Exhibit 10.1  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:  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.


ARTICLE IDEFINITIONS  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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  2


provides for marketing approval for Aldurazyme in the
United States, as the same may be updated or amended from time to time.  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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  3


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, 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).  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  4


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) 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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  5


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 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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  6


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.  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; providedthat 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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  7


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 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,  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  8


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.  ARTICLE III
GRANTS AND RESERVATIONS OF RIGHTS  3.1 Licenses of Rights
to BioMarin/Genzyme LLC.  3.1.1Grants
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.  3.1.2Grants
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.  3.1.3BioMarin/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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  9


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.  3.1.4Rights 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 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.  3.1.5External
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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  10


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.  3.2.1General.  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.  3.2.2Further
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.  3.3.1Reservation
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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  11


3.3.2Reservation
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 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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  12


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.  4.2.1Quarterly
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.  4.2.2Monthly
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 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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  13


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 [**].  ARTICLE V
THE DEVELOPMENT PROGRAM  5.1 Conduct of the
Development Program.  5.1.1General.  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,  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  14


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.  5.1.2Development
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.  5.1.3Initial
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 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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  15


5.1.4Studies
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).  5.1.5Other
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 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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  16


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.  5.1.6Execution
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.  5.1.7Attendance
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.  5.2.1Reports
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 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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  17


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.  5.2.2Adverse
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.  5.2.3Clinical
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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  18


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.  5.4Clinical
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.  5.5Facilities
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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  19


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):  7.2.1General.
All decisions relating to the manufacture of Collaboration Products shall be
subject to the approval of, or modification by, the Steering Committee.  7.2.2Facilities.  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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  20


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.  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.  8.1.1General.  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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  21


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.  8.1.2Development
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.  8.1.3Minutes.  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.  8.2.1General.  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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  22


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.  8.2.2Functions.  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.  8.2.3Minutes.  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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  23


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.  9.1.1Ownership
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 (BioMarinInventions) 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 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.  9.1.2Ownership
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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  24


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.  9.1.3Cooperation
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 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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  25


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 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.  9.7.1Enforcement.  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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  26


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.  9.7.2Information.  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.  9.7.3Enforcement
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.  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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  27


ARTICLE X
CONFIDENTIALITY  10.1Nondisclosure
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, providedthat 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, providedthat 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, providedthat
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).  10.2Terms
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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  28


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; providedthat 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.  10.3Publications.  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.  10.3.1Patents.  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.  10.3.2Other.  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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  29


ARTICLE XI
REPRESENTATIONS AND WARRANTIES  11.1Authorization.  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.  11.2Intellectual
Property Rights.  11.2.1BioMarin 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.  11.2.2Each 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.  11.2.3Genzyme 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.  11.3Warranties.  11.3.1Genzyme
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.  11.3.2BioMarin
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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  30


11.4Disclaimer
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.  11.5Limitation
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  12.1BioMarin/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; providedthat 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, [**].  12.2Insurance.  BioMarin/Genzyme LLC shall maintain clinical
trial and product liability insurance with respect to development, manufacture
and sales of Collaboration Products in an  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  31


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  13.1Term.  The term of this Agreement shall be perpetual
unless terminated pursuant to Section 13.3 below.  13.2Termination
of the Development Program Only.  13.2.1By Mutual
Agreement.  The Parties may terminate
the Development Program (but not this Agreement) at any time by mutual written
consent.  13.2.2For 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.  13.3Termination
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.  13.4Effects
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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  32


13.5Survival
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 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  14.1Cooperation.  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.  14.2Exchange
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.  14.3Withholding
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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  33


14.4Interest
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.  14.5Force
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.  14.6Assignment.  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.  14.7Severability.  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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  34


14.8Notices.  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.  If to BioMarin:
  
  BioMarin Pharmaceutical
  Inc.
  105 Digital Drive
  Novato, California 94949 
  Attention: Chief Executive Officer 
  Facsimile: (415) 382-7889with a copy to:
  
  BioMarin Pharmaceutical
  Inc.
  105 Digital Drive
  Novato, California 94949 
  Attention: General Counsel 
  Facsimile: (415) 382-7889If to Genzyme:
  
  Genzyme Corporation
  500 Kendall Street
  Cambridge, Massachusetts 02142 
  Attention: President, LSD Therapeutics 
  Facsimile: (617) 768-6419with a copy to:
  
  Genzyme Corporation
  500 Kendall Street
  Cambridge, Massachusetts 02142
  Attention: General Counsel 
  Facsimile: (617) 252-7553If to
  BioMarin/Genzyme
  LLC (if such notice is
  sent by BioMarin):
  
  BioMarin/Genzyme LLCc/o Genzyme Corporation
  500 Kendall Street
  Cambridge, Massachusetts 02142 
  Attention: President, LSD Therapeutics 
  Facsimile: (617) 768-6419  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  35


with a copy to:
  
  Genzyme Corporation
  500 Kendall Street
  Cambridge, Massachusetts 02142
  Attention: General Counsel 
  Facsimile: (617) 252-7553If 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) 382-7889with a copy to:
  
  BioMarin Pharmaceutical
  Inc.
  105 Digital Drive
  Novato, California 94949 
  Attention: General Counsel 
  Facsimile: (415) 382-7889  14.9Applicable
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.  14.10Arbitrate.  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.  14.10.1General.  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).  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  36


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.  14.10.2Procedure.  (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.  (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.  [**] = Portions of this exhibit have been omitted pursuant to a confidential
treatment request.  An unredacted version of this exhibit has been filed
separately with the Commission.  37


14.11Injunctive
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.  14.12Entire
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.  14.13Section 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.  14.14Headings.  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.  14.15Independent
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  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  38


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.  14.16Waiver.  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.  14.17Counterparts.  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]  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  39


IN WITNESS WHEREOF, the
Parties have executed this Agreement as of the date first set forth above.  GENZYME CORPORATIONBy:
  /s/
  David P. MeekerPrint
  Name: David P. MeekerTitle:
  President LSD TherapeuticsDate:
  12/31/07BIOMARIN PHARMACEUTICAL INC.By:
  /s/
  G. Eric DavisPrint
  Name: G. Eric DavisTitle:
  Vice President, General CounselDate:
  12/31/07BIOMARIN/GENZYME LLCBy:
  BIOMARIN PHARMACEUTICAL INC.By:
  /s/
  G. Eric DavisPrint
  Name: G. Eric DavisTitle:
  Vice President, General CounselDate:
  12/31/07By:
  GENZYME CORPORATIONBy:
  /s/
  David P. MeekerPrint
  Name: David P. MeekerTitle:
  President LSD TherapeuticsDate:
  12/31/07  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  40


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.  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  41


APPENDIX
A  [**]  [**] = Portions of this exhibit have been omitted pursuant to a
confidential treatment request.  An unredacted version of this exhibit has
been filed separately with the Commission.  42