses incurred by the Parties and their Affiliates arising from such a Third Party Claim. To the extent that the Specifications are modified
after the Effective Date pursuant to Sections 1.50 and 3.11 and any of the Parties is found to be responsible for any claims related to a design defect in the production of Aldurazyme pursuant to such modified Specifications, the Parties
acknowledge and agree that BioMarin shall be responsible for one hundred percent (100%) of all Losses incurred by the Parties and their Affiliates arising from such a Third Party Claim except to the extent such Losses also arise from
Genzyme’s breach of any warranty or representation herein, Genzyme’s failure to perform any covenant herein, or the negligent or willful act or omission of Genzyme in performing its obligations under this Agreement, in which case liability
shall be apportioned between BioMarin and Genzyme as appropriate. 

  

	 	11.3.2	Alleged Manufacturing Defect Regarding Aldurazyme. Notwithstanding Section 11.3.1, the Parties acknowledge and agree that to the extent that a Third Party Claim
is asserted against Genzyme or any of its Affiliates related to the manufacturing, storing, handling, delivery, US regulatory compliance of Aldurazyme by BioMarin or its Affiliates or Third Parties engaged by or on behalf of BioMarin (including
without limitation Hollister-Stier), any and all Losses incurred by Genzyme or its Affiliates arising from such Third Party Claim shall be the sole responsibility of BioMarin except to the extent such Losses arise from Genzyme’s breach of any
warranty or representation herein, Genzyme’s failure to perform any covenant herein, or the negligent or willful act or omission of Genzyme in performing its obligations under this Agreement, in which case liability shall be apportioned between
BioMarin and Genzyme as appropriate. 

  

 -34- 

	 	11.3.3	Alleged Non-Manufacturing Defect Regarding Aldurazyme. Notwithstanding Section 11.3.1, the Parties acknowledge and agree that to the extent that a Third Party
Claim is asserted against BioMarin or any of its Affiliates related to the storage, handling, delivery, non-US regulatory compliance, labeling, marketing, distribution or sale of Aldurazyme by Genzyme or its Affiliates or Third Parties engaged by or
on behalf of Genzyme, any and all Losses incurred by BioMarin or its Affiliates arising from such Third Party Claim shall be the sole responsibility of Genzyme except to the extent such Losses arise from BioMarin’s breach of any warranty or
representation herein, BioMarin’s failure to perform any covenant herein, or the negligent or willful act or omission of BioMarin in performing its obligations under this Agreement, in which case liability shall be apportioned between BioMarin
and Genzyme as appropriate. 

  

	11.4	Indemnification and Contribution Procedures. 

  

	 	11.4.1	Indemnification Procedure. In a circumstance where one Party is required to indemnify the other Party for one hundred percent (100%) of the Losses arising from any Third
Party Claim, a Party or any of its Affiliates or their respective directors, officers, employees or agents (the “Indemnitee”) that intends to claim indemnification under this Article 11 shall promptly notify the other Party
(the “Indemnitor”) of any Losses in respect of which the Indemnitee intends to claim such indemnification, and if the Indemnitor confirms in writing that it will indemnify the Indemnitee for one hundred percent (100%) of such
Loss, then the Indemnitor shall have the right to assume the defense thereof with counsel of its choice, subject to the consent of the non-Indemnifying Party, which consent will not be unreasonably withheld, delayed or conditioned; provided,
however, that an Indemnitee shall have the right to retain its own counsel, with the fees and expenses to be paid by the Indemnitor, if representation of such Indemnitee by the counsel retained by the Indemnitor would be inappropriate due to actual
or potential conflicting interests between such Indemnitee and any other Party represented by such counsel in such proceedings. The Indemnitor shall not be responsible for the fees and expenses of more than one counsel to all Indemnitees. The
indemnity in this Article 11 shall not apply to amounts paid in settlement of any Third Party Claim if such settlement is effected without the prior written consent of any Indemnitor, which consent shall not be unreasonably withheld, delayed
or conditioned. The failure to deliver notice to an Indemnitor within a reasonable time after the commencement of any such Third Party Claim shall not relieve such Indemnitor of any liability to the Indemnitee under this Article 11 with
respect to such action, except to the extent that such failure materially prejudiced the Indemnitor’s ability to defend such action. Each Indemnitee under this Article 11, its employees and agents, shall cooperate fully with the
Indemnitor and its legal representatives in the investigation of any Claim or action covered by this indemnification. 

  

 -35- 

	 	11.4.2	Contribution Procedure. In a circumstance where responsibility for Losses arising out of any Third Party Claim is to be apportioned between Genzyme and BioMarin pursuant to
Sections 11.1, 11.2, and 11.3, a Party that intends to seek partial indemnification or contribution from the other Party for any such Losses must notify the other Party as promptly as practical. The failure to deliver such notice within a
reasonable time will not relieve a Party of any obligations for indemnification or contribution hereunder with respect to such Losses, except to the extent that such failure materially prejudiced the Indemnitor’s ability to defend such Third
Party Claim. If the Parties agree that one Party will have responsibility for more than fifty percent (50%) of the Losses arising out of a Third Party Claim, such Party shall have the right to assume the defense of the Third Party Claim with
counsel of its choice, subject to the consent of the non-Indemnifying Party, which consent will not be unreasonably withheld, delayed or conditioned; provided, however, the Party not defending the Third Party Claim shall have the right to retain its
own counsel to participate in the defense of the Third Party Claim at its own expense. In the absence of such agreement, each Party seeking indemnification or contribution may defend the Third Party Claim with counsel of its choosing at its own
expense, and each Party’s counsel fees and costs related to the Third Party Claim will be excluded from the Losses to be apportioned in accordance with Section 11.1, 11.2, and 11.3. No Party shall be required to contribute to or
indemnify the other Party for any Losses arising out of a Third Party Claim if the claim is settled without its written consent, which consent shall not be unreasonably withheld, delayed or conditioned. Each Party, its employees and agents, shall
cooperate fully with the other Party and its legal representatives in the investigation of any Third Party Claim covered by this Section 11.4.2. The Parties shall negotiate in good faith to enter into a joint defense agreement with
respect to the defense of any Third Party Claim covered by this Section 11.4.2. 

  

	11.5	Insurance. Each Party shall, during the term of this Agreement, maintain commercially reasonable amounts of insurance or self-insurance given the size, nature and scope of
its business from a reputable insurance carrier to cover against liability risks, including product liability insurance and business interruption insurance (for commercial value) for the benefit of the other Party. Each Party shall provide the other
Party with evidence of such insurance. 

  

	12.	TERM AND TERMINATION. 

  

	12.1	Term. The term of this Agreement shall be perpetual unless terminated pursuant to Section 12.2 below. 

  

 -36- 

	12.2	Termination. This Agreement may be terminated in the following circumstances: 

  

	 	12.2.1	For Certain Material Breaches. If either BioMarin or Genzyme fails to use commercially reasonable and diligent efforts to perform any material duty imposed upon such Party
under this Agreement and such failure to perform is not cured within ninety (90) days of written notice thereof from the non-breaching Party, the non-breaching Party may elect, in its sole discretion, to terminate this Agreement with the
consequences set forth in Section 12.3.1 below. Such 90-day period shall be extended to one hundred eighty (180) days if the breaching Party has engaged in good faith efforts to remedy such default within such 90-day period and
indicated in writing to the non-breaching Party prior to the expiration of such 90-day period that it believes that it will be able to remedy the default within such 180-day period, but such extension shall apply only so long as the breaching Party
is engaging in good faith efforts to remedy such default. 

  

	 	12.2.2	For Convenience. Either BioMarin or Genzyme may elect to terminate this Agreement for any reason upon one (1) year prior written notice to the other Party, during which
one-year period the obligations of the Parties shall continue in full force and effect. 

  

	 	12.2.3	Upon Change of Control. Either BioMarin or Genzyme may terminate this Agreement in the event that the other Party is a party to a transaction involving (a) a merger or
consolidation in which such Party is not the surviving entity or (b) the sale of all or substantially all of the assets of such Party to a Third Party. Termination of this Agreement pursuant to this Section 12.2.3 shall be effective
as of the effective date of such transaction. 

  

	 	12.2.4	Upon Bankruptcy. Either BioMarin or Genzyme may terminate this Agreement if: (a) the other Party fails to meet any material obligation hereunder and (i) applies for
or consents to the appointment of a receiver, trustee, liquidation or custodian of itself or of all or a substantial part of its property; (ii) becomes unable, or admits in writing its inability, to pay its debts generally as they mature;
(iii) makes a general assignment for the benefit of its or any of its creditors; (iv) is dissolved or liquidated in full or in part; (v) commences 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 (vi) takes any action for the purpose of effecting any of the foregoing; or (b) proceedings for the appointment of a receiver, trustee, liquidator or custodian of the other Party 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 other Party 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 sixty (60) calendar days of commencement. 

  

 -37- 

	12.3	Effects of Termination. 

  

	 	12.3.1	For Certain Material Breaches. In addition to the rights and duties set forth in Section 12.4 below, BioMarin and Genzyme shall have the following rights and
duties upon termination of this Agreement pursuant to Section 12.2.1 above: 

  

	 	(a)	the non-breaching Party shall have an irrevocable right and license, with the right to grant and authorize sublicenses, to develop, make, have made, use, offer for sale,
sell, have sold, import and export Aldurazyme and the breaching Party shall execute such documents and take all action as may be necessary or desirable to effect the foregoing; provided, that such license shall be for the same level of
exclusivity as the rights granted with respect thereto under Article 3 or Article 4 hereof, as the case may be, immediately prior to such termination; provided further that any license granted hereunder shall be subject
to the obligation of the non-breaching Party to use commercially reasonable and diligent efforts to manufacture, develop and market Aldurazyme pursuant to such license; 

  

	 	(b)	the breaching Party shall assign and transfer all of its interest in BioMarin/Genzyme LLC to the non-breaching Party, and the non-breaching Party may dissolve
BioMarin/Genzyme LLC in its sole discretion; provided that in the event that BioMarin is the breaching Party, it shall also cause BioMarin Genetics to assign and transfer all of its interest in BioMarin/Genzyme LLC to Genzyme.

  

	 	(c)	upon exercise of its license option by the non-breaching party provided in paragraph (a) of this Section 12.3.1: (i) all rights and licenses or rights
granted pursuant to Article 3 or Article 4, as the case may be, shall be revoked; (ii) if BioMarin/Genzyme LLC is dissolved, any applicable Regulatory Approvals (other than any Regulatory Approvals filed in the name of an entity
other than BioMarin/Genzyme LLC), “Orphan Drug” designations and clinical data owned or licensed by BioMarin/Genzyme LLC and any trademarks owned or licensed by BioMarin/Genzyme LLC (other than any trademarks registered in the name of an
entity other than BioMarin/Genzyme LLC pursuant to Section 9.1.2 of the Amended and Restated Collaboration Agreement) shall be assigned or exclusively licensed to the non-breaching Party; and (iii) any Regulatory Approvals filed and
any trademarks registered in the name of an entity other than BioMarin/Genzyme LLC shall be (A) exclusively licensed to BioMarin/Genzyme LLC, the non-breaching Party or any Third Party or Affiliate designated by the non-breaching Party until
such time as BioMarin/Genzyme LLC, the non-breaching Party or its designee is qualified to hold such Regulatory Approvals or trademarks under the applicable provisions of the Regulatory Scheme and (B) transferred or assigned to BioMarin/Genzyme
LLC, the non-breaching Party or its designee, as appropriate, as soon as practicable thereafter; 

  

 -38- 

	 	(d)	the non-breaching Party shall become obligated to pay the breaching Party an amount equal to: [****] (iii) interest thereon at the Base Rate of interest declared from
time to time by Bank of America, N.A. in Boston, Massachusetts from the date of termination to the date payment is made (the “Breach Buyout Amount”), payable as follows: 

  

	 	(i)	if the non-breaching Party elects to sell or otherwise dispose of all or any portion of its or its Affiliates’ right, title and interest in Aldurazyme, then the
non-breaching Party shall, upon any such sale or other disposition, pay the breaching Party an amount equal to [****]; 

  

	 	(ii)	for as long as the non-breaching Party (together, in the case of BioMarin with BioMarin Genetics) has not sold or otherwise disposed of all or a portion of its (together in
the case of BioMarin, with BioMarin Genetics) right, title and interest in Aldurazyme which is equal to or greater than the breaching Party’s (together in the case of BioMarin, with BioMarin Genetics) Percentage Interest as of the date of
termination, the non-breaching Party shall pay the breaching Party (and, in the event that BioMarin is the breaching Party, BioMarin Genetics) [****] as described in the preceding paragraph; and 

  

	 	(iii)	on the [****] of the date of termination, the non-breaching Party shall pay the breaching Party (and, in the event that BioMarin is the breaching Party, BioMarin Genetics)
the difference between the aggregate amounts paid pursuant to clauses (i) and (ii) above and the Breach Buyout Amount; provided, that the aggregate amount of all payments made under clauses (i), (ii) and (iii) shall not
exceed the Breach Buyout Amount. 

  

	 	12.3.2	For Convenience. In addition to the rights and duties set forth in Section 12.4 below, BioMarin and Genzyme shall have the following rights and duties upon
termination of this Agreement pursuant to Section 12.2.2 above: 

  

	 	(a)	the non-terminating Party shall have an option exercisable upon written notice to the terminating Party within the one-year period provided in Section 12.2.2
hereof to obtain from the terminating Party the irrevocable right and license, with the right to grant and authorize sublicenses, to develop, make, have made, use, offer for sale, sell, have sold, import and export Aldurazyme, and the
terminating Party shall execute such documents and take all action as may be necessary or desirable to affect the foregoing; provided, that such license shall be for the same level of exclusivity as the rights granted with respect thereto
under Article 3 or Article 4 hereof, as the case may be; provided further that any license granted hereunder shall be subject to the obligation of the non-terminating Party to use commercially reasonable and diligent
efforts to develop and market Aldurazyme pursuant to such license; 

  

 -39- 

	 	(b)	upon exercise of its license option provided in paragraph (a) of this Section 12.3.2, the terminating Party shall assign and transfer all of its interest in
BioMarin/Genzyme LLC to the non-terminating Party, and the non-terminating Party may dissolve BioMarin/Genzyme LLC in its sole discretion; 

  

	 	(c)	upon exercise of its license option provided in paragraph (a) of this Section 12.3.2, (i) all licenses granted pursuant to Article 3 or
Article 4, as the case may be, shall be revoked, (ii) if BioMarin/Genzyme LLC is dissolved, any applicable Regulatory Approvals (other than any Regulatory Approvals filed in the name of an entity other than BioMarin/Genzyme LLC pursuant
to Section 5.3 of the Amended and Restated Collaboration Agreement), “Orphan Drug” designations and clinical data owned or licensed by BioMarin/Genzyme LLC and any trademarks owned or licensed by BioMarin/Genzyme LLC (other than any
trademarks registered in the name of an entity other than BioMarin/Genzyme LLC pursuant to Section 9.1.2 of the Amended and Restated Collaboration Agreement) shall be assigned to the non-terminating Party and (iii) any Regulatory Approvals
filed and any trademarks registered in the name of an entity other than BioMarin/Genzyme LLC shall be (A) exclusively licensed to BioMarin/Genzyme LLC, the non-terminating-Party or any Third Party or Affiliate designated by such Party until
such time as BioMarin/Genzyme LLC, the non-terminating Party or its designee is qualified to hold such Regulatory Approvals or trademarks under the applicable provisions of the Regulatory Scheme and (B) transferred or assigned to
BioMarin/Genzyme LLC, the non-terminating Party or its designee, as appropriate, as soon as practicable thereafter; 

  

	 	(d)	upon the exercise of its license option provided in paragraph (a) of this Section 12.3.2, the non-terminating Party shall become obligated to pay to the
terminating Party an amount equal to: [****] (iii) interest thereon at the Base Rate of interest declared from time to time by Bank of America, N.A. in Boston, Massachusetts from the date of termination to the date payment is made; payable on
the terms and conditions and in accordance with the schedule of payments set forth in Section 12.3.1(d), mutatis mutandis; and 

  

	 	(e)	if the license option provided in paragraph (a) of this Section 12.3.2 is not exercised, then all right, title and interest in Aldurazyme shall be sold to
the highest bidder within eighteen (18) months from the date of termination and the proceeds shall be allocated between the Members in proportion to their Percentage Interests in BioMarin/Genzyme LLC as of the date of termination and
BioMarin/Genzyme LLC shall be dissolved. 

  

 -40- 

	 	12.3.3	Upon a Change of Control. In addition to the rights and duties set forth in Section 12.4 below, BioMarin and Genzyme shall have the following rights and duties
upon termination of this Agreement pursuant to Section 12.2.3 above: 

  

	 	(a)	the terminating Party shall have the exclusive, irrevocable and, except as provided in Section 12.3.3(d), royalty-free right and license, with the right to grant
sublicenses, to develop, make, have made, use, offer for sale, sell, have sold, import and export Aldurazyme, and the non-terminating Party shall execute such documents and take all action as may be necessary or desirable to effect the foregoing;
provided, that any license granted hereunder shall be subject to the obligation of the terminating Party to use commercially reasonable and diligent efforts to develop and market Collaboration Products pursuant to such license;

  

	 	(b)	the non-terminating Party shall assign and transfer all of its interest in BioMarin/Genzyme LLC to the terminating Party, and the terminating Party may dissolve
BioMarin/Genzyme LLC in its sole discretion; provided that in the event that BioMarin is the non-terminating Party, it shall also cause BioMarin Genetics to assign and transfer all of its interest in BioMarin/Genzyme LLC to Genzyme;

  

	 	(c)	all licenses or rights granted pursuant to Article 3 or Article 4, as the case may be, shall be revoked and, if BioMarin/Genzyme LLC is dissolved, any
applicable Regulatory Approval, “Orphan Drug” designations and clinical data owned or licensed by BioMarin/Genzyme LLC and any trademarks owned or licensed by BioMarin/Genzyme LLC shall be assigned or licensed to the terminating Party; and

  

	 	(d)	 the terminating Party (the “Offeror”) shall, pursuant to the conditions set forth in this Section 12.3.3(d), give the other
Party (Genzyme in the case BioMarin is terminating or the BioMarin Companies in the case Genzyme is terminating, in either case the “Offeree”) at the time of termination written notice of the Offeror’s intention to purchase
Offeree’s entire interest in and to (i) Aldurazyme as of the date of termination and (ii) the Percentage Interest of the net asset value of BioMarin/Genzyme LLC as of the date of termination (the “Notice of Offer”).
The Notice of Offer shall state therein the specific price, terms and conditions under which the Offeror agrees to purchase Offeree’s entire interest in and to (i) Aldurazyme as of the date of termination and (ii) the Percentage
Interest of the net asset value of BioMarin/Genzyme LLC as of the date of termination; provided, however, that the purchase price shall be paid in cash, publicly-traded and registered securities or as the Parties otherwise agree. The
Offeree shall then have ninety (90) days (the “Acceptance Period”) from the receipt of the Notice of Offer to give notice (the “Notice of Acceptance”) of the Offeree’s intention to accept the offer of the Offeror
and shall sell Offeree’s entire interest in and to (i) Aldurazyme as of the date of termination and (ii) the Percentage Interest of the net asset value of BioMarin/Genzyme LLC as of the date of termination to Offeror for the price and
upon such terms and conditions as set forth in the Notice of Offer. In the event the Offeree gives such Notice of 

  

 -41- 

	 	 
Acceptance, a closing shall be held within ninety (90) days of the receipt of the Notice of Acceptance by the Offeror. In the event the Offeree elects
not to accept the Offeror’s offer to purchase, by giving the Offeror written notice thereof, or by failing to give the appropriate Notice of Acceptance within the Acceptance Period, the Offeree shall thereby automatically be bound to purchase
Offeror’s entire interest in and to (i) Aldurazyme as of the date of termination and (ii) the Percentage Interest of the net asset value of BioMarin/Genzyme LLC as of the date of termination for the same price (as adjusted for
Percentage Interest, if necessary) and upon such terms and conditions as specified in the Notice of Offer. In such event, a closing shall be held within ninety (90) days of the earlier to occur of the expiration of the Acceptance Period and the
date of receipt of the written rejection, whichever is the first to occur. In addition to any other remedies provided by this Agreement, in the event the Offeree rejects the offer contained in the Notice of Offer, but thereafter fails for any reason
to timely close as provided herein above, the Offeree shall, by such failure to close, be deemed to have accepted the original offer contained in the Notice of Offer, and shall thereafter sell Offeree’s entire interest in and to
(i) Aldurazyme as of the date of termination and (ii) the Percentage Interest of the net asset value of BioMarin/Genzyme LLC as of the date of termination to the Offeror pursuant to the terms of the Notice of Offer. For purposes of
Sections 12.3.3 (a)-(c) above, the Party purchasing the other Party’s interest in (i) Aldurazyme and (ii) the Percentage Interest of the net asset value of BioMarin/Genzyme LLC shall be deemed to be the terminating Party
and the other Party shall be deemed to be the non-terminating Party. 

  

	 	12.3.4	Upon Bankruptcy. In addition to the rights and duties set forth in Section 12.4 below, BioMarin and Genzyme shall have the following rights and duties upon
termination of this Agreement pursuant to Section 12.2.4 above: 

  

	 	(a)	the terminating Party shall obtain from the non-terminating Party the irrevocable right and license, with the right to grant sublicenses, under the non-terminating
Party’s Patent Rights, Technology and Manufacturing Know-How to develop, make, have made, use, offer for sale, sell, have sold, import and export Aldurazyme in the Field and in the Territory, and the non-terminating Party shall execute such
documents and take all action as may be necessary or desirable to affect the foregoing; provided, that such license shall be for the same level of exclusivity as the rights granted with respect thereto under Article 3 or Article
4 hereof, as the case may be; provided further that any license granted hereunder shall be subject to the obligation of the terminating Party to use commercially reasonable and diligent efforts to develop and market Aldurazyme
pursuant to such license; 

  

	 	(b)	the non-terminating Party shall assign and transfer all of its interest in BioMarin/Genzyme LLC to the terminating Party, and the terminating Party may dissolve
BioMarin/Genzyme LLC in its sole discretion; provided that in the event that BioMarin is the non-terminating Party, it shall also cause BioMarin Genetics to assign and transfer all of its interest in BioMarin/Genzyme LLC to Genzyme.

  

 -42- 

	 	(c)	all licenses granted pursuant to Article 3 or Article 4, as the case may be, shall be revoked and, if BioMarin/Genzyme LLC is dissolved, any applicable
Regulatory Approvals (other than any Regulatory Approvals filed in the name of an entity other than BioMarin/Genzyme LLC pursuant to Section 5.3 of the Amended and Restated Collaboration Agreement), “Orphan Drug” designations and
clinical data owned or licensed by BioMarin/Genzyme LLC and any trademarks owned or licensed by BioMarin/Genzyme LLC (other than any trademarks registered in the name of an entity other than BioMarin/Genzyme LLC pursuant to Section 9.1.2 of the
Amended and Restated Collaboration Agreement) shall be assigned or licensed to the terminating Party and (iii) any Regulatory Approvals filed and any trademarks registered in the name of an entity other than BioMarin/Genzyme LLC shall be
(A) exclusively licensed to BioMarin/Genzyme LLC, the terminating Party or any Third Party or Affiliate designated by such Party until such time as BioMarin/Genzyme LLC, the terminating Party or its designee is qualified to hold such Regulatory
Approvals or trademarks under the applicable provisions of the Regulatory Scheme and (B) transferred or assigned to BioMarin/Genzyme LLC, the terminating Party or its designee, as appropriate, as soon as practicable thereafter,

  

	 	(d)	the terminating Party shall become obligated to pay to the non-terminating Party an amount equal to [****] from the date of termination to the date payment is made, payable
on the terms and conditions and in accordance with the schedule of payments set forth in Section 12.3.1(d), mutatis mutandis. 

  

	 	12.3.5	Fair Value. For purposes of this Section 12.3, the “Fair Value” of a Party’s interest in Aldurazyme shall be [****], the Fair Value shall be
determined by an investment, banking firm selected by mutual agreement of BioMarin and Genzyme, and the costs and expenses incurred in connection with the engagement of such investment banking firm shall be shared equally by BioMarin and Genzyme.

  

	 	12.3.6	Other. In the event that a Party (the “Purchasing Party”) purchases the other Party’s (or in the case of BioMarin, the BioMarin Companies’, in each
case the “Selling Party”) entire interest in and to (i) Aldurazyme and (ii) the Percentage Interest of the net asset value of BioMarin/Genzyme LLC pursuant to this Section 12.3, the Purchasing Party shall be
deemed to assume all of the liabilities inuring to the Selling Party’s Percentage Interest being acquired. At closing, the Parties shall execute any and all documents necessary to effectuate such transfer, including an assignment of all of the
Selling Party’s Percentage Interest and mutual releases which shall have the effect of releasing each Party from all claims or liabilities pertaining to BioMarin/Genzyme LLC (except for any liabilities specifically included in the terms of such
sale). 

  

 -43- 

	12.4	Cooperation. If either BioMarin or Genzyme (the “Assuming Party”) shall assume Aldurazyme rights from the other Party (the “Responsible
Party”) in accordance with the provisions of this Article 12, the Responsible Party shall promptly provide to the Assuming Party (or any Third Party or Affiliate designated by the Assuming Party) all Technology, Manufacturing
Know-How and access to regulatory filings filed hereunder reasonably necessary to allow the Assuming Party to perform the duties assumed and otherwise exercise the rights and licenses granted hereunder. The Responsible Party shall further use its
commercially reasonable and diligent efforts to provide reasonable assistance required by the Assuming Party with respect to such transfer so as to permit the Assuming Party to begin to perform such duties as soon as possible to minimize any
disruption in the continuity of supply or marketing of Aldurazyme. If the Responsible Party is manufacturing Aldurazyme the Responsible Party shall, at the option of the Assuming Party, supply Aldurazyme until the earlier of [****].

  

	12.5	Survival of Rights and Duties. No termination of this Agreement shall eliminate any rights or duties of the Parties accrued prior to such termination. The provisions of
Article 1, Sections 4.9, 5.2.2, 6.5, 6.9, 6.10 and 9.4, Article 8, Article 11, Sections 12.3 through 12.5, 13.2, 13.3, 13.6 through 13.15 and the first sentence of Section 3.15 and the last sentence of
Section 3.14 hereof shall survive any termination of this Agreement. 

  

	13.	MISCELLANEOUS 

  

	13.1	Exchange Controls. All payments due hereunder shall be paid in United States dollars. If at any time legal restrictions prevent the prompt remittance of part or all payments
with respect to any country in which Aldurazyme is sold, payment shall be made through such lawful means or methods as the Parties may determine in good faith. 

  

	13.2	Withholding Taxes. If applicable laws or regulations require that taxes be withheld from payments made hereunder, the Party paying such taxes will (a) deduct such taxes,
(b) timely pay such taxes to the proper authority and (c) send written evidence of payment to the Party from whom such taxes were withheld within sixty (60) days after payment. Each Party will assist the other Party or Parties in
claiming tax refunds, deductions or credits at such other Party’s request and will cooperate to minimize the withholding tax, if available, under various treaties applicable to any payment made hereunder. 

  

	13.3	Force Majeure. Neither Party shall be held liable or responsible to the other Party nor be deemed to have defaulted under or breached this Agreement for failure or delay in
fulfilling or performing any term of this Agreement when such failure or delay is caused by or results from causes beyond the reasonable control of the affected Party, including without limitation fire, floods, embargoes, war, acts of war (whether
war is declared or not), insurrections, riots, civil commotions, strikes, lockouts or other labor disturbances, acts of God or acts, omissions or delays in acting by any governmental authority or the other Party; provided, however,
that the Party so affected shall use commercially reasonable and diligent efforts to avoid or remove such causes of non-performance, and shall continue performance hereunder with reasonable dispatch wherever such causes are removed. Each Party shall
provide the other Parties with prompt written notice of any delay or failure to perform that occurs by reason of force majeure. The Parties shall mutually seek a resolution of the delay or the failure to perform in good faith.

  

 -44- 

	13.4	Assignment. This Agreement may not be assigned or otherwise transferred by any Party without the consent of the other Parties; provided, however, that
either BioMarin or Genzyme may, without such consent, assign its rights and obligations under this Agreement (a) in connection with a corporate reorganization, to any member of an affiliated group, all or substantially all of the equity
interest of which is owned and controlled by such Party or its direct or indirect parent corporation or (b) in connection with a merger, consolidation or sale of substantially all of such Party’s assets to an unrelated Third Party;
provided, however, that such Party’s rights and obligations under this Agreement shall be assumed by its successor in interest in any such transaction and shall not be transferred separate from all or substantially all of its
other business assets, including without limitation those business assets that are the subject of this Agreement and the Related Agreements. Any permitted assignee shall assume all obligations of its assignor under this Agreement; accordingly, all
references herein to the assigning Party shall be deemed references to the assignee to whom the Agreement is so assigned. Any purported assignment in violation of this Section 13.4 shall be void. 

  

	13.5	Severability. Each Party hereby agrees that it does not intend to violate any public policy, statutory or common laws, rules, regulations, treaty or decision of any
government agency or executive body thereof of any country or community or association of countries. Should one or more provisions of this Agreement be or become invalid, the Parties hereto shall substitute, by mutual consent, valid provisions for
such invalid provisions which valid provisions in their economic effect are sufficiently similar to the invalid provisions that it can be reasonably assumed that the Parties would have entered into this Agreement with such valid provisions. In case
such valid provisions cannot be agreed upon, the invalidity of one or several provisions of this Agreement shall not affect the validity of this Agreement as a whole, unless the invalid provisions are of such essential importance to this Agreement
that it is to be reasonably assumed that the Parties would not have entered into this Agreement without the invalid provisions. In the event a Party seeks to avoid a material provision of this Agreement upon an assertion that such provision is
invalid, illegal or otherwise unenforceable, the other Party shall have the right to terminate this Agreement upon sixty (60) days prior written notice to the asserting Party, unless such assertion is eliminated and cured within such
sixty (60) day period. Such a termination shall be deemed a termination by such Party for breach pursuant to Section 12.2.1. 

  

	13.6	Notices. Any consent, notice or report required or permitted to be given or made under this Agreement by one of the Parties hereto to the other shall be in writing, delivered
personally or by facsimile (and promptly confirmed by personal delivery or courier), by a next business day delivery service of a nationally recognized overnight courier service or by courier, postage prepaid (where applicable), addressed to such
other Party at its address indicated below, or to such other address as the addressee shall have last furnished in writing to the addressor in accordance with this Section 13.6 and shall be effective upon receipt by the addressee.

  

 -45- 

			
	If to BioMarin:	  	 BioMarin Pharmaceutical Inc.
 105 Digital
Drive
 Novato, California 94949
 Attention: Chief Executive
Officer
 Facsimile: (415) 382-7889

		
	with a copy to:	  	 BioMarin Pharmaceutical Inc.
 105 Digital
Drive
 Novato, California 94949
 Attention: General
Counsel
 Facsimile: (415) 382-7889

		
	If to Genzyme:	  	 Genzyme Corporation
 500 Kendall Street
 Cambridge, Massachusetts 02142
 Attention: President, LSD
Therapeutics
 Facsimile: (617) 768-6419

		
	with a copy to:	  	 Genzyme Corporation
 500 Kendall Street
 Cambridge, Massachusetts 02142
 Attention: General Counsel
 Facsimile: (617) 252-7553

		
	 If to
 BioMarin/Genzyme
 LLC (if such notice is
 sent by BioMarin):
	  	 BioMarin/Genzyme LLC
 c/o Genzyme Corporation

500 Kendall Street
 Cambridge, Massachusetts 02142
 Attention: President, LSD Therapeutics
 Facsimile: (617)
768-6419

		
	with a copy to:	  	 Genzyme Corporation
 500 Kendall Street
 Cambridge, Massachusetts 02142
 Attention: General Counsel
 Facsimile: (617) 252-7553

		
	 If to
 BioMarin/Genzyme
 LLC (if such notice is
 sent by Genzyme):
	  	 BioMarin/Genzyme LLC
 c/o BioMarin Pharmaceutical Inc.

 105 Digital Drive
 Novato, California 94949
 Attention: Chief Executive Officer
 Facsimile: (415)
382-7889

		
	with a copy to:	  	 BioMarin Pharmaceutical Inc.
 105 Digital
Drive
 Novato, California 94949
 Attention: General
Counsel
 Facsimile: (415) 382-7889

  

 -46- 

	13.7	Applicable Law. This Agreement shall be governed by and construed in accordance with the laws of the State of Delaware without regard to any choice of law principle that
would dictate the application of the laws of another jurisdiction. 

  

	13.8	Arbitrate. Any disputes arising between the Parties relating to, arising out of or in any way connected with this Agreement or any term or condition hereof, or the
performance by either Party of its obligations hereunder, whether before or after termination of this Agreement (a “Dispute”) shall be finally resolved by binding arbitration as herein provided. 

  

	 	13.8.1	General. Except as otherwise provided in this Section 13.8, any arbitration hereunder shall be conducted under the commercial rules of the American Arbitration
Association. Each such arbitration shall be conducted in the English language by a single arbitrator appointed in accordance with such rules, provided that if either Party requests the arbitration shall be conducted by a panel of
three (3) arbitrators (the “Arbitration Panel”). In the case of three (3) arbitrators, each of BioMarin and Genzyme shall appoint one (1) arbitrator to the Arbitration Panel and the third arbitrator
shall be appointed by the two (2) arbitrators appointed by BioMarin and Genzyme. The Arbitration Panel shall be convened upon delivery of the Notice of Arbitration (as herein defined). Any such arbitration shall be held in Chicago,
Illinois. The Arbitration Panel shall have the authority to grant specific performance, and to allocate between the Parties the costs of arbitration in such equitable manner as it shall determine. Judgment upon the award so rendered may be entered
in any court having jurisdiction or application may be made to such court for judicial acceptance of any award and an order of enforcement, as the case may be. 

  

	 	13.8.2	Procedure. 

  

	 	(a)	Whenever a Party (the “Claimant”) shall decide to institute arbitration proceedings, it shall give written notice to that effect (the “Notice of
Arbitration”) to the other Party (the “Respondent”). The Notice of Arbitration shall set forth in detail the nature of the Dispute, the facts upon which the Claimant relies and the issues to be arbitrated (collectively, the
“Arbitration Issues”). Within fifteen (15) days of its receipt of the Notice of Arbitration, the Respondent shall send the Claimant and the Arbitration Panel a written Response (the “Response”). The
Response shall set forth in detail the facts upon which the Respondent relies. In addition, the Response shall contain all counterclaims which the Respondent may have against the Claimant which are within the Arbitration Issues, whether or not such
claims have previously been identified. If the Response sets forth a counterclaim, the Claimant may, within fifteen (15) days of the receipt of the Response, deliver to the Respondent and the Arbitration Panel a rejoinder answering such
counterclaim. 

  

 -47- 

	 	(b)	Within fifteen (15) days after the later of (i) the expiration of the period provided in Section 13.8.2(a) above for the Claimant to deliver a
rejoinder or (ii) the completion of any discovery proceedings authorized by the Arbitration Panel: (A) the Claimant shall send to the Arbitration Panel a proposed resolution of the Arbitration Issues and a proposed resolution of any
counterclaims set forth in the Response, including without limitation the amount of monetary damages, if any, or other relief sought (the “Claimant’s Proposal”); and (B) the Respondent shall send to the Arbitration Panel a
proposed resolution of the Arbitration Issues, a proposed resolution of any counterclaims set forth in the Response and a proposed resolution of any rejoinder submitted by the Claimant, including without limitation the amount of monetary damages, if
any, or other relief sought (the “Respondent’s Proposal”). Once both the Claimant’s Proposal and the Respondent’s Proposal have been submitted, the Arbitration Panel shall deliver to each Party a copy of the other
Party’s proposal. 

  

	 	(c)	The Arbitration Panel shall issue an opinion with respect to any Dispute, which opinion shall explicitly accept either the Claimant’s Proposal or the Respondent’s
Proposal in its entirety (the “Final Decision”). The Arbitration Panel shall not have the authority to reach a Final Decision that provides remedies or requires payments other than those set forth in the Claimant’s Proposal or
the Respondent’s Proposal. The concurrence of two (2) arbitrators shall be sufficient for the entry of a Final Decision. The arbitrators shall issue a Final Decision within one (1) month from the later of (i) the last
day for submission of proposals under Section 13.8.2(b) above or (ii) the date of the final hearing on any Dispute held by the Arbitration Panel. A Final Decision shall be binding on both Parties. 

  

	13.9	Injunctive Relief. The Parties hereby acknowledge that a breach of their respective obligations under Article 8 hereof may cause irreparable harm and that the
remedy or remedies at law for any such breach may be inadequate. The Parties hereby agree that, in the event of any such breach, in addition to all other available remedies hereunder, the non-breaching Party or Parties shall have the right to seek
equitable relief to enforce Article 8 hereof. 

  

	13.10	Entire Agreement. This Agreement, together with the Related Agreements, contains the entire understanding of the Parties with respect to the subject matter hereof. All
express or implied agreements and understandings, either oral or written, heretofore made are expressly merged in and made a part of this Agreement. This Agreement may be amended, or any term hereof modified, only by a written instrument duly
executed by both Parties hereto. Each of the Parties hereby acknowledges that this Agreement is the result of mutual negotiation and therefore any ambiguity in their respective terms shall not be construed against the drafting Party.

  

 -48- 

	13.11	Bankruptcy Provision. All rights and licenses now or hereafter granted under or pursuant to this Agreement, including Sections 12.3 and 12.4, are rights to
“intellectual property” (as defined in Section 101(35A) of Title 11 of the United States Code, as amended (such Title 11, the “Bankruptcy Code”)). The licensing Party hereby grants to the licensee Party a right of
access and to obtain possession of and to benefit from (i) copies of research data, (ii) laboratory samples, (iii) product samples, (iv) formulas, (v) laboratory notes and notebooks, (vi) data and results related to
clinical trials, (vii) regulatory filings and approvals, (viii) rights of reference in respect of regulatory filings and approvals, (ix) manufacturing procedure documentation and manufacturing records, (x) marketing, advertising
and promotional materials, and (xi) all other embodiments of such intellectual property, that are in the licensing Party’s possession or control, freely licenseable without further payment or restriction by the licensing Party, and
necessary for the licensee Party’s exercise of the rights and licenses to such intellectual property, all of which constitute “embodiments” of intellectual property pursuant to Section 365(n) of the Bankruptcy Code. The licensing
Party agrees not to interfere with the licensee’s exercise of rights and licenses to intellectual property licensed under this Agreement and embodiments thereof in accordance with this Agreement and agrees to use commercially reasonable and
diligent efforts to assist the licensee Party to obtain such intellectual property and embodiments thereof in the possession or control of Third Parties as reasonably necessary or desirable for the licensee Party to exercise such rights and licenses
in accordance with this Agreement. The Parties acknowledge and agree that only the payments due under Section 6.1, as adjusted pursuant to this Agreement, constitute “royalties” within the meaning of Bankruptcy Code
§365(n). 

  

	13.12	Headings. The captions to the several Articles and Sections hereof are not a part of this Agreement, but are merely guides or labels to assist in locating and reading the
several Articles and Sections hereof. 

  

	13.13	Independent Contractors. It is expressly agreed that BioMarin and Genzyme shall be independent contractors and that the relationship between the two Parties shall not
constitute a partnership, joint venture or agency. Neither BioMarin nor Genzyme shall have the authority to make any statements, representations or commitments of any kind, or to take any action, which shall be binding on the other, without the
prior consent of the other Party to do so. 

  

	13.14	Waiver. Except as expressly provided herein, the waiver by either Party hereto of any right hereunder or of any failure to perform or any breach by the other Party shall not
be deemed a waiver of any other right hereunder or of any other failure to perform or breach by said other Party, whether of a similar nature or otherwise, nor shall any singular or partial exercise of such right preclude any further exercise
thereof or the exercise of any other such right. 

  

 -49- 

	13.15	Counterparts. This Agreement 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. Signature pages may be exchanged by facsimile. 

  
 [SIGNATURE PAGE FOLLOWS] 
  

 -50- 

 IN WITNESS WHEREOF, the Parties have executed this Agreement effective as of the date first set forth
above. 
  

			
	GENZYME CORPORATION
		
	By:	 	/s/ David P. Meeker
	Print Name:	 	David P. Meeker
	Title:	 	President LSD Therapeutics
	Date:	 	12/31/07
	
	BIOMARIN PHARMACEUTICAL INC.
		
	By:	 	/s/ G. Eric Davis
	Print Name:	 	G. Eric Davis
	Title:	 	Vice President, General Counsel
	Date:	 	12/31/07
	
	BIOMARIN/GENZYME LLC
	
	By: BIOMARIN PHARMACEUTICAL INC.
		
	By:	 	/s/ G. Eric Davis
	Print Name:	 	G. Eric Davis
	Title:	 	Vice President, General Counsel
	Date:	 	12/31/07
	
	 By: GENZYME CORPORATION

		
	By:	 	/s/ David P. Meeker
	Print Name:	 	David P. Meeker
	Title:	 	President LSD Therapeutics
	Date:	 	12/31/07

 Schedule 1.56 
  

 Third Party Licenses 
  
 1.) License Agreement effective as of September 4, 1998 entered into by and between the University of Iowa Research Foundation, an Iowa corporation and
BioMarin/Genzyme LLC, a Delaware limited liability company. 
  
 2.) License
Agreement effective as of September 4, 1998 entered into by and between Research Corporation Technologies, Inc., a Delaware nonprofit corporation and BioMarin/Genzyme LLC, a Delaware limited liability company. 
  
 3.) Grant Terms and Conditions Agreement dated April 1, 1997 entered into by and among
BioMarin Pharmaceuticals, Harbor-UCLA Research and Education Institute and Emil D. Kakkis, as amended through the Effective Date. 

 Schedule 3.12 
  

 [****] 

 Exhibit A 
  
 [****] 

 Exhibit B 
  
 [****]Amended and Restated Collaboration Agreement

 EXHIBIT 10.31 
  
 CONFIDENTIAL TREATMENT REQUESTED 
 Redacted Portions are indicated by [****] 
  
 AMENDED AND RESTATED COLLABORATION AGREEMENT 
  
 THIS AMENDED AND RESTATED COLLABORATION AGREEMENT effective as of January 1, 2008 (the “Agreement”) is made by and among Genzyme Corporation, a Massachusetts corporation having its principal place of
business at 500 Kendall Street, Cambridge, Massachusetts 02142 (“Genzyme”), BioMarin Pharmaceutical, Inc., a Delaware corporation having its principal place of business at 105 Digital Drive, Novato, California 94949
(“BioMarin”) and BioMarin/Genzyme LLC, a Delaware limited liability company having its principal place of business at 500 Kendall Street, Cambridge, Massachusetts 02142 (“BioMarin/Genzyme LLC”). Genzyme, BioMarin
and BioMarin/Genzyme LLC are sometimes referred to herein individually as a “Party” and collectively as the “Parties”. 
  
 RECITALS 
  
 A. BioMarin, Genzyme and BioMarin/Genzyme LLC are parties to a Collaboration Agreement dated as of September 4, 1998 (the “Original
Collaboration Agreement”) pursuant to which BioMarin and Genzyme through BioMarin/Genzyme LLC develop, manufacture, market and sell Aldurazyme (as defined herein). 
  
 B. The Parties no longer desire to develop, manufacture, market and sell Aldurazyme through a joint venture and instead have
agreed that: (1) BioMarin will manufacture Aldurazyme and sell finished product to Genzyme; (2) Genzyme will label and commercially distribute, market and sell Aldurazyme globally; (3) each of Genzyme and BioMarin may conduct its own
research and development of Aldurazyme and other Collaboration Products (as defined herein) in accordance with the terms of this Agreement and the Manufacturing, Marketing and Sales Agreement (as defined herein); and (4) BioMarin/Genzyme LLC
will maintain and provide intellectual property licenses and sublicenses to BioMarin and Genzyme so that they may fulfill their respective obligations under this Agreement, the Manufacturing, Marketing and Sales Agreement and the Fill Agreement (as
defined herein). 
  
 C. BioMarin and Genzyme are hereby amending
and restating the Original Collaboration Agreement so that hereafter BioMarin/Genzyme LLC will no longer engage in commercial activities and will solely (1) hold the intellectual property relating to Aldurazyme and license all such intellectual
property on the terms set forth herein to BioMarin and Genzyme on the terms set forth herein and (2) engage in research and development activities that are mutually selected and funded by BioMarin and Genzyme. 

 NOW THEREFORE, in consideration of the premises and of the covenants herein contained, the Parties
mutually agree as follows: 
  
 ARTICLE I 
  
 DEFINITIONS 
  
 For purposes of this Agreement, the terms defined in this Article shall have
the meanings specified below. Certain other capitalized terms are defined elsewhere in this Agreement. 
  
 1.1 “Affiliate” shall mean any corporation or other entity which controls, is controlled by, or is under common control with a Party. A
corporation or other entity shall be regarded as in control of another corporation or entity if it owns or directly or indirectly controls more than fifty percent (50%) of the voting stock or other ownership interest of the other corporation or
entity, or if it possesses, directly or indirectly, the power to direct or cause the direction of the management and policies of the corporation or other entity or the power to elect or appoint more than fifty percent (50%) of the members of
the governing body of the corporation or other entity. 
  
 1.2
“Aldurazyme” shall mean alpha-L-iduronidase meeting the Specifications. 
  
 1.3 “alpha-L-iduronidase” shall mean recombinant human alpha-L-iduronidase. 
  
 1.4 “BioMarin Companies” shall mean BioMarin and BioMarin Genetics, Inc., a Delaware corporation and a wholly-owned subsidiary of
BioMarin (“BioMarin Genetics”). 
  
 1.5
“BioMarin/Genzyme Patent Rights” shall mean the Patent Rights that claim Joint Inventions (as such term is defined in Section 9.1.1 hereof) that are discovered, made or conceived during and in connection with the Program
jointly by employees or consultants of Genzyme and BioMarin to the extent that such Patent Rights relate to or are useful for the research, development, manufacture or commercialization of Collaboration Products for use in the Field. 
  
 1.6 “BioMarin/Genzyme Technology” shall mean all Technology
discovered, made or conceived during and in connection with the Program jointly by employees or consultants of Genzyme and BioMarin relating to or useful for the research, development, manufacture or commercialization of Collaboration Products for
use in the Field. 
  
 1.7 “BioMarin Patent
Rights” shall mean all Patent Rights Controlled by BioMarin during the Term to the extent that such Patent Rights relate to or are useful for the research, development, manufacture or commercialization of Collaboration Products for use in
the Field. 
  
 1.8 “BioMarin Technology” shall
mean all Technology Controlled by BioMarin during the Term to the extent that such Technology relates to or is useful for the research, development, manufacture or commercialization of Collaboration Products for use in the Field. 
  
 1.9 “BLA” shall mean a Biologics License Application or
similar application filed with the FDA after completion of human clinical trials to obtain marketing approval for a Collaboration Product, including without limitation the BLA as approved by the FDA on April 30, 2003 that provides for marketing
approval for Aldurazyme in the United States, as the same may be updated or amended from time to time. 
  

 -2- 

 1.10 “Collaboration Product” shall mean any pharmaceutical compositions of
alpha-L-iduronidase (including Aldurazyme unless otherwise indicated herein), including without limitation any and all improvements, derivatives, analogs, combination products, delivery systems and dosage forms related thereto. 
  
 1.11 “Commercialization Costs” with respect to a
Collaboration Product shall mean the variable costs and fixed costs incurred by BioMarin/Genzyme LLC with respect to work performed by the Parties and their Affiliates and subcontractors in connection with the performance of and in accordance with
the commercialization plan for such Collaboration Product, including without limitation, sales and marketing costs related to performing market research, advertising, producing promotional literature, sponsoring seminars and symposia, sales training
meetings and seminars, originating sales, providing reimbursement and other patient support services. For purposes of this Section 1.11, “variable costs” shall be deemed to be the cost of labor, raw materials, supplies and
other resources directly consumed in the conduct of the Commercialization Plan and manufacture of Collaboration Product for use in commercialization activities, as well as amounts paid to Third Parties under a Third Party Agreement as a result of
performance of the Commercialization Plan, to the extent not included in Development Costs or Fully Absorbed Cost of Goods. For purposes of this Section 1.11, “fixed costs” shall be deemed to be the cost of facilities,
utilities, insurance, equipment depreciation and other fixed costs directly related to the conduct of and in accordance with the Commercialization Plan and the manufacture of Collaboration Product for use in commercialization activities, allocated
based upon the proportion of such costs directly attributable to support or performance of the Commercialization Plan and the manufacture of Collaboration Product for use in commercialization activities or by such other method of cost allocation as
may be approved by the Steering Committee. All cost determinations made hereunder shall be made in accordance with GAAP. 
  
 1.12 “Commercialization Party” shall have the meaning set forth in Section 7.1 below. 
  
 1.13 “Control” shall mean possession of the ability to grant
a license or sublicense as provided for herein without violating the terms of an agreement with a Third Party. 
  
 1.14 “Development Costs” with respect to a Collaboration Product shall mean the variable costs and fixed costs incurred by
BioMarin/Genzyme LLC with respect to work performed by the Parties and their Affiliates and subcontractors in connection with the conduct of and in accordance with the Development Plan for such Collaboration Product, including without limitation
(a) direct, out-of-pocket external costs, including clinical grants, clinical laboratory fees, positive controls and the cost of studies conducted and services provided by contract research organizations and individuals, consultants, toxicology
contractors, and manufacturers necessary or useful for the purpose of obtaining Regulatory Approvals for such Collaboration Product, (b) amounts paid to Genzyme and BioMarin by BioMarin/Genzyme LLC with respect to research and development and
pre-commercialization sales and marketing efforts as set forth in the Development Plan for such Collaboration Product, including without limitation the efforts of Genzyme and BioMarin to develop and document process methods and procedures for the
manufacture of such Collaboration Product and the Fully Absorbed Cost of Goods for batches of such Collaboration Product manufactured and supplied for use in preclinical and clinical trials and pre-commercialization activities, (c) costs
related to data management, statistical designs and studies, document preparation and other expenses associated with the clinical testing program for such Collaboration Product, (d) costs for preparing, 

  

 -3- 

 
submitting, reviewing or developing data or information for the purpose of submission of applications to obtain Regulatory Approvals for such Collaboration
Product, (e) license fees and other amounts paid to a Third Party pursuant to any Third Party Agreement other than those fees and other amounts payable with respect to Aldurazyme under the Third Party Agreements in effect as of the Effective
Date, and (f) costs relating to the prosecution and maintenance of Patent Rights which claim, or disclose subject matter relevant to, the manufacture or use of such Collaboration Product. For purposes of this Section 1.14,
“variable costs” shall be deemed to be the cost of labor, raw materials, supplies and other resources directly consumed in the conduct of and in accordance with the Development Program and the manufacture of the Collaboration
Product for use in preclinical and clinical trials and pre-commercialization activities. For purposes of this Section 1.14, “fixed costs” shall be deemed to be the cost of facilities, utilities, insurance, facility and
equipment depreciation and other fixed costs directly related to the conduct of the Development Program and the manufacture of the Collaboration Product for use in preclinical and clinical trials and pre-commercialization activities, allocated based
upon the proportion of such costs directly attributable to the support or performance of the Development Program and the manufacture of the Collaboration Product for use in preclinical and clinical trials and pre-commercialization activities or by
such other method of cost allocation as may be approved by the Steering Committee. All cost determinations made hereunder shall be made in accordance with GAAP. 
  

1.15 “Development Plan” shall mean, with respect to a particular Collaboration Product, the comprehensive three (3) year rolling
plan and budget for the development of such Collaboration Product under the Development Program, as more specifically described in Section 5.1.2 hereof. 
  
 1.16 “Development Program” shall mean, with respect to a particular Collaboration Product, the preclinical and clinical development of
such Collaboration Product including post-marketing studies and the preparation and filing of all applications for Regulatory Approvals for such Collaboration Product, all as determined by the Steering Committee or as otherwise set forth herein.

  
 1.17 “Effective Date” shall mean
January 1, 2008. 
  
 1.18 “Field” shall mean
any and all therapeutic applications of alpha-L-iduronidase for MPS I and other alpha-L-iduronidase deficiencies. Notwithstanding the foregoing, the Field shall not include Gene Therapy for MPS I or other alpha-L-iduronidase deficiencies. For
purposes of this Agreement, “Gene Therapy” shall mean treatment or prevention of MPS I or other alpha-L-iduronidase deficiencies by means of ex vivo or in vivo introduction (via viral or nonviral gene transfer systems)
of nucleotide sequences (including without limitation, DNA, RNA and complementary and reverse complementary nucleotide sequences thereto, whether coding or noncoding). 
  

 1.19 “FDA” shall mean the United States Food and Drug Administration, any successor agency, or the regulatory authority of
any country other than the United States with responsibilities comparable to those of the United States Food and Drug Administration. 
  
 1.20 “Fully Absorbed Cost of Goods” with respect to units of Collaboration Product shall mean (a) the variable costs and fixed costs
incurred by a Party associated with the manufacture (inclusive of finishing processes including filling, packaging, labeling and/or other preparation) 

  

 -4- 

 
quality assurance, quality control and other testing, storage and shipping of batches of such units of Collaboration Product or (b) if such units or
portions of Collaboration Product are not manufactured by the Parties, the amounts paid to the vendor plus costs associated with acquisition from such vendor. For purposes of this Section 1.20, “variable costs” shall be deemed
to be the cost of labor, raw materials, supplies and other resources directly consumed in the manufacture, quality assurance, quality control and other testing, storage and shipping of batches of such Collaboration Product. For purposes of this
Section 1.20, “fixed costs” shall be deemed to be the cost of facilities, utilities, insurance, facility and equipment depreciation and other fixed costs directly related to the manufacture, quality assurance, quality control
and other testing, storage and shipping of batches of such Collaboration Product, as well as amounts paid to Third Parties under a Third Party Agreement as a result of the manufacture, use or sale of such units of Collaboration Products. Fixed costs
shall be allocated to such units of Collaboration Product based upon the proportion of such costs directly attributable to support of the manufacturing, quality assurance, quality control and other testing, storage and shipping processes for such
Collaboration Product. If a facility is used to manufacture Collaboration Products and has the capacity to manufacture products for other programs of either Genzyme or BioMarin, fixed costs shall be allocated in proportion to the actual use of such
facility for the manufacture of Collaboration Products and the capacity to manufacture products for such other programs. For the avoidance of doubt, no idle capacity of a manufacturing facility shall be included in Fully Absorbed Cost of Goods
unless such facility is appropriately sized and dedicated solely to the manufacture of Collaboration Products. Fully Absorbed Cost of Goods shall exclude all costs otherwise reimbursed pursuant to this Agreement. In the event that either BioMarin or
Genzyme subcontracts with the other Party to perform any work on its behalf in connection with the manufacturing responsibilities assigned to BioMarin or Genzyme, respectively, pursuant to Section 7.1.1 hereof, BioMarin and Genzyme
(i) shall each directly charge BioMarin/Genzyme LLC their respective Fully Absorbed Cost of Goods and (ii) shall not include any part of the other Party’s Fully Absorbed Cost of Goods in the amount so charged to BioMarin/Genzyme LLC.
Except as otherwise provided in this Agreement, all cost determinations made hereunder shall be made in accordance with GAAP. 
  
 1.21 “GAAP” shall mean the then-current United States generally accepted accounting principles, consistently applied, except when
different accounting principles are required under the terms of the Operating Agreement, in which case the accounting principles mandated under the Operating Agreement shall control. 
  
 1.22 “Genzyme Patent Rights” shall mean all Patent Rights Controlled by Genzyme during the Term to the
extent that such Patent Rights relate to or are useful for the research, development, manufacture or commercialization of Collaboration Products for use in the Field. 
  
 1.23 “Genzyme Technology” shall mean all Technology Controlled by Genzyme during the Term to the extent
such Technology relates to or is useful for the research, development manufacture or commercialization of Collaboration Products for use in the Field. 
  
 1.24 “Manufacturing Party” shall have the meaning set forth in Section 7.1.1. 
  
 1.25 “Manufacturing Know-How” shall mean all information,
techniques, inventions, discoveries, improvements, practices, methods, knowledge, skill, experience and other technology, whether or not patentable or copyrightable, and any copyrights based thereon, relating to or 

  

 -5- 

 
necessary or useful for the production, purification, packaging, storage and transportation of Collaboration Products, including without limitation
specifications, acceptance criteria, manufacturing batch records, standard operating procedures, engineering plans, installation, operation and process qualification protocols for equipment, validation records, master files submitted to the FDA,
process validation reports, environmental monitoring processes, test data including pharmacological, toxicological and clinical test data, cost data and employee training materials. 
  
 1.26 “Manufacturing, Marketing and Sales Agreement” shall mean the Manufacturing, Marketing and Sales
Agreement of even date herewith by and among the Parties. 
  
 1.27
“Member” shall have the meaning set forth in the Operating Agreement. 
  
 1.28 “Members Agreement” shall have the meaning set forth in the recitals. 
  
 1.29 “MPS-I” shall mean mucopolysaccharidosis I. 
  

1.30 “Operating Agreement” shall mean the Operating Agreement of BioMarin/Genzyme LLC dated as of September 4, 1998 by and among
the BioMarin Companies and Genzyme. 
  
 1.31 “Original
Date of Execution” shall mean September 4, 1998. 
  
 1.32 “Patent Rights” shall mean any U.S. and foreign patents and patent applications (including continuations, continuations-in-part, divisionals, reissues, re-examinations, renewals, supplemental protection certificates
and extensions). 
  
 1.33 “Percentage Interest”
shall have the meaning set forth in the Operating Agreement. 
  
 1.34 “Program” shall mean the collaboration among BioMarin/Genzyme LLC, BioMarin and Genzyme described in this Agreement. 
  
 1.35 “Program Costs” shall mean all Program-related costs, including without limitation Development Costs and Commercialization Costs, in
each case as such costs are incurred or accrued by BioMarin/Genzyme LLC on or after the Original Date of Execution. Notwithstanding anything herein to the contrary, it is understood that the Parties shall apply mutually agreed upon cost allocation
methods in determining Program Costs hereunder. It is understood and agreed that, with respect to Aldurazyme, “Program Costs” shall be limited to Program Costs incurred (i) on or prior to December 31, 2007 or (ii) solely
pursuant to development activities identified in the Development Plan attached hereto as Appendix A and other development activities added pursuant to Section 5.1.4 hereof or agreed to by the Steering Committee on or after the Effective
Date, and shall exclude (x) all Commercialization Costs incurred on or after January 1, 2008, (y) all Fully Absorbed Costs incurred on or after January 1, 2008 except with respect to quantities of Collaboration Products required
for the activities described in clause (ii) above and (z) all other costs that are allocated between the Parties under the Manufacturing, Marketing and Sales Agreement or the Fill Agreement of even date herewith by and between BioMarin and
Genzyme (the “Fill Agreement”). The Parties shall maintain separate records for Program Costs incurred on or prior to December 31, 2007 and for Program Costs incurred on or after the Effective Date. 
  

 -6- 

 1.36 “Program Management Team” shall mean the joint team composed of representatives of
Genzyme and BioMarin described in Section 8.1.1 hereof. 
  
 1.37 “Regulatory Approvals” shall mean all approvals from regulatory authorities in any country in the Territory required lawfully to manufacture and market Collaboration Products in any such country, including without
limitation approval of any BLA, any establishment license application filed with the FDA to obtain approval of the facilities and equipment to be used to manufacture a Collaboration Product, and any product pricing approvals where applicable.

  
 1.38 “Regulatory Scheme” shall mean the
United States Public Health Service Act and the regulations, interpretations and guidelines promulgated thereunder by the FDA or the regulatory scheme applicable to the Collaboration Products in any country other than the United States, as such
statutes, regulations, interpretations and guidelines or regulatory schemes may be amended from time to time. 
  
 1.39 “Related Agreements” shall mean the Manufacturing, Marketing and Sales Agreement, the Members Agreement, the Fill/Finish
Manufacturing Agreement and the Operating Agreement. 
  
 1.40
“Specifications” with respect to a particular Collaboration Product (other than Aldurazyme) shall mean the written specifications for such Collaboration Product determined by the Program Management Team and approved by the Steering
Committee; provided that such specifications shall at all times comply with the relevant Regulatory Scheme in the country of sale and in the country of use. Such Specifications may be amended from time to time by the Program Management Team
provided that such amendments are approved by the Steering Committee or the written agreement of the Parties, as the case may be. Copies of the then-current Specifications shall be maintained by both BioMarin and Genzyme and shall become a
part of this Agreement as if incorporated herein. The term “Specifications” with respect to Aldurazyme shall have the meaning set forth in the Manufacturing, Marketing and Sales Agreement. 
  
 1.41 “Steering Committee” shall mean the governing body of
BioMarin/Genzyme LLC composed of representatives of BioMarin and Genzyme appointed as described in Section 8.2.1 hereof. 
  
 1.42 “Technology” shall mean inventions, trade secrets, copyrights, know-how, data and other intellectual property of any kind (including
without limitation any proprietary biological or other materials, compounds or reagents, but not including Patent Rights). 
  
 1.43 “Term” shall mean the period commencing on September 4, 1998 and ending with the effective date of any termination of this
Agreement pursuant to Article 13 hereof. 
  
 1.44
“Territory” shall mean the world. 
  
 1.45
“Third Party” shall mean any entity other than BioMarin/Genzyme LLC, BioMarin or Genzyme and their respective Affiliates. 
  
 1.46 “Third Party Agreements” shall mean collectively those Third Party agreements listed on Schedule 1.47 hereto or such other
Third Party agreements pursuant to which a Party obtains 

  

 -7- 

 
rights applicable to the development, manufacture, sale or use of Collaboration Products hereunder. If after the Effective Date any of BioMarin, Genzyme
and/or BioMarin/Genzyme LLC enter into an agreement to license or acquire rights from a Third Party with respect to subject matter to be utilized in connection with Collaboration Products in accordance with Section 3.1.4 below, such agreements
shall also be included in the definition of Third Party Agreements for purposes of this Agreement. 
  
 ARTICLE II 
  
 SCOPE AND STRUCTURE OF THE COLLABORATION 
  
 2.1
General. BioMarin/Genzyme LLC will undertake the Development Program, with each of the Parties assuming responsibility for those portions of the Development Program allocated to it under this Agreement in accordance with the Development Plan
then in effect. Upon receipt of Regulatory Approval in any country within the Territory, the Manufacturing Party or Parties will manufacture the Collaboration Products and the Commercialization Party will distribute, market and sell the
Collaboration Products in such country all on the terms and conditions set forth in the Manufacturing, Marketing and Sales Agreement and such other terms and conditions as the Parties may agree upon. All services provided by or on behalf of
BioMarin, Genzyme or their respective Affiliates for BioMarin/Genzyme LLC in connection with the Program shall be provided at cost. For avoidance of doubt, “cost” for services provided by a Third Party on behalf of BioMarin, Genzyme or
their respective Affiliates for BioMarin/Genzyme LLC in connection with the Program shall be the amount paid for such services plus costs associated with the acquisition, including quality control, of such services from such Third Party. 

 
 2.2 Exclusive Relationship. Except as otherwise expressly provided
herein or in any Related Agreement, during the Term, neither BioMarin/Genzyme LLC, Genzyme nor BioMarin, nor any of their respective Affiliates shall independently, or with or through a Third Party, conduct research or development activities
regarding, or engage in the manufacture, marketing, sale or distribution of, Collaboration Products in the Field and in the Territory other than as part of the Program. In addition, during the two-year period following termination of this Agreement,
neither (a) the breaching Party and its Affiliates in the case of termination pursuant to Section 12.2.1 of the Manufacturing, Marketing and Sales Agreement, (b) (c) the terminating Party and its Affiliates in the case of
termination pursuant to Section 12.2.2 of the Manufacturing, Marketing and Sales Agreement or (d) the non-terminating Party and its Affiliates in the case of termination pursuant to Sections 12.2.3 or 12.2.4 of the Manufacturing,
Marketing and Sales Agreement shall independently, or with or through a Third Party, conduct research regarding, or engage in the manufacture, marketing, sale or distribution of, Collaboration Products in the Field and in the Territory; provided,
however, that in the event that this Agreement is terminated pursuant to Section 12.2.2 of the Manufacturing, Marketing and Sales Agreement and the non-terminating Party does not exercise its option under Section 12.3.2(a) thereof,
then the restrictions set forth in this sentence shall not apply. Notwithstanding the foregoing, except as provided in Section 3.1.5 nothing herein is intended to restrict BioMarin, Genzyme or their respective Affiliates from conducting
research or development activities regarding, or engaging in the manufacture, marketing, sale or distribution of Gene Therapy products targeted to MPS I and other alpha-L-iduronidase deficiencies. 
  

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 ARTICLE III 
  
 GRANTS AND RESERVATIONS OF RIGHTS 
  
 3.1 Licenses of Rights to BioMarin/Genzyme LLC. 
  
 3.1.1 Grants from BioMarin. Except as otherwise expressly provided herein, BioMarin hereby grants to
BioMarin/Genzyme LLC a worldwide, exclusive, royalty-free right and license during the term of this Agreement under the BioMarin Patent Rights, BioMarin Technology, BioMarin/Genzyme Patent Rights and the BioMarin/Genzyme Technology and Manufacturing
Know-How Controlled by BioMarin to develop, make, have made, use, offer for sale, sell, have sold, import and export Collaboration Products for use in the Field and in the Territory. 
  
 3.1.2 Grants from Genzyme. Except as otherwise expressly provided herein, Genzyme hereby grants to
BioMarin/Genzyme LLC a worldwide, exclusive, royalty-free right and license during the term of this Agreement under the Genzyme Patent Rights, Genzyme Technology, BioMarin/Genzyme Patent Rights and the BioMarin/Genzyme Technology and Manufacturing
Know-How Controlled by Genzyme to develop, make, have made, use, offer for sale, sell, have sold, import and export Collaboration Products for use in the Field and in the Territory. 
  
 3.1.3 BioMarin/Genzyme LLC Undertakings; Sublicenses. In consideration of the licenses granted under
this Section 3.1, except as provided herein, BioMarin/Genzyme LLC hereby undertakes to pay all royalties, sublicense fees and other costs or expenses payable to Third Parties under a Third Party Agreement associated with the acquisition or
exercise of such licenses by or on behalf of BioMarin/Genzyme LLC for use in connection with the Program. The licenses granted or to be granted under Sections 3.1.1 and 3.1.2 above shall include the right to grant and further authorize
sublicenses within the scope of such licenses; provided, however, all sublicenses granted by BioMarin/Genzyme LLC (other than those provided in Section 3.2.1 below) shall be subject to prior approval by the Steering Committee.
Notwithstanding anything to the contrary herein, pursuant to the Manufacturing, Marketing and Sales Agreement, Genzyme shall be solely responsible for all royalties payable to Third Parties under Third Party Agreements that are payable based on the
commercialization of Aldurazyme. 
  
 3.1.4
Rights of BioMarin/Genzyme LLC to Patent Rights or Technology Developed Outside the Program. In the event that either BioMarin or Genzyme develops, acquires or otherwise Controls Patent Rights, Technology or Manufacturing Know-How after the
Original Date of Execution other than in connection with the Program and such Patent Rights, Technology or Manufacturing Know-How are useful in the Field (“Additional Technology”), the Party Controlling such Additional Technology
hereby grants to BioMarin/Genzyme LLC an option exercisable at the discretion of the Steering Committee to obtain an exclusive, irrevocable (during the Term) right and license, with the right to grant sublicenses, to such Additional Technology
limited to use in the Field and in the Territory to the extent necessary or appropriate to enable BioMarin/Genzyme LLC to develop, make, have made, use, offer for sale, sell, have sold, import and export Collaboration Products, in each case subject
only to BioMarin/Genzyme LLC’s undertaking to pay (a) a commercially reasonable portion of all costs incurred by BioMarin or Genzyme, as the case may be, to acquire or develop such Additional Technology, (b) a commercially reasonable
portion of any and 

  

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all development costs relating to the Additional Technology incurred by BioMarin or Genzyme, as the case may be, since the date such Party acquired or
developed such Additional Technology and (c) a pro rata share of all royalties, sublicense fees and other costs or expenses payable to Third Parties under a Third Party Agreement associated with the acquisition or exercise of such
license by or on behalf of BioMarin/Genzyme LLC, allocated based upon the proportion of such costs attributable to the acquisition or use of such Additional Technology by BioMarin/Genzyme LLC; provided, however, that if BioMarin or Genzyme,
as the case may be, has more limited rights to such Additional Technology that those described above, the license subject to BioMarin/Genzyme LLC’s option hereunder shall be consistent with the rights held by BioMarin or Genzyme, as the case
may be, with respect to such Additional Technology. Subject to BioMarin/Genzyme LLC agreeing to pay the appropriate amounts due to a Third Party under an agreement with a Party as a result of the acquisition of Additional Technology and/or exercise
of the rights therein by or on behalf of BioMarin/Genzyme LLC, the same shall be a “Third Party Agreement” for purposes of this Agreement. 
  
 3.1.5 External Products. If at any time during the Term either Genzyme, BioMarin or their respective Affiliates intends to
collaborate with a Third Party regarding the development and/or commercialization of a Gene Therapy product for the treatment or prevention of MPS I or other alpha-L-iduronidase deficiencies (an “External Product”), such Party (the
“Proposing Party”) shall provide written notice of its intent to the Steering Committee. The Proposing Party and the Steering Committee shall negotiate in good faith the terms and conditions upon which the Proposing Party and
BioMarin/Genzyme LLC would be willing to collaborate for such purposes. If the Proposing Party and the Steering Committee are unable to agree upon such terms and conditions within sixty (60) days after receipt by the Steering Committee of the
Proposing Party’s notice, the Proposing Party shall have the right to develop or commercialize such External Product with a Third Party. 
  
 3.2 Sublicenses of Rights from BioMarin/Genzyme LLC to BioMarin and Genzyme. 
  
 3.2.1 General. BioMarin/Genzyme LLC hereby grants to each of BioMarin and Genzyme a worldwide,
non-exclusive, royalty-free right and sublicense during the Term under the Patent Rights, Technology and Manufacturing Know-How licenses granted to BioMarin/Genzyme LLC pursuant to Section 3.1 or under the Third Party Agreements solely to the
extent required to permit such Party to perform its duties and obligations and exercise its rights under this Agreement and any Related Agreement. BioMarin/Genzyme LLC also hereby agrees to grant to each of BioMarin and Genzyme a worldwide,
non-exclusive, royalty-free right and license during the Term under any Additional Technology as to which BioMarin/Genzyme LLC obtains a license pursuant to Section 3.1.4 above solely to the extent required to permit such Party to perform its
duties and obligations and exercise its rights under this Agreement and any Related Agreement. BioMarin/Genzyme LLC also hereby grants a worldwide, non-exclusive, royalty-free right and license during the Term to use any and all present and future
trademarks Controlled by BioMarin/Genzyme LLC (i) to Genzyme in connection with the commercialization of Aldurazyme in the Territory to the extent required to permit Genzyme to perform its duties and obligations and exercise its rights under
this Agreement and any Related Agreement with respect to Aldurazyme and (ii) the Commercialization Party in connection with the commercialization of any other Collaboration Product to the extent required to permit such Commercialization Party
to perform its duties and obligations and exercise its rights under this Agreement and any Related Agreement with respect to such Collaboration Product, in each case such licenses are subject to the quality-related requirements for Collaboration
Products set forth in this Agreement and the Related Agreements. 
  

 -10- 

 3.2.2 Further Sublicenses. The foregoing licenses granted to Genzyme and BioMarin,
respectively, shall include the right to grant and further authorize sublicenses to Third Parties within the scope of such licenses. 
  
 3.3 Reservation of Rights. 
  
 3.3.1 Reservation by BioMarin. Notwithstanding the license grants set forth in Section 3.1 but subject to the terms and
conditions set forth in this Agreement and the Manufacturing, Marketing and Sales Agreement, BioMarin at all times reserves the rights under the BioMarin Patent Rights, the BioMarin Technology, the BioMarin/Genzyme Patent Rights, the
BioMarin/Genzyme Technology and the Manufacturing Know-How Controlled by BioMarin (a) to make, have made and use Collaboration Products for research and development purposes only; (b) to develop, make, have made, use, offer for sale, sell,
have sold, import and export (i) products outside the Field and (ii) products other than a Collaboration Product; and (c) to grant licenses to Third Parties for the foregoing purposes. 
  
 3.3.2 Reservation by Genzyme. Notwithstanding the
license grants set forth in Section 3.1 but subject to the terms and conditions set forth in this Agreement and the Manufacturing, Marketing and Sales Agreement, Genzyme at all times reserves the rights under the Genzyme Patent Rights, the
Genzyme Technology, the BioMarin/Genzyme Patent Rights, the BioMarin/Genzyme Technology and Manufacturing Know-How Controlled by Genzyme (a) to make, have made and use Collaboration Products for research and development purposes only;
(b) to develop, make, have made, use, offer for sale, sell, have sold, import and export (i) products outside the Field and/or outside the Territory and (ii) products other than a Collaboration Product; and (c) to grant licenses
to Third Parties for the foregoing purposes. 
  
 3.4 Assignment
of Orphan Drug Designation. Except to the extent prohibited by the applicable Regulatory Scheme, BioMarin hereby assigns and BioMarin and Genzyme each hereby agree to assign to BioMarin/Genzyme LLC any “Orphan Drug” (or similar
designation outside the United States) for any Collaboration Product which BioMarin has received or which BioMarin or Genzyme may receive during the Term in the Territory. 
  

 3.5 Third Party Agreements. Each Party shall exercise their rights under the Third Party Agreements in a manner that is as
consistent as possible with the terms of this Agreement and the Related Agreements in consultation with and as reasonably requested by the other Parties. Without the prior written consent of the other Parties (which consent shall not be
unreasonably withheld, delayed or conditioned), none of the Parties shall voluntarily (i) amend or modify, or consent to any action that may be taken under, any Third Party Agreement, the effect of which would change any financial terms or
conditions thereunder or materially adversely affect the Parties’ rights under this Agreement or any Related Agreement, (ii) take or consent to any action taken with respect to any Patent Rights, Technology or Manufacturing Know-How
licensed under the Third Party Agreement, the effect of which would materially adversely affect the Party’s rights under this Agreement or any Related Agreement, or (iii) terminate or engage in any act or omission that constitutes or would
constitute, with or without the giving of notice or the passage of time, an event that would permit the 

  

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licensor under the Third Party Agreements to terminate the Third Party Agreements. Each Party shall immediately notify the other Parties of any such
event or of the receipt any notice of breach or termination of any Third Party Agreement. The Party who is a party to a Third Party Agreement shall take all reasonable actions necessary, or permit such actions to be taken in its name by the other
Parties, to maintain and enforce the Parties’ rights under such Third Party Agreement in a manner consistent with the terms of this Agreement and the Related Agreement. 
  
 ARTICLE IV 
  
 PROGRAM FUNDING 
  
 4.1 Program Funding Commitments. Genzyme hereby undertakes to make capital contributions to BioMarin/Genzyme LLC in an amount equal to fifty
percent (50%) of all Program Costs and BioMarin, on behalf of the BioMarin Companies, hereby undertakes to make capital contributions to BioMarin/Genzyme LLC in an aggregate amount equal to fifty percent (50%) of all Program Costs. In the
event that either BioMarin, on behalf of the BioMarin Companies, or Genzyme fails to make a capital contribution pursuant to this Section 4.1 and Section 4.2 below, and the other Party does not elect to terminate the Development Program
pursuant to Article 13 hereof, then the Percentage Interests in BioMarin/Genzyme LLC and the future funding responsibility of the Members shall be adjusted as provided in Section 4.1(b) of the Operating Agreement. 
  
 4.2 Program Funding Capital Contributions. 
  
 4.2.1 Quarterly Capital Contributions. Genzyme and
BioMarin, on behalf of the BioMarin Companies, shall each make capital contributions to BioMarin/Genzyme LLC, quarterly in advance, not later than the fifteenth (15th) day prior to the end of the prior calendar quarter, in an aggregate amount
equal to the Program Costs budgeted to be incurred by BioMarin/Genzyme LLC in the then-current Development Plan for such calendar quarter, allocated between such Parties in accordance with the funding responsibility assumed by Genzyme and BioMarin,
on behalf of the BioMarin Companies, pursuant to Section 4.1 above. Upon receipt of each such capital contribution from Genzyme or BioMarin, as the case may be, BioMarin/Genzyme LLC shall promptly pay each of the Parties an amount equal to that
portion of the budgeted Program Costs to which they are respectively entitled in accordance with this Agreement. 
  
 4.2.2 Monthly Statements; Quarterly Reconciliation. As soon as practicable, but in any event prior to the tenth
(10th) business day after the end of each calendar month, each of BioMarin and Genzyme shall provide BioMarin/Genzyme LLC with a detailed itemization of Program Costs actually incurred by such Party during the previous month. Within thirty
(30) days following receipt of the third monthly statement for each calendar quarter of actual Program Costs provided by each of BioMarin and Genzyme, BioMarin, on behalf of the BioMarin Companies, and Genzyme shall each make an additional
capital contribution to BioMarin/Genzyme LLC in the amount of any actual Program Costs shown on the three (3) monthly statements for such calendar quarter, taken in the aggregate, and not yet paid for which such Party has assumed funding
responsibility pursuant to Section 4.1 above but only to the extent that such amount, together with all prior capital contributions to date during such year, does not exceed [****] of the total Program 

  

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Costs budgeted year-to-date through the end of the quarter to which such statement relates (except to the extent such excess is approved by the Steering
Committee pursuant to Section 5.1.3 hereof). If the aggregate amount stated to be due from BioMarin/Genzyme LLC based on such quarterly reconciliation for actual Program Costs is less than the amount already contributed by the Parties to the
capital of BioMarin/Genzyme LLC with respect to budgeted Program Costs for such calendar quarter, such excess shall be credited against the next successive quarterly capital contribution(s) due from Genzyme or BioMarin hereunder. 
  
 4.3 Distributions. Distributions to each Member shall be made at such
times and in such amounts as determined in accordance with the Operating Agreement. 
  
 4.4 Books of Account; Audit. Genzyme shall keep and maintain proper and complete books of account, and shall maintain a bank account, on behalf of BioMarin/Genzyme LLC. In the event that either BioMarin or
Genzyme reasonably deems the Program to be material to BioMarin or Genzyme, as the case may be, for financial accounting purposes, then, upon such Party’s request, audited financial statements of BioMarin/Genzyme LLC shall be prepared by an
independent accounting firm to be selected by the Steering Committee. Each of BioMarin and Genzyme shall keep and maintain proper and complete records and books of account documenting all Program Costs incurred by such Party. Each of
BioMarin/Genzyme LLC, BioMarin and Genzyme shall permit independent accountants retained by BioMarin or Genzyme (the “Auditing Party”) to have access to its records and books for the sole purpose of determining the appropriateness of
Program Costs charged by or accrued to the Party being audited hereunder. Such examination shall be conducted during regular business hours and upon reasonable notice, at the Auditing Party’s own expense and no more than once in each calendar
year during the Term and once during the three (3) calendar years following the expiration or termination hereof. If such examination reveals that such Program Costs have been misstated, any adjustment shall be promptly refunded or paid, as
appropriate. The Auditing Party shall pay the fees and expenses of the accountant engaged to perform the audit, unless such audit reveals an overcharge or accrual of [****] or more for the period examined, in which case the Party who received such
overpayment shall pay all reasonable costs and expenses incurred by the Auditing Party in the course of making such determination, including the fees and expenses of the accountant along with interest at the rate set forth in Section 14.4.

  
 4.5 Enforceability of Sections 4.1 and 4.2. The
agreements regarding capital contributions set forth in Sections 4.1 and 4.2 hereof are by and between, and for the benefit of, Genzyme and BioMarin only, and are not enforceable by BioMarin/Genzyme LLC or any Third Party. 
  
 4.6 General and Administrative Services. Except as provided in this
Section 4.6, each of BioMarin and Genzyme shall continue to provide general and administrative services to BioMarin/Genzyme LLC after the Effective Date (in substantially the same manner and to the same extent as such Party has provided such
general and administrative services to BioMarin/Genzyme LLC prior to the Effective Date) at no cost to BioMarin/Genzyme LLC. To the extent that there are any Third Party costs (such as legal or accounting costs or insurance premiums), all such costs
shall be paid for [****]. 
  

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 ARTICLE V 
  

THE DEVELOPMENT PROGRAM 
  
 5.1 Conduct of the Development Program. 
  
 5.1.1 General. The Parties agree to use commercially reasonable and diligent efforts to execute and substantially perform and to
cooperate with each other in carrying out the Development Plan for each Collaboration Product. Neither BioMarin nor Genzyme shall be required to undertake activities in furtherance of the Development Plan in the absence of funding from
BioMarin/Genzyme LLC pursuant to the provisions of this Agreement. As used in this Agreement, the phrase “commercially reasonable and diligent efforts” will mean that level of effort which, consistent with the exercise of prudent
scientific and business judgment, is applied by the Party in question to its other therapeutic products at a similar stage of development and with similar commercial potential. 
  
 5.1.2 Development Plan. The Development Program shall be conducted by the Parties for
BioMarin/Genzyme LLC in accordance with the then-current Development Plan which shall describe the proposed overall program of development for each Collaboration Product, including preclinical studies, toxicology, formulation, manufacturing,
clinical trials and regulatory plans and other key elements. Pursuant to the Development Plan, development work may be subcontracted to Genzyme and BioMarin or their respective Affiliates, at fully absorbed costs determined by GAAP. The respective
charges to BioMarin/Genzyme LLC for Development Costs incurred by a Party shall be invoiced following completion of the work, and shall be payable by BioMarin/Genzyme LLC within a commercially reasonable time thereafter (but in no event later than
forty-five (45) days of the date of invoice therefor). The Development Plan shall include (i) a summary of estimated Development Costs expected to be incurred by each Party hereunder in performing activities of the Development Program
assigned to such Party pursuant to Section 5.1.4 below and (ii) a summary budget for all development projects proposed for the applicable period and for each Collaboration Product. 
  
 5.1.3 Initial and Updated Development Plan. The
Parties have agreed to an initial three (3) year development plan and budget for the period beginning on the Effective Date and ending on December 31, 2010, which is attached hereto as Appendix A. The rolling three (3) year
Development Plan shall be updated annually by the Program Management Team and submitted to the Steering Committee for review and approval not later than sixty (60) days prior to January 1 of each year during the Development Program. Each
such updated Development Plan shall include (a) an overall development plan for each Collaboration Product which sets forth all major development tasks and (b) a detailed description and budget for the activities proposed for the covered
period. The Project Management Team shall be primarily responsible for preparing the annual updates to the Development Plan and, in connection with the preparation of such updates, shall consult with Genzyme and BioMarin regarding the
identification, timing and execution of and budget for the major tasks and detailed activities required to perform the updated Development Plan. Each such updated Development Plan approved by the Steering Committee shall be signed by an authorized
representative of each of BioMarin and Genzyme. The members of the Program Management Team shall actively consult with one another throughout the term of the Development Plan so as to adjust 

  

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the specific work performed under the Development Plan to conform to evolving developments in technology and the results of the development work performed.
Any changes in the scope or direction of the work and any changes to the total amount budgeted in any calendar year for the Development Program must be approved by the Steering Committee, in the absence of which approval the most recently approved
Development Plan shall remain in effect. 
  
 5.1.4 Studies Required by Regulatory Authorities. 
  
 (a) Existing Markets. In the event that any regulatory authority in any country in which Aldurazyme has received all necessary Regulatory Approvals as of the Effective Date (“Existing Markets”)
requires a post-marketing study related to Aldurazyme (other than any registry program existing on the Effective Date or post-marketing studies related to the manufacture of Aldurazyme, each of which shall be governed by the terms and conditions of
the Manufacturing, Marketing and Sales Agreement) that is not contemplated by the initial Development Plan described above, then such study shall automatically be added to the Development Program and (i) the Program Management Team shall
promptly prepare a plan for the conduct of such required post-marketing study (including without limitation a timeline and reasonably detailed budget) and submit the plan to the Steering Committee for review and approval and (ii) the Steering
Committee shall promptly review such plan and, upon approval of such plan by the Steering Committee, the then-current Development Plan shall automatically be updated to include the study and the budget included in the approved plan; provided,
however, that in the event that any regulatory authority requires a post-marketing study to be conducted through or as part of the registry program, then, notwithstanding anything to the contrary herein, such study and the incremental costs
associated with conducting such study (i.e., such costs above the general cost of maintaining the registry program) shall be added to the Development Plan in accordance with the process set forth in this Section 5.1.4(a). 
  
 (b) New Markets. In the event that clinical studies
are required to apply for Regulatory Approval for Aldurazyme in any country that is not an Existing Market and/or the regulatory authority in any such country requires a post-marketing study(ies) as a condition to granting or maintaining Regulatory
Approval for Aldurazyme, then (i) the Program Management Team shall promptly prepare a plan for the conduct of such study(ies) (including without limitation a timeline and reasonably detailed budget) and submit the plan to the Steering
Committee for review and approval and (ii) if the Steering Committee approves the plan, such study(ies) will be added to the Development Program and the then-current Development Plan shall automatically be updated to include such study(ies) and
the budget included in the approved plan; provided, however, that in the event that any regulatory authority requires any such post-marketing study to be conducted through or as part of the registry program, then the budget for such study
shall reflect the incremental costs associated with conducting such study (i.e., such costs above the general cost of maintaining the registry program). 
  
 5.1.5 Other Development Activities. In the event that either BioMarin or Genzyme desires to engage in any research or development
activities related to Aldurazyme or other Collaboration Products that are not contemplated by an existing Development Plan or covered by Section 5.1.4 above (including without limitation through investigator sponsored studies), then such Party
shall submit a proposal to the Steering Committee for such activities (including a reasonably detailed budget therefore), and the Steering Committee shall decide within ninety (90) days after 

  

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receipt thereof whether such activities will be added to the Development Program. If the Steering Committee elects to add such activities to the Development
Program, then it shall promptly amend the then-current Development Plan to include such activities and the related budget. If the Steering Committee declines or fails to make such election, then the Party who submitted the proposal shall be free to
conduct such research or development activities independently at its own expense subject to the terms of the Manufacturing, Marketing and Sales Agreement ; provided, however, that any and all Patent Rights, Technology and Manufacturing
Know-How developed as a result of those activities shall be subject to the rights and licenses granted to the Parties pursuant to Article 3 of this Agreement. 
  

5.1.6 Execution and Performance. The Development Program shall allocate among the Parties responsibility for each of the
activities described therein. The Parties shall use commercially reasonable and diligent efforts to conduct the activities described in the Development Plan. The Development Plan shall be supervised by the Program Management Team. The Program
Management Team will coordinate preclinical and clinical testing of the Collaboration Products in the Territory and work with designated individuals at BioMarin and Genzyme in the preparation of Regulatory Approval filings for the Collaboration
Products and filing the same with regulatory agencies designated by the Steering Committee. 
  
 5.1.7 Attendance at Regulatory Meetings; Correspondence. Each Party shall provide the others with prior notice of all meetings and
teleconferences between representatives of the notifying Party and regulatory authorities regarding any Collaboration Product for use in the Territory. Except as otherwise provided herein, the Party receiving such notice shall have the right to have
representatives participate in all such meetings and teleconferences. Each Party shall use reasonable efforts to provide the other Party with a reasonable opportunity to review and comment upon submissions to, and correspondence with, any regulatory
agency in the Territory with respect to Collaboration Products prior to the filing or delivery of such submissions or correspondence. Without limiting the foregoing, each Party shall use reasonable efforts to confirm in writing to the other Party
all communications with a regulatory authority with respect to a Regulatory Approval (including filings therefor) and to provide to the other Party copies of all documents sent to or received from such regulatory authority regarding such Regulatory
Approvals. 
  
 5.2 Development Information. 
  
 5.2.1 Reports and Information Exchange. As between
the Parties hereto, (a) BioMarin/Genzyme LLC shall own all clinical trial data accumulated from all clinical trials of Collaboration Products conducted as part of the Program or otherwise funded or partially funded by BioMarin/Genzyme LLC and
(b) subject to the license granted pursuant to Section 3.1 above, BioMarin or Genzyme, as the case may be, shall own all clinical trial data accumulated from all clinical trials of Collaboration Products that are not conducted as part of
the Program. Each of BioMarin and Genzyme shall use commercially reasonable and diligent efforts to disclose to BioMarin/Genzyme LLC and to the other Party all material information relating to any Collaboration Product promptly after it is learned
or its materiality is appreciated. The Party performing or supervising clinical trials of Collaboration Products in accordance with the Development Plan shall, on behalf and in the name of BioMarin/Genzyme LLC, maintain the database of clinical
trial data accumulated from all clinical trials of Collaboration Products and of adverse reaction information for all such Collaboration Products. Each Party shall also keep the 

  

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Program Management Team informed as to its progress in the Development Plan. All protocols for clinical trials to be conducted, and all product registration
plans, for Collaboration Products for applications within the Field in the Territory shall be submitted to the Program Management Team for review and comment by the Program Management Team prior to filing of such protocols or registrations with any
regulatory agency. Within sixty (60) days following the end of each calendar quarter during the Development Program, each of BioMarin and Genzyme shall provide the other Parties with a reasonably detailed written report describing the progress
to date of all activities for which such Party was allocated responsibility during such quarter under the Development Plan. 
  
 5.2.2 Adverse Reaction Reporting. Each of BioMarin and Genzyme shall notify the other Parties of any Adverse Reaction Information
relating to any Collaboration Product within twenty-four (24) hours of the receipt of such information and as necessary for compliance with regulatory requirements. “Adverse Reaction Information” includes without limitation
information relating to any experience that (a) suggests a significant hazard, contraindication, side effect or precaution, (b) is fatal or life threatening, (c) is permanently disabling, (d) requires or prolongs inpatient
hospitalization, (e) involves a congenital anomaly, cancer or overdose or (f) is one not identified in nature, specificity, severity or frequency in the current investigator brochure or the United States labeling for the Collaboration
Product. 
  
 5.2.3 Clinical and Regulatory
Audits. Each of BioMarin and Genzyme shall permit BioMarin/Genzyme LLC and the other Party or the representatives of BioMarin/Genzyme LLC or the other Party to have access during regular business hours and upon reasonable advance notice, at the
auditing Party’s own expense and no more than once in each calendar year during the Term, to the non-auditing Party’s records and facilities relating to the Development Program for the purpose of monitoring compliance with Good Clinical
Practice and other applicable requirements of the Regulatory Scheme in connection with such Party’s performance of its obligations hereunder. 
  
 5.3 Regulatory Approval Filings. Except as set forth in the Manufacturing, Marketing and Sales Agreement, Regulatory Approval filings in the
Territory for the Collaboration Products and for the facilities used to manufacture such Collaboration Products shall be filed in the name of BioMarin/Genzyme LLC or, if required with respect to filings to be made with governmental authorities or
deemed to be in the best interest of the Parties by the Steering Committee, in the name of such other entity as may be agreed upon by the Steering Committee (such as filings with European regulatory authorities). Prior to submission to the FDA, the
Parties, through the Program Management Team, shall consult, cooperate in preparing and mutually agree on the content and scope of such Regulatory Approval filings. In the event that Regulatory Approvals are required to be filed in the name of an
entity other than BioMarin/Genzyme LLC, the Steering Committee shall ensure that a duly authorized officer of such entity agrees in writing that (a) such entity shall hold the licenses issued in respect of such Regulatory Approval filings,
maintain control over the manufacturing facilities, equipment and personnel, and engage in pharmacovigilence to the extent required by the Regulatory Scheme, (b) such entity shall maintain compliance with applicable Regulatory Schemes,
(c) such entity shall provide manufacturing and supply services to BioMarin/Genzyme LLC at the Fully Absorbed Cost of Goods of Collaboration Products so manufactured and supplied, (d) the Parties shall have an irrevocable right of access
and reference to such Regulatory Approval filings, licenses and facilities and (e) such entity agrees to comply with the provisions of Article 12 of the Manufacturing, Marketing and Sales Agreement with respect to the ownership and/or
disposition of such Regulatory Approvals in the event this Agreement is terminated and to provide the level of cooperation described in Section 14.1 hereof in connection therewith. 
  

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 5.4 Clinical Data. In all agreements with Third Parties or Affiliates involving the development of
preclinical or clinical data for a Collaboration Product, Genzyme and BioMarin shall require that such Third Parties and Affiliates provide BioMarin/Genzyme LLC and the other Party access to all such data, to the extent such data is required to be
obtained from such Third Parties by the Japanese Ministry of Health and Welfare, the U.S. FDA, the Commission of Proprietary Medicines of the European Community, the European Medicines Evaluation Agency or other regulatory agency, in each case with
respect to Regulatory Approvals. 
  
 5.5 Facilities Visit.
Representatives of BioMarin and Genzyme may visit all manufacturing sites and the sites of any clinical trials or other experiments being conducted by the other Party or BioMarin/Genzyme LLC in connection with the Development Program. If requested
by the other Party, BioMarin and Genzyme shall cause appropriate individuals working on the Development Program to be available for meetings at the location of the facilities where such individuals are employed at times reasonably convenient to the
Party responding to such request. 
  
 ARTICLE VI 

 
 [RESERVED] 
  
 ARTICLE VII 
  
 MANUFACTURE AND SUPPLY; SALES AND MARKETING 
  
 Subject to the terms and conditions of this Agreement, Collaboration Products
shall be manufactured and supplied for preclinical and clinical testing and for commercial sale upon the following terms and conditions. For purposes of this Article 7, the term “Collaboration Products” shall be deemed to exclude
Aldurazyme except as explicitly provided below. 
  
 7.1
General. For any Collaboration Product that may be commercialized, the Parties shall negotiate in good faith to include the additional Collaboration Product to the Manufacturing Marketing and Sales Agreement to allow for BioMarin to
manufacture such Collaboration Product and for Genzyme to commercialize such Collaboration Product (the “Commercializing Party”) on financial terms and such other terms and conditions appropriate for such Collaboration Product as
may be mutually agreed upon by the Parties. 
  
 7.2 Manufacture
and Supply of Collaboration Products. BioMarin/Genzyme LLC shall manufacture (or, subject to Section 7.2.1, have manufactured) and supply Collaboration Products for preclinical and clinical activities upon the following terms and conditions
(and such other terms and conditions established by the Steering Committee consistent with the provisions of this Agreement): 
  
 7.2.1 General. All decisions relating to the manufacture of Collaboration Products shall be subject to the approval of, or
modification by, the Steering Committee. 
  

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 7.2.2 Facilities. Notwithstanding any provision of this Agreement to the contrary,
the Parties acknowledge and agree that BioMarin/Genzyme LLC shall not bear any costs relating to the construction of manufacturing facilities for a Collaboration Product (other than through normal depreciation and amortization included in Fully
Absorbed Cost of Goods). 
  
 7.3 Manufacture and Supply of
Aldurazyme for the Development Program. BioMarin shall use commercially reasonable and diligent efforts to manufacture and supply Aldurazyme (either itself or through Third Parties) for activities undertaken pursuant to the Development Plan in
quantities and within a time period sufficient to conduct the activities set forth in the Development Plan and BioMarin/Genzyme LLC and the Fully Absorbed Cost of Goods of such Aldurazyme shall be included as Development Costs. 
  
 7.4 Certificates of Analysis. The Manufacturing Party shall perform,
or cause its contract manufacturer(s) to perform, quality assurance and control tests on each lot of Collaboration Products before delivery and shall prepare, or cause its contract manufacturer(s) to prepare and deliver, a written report of the
results of such tests (for purposes of Sections 7.2, 7.3 and 7.4, such contract manufacturer(s) shall be included in the definition of the term “Manufacturing Party”). Each test report shall set forth for each lot delivered the items
tested, specifications and results in a certificate of analysis containing the types of information which shall have been approved by the Program Management Team or required by the FDA or other applicable regulatory authority. The Manufacturing
Party shall maintain such certificates for a period of not less than five (5) years from the date of manufacture or for such longer period as required under applicable requirements of the FDA or other applicable regulatory authority.

  
 7.5 Certificates of Manufacturing Compliance. The
Manufacturing Party shall prepare, or cause to be prepared and delivered, and maintain for a period of not less than five (5) years or for such longer period as required under applicable requirements of the FDA or other applicable regulatory
authority for each lot of Collaboration Products manufactured a certificate of manufacturing compliance containing the types of information which shall have been approved by the Program Management Team or required by the FDA or other applicable
regulatory authority, which certificate will certify that the lot of Collaboration Products was manufactured in accordance with the Specifications and the Good Manufacturing Practices of the FDA or other applicable regulatory authority as the same
may be amended from time to time. The Manufacturing Party shall advise the other Parties immediately if an authorized agent of the FDA or other regulatory authority visits any of the Manufacturing Party’s manufacturing facilities, or the
facilities where the Collaboration Products are being manufactured, for an inspection with respect to the Collaboration Products. The Manufacturing Party shall furnish to the other Parties the report by such agency of such visit, to the extent that
such report relates to Collaboration Products, within ten (10) business days of the Manufacturing Party’s receipt of such report, and the other Parties shall have the right to comment on any response by the Manufacturing Party to such
inspecting agency. 
  
 7.6 Access to Facilities. Each Party
shall have the right to audit annually those portions of the manufacturing, finish processing or storage facilities of the Manufacturing Party where Collaboration Products are being manufactured, finished or stored, or any subcontractor who is
manufacturing, finishing or storing Collaboration Products for the Manufacturing Party, at any time during regular business hours and upon reasonable advance notice to ascertain compliance with the Good Manufacturing Practices of the FDA or other
applicable regulatory authority, as the same may be amended from time to time. Subject to the terms and conditions of Section 10.1 below, confidential information disclosed to or otherwise gathered by the Party conducting such inspection during
any such inspection shall be maintained as confidential. 
  

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 7.7 Responsibilities of the Other Parties. No Party other than the Commercialization Party shall
actively solicit for its own account sales of Collaboration Products in the Territory. Any solicitations or requests to purchase Collaboration Products received by a Party other than the Commercialization Party from any customer or prospective shall
be immediately referred to the Commercialization Party. 
  
 ARTICLE VIII 
  
 MANAGEMENT 
  
 8.1 Program Management Team. 
  
 8.1.1 General. The Parties have established a Program
Management Team to oversee and control development of Collaboration Products. The Program Management Team is and shall continue to be composed of four (4) representatives appointed by BioMarin and four (4) representatives appointed by
Genzyme. Such representatives will include individuals with expertise and responsibilities in such areas as preclinical development, clinical development, manufacturing, and regulatory affairs. The Program Management Team shall meet as needed but
not less than monthly. The Program Management Team shall appoint one of its members to act as Secretary. Such meetings shall be at times and places or in such form (e.g., telephone or video conference) as the members of the Program Management Team
shall agree. A Party may change one or more of its representatives to the Program Management Team at any time. Members of the Program Management Team may be represented at any meeting by another member of the Program Management Team or by a deputy.
Any approval, determination or other action agreed to by a majority of the members of the Program Management Team appointed by each of BioMarin and Genzyme or their deputies present at the relevant Team meeting shall be the approval, determination
or other action of the Program Management Team, provided at least two (2) representatives of each of BioMarin and Genzyme are present at such meeting. Representatives of either BioMarin and Genzyme who are not members of the Program Management
Team may attend meetings of the Program Management Team as agreed to by the representative members of the other Party. The Program Management Team may designate project leaders to the extent it deems it necessary or advisable. The Parties
specifically agree that the Product Management Team has no authority to oversee or control the manufacture or commercialization of Aldurazyme and that all such activities shall be governed by the Manufacturing, Marketing and Sales Agreement.

  
 8.1.2 Development Program Functions.
During the term of the Development Program, the Program Management Team shall coordinate, expedite and control the development of Collaboration Products. The Program Management Team will (a) develop and recommend to the Steering Committee
Development Plans (including annual development budgets), (b) facilitate the flow of information with respect to development work being conducted for each Collaboration Product throughout the Territory and (c) discuss and cooperate
regarding the conduct of such development work. 
  

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 8.1.3 Minutes. The Program Management Team shall keep accurate minutes of its
deliberations which shall record all proposed decisions and all actions recommended or taken. The Secretary shall be responsible for the preparation of draft minutes. Draft minutes shall be sent to all members of the Program Management Team within
five (5) working days after each meeting and shall be approved, if appropriate, at the next meeting. All records of the Program Management Team shall at all times be available to all of the Parties. 
  
 8.2 Steering Committee. 
  
 8.2.1 General. The Parties have established a
Steering Committee to oversee and manage the collaboration contemplated by this Agreement. The Steering Committee is and shall continue to be composed of three (3) representatives appointed by BioMarin and three (3) representatives
appointed by Genzyme. Such representatives will be senior officers and/or managers of their respective companies. Genzyme and BioMarin shall each designate one (1) of their respective representatives on the Steering Committee to act as
Co-Chairman. The Steering Committee shall appoint one (1) of its members to act as Secretary. The Steering Committee will meet as needed but not less than once each calendar quarter. Such meetings shall be at times and places or in such form
(e.g., telephone or video conference) as the members of the Steering Committee shall agree. A Party may change one or more of its representatives to the Steering Committee at any time. Members of the Steering Committee may be represented at any
meeting by another member of the Steering Committee or by a deputy. Any approval, determination or other action agreed to by unanimous consent of the members of the Steering Committee or their deputies present at the relevant Steering Committee
meeting shall be the approval, determination or other action of the Steering Committee, provided at least two (2) representatives of each of BioMarin and Genzyme are present at such meeting. Representatives of either BioMarin and Genzyme who
are not members of the Steering Committee may attend meetings of the Steering Committee as agreed to by the representative members of the other Party. Each Party shall bear its own personnel and travel costs and expenses relating to Steering
Committee meetings, which costs and expenses shall not be included in the Program Costs. The Parties specifically agree that the Steering Committee has no authority to oversee or control the manufacture or commercialization of Aldurazyme and that
all such activities shall be governed by the Manufacturing, Marketing and Sales Agreement. 
  
 8.2.2 Functions. The Steering Committee shall perform the following functions: (a) determine the overall strategy for the
Program in the manner contemplated by this Agreement; (b) coordinate the activities of the Parties hereunder; (c) settle disputes or disagreements that are unresolved by the Program Management Team; (d) approve any agreements with
Third Parties regarding a Collaboration Product or which involve the grant of any rights related to the development of a Collaboration Product or manufacturing of a Collaboration Product other than Aldurazyme; (e) review and approve each
Development Plan, including each change and annual update thereto, submitted to it pursuant to Section 5.1.3 hereof; (f) serve as the governing body of BioMarin/Genzyme LLC; and (g) perform such other functions as appropriate to
further the purposes of this Agreement as determined by the Parties. 
  

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 8.2.3 Minutes. The Steering Committee shall keep accurate minutes of its
deliberations which shall record all proposed decisions and all actions recommended or taken. The Secretary shall be responsible for the preparation of draft minutes. Draft minutes shall be sent to all members of the Steering Committee within ten
(10) working days after each meeting and shall be approved, if appropriate, at the next meeting. All records of the Steering Committee shall at all times be available to both BioMarin and Genzyme. 
  
 8.3 General Disagreements. All disagreements within the Program
Management Team or the Steering Committee shall be subject to the following: 
  
 (a) The representatives to the Program Management Team or Steering Committee (as the case may be) will negotiate in good faith for a period of not less than thirty (30) days to attempt to resolve the dispute. In
the case of the Program Management Team, any unresolved dispute shall be referred to the Steering Committee for good faith negotiations for an additional period of not less than thirty (30) days to attempt to resolve the dispute. 
  
 (b) In the event that the dispute is not resolved after the
period specified in clause (a), the representatives shall promptly present the disagreement to the Chief Executive Officers of BioMarin and Genzyme or a designee of such Chief Executive Officer reasonably acceptable to the other Party. 

 
 (c) Such executives shall meet or discuss in a telephone
or video conference each of BioMarin and Genzyme’s views and explain the basis for such dispute. 
  
 (d) If such executives cannot resolve such disagreement within thirty (30) days after such issue has been referred to them, then such
dispute shall be referred to arbitration as described in Section 14.10 hereof. 
  
 ARTICLE IX 
  
 INTELLECTUAL PROPERTY RIGHTS 
  
 9.1
Ownership. The Parties acknowledge that the ownership rights set forth herein (a) shall not be affected by the participation in the discovery or development of an Invention (as defined below) by the Program Management Team or the
Steering Committee in the course of discharging their duties hereunder and (b) are subject to the license grants set forth in Article 3 above. 
  
 9.1.1 Ownership and Assignment of Discoveries and Improvements. All right, title and interest in all writings, inventions,
discoveries, improvements and other technology, whether or not patentable or copyrightable, and any patent applications, patents or copyrights based thereon (collectively, the “Inventions”) that are discovered, made or conceived
during and in connection with the Program solely by employees of BioMarin or others acting on behalf of BioMarin (“BioMarin Inventions”) shall be owned by BioMarin. All right, title and interest in all Inventions that are
discovered, made or conceived during and in connection with the Program solely by employees of Genzyme or others acting on behalf of Genzyme (“Genzyme Inventions”) shall be owned by Genzyme. All right, title and interest in all
Inventions that are discovered, made or conceived during 

  

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and in connection with the Program jointly by employees of BioMarin and Genzyme (“Joint Inventions”) shall be jointly owned by Genzyme and
BioMarin. Each of BioMarin and Genzyme shall promptly disclose to BioMarin/Genzyme LLC and the other Party the making, conception or reduction to practice of Inventions by employees or others acting on behalf of such Party. All BioMarin Inventions,
Genzyme Inventions and Joint Inventions shall be automatically licensed to BioMarin/Genzyme LLC pursuant to Section 3.1 hereof. Except as expressly provided in this Agreement, it is understood that neither BioMarin nor Genzyme shall have any
obligation to account to the other Party for profits, or obtain any approval of the other to grant a license or exploit a Joint Invention outside of the Field by reason of joint ownership of such Invention or other intellectual property. For
avoidance of doubt, in any jurisdiction where consent of all owners of a Joint Invention is required in order to grant a license to such Invention, BioMarin and Genzyme each grants the other Party consent to grant a non-exclusive license to Joint
Invention outside the Field. 
  
 9.1.2
Ownership of Trademarks. The Steering Committee shall select and as between the Parties hereto BioMarin/Genzyme LLC shall own all trademarks for the sale and use of Collaboration Products in the Territory (collectively, “Product
Marks”) and all goodwill therein shall inure to the benefit of BioMarin/Genzyme LLC, and all expenses incurred by a Party with respect thereto shall be considered Program Costs. All Product Marks shall be registered in the name of
BioMarin/Genzyme LLC if and when registered. In the event that the applicable laws and regulations of any country in which the Steering Committee elects to register any Product Marks require that such trademark(s) be registered in the name of an
entity other than BioMarin/Genzyme LLC, or if the Steering Committee determines that it is in the best interests of the Parties, then the Steering Committee shall select such entity and ensure that a duly authorized officer of such entity agrees in
writing that such entity shall (a) grant BioMarin/Genzyme LLC a worldwide, exclusive, fully-paid, royalty-free, irrevocable right and license (with the right to grant and authorize sublicenses) to use such Product Marks and (b) comply with
the provisions of Article 12 of the Manufacturing, Marketing and Sales Agreement with respect to the ownership and/or disposition of such Product Marks in the event this Agreement is terminated and provide the level of cooperation described in
Section 14.1 hereof in connection therewith. Each Party hereby acknowledges agrees that at no time during of this Agreement to challenge or assist others to challenge the Product Marks or the registration thereof or attempt to register any
trademarks, marks or trade names confusingly similar to such Product Marks. 
  
 9.1.3 Cooperation of Employees. Each of BioMarin and Genzyme represents and agrees that all employees or others acting on its behalf in performing its obligations under this Agreement shall be obligated under a
binding written agreement to assign to such Party, or as such Party shall direct, all Inventions made or conceived by such employee or other person. In the case of non-employees working for other companies or institutions on behalf of BioMarin or
Genzyme, BioMarin or Genzyme, as applicable, shall have the right to obtain licenses for all Inventions made by such non-employees on behalf of BioMarin or Genzyme, as applicable, in accordance with the policies of said company or institution.
BioMarin and Genzyme agree to undertake to enforce such agreements (including, where appropriate, by legal action) considering, among other things, the commercial value of such Inventions. 
  
 9.2 Filing, Prosecution and Maintenance of Patent Rights. BioMarin
shall be responsible, at BioMarin’s expense, for the filing, prosecution and maintenance of Patent Rights within the BioMarin Patent Rights and BioMarin/Genzyme Patent Rights, and Genzyme shall be responsible, at 

  

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Genzyme’s expense, for the filing, prosecution and maintenance of Patent Rights within the Genzyme Patent Rights. For so long as any of the license
grants set forth in Article 3 hereof remain in effect and upon request of the other Party, each of BioMarin and Genzyme agrees to file and prosecute patent applications and maintain the Patent Rights for which it is responsible in all countries in
the Territory selected by the Steering Committee. Each of BioMarin and Genzyme shall consult with and keep the other Party fully informed of important issues relating to the preparation and filing (if time permits), prosecution and maintenance of
such patent applications and patents, and shall furnish to the other Party copies of documents relevant to such preparation, filing, prosecution or maintenance in sufficient time prior to filing such document or making any payment due thereunder to
allow for review and comment by the other Party and, to the extent possible in the reasonable exercise of its discretion, the responsible Party shall incorporate all such comments. 
  
 9.3 Cooperation. Each of BioMarin and Genzyme shall make available to the other Party (or to the other Party’s
authorized attorneys, agents or representatives) its employees, agents or consultants to the extent necessary or appropriate to enable BioMarin to file, prosecute and maintain patent applications and resulting patents with respect to inventions
owned by a Party and for periods of time sufficient for such Party to obtain the assistance it needs from such personnel. Where appropriate, each of BioMarin and Genzyme shall sign or cause to have signed all documents relating to said patent
applications or patents at no charge to the other Party. 
  
 9.4
Notification of Patent Term Restoration. BioMarin shall notify Genzyme of (a) the issuance of each patent included within the Patent Rights, giving the date of issue and patent number for each such patent, and (b) each notice
pertaining to any patent included within the Patent Rights which it receives as patent owner pursuant to the Drug Price Competition and Patent Term Restoration Act of 1984, including notices pursuant to §§101 and 103 of such Act from
persons who have filed an abbreviated NDA. Such notices shall be given promptly, but in any event within ten (10) business days after receipt of each such notice pursuant to such Act. BioMarin shall notify Genzyme of each filing for patent term
restoration under such Act, any allegations of failure to show due diligence and all awards of patent term restoration (extensions) with respect to the Patent Rights. 
  
 9.5 No Other Technology Rights. Except as otherwise expressly provided in this Agreement and the Related Agreements,
under no circumstances shall a Party hereto, as a result of this Agreement, obtain any ownership interest in or other right to the Patent Rights, Technology or Manufacturing Know-How of the other Party, including items owned, controlled or developed
by the other Party, or transferred by the other Party to said Party at any time pursuant to this Agreement. It is understood and agreed that this Agreement does not grant either Party any license or other right in the Patent Rights of the other
Party except as expressly provided in Article 3 hereof and this Article 9. 
  
 9.6 Defense of Third Party Infringement Claims. If the manufacture, production, sale or use of any Collaboration Product pursuant to this Agreement results in a claim, suit or proceeding (collectively,
“Actions”) alleging patent infringement against BioMarin or Genzyme (or their respective Affiliates), such Party shall promptly notify the other Party hereto in writing. The Party subject to such Action (for purposes of this
Section 9.6, the “Controlling Party”) shall have the exclusive right to defend and control the defense of any such Action using counsel of its own choice; provided, however, that if such Action is directed to the subject
of the Patent Rights of the other Party (i.e., the BioMarin Patent Rights or the Genzyme Patent Rights), such other Party may 

  

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participate in the defense and/or settlement thereof at its own expense with counsel of its choice. Except as agreed in writing by Genzyme and BioMarin, the
Controlling Party shall not enter into any settlement relating to a Collaboration Product, if such settlement admits the invalidity or unenforceability of any Patent Rights within the BioMarin Patent Rights or the Genzyme Patent Rights, as
applicable, of the other Party. The Controlling Party agrees to keep the other Party hereto reasonably informed of all material developments in connection with any such Action. Any cost, liability or expense (including amounts paid in settlement)
incurred by the Controlling Party as a result of such Action shall be included in Development Costs for the Collaboration Product(s) that are the subject of such Action and shall not be subject to the limitations of Sections 1.11, 4.2 and 5.1.3
above provided that the other Party consents to incurrence of such cost, liability or expense, with such consent not to be unreasonably withheld, delayed or conditioned. 
  

 9.7 Enforcement of Patent Rights. 
  
 9.7.1 Enforcement. Subject to the provisions of this Section 9.7, in the event that BioMarin or Genzyme reasonably believes
that any BioMarin Patent Rights, BioMarin Technology, Genzyme Patent Rights, Genzyme Technology, BioMarin/Genzyme Patent Rights or BioMarin/Genzyme Technology necessary for the manufacture, use or sale of a Collaboration Product in the Field is
infringed or misappropriated by a Third Party or is subject to a declaratory judgment action arising from such infringement in a country, in each case with respect to the manufacture, sale or use of a product potentially competitive with a
Collaboration Product within the Field, Genzyme or BioMarin (respectively) shall promptly notify the other Party hereto. Promptly after such notice the Parties shall meet to discuss the course of action to be taken with respect to an Enforcement
Action (as defined below) with respect to such infringement or misappropriation, including the control thereof and sharing of costs and expenses related thereto, for the purposes of entering into a litigation agreement setting forth the same
(“Litigation Agreement”). If the Parties do not enter such Litigation Agreement, the Party whose Patent Rights or Technology is so allegedly infringed or misappropriated, or is subject to such declaratory judgment action, (for
purposes of this Section 9.7, the “Owner”) shall have the initial right (but not the obligation) to enforce the intellectual property rights within such Patent Rights or Technology, or defend any declaratory judgment action
with respect thereto (for purposes of this Section 9.7, an “Enforcement Action”); provided that the Owner agrees to indemnify the other Party for any and all liabilities and expenses (including, without limitation, reasonable
attorneys’ fees and other expenses of litigation) incurred by such other Party as a result of such Enforcement Action. 
  
 9.7.2 Information. Absent a Litigation Agreement, the Party initiating or defending any such Enforcement Action shall keep the
other Party hereto reasonably informed of the progress of any such Enforcement Action, and such other Party shall have the right to participate with counsel of its own choice at its own expense. 
  
 9.7.3 Enforcement Costs; Recoveries. Unless otherwise
agreed, the Party initiating an Enforcement Action shall, at the option of such Party, have the right to either: (i) assume responsibility for all costs and expenses of such Enforcement Action, in which case all amounts recovered in the
Enforcement Action (including without limitation amounts resulting from a settlement thereof) shall be retained by such Party; or (ii) include such costs and expenses within the Development Costs, in which case all amounts recovered in the
Enforcement Action, after reimbursing the Party initiating the Action for any costs and expenses not previously so offset, shall be shared by BioMarin and Genzyme in accordance with their respective Percentage Interests. 
  

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 9.8 Third Party Rights. The foregoing provisions of this Article 9 shall be subject to and limited
by any agreements pursuant to which BioMarin and Genzyme, as the case may be, acquired any particular BioMarin Patent Rights, BioMarin Technology or Genzyme Patent Rights or Genzyme Technology. 
  
 9.9 Third Party Agreements—Reports. To the extent that a Party is
obligated to provide reports to a Third Party pursuant to a Third Party Agreement as a result of or reporting on the status of activities of the other Party hereunder, the other Party hereto shall reasonably assist the reporting Party by providing
information in its possession or control and in sufficient detail to complete and submit such reports as required. 
  
 ARTICLE X 
  
 CONFIDENTIALITY 
  
 10.1 Nondisclosure
Obligations. Except as otherwise provided in this Article 10, during the Term and for a period of five (5) years thereafter, the Parties shall, and BioMarin shall cause BioMarin Genetics to, maintain in confidence and use only for purposes
specifically authorized under this Agreement any information furnished to it by the other Party hereto pursuant to this Agreement which if disclosed in tangible form is marked “Confidential” or with other similar designation to indicate
its confidential or proprietary nature or if disclosed orally or by inspection is indicated orally to be confidential or proprietary by the Party disclosing such information at the time of such disclosure and is confirmed in writing as confidential
or proprietary by the disclosing Party (describing in reasonable detail the information to be treated as confidential) within a reasonable time after such disclosure (collectively, “Information”). 
  
 To the extent it is reasonably necessary or appropriate to fulfill its
obligations or exercise its rights under this Agreement or a Related Agreement, a Party may disclose Information of the other Party it is otherwise obligated under this Section 10.1 not to disclose to its Affiliates, permitted sublicensees,
consultants, outside contractors and clinical investigators, on a need-to-know basis and on the condition that such entities or persons agree to keep the Information confidential for the same time periods and to substantially the same extent as such
Party is required to keep such Information confidential; and a Party or its permitted sublicensees may disclose such Information to government or other regulatory authorities to the extent that such disclosure is reasonably necessary to obtain
patents or authorizations to conduct clinical trials or to file and maintain Regulatory Approvals with and to market commercially Collaboration Products. The obligation not to disclose Information shall not apply to any part of such Information
that: (i) is or becomes patented, published or otherwise becomes publicly known other than by acts of the Party obligated not to disclose such Information or its Affiliates or sublicensees in contravention of this Agreement; (ii) can be
shown by written documents to have been disclosed to the receiving Party or its Affiliates or sublicensees by a Third Party, provided that such Information was not obtained by such Third Party directly or indirectly from the disclosing Party
under this Agreement; (iii) prior to disclosure under this 

  

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Agreement, was already in the possession of the receiving Party or its Affiliates or sublicensees, provided that such Information was not obtained
directly or indirectly from the disclosing Party under this Agreement; (iv) can be shown by written documents to have been independently developed by the receiving Party or its Affiliates without breach of any of the provisions of this
Agreement; or (v) is required to be disclosed by the receiving Party to comply with applicable laws or regulations, or with a court or administrative order, provided that the receiving Party notifies the disclosing Party in writing prior
to any such disclosure and agrees to use reasonable efforts to secure confidential treatment thereof prior to its disclosure (whether by protective order or otherwise). 
  

 10.2 Terms of this Agreement; Press Releases. The Parties agree to seek confidential treatment for any filing of this Agreement with
the Securities and Exchange Commission and shall agree upon the content of the request for confidential treatment made by each Party in respect of such filing. Except as permitted by the foregoing provisions or as otherwise required by law, BioMarin
and Genzyme each agree not to disclose any terms or conditions of this Agreement to any Third Party without the prior consent of the other Party; provided that each Party shall be entitled to disclose the terms of this Agreement without such
consent to its advisors and potential investors or other financing sources on the condition that such entities or persons agree to keep such terms confidential for the same time periods and to the same extent as such Party is required to keep such
terms confidential. The Parties agree that all press releases related to the Program shall be issued jointly by BioMarin and Genzyme and that the Party preparing any such press release shall provide the other Party with a draft thereof reasonably in
advance of disclosure so as to permit the other Party to review and comment on such press release. Notwithstanding the foregoing, the Parties shall agree upon a press release to announce the execution of this Agreement, together with a corresponding
Question & Answer outline for use in responding to inquiries about the Agreement; thereafter, BioMarin and Genzyme may each disclose to Third Parties the information contained in such press release and Question & Answer outline
without the need for further approval by the other. 
  
 10.3
Publications. Each Party recognizes the mutual interest in obtaining valid patent protection. Consequently, any Party, its employees or consultants wishing to make a publication (including any oral disclosure made without obligation of
confidentiality) relating to work performed by such Party as part of the Program (the “Publishing Party”) shall transmit to the other Party (the “Reviewing Party”) a copy of the proposed written publication at least
forty-five (45) days prior to submission for publication, or an abstract of such oral disclosure at least fifteen (15) days prior to submission of the abstract or the oral disclosure, whichever is earlier. The Reviewing Party shall have
the right to (a) request a delay in publication or presentation in order to protect patentable information, (b) propose modifications to the publication for patent reasons or (c) request that the information be maintained as a trade
secret. With respect to publications or disclosures by investigators or other Third Parties, such publications and disclosures shall be subject to review by the Reviewing Party under this Section 10.3 only to the extent that the submitting
Party has the right to do so. 
  
 10.3.1
Patents. If the Reviewing Party requests a delay as described in clause (a) above, the Publishing Party shall delay submission or presentation of the publication for a period of ninety (90) days to enable patent applications
protecting each Party’s rights in such information to be filed. Upon the expiration of forty-five (45) days, in the case of proposed written disclosures, or fifteen (15) days, in the case of an abstract of proposed oral disclosures,
from transmission of such proposed disclosures to the Reviewing Party, the Publishing Party shall be free to proceed with the written publication or the oral presentation, respectively, unless the Reviewing Party has requested the delay described
above. 
  

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 10.3.2 Other. To the extent possible in the reasonable exercise of its discretion,
the Publishing Party shall incorporate all modifications proposed under clause (b) above. If a trade secret that is the subject of a request made under clause (c) above cannot be otherwise protected without unreasonable expense to the
Reviewing Party, such information shall be omitted from the publication. 
  
 ARTICLE XI 
  
 REPRESENTATIONS AND WARRANTIES

  
 11.1 Authorization. Each Party warrants and
represents to the other Parties that (a) it has the legal right and power to enter into this Agreement, to extend the rights and licenses granted to the other in this Agreement, and to perform fully its obligations hereunder, (b) this
Agreement has been duly executed and delivered and is a valid and binding agreement of such Party, enforceable in accordance with its terms, (c) such Party has obtained all necessary approvals to the transactions contemplated hereby and
(d) such Party has not made and will not make any commitments to others in conflict with or in derogation of such rights or this Agreement. 
  
 11.2 Intellectual Property Rights. 
  
 11.2.1 BioMarin hereby represents and warrants that that as of the Effective Date (a) it possesses an exclusive right, title and
interest in or to, the BioMarin Patent Rights and the BioMarin Technology, (b) the BioMarin Patent Rights and the BioMarin Technology are free and clear of any lien or other encumbrance and (c) it has the right to (i) enter into the
obligations set forth in this Agreement and (ii) grant the rights and licenses set forth in Article 3 hereof. 
  
 11.2.2 Each of the Parties hereby represents and warrants that it is not aware of any issued patent that would be infringed by the
manufacture and sale of Collaboration Products as contemplated by this Agreement. 
  
 11.2.3 Genzyme hereby represents and warrants that as of the Effective Date (a) it possesses an exclusive right, title and interest
in the Genzyme Patent Rights and the Genzyme Technology, (b) the Genzyme Patent Rights and the Genzyme Technology are free and clear of any lien or other encumbrance and (c) it has the right to (i) enter into the obligations set forth
in this Agreement and (ii) grant the rights and licenses set forth in Article 3 hereof. 
  
 11.3 Warranties. 
  
 11.3.1 Genzyme Warranties. Genzyme warrants that the Collaboration Products delivered by Genzyme pursuant to Section 7.1 hereof, if any, will conform in all material respects to the Specifications, the
conditions of any applicable Regulatory Approvals regarding the manufacturing process and any applicable requirements of the Regulatory Scheme regarding the manufacturing process. 
  

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 11.3.2 BioMarin Warranties. BioMarin warrants that the Collaboration Products
delivered by BioMarin pursuant to Section 7.1 hereof will conform in all material respects to the Specifications, the conditions of any applicable Regulatory Approvals regarding the manufacturing process and any applicable requirements of the
Regulatory Scheme regarding the manufacturing process. 
  
 11.4
Disclaimer of Representations and Warranties. EXCEPT AS OTHERWISE EXPRESSLY SET FORTH IN THIS AGREEMENT OR IN A RELATED AGREEMENT, NONE OF BIOMARIN, GENZYME OR BIOMARIN/GENZYME LLC MAKES ANY REPRESENTATIONS OR EXTENDS ANY WARRANTIES OF ANY
KIND, EITHER EXPRESS OR IMPLIED, INCLUDING WITHOUT LIMITATION WARRANTIES OF MERCHANTABILITY, FITNESS FOR A PARTICULAR PURPOSE, AND THE NON-INFRINGEMENT OF ANY THIRD-PARTY PATENTS OR PROPRIETARY RIGHTS. ALL UNIFORM COMMERCIAL CODE WARRANTIES ARE
EXPRESSLY DISCLAIMED BY THE PARTIES. 
  
 11.5 Limitation of
Liability. EXCEPT WITH RESPECT TO CLAIMS FOR INDEMNIFICATION UNDER ARTICLE 12 HEREOF AND AS OTHERWISE EXPRESSLY SET FORTH IN THIS AGREEMENT OR ANY RELATED AGREEMENT, IT IS AGREED BY THE PARTIES THAT NO PARTY SHALL BE LIABLE TO ANOTHER PARTY FOR
ANY SPECIAL, CONSEQUENTIAL, EXEMPLARY OR INCIDENTAL DAMAGES (INCLUDING LOST OR ANTICIPATED REVENUES OR PROFITS RELATING TO THE SAME), ARISING FROM ANY CLAIM RELATING TO THIS AGREEMENT OR THE RELATED AGREEMENTS, WHETHER SUCH CLAIM IS BASED ON
CONTRACT, TORT (INCLUDING NEGLIGENCE) OR OTHERWISE, EVEN IF AN AUTHORIZED REPRESENTATIVE OF SUCH PARTY IS ADVISED OF THE POSSIBILITY OR LIKELIHOOD OF SAME. 
  
 ARTICLE XII 
  
 INDEMNITY 
  
 12.1 BioMarin/Genzyme LLC Indemnity Obligations. The Operating Agreement shall provide that BioMarin/Genzyme LLC shall indemnify each of the Members and its Affiliates, employees and agents (each an “Indemnified
Person”) for any act performed by such Indemnified Person within the scope of the authority conferred upon such Indemnified Person under this Agreement; provided that it shall be a condition to such indemnity that (a) the
Indemnified Person seeking indemnification acted in good faith and in a manner reasonably believed to be in, or not opposed to, the best interests of BioMarin/Genzyme LLC, (b) the act for which indemnification is sought did not constitute gross
negligence or willful misconduct by such Indemnified Person and (c) payment and indemnification of any matter disposed of by a compromise payment by such Indemnified Person, pursuant to consent decree or otherwise, shall have been approved by
the Members, which approval shall not be unreasonably withheld or delayed, or by a court of competent jurisdiction. For the avoidance of doubt, such indemnification of the Indemnified Persons shall include indemnification for acts performed by such
Indemnified Person since the Original Date of Execution, [****]. 
  

 -29- 

 12.2 Insurance. BioMarin/Genzyme LLC shall maintain clinical trial and product liability insurance
with respect to development, manufacture and sales of Collaboration Products in an amount reasonably believed by Genzyme and BioMarin to be adequate and customary for the development, manufacture and sale of novel therapeutic products. Genzyme and
BioMarin shall be named as additional insureds on any such policy. Genzyme and BioMarin shall each maintain similar clinical trial and product liability insurance coverage in amounts reasonably determined by the Steering Committee from time to time.

  
 ARTICLE XIII 
  
 TERM AND TERMINATION 
  
 13.1 Term. The term of this Agreement shall be perpetual unless
terminated pursuant to Section 13.3 below. 
  
 13.2
Termination of the Development Program Only. 
  
 13.2.1 By Mutual Agreement. The Parties may terminate the Development Program (but not this Agreement) at any time by mutual written consent. 
  

13.2.2 For Default. If either BioMarin or Genzyme (a) fails to use commercially reasonable and diligent efforts to perform
any material duty imposed upon such Party under this Agreement or the Development Plan or (b) fails to make [****] or more capital contributions in accordance with Section 4.2 hereof, and such failure to perform is not cured within ninety
(90) days of written notice thereof from the non-breaching Party, the non-breaching Party may elect, in its sole discretion, to (i) in the case of clause (b) above, waive the terms of Article 4 hereof with respect to any one or more
required capital contributions and cause the respective Percentage Interests and future funding responsibilities of the Parties to be adjusted in accordance with Section 4.1 of the Operating Agreement or (ii) terminate the Development
Program. Such 90-day period shall be extended to one hundred eighty (180) days if the breaching Party has engaged in good faith efforts to remedy such default within such 90-day period and indicated in writing to the non-breaching Party prior
to the expiration of such 90-day period that it believes that it will be able to remedy the default within such 180-day period, but such extension shall apply only so long as the breaching Party is engaging in good faith efforts to remedy such
default. 
  
 13.3 Termination of this Agreement in its
Entirety. This Agreement shall automatically terminate upon the effective date of the termination of the Manufacturing, Marketing and Sales Agreement. This Agreement may not be terminated independently from the Manufacturing, Marketing and Sales
Agreement. 
  
 13.4 Effects of Termination of this
Agreement. Upon any termination of this Agreement in its entirety, Parties shall have the respective rights and duties set forth under Article 12 of Manufacturing, Marketing and Sales Agreement. 
  
 13.5 Survival of Rights and Duties. No termination of this Agreement
shall eliminate any rights or duties of the Parties accrued prior to such termination. The provisions of Article 1, 

  

 -30- 

 
Sections 3.3, 4.3 through 4.5, 9.1.1, 9.3, 9.5, Article 10, Section 11.5, Article 12, Sections 13.3 through 13.5, 14.1, 14.3 through 14.5, and 14.8
through 14.17 and the first sentence of Section 7.5, the second to last sentence of Section 2.2 and the last sentences of Sections 2.2, 7.4 and 9.1.3 hereof shall survive any termination of this Agreement. 
  
 ARTICLE XIV 
  
 MISCELLANEOUS 
  
 14.1 Cooperation. If either BioMarin or Genzyme (the “Assuming
Party”) shall assume the Program rights from the other Party (the “Responsible Party”) in accordance with the provisions of Article 12 of the Manufacturing, Marketing and Sales Agreement, the Responsible Party shall
promptly provide to the Assuming Party (or any Third Party or Affiliate designated by the Assuming Party) all Technology, Manufacturing Know-How and access to regulatory filings filed hereunder reasonably necessary to allow the Assuming Party to
perform the duties assumed and otherwise exercise the rights and licenses granted hereunder. The Responsible Party shall further use its best efforts to provide reasonable assistance required by the Assuming Party with respect to such transfer so as
to permit the Assuming Party to begin to perform such duties as soon as possible to minimize any disruption in the continuity of supply or marketing of Collaboration Products. If the Responsible Party is the Manufacturing Party for a Collaboration
Product, the Responsible Party shall, at the option of the Assuming Party, supply such Collaboration Product until the earlier of [****]. In addition, if upon the date this Agreement is terminated Collaboration Products are being manufactured in
facilities owned or leased by the Responsible Party (including facilities subleased by BioMarin/Genzyme LLC from the Responsible Party), the Responsible Party agrees to lease such facilities to the Assuming Party on commercially reasonable terms for
a period of up to [****] at the option of the Assuming Party. 
  
 14.2 Exchange Controls. All payments due hereunder shall be paid in United States dollars. If at any time legal restrictions prevent the prompt remittance of part or all payments with respect to any country in which Collaboration
Products (other than Aldurazyme) are sold, payment shall be made through such lawful means or methods as the Parties may determine in good faith. 
  
 14.3 Withholding Taxes. If applicable laws or regulations require that taxes be withheld from payments made hereunder, the Party paying such taxes
will (a) deduct such taxes, (b) timely pay such taxes to the proper authority and (c) send written evidence of payment to the Party from whom such taxes were withheld within sixty (60) days after payment. Each Party will assist
the other Party or Parties in claiming tax refunds, deductions or credits at such other Party’s request and will cooperate to minimize the withholding tax, if available, under various treaties applicable to any payment made hereunder.

  
 14.4 Interest on Late Payments. Any payments to be made
hereunder that are not paid on or before the date such payments are due under this Agreement shall bear interest, to the extent permitted by applicable law, at the Base Rate of interest declared from time to time by Bank of America, N.A. in Boston,
Massachusetts, calculated on the number of days payment is delinquent. 
  

 -31- 

 14.5 Force Majeure. Neither Party shall be held liable or responsible to the other Party nor be
deemed to have defaulted under or breached this Agreement for failure or delay in fulfilling or performing any term of this Agreement when such failure or delay is caused by or results from causes beyond the reasonable control of the affected Party,
including without limitation fire, floods, embargoes, war, acts of war (whether war is declared or not), insurrections, riots, civil commotions, strikes, lockouts or other labor disturbances, acts of God or acts, omissions or delays in acting by any
governmental authority or the other Party; provided, however, that the Party so affected shall use commercially reasonable and diligent efforts to avoid or remove such causes of non-performance, and shall continue performance hereunder with
reasonable dispatch wherever such causes are removed. Each Party shall provide the other Parties with prompt written notice of any delay or failure to perform that occurs by reason of force majeure. The Parties shall mutually seek a
resolution of the delay or the failure to perform in good faith. 
  
 14.6 Assignment. This Agreement may not be assigned or otherwise transferred by any Party without the consent of the other Parties; provided, however, that either BioMarin or Genzyme may, without such consent, assign its
rights and obligations under this Agreement (a) in connection with a corporate reorganization, to any member of an affiliated group, all or substantially all of the equity interest of which is owned and controlled by such Party or its direct or
indirect parent corporation or (b) in connection with a merger, consolidation or sale of substantially all of such Party’s assets to an unrelated Third Party; provided, however, that such Party’s rights and obligations under
this Agreement shall be assumed by its successor in interest in any such transaction and shall not be transferred separate from all or substantially all of its other business assets, including without limitation those business assets that are the
subject of this Agreement and the Manufacturing, Marketing and Sales Agreement. Any permitted assignee shall assume all obligations of its assignor under this Agreement; accordingly, all references herein to the assigning Party shall be deemed
references to the assignee to whom the Agreement is so assigned. Any purported assignment in violation of this Section 14.6 shall be void. 
  
 14.7 Severability. Each Party hereby agrees that it does not intend to violate any public policy, statutory or common laws, rules, regulations,
treaty or decision of any government agency or executive body thereof of any country or community or association of countries. Should one or more provisions of this Agreement be or become invalid, the Parties hereto shall substitute, by mutual
consent, valid provisions for such invalid provisions which valid provisions in their economic effect are sufficiently similar to the invalid provisions that it can be reasonably assumed that the Parties would have entered into this Agreement with
such valid provisions. In case such valid provisions cannot be agreed upon, the invalidity of one or several provisions of this Agreement shall not affect the validity of this Agreement as a whole, unless the invalid provisions are of such essential
importance to this Agreement that it is to be reasonably assumed that the Parties would not have entered into this Agreement without the invalid provisions. 
  
 14.8 Notices. Any consent, notice or report required or permitted to be given or made under this Agreement by one of the Parties hereto to the
other shall be in writing, delivered personally or by facsimile (and promptly confirmed by personal delivery or courier), by a next business day delivery service of a nationally recognized overnight courier service or by courier, postage prepaid
(where applicable), addressed to such other Party at its address indicated below, or to such other address as the addressee shall have last furnished in writing to the addressor in accordance with this Section 14.8 and shall be effective upon
receipt by the addressee. 
  

 -32- 

			
	If to BioMarin:	  	 BioMarin Pharmaceutical Inc.
 105 Digital
Drive
 Novato, California 94949
 Attention: Chief Executive
Officer
 Facsimile: (415) 382-7889

		
	with a copy to:	  	 BioMarin Pharmaceutical Inc.
 105 Digital
Drive
 Novato, California 94949
 Attention: General
Counsel
 Facsimile: (415) 382-7889

		
	If to Genzyme:	  	 Genzyme Corporation
 500 Kendall Street
 Cambridge, Massachusetts 02142
 Attention: President, LSD
Therapeutics
 Facsimile: (617) 768-6419

		
	with a copy to:	  	 Genzyme Corporation
 500 Kendall Street
 Cambridge, Massachusetts 02142
 Attention: General Counsel
 Facsimile: (617) 252-7553

		
	 If to
 BioMarin/Genzyme
 LLC (if such notice is
 sent by BioMarin):
	  	 BioMarin/Genzyme LLC
 c/o Genzyme Corporation

500 Kendall Street
 Cambridge, Massachusetts 02142
 Attention: President, LSD Therapeutics
 Facsimile: (617)
768-6419

		
	with a copy to:	  	 Genzyme Corporation
 500 Kendall Street
 Cambridge, Massachusetts 02142
 Attention: General Counsel
 Facsimile: (617) 252-7553

  

 -33- 

			
		
	 If to
 BioMarin/Genzyme
 LLC (if such notice is
 sent by Genzyme):
	  	 BioMarin/Genzyme LLC
 c/o BioMarin Pharmaceutical Inc.

 105 Digital Drive
 Novato, California 94949
 Attention: Chief Executive Officer
 Facsimile: (415)
382-7889

		
	with a copy to:	  	 BioMarin Pharmaceutical Inc.
 105 Digital
Drive
 Novato, California 94949
 Attention: General
Counsel
 Facsimile: (415) 382-7889

  
 14.9 Applicable
Law. This Agreement shall be governed by and construed in accordance with the laws of the State of Delaware without regard to any choice of law principle that would dictate the application of the laws of another jurisdiction. 
  
 14.10 Arbitrate. Any disputes arising between the Parties relating to,
arising out of or in any way connected with this Agreement or any term or condition hereof, or the performance by either Party of its obligations hereunder, whether before or after termination of this Agreement (a “Dispute”), which
has not resolved in accordance with the provisions of Section 8.3 hereof, shall be finally resolved by binding arbitration as herein provided. 
  
 14.10.1 General. Except as otherwise provided in this Section 14.10, any arbitration hereunder shall be conducted under the
commercial rules of the American Arbitration Association. Each such arbitration shall be conducted in the English language by a single arbitrator appointed in accordance with such rules, provided that if either Party requests the arbitration shall
be conducted by a panel of three (3) arbitrators (the “Arbitration Panel”). In the case of three (3) arbitrators, each of BioMarin and Genzyme shall appoint one (1) arbitrator to the Arbitration Panel and the third
arbitrator shall be appointed by the two (2) arbitrators appointed by BioMarin and Genzyme. The Arbitration Panel shall be convened upon delivery of the Notice of Arbitration (as herein defined). Any such arbitration shall be held in Chicago,
Illinois. The Arbitration Panel shall have the authority to grant specific performance, and to allocate between the Parties the costs of arbitration in such equitable manner as it shall determine. Judgment upon the award so rendered may be entered
in any court having jurisdiction or application may be made to such court for judicial acceptance of any award and an order of enforcement, as the case may be. 
  

14.10.2 Procedure. 
  
 (a) Whenever a Party (the “Claimant”) shall decide to institute arbitration proceedings, it shall give written notice to
that effect (the “Notice of Arbitration”) to the other Party (the “Respondent”). The Notice of Arbitration shall set forth in detail the nature of the Dispute, the facts upon which the Claimant relies and the issues
to be arbitrated (collectively, the “Arbitration Issues”). Within fifteen (15) days of its receipt of the Notice of Arbitration, the Respondent shall 

  

 -34- 

 
send the Claimant and the Arbitration Panel a written Response (the “Response”). The Response shall set forth in detail the facts upon which
the Respondent relies. In addition, the Response shall contain all counterclaims which the Respondent may have against the Claimant which are within the Arbitration Issues, whether or not such claims have previously been identified. If the Response
sets forth a counterclaim, the Claimant may, within fifteen (15) days of the receipt of the Response, deliver to the Respondent and the Arbitration Panel a rejoinder answering such counterclaim. 
  
 (b) Within fifteen (15) days after the later of
(i) the expiration of the period provided in Section 14.10.2(a) above for the Claimant to deliver a rejoinder or (ii) the completion of any discovery proceedings authorized by the Arbitration Panel: (A) the Claimant shall send to
the Arbitration Panel a proposed resolution of the Arbitration Issues and a proposed resolution of any counterclaims set forth in the Response, including without limitation the amount of monetary damages, if any, or other relief sought (the
“Claimant’s Proposal”); and (B) the Respondent shall send to the Arbitration Panel a proposed resolution of the Arbitration Issues, a proposed resolution of any counterclaims set forth in the Response and a proposed
resolution of any rejoinder submitted by the Claimant, including without limitation the amount of monetary damages, if any, or other relief sought (the “Respondent’s Proposal”). Once both the Claimant’s Proposal and the
Respondent’s Proposal have been submitted, the Arbitration Panel shall deliver to each Party a copy of the other Party’s proposal. 
  
 (c) The Arbitration Panel shall issue an opinion with respect to any Dispute, which opinion shall explicitly accept either the
Claimant’s Proposal or the Respondent’s Proposal in its entirety (the “Final Decision”). The Arbitration Panel shall not have the authority to reach a Final Decision that provides remedies or requires payments other than
those set forth in the Claimant’s Proposal or the Respondent’s Proposal. The concurrence of two (2) arbitrators shall be sufficient for the entry of a Final Decision. The arbitrators shall issue a Final Decision within one
(1) month from the later of (i) the last day for submission of proposals under Section 14.10.2(b) above or (ii) the date of the final hearing on any Dispute held by the Arbitration Panel. A Final Decision shall be binding on both
Parties. 
  
 14.11 Injunctive Relief. The Parties hereby
acknowledge that a breach of their respective obligations under Article 10 hereof may cause irreparable harm and that the remedy or remedies at law for any such breach may be inadequate. The Parties hereby agree that, in the event of any such
breach, in addition to all other available remedies hereunder, the non-breaching Party or Parties shall have the right to seek equitable relief to enforce Article 10 hereof. 
  
 14.12 Entire Agreement. This Agreement, together with the Related Agreements, contain the entire understanding of the
Parties with respect to the subject matter hereof. All express or implied agreements and understandings, either oral or written, heretofore made are expressly merged in and made a part of this Agreement. This Agreement may be amended, or any term
hereof modified, only by a written instrument duly executed by both Parties hereto. Each of the Parties hereby acknowledges that this Agreement and the Related Agreements are all the result of mutual negotiation and therefore any ambiguity in their
respective terms shall not be construed against the drafting Party. 
  

 -35- 

 14.13 Section 365(n) of the Bankruptcy Code. All rights and licenses now or hereafter granted
under or pursuant to this Agreement, including in the event of termination pursuant to Article 12 of the Manufacturing, Marketing and Sales Agreement, are rights to “intellectual property” (as defined in Section 101(35A) of Title 11
of the United States Code, as amended (such Title 11, the “Bankruptcy Code”)). The licensing Party hereby grants to the licensee Party a right of access and to obtain possession of and to benefit from (i) copies of research
data, (ii) laboratory samples, (iii) product samples, (iv) formulas, (v) laboratory notes and notebooks, (vi) data and results related to clinical trials, (vii) regulatory filings and approvals, (viii) rights of
reference in respect of regulatory filings and approvals, (ix) manufacturing procedure documentation and manufacturing records, (x) marketing, advertising and promotional materials, and (xi) all other embodiments of such intellectual
property, that are in the licensing Party’s possession or control, freely licenseable without further payment or restriction by the licensing Party, and necessary for the licensee Party’s exercise of the rights and licenses to such
intellectual property, all of which constitute “embodiments” of intellectual property pursuant to Section 365(n) of the Bankruptcy Code. The licensing Party agrees not to interfere with the licensee’s exercise of rights and
licenses to intellectual property licensed under this Agreement and embodiments thereof in accordance with this Agreement and agrees to use commercially reasonable efforts to assist the licensee Party to obtain such intellectual property and
embodiments thereof in the possession or control of Third Parties as reasonably necessary or desirable for the licensee Party to exercise such rights and licenses in accordance with this Agreement. 
  
 14.14 Headings. The captions to the several Articles and Sections
hereof are not a part of this Agreement, but are merely guides or labels to assist in locating and reading the several Articles and Sections hereof. 
  
 14.15 Independent Contractors. It is expressly agreed that BioMarin and Genzyme shall be independent contractors and that, except as Members of
BioMarin/Genzyme LLC, the relationship between the two Parties shall not constitute a partnership, joint venture or agency. Neither BioMarin nor Genzyme shall have the authority to make any statements, representations or commitments of any kind, or
to take any action, which shall be binding on the other, without the prior consent of the other Party to do so. 
  
 14.16 Waiver. Except as expressly provided herein, the waiver by either Party hereto of any right hereunder or of any failure to perform or any
breach by the other Party shall not be deemed a waiver of any other right hereunder or of any other failure to perform or breach by said other Party, whether of a similar nature or otherwise, nor shall any singular or partial exercise of such right
preclude any further exercise thereof or the exercise of any other such right. 
  
 14.17 Counterparts. This Agreement 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. Signature pages
may be exchanged by facsimile. 
  
 [SIGNATURE PAGE FOLLOWS]

  

 -36- 

 IN WITNESS WHEREOF, the Parties have executed this Agreement as of the date first set forth above.

  

			
	GENZYME CORPORATION
		
	By:	 	 /s/ David P. Meeker

	Print Name:	 	 David P. Meeker

	Title:	 	 President LSD Therapeutics

	Date:	 	 12/31/07

	
	BIOMARIN PHARMACEUTICAL INC.
		
	By:	 	/s/ G. Eric Davis
	Print Name:	 	G. Eric Davis
	Title:	 	Vice President, General Counsel
	Date:	 	12/31/07
	
	BIOMARIN/GENZYME LLC
	
	By: BIOMARIN PHARMACEUTICAL INC.
		
	By:	 	/s/ G. Eric Davis
	Print Name:	 	G. Eric Davis
	Title:	 	Vice President, General Counsel
	Date:	 	12/31/07
	
	 By: GENZYME CORPORATION

		
	By:	 	/s/ David P. Meeker
	Print Name:	 	David P. Meeker
	Title:	 	President LSD Therapeutics
	Date:	 	12/31/07

  

 -37- 

 SCHEDULE 1.47 
  

 THIRD PARTY AGREEMENTS 
  
 1.) License Agreement effective as of September 4, 1998 entered into by and between the University of Iowa Research Foundation, an Iowa corporation and
BioMarin/Genzyme LLC, a Delaware limited liability company. 
  
 2.) License
Agreement effective as of September 4, 1998 entered into by and between Research Corporation Technologies, Inc., a Delaware nonprofit corporation and BioMarin/Genzyme LLC, a Delaware limited liability company. 
  
 3.) Grant Terms and Conditions Agreement dated April 1, 1997 entered into by and among
BioMarin Pharmaceuticals, Harbor-UCLA Research and Education Institute and Emil D. Kakkis, as amended. 
  

 -38- 

 APPENDIX A 
  

 [****] 
  

 -39-

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