<PAGE>

                                                                    Exhibit 10.6

                           EXCLUSIVE LICENSE AGREEMENT

                                     between

NOVO NORDISK A/S, Novo Alle, 2880 Bagsvaerd, Denmark - Danish company
identification number CVR 24 25 67 90 (hereinafter referred to as "NOVO
NORDISK")

                                       and

AMICUS THERAPEUTICS, Inc., 675 U.S. Highway One, North Brunswick, NJ 08902, USA
(hereinafter referred to as "AMICUS THERAPEUTICS").

Hereinafter individually referred to as "Party" and collectively as "Parties";

                                   WITNESSETH:

WHEREAS,   AMICUS THERAPEUTICS is involved in development of small molecule
           enzyme chaperones for treatment of genetic and metabolic diseases;

WHEREAS,   NOVO NORDISK is the owner of certain Intellectual Property Rights
           relating to glycogen phosphorylase inhibitors, its use and in
           particular patent rights relating to a specific glycogen
           phosphorylase inhibitor NN4201;

WHEREAS,   NOVO NORDISK wishes to license to AMICUS THERAPEUTICS such
           Intellectual Property Rights; and

WHEREAS,   AMICUS THERAPEUTICS wishes to acquire a license to such Intellectual
           Property Rights from NOVO NORDISK;

NOW, THEREFORE, the Parties agree as follows:

1.   BACKGROUND

1.1  As of the Effective Date and upon the terms and subject to the conditions
     of this Agreement, NOVO NORDISK agrees to grant to AMICUS THERAPEUTICS, and
     AMICUS THERAPEUTICS agrees to acquire from NOVO NORDISK, a license to the
     Intellectual Property Rights (as further defined in Article 2.1.9 below),
     free of any and all security interests, options or other third party rights
     (including but not limited to rights of pre-emption and royalties) of any
     nature what so ever.

2.   DEFINITIONS

2.1  For the purpose of this Agreement, the following terms shall have the
     following meanings in this Exclusive License Agreement and its appendices:

     2.1.1 "Affiliate" means any company, corporation, or other business entity
          which controls, is controlled by, or is under common control with, a
          Party hereto.

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.


                                       1
<PAGE>

          "Control," including the terms "controlled by" or "under common
          control with," shall mean (a) in the case of corporate entities,
          direct or indirect ownership of stock or shares having the power to
          elect a majority of directors or similar body which governs the
          affairs of such corporate entity; and b) in the case of non-corporate
          entities, direct or indirect ownership of equity interest with the
          power to direct the management and policies of such non-corporate
          entities.

     2.1.2 "Agreement" shall mean this Exclusive License Agreement including its
          appendices.

     2.1.3 "Analogue" shall mean any chemical structure that is a structural
          homolog to, or derived from, the Compound and is covered by NOVO
          NORDISK Intellectual Property Rights.

     2.1.4 "Annual Net Sales" means the gross invoice price of the Licensed
          Product per year sold by AMICUS THERAPEUTICS, its Affiliates or
          sublicensees to independent Third Party customers in bona fide
          arms-length transactions, less the following deductions:

          (a)  trade, cash and/or quantity discounts actually taken;

          (b)  sales taxes, use taxes, tariffs, customs duties and value added
               or other taxes;

          (c)  Outbound transportation prepaid or allowed;

          (d)  refunds, rebates, allowances, credits or returns, including
               amounts repaid or credited by reason of rejections, return of
               goods or retroactive price reductions.

          For Annual Net Sales of a Licensed Product sold or supplied as a
          Combination, the Annual Net Sales of such a Combination in a country
          shall be determined as follows:

          A) by multiplying the Annual Net Sales of the Combination by the
          fraction A/(A+B), where A is the invoice price of the Licensed Product
          in that country if sold separately and B is the total invoice price of
          any other active component or components in the Combination in that
          country if sold separately; or If the Licensed Product and the other
          active component or components in the Combination are not sold
          separately, the Annual Net Sales, for purposes of determining
          royalties on the Combination, will be calculated by multiplying the
          Annual Net Sales of the Combination by the fraction determined by
          mutual agreement of the Parties, that reflects the relative
          contribution in value that the Licensed Product contained in the
          Combination makes to the total value of such Combination to the end
          user.; and

          B) if the Licensed Product contained in the Combination is not sold in
          that country in a vial, the Parties shall negotiate in good faith the
          value of the cartridge or prefilled device and/or other biologically
          active pharmaceutical(s) to be deducted from the Annual Net Sales of
          the Combination.

     2.1.5 "Combination" means A) where a Licensed Product is sold or supplied
          as a pharmaceutical product containing, in addition to the Licensed
          Product, one or more biologically active pharmaceuticals which are not
          a Licensed

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

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          Product, and/or B) where the Licensed Product is sold or supplied
          incorporated in a cartridge or prefilled device.

     2.1.6 "Compound" shall refer specifically to the compound identified as NNC
          42-1001 or NN4201 having the systematic name (2R, 3R,
          4R)-2-hydroxymethyl-pyrrolidine-3,4-diol. The IUPAC name for the
          tartaric salt of this compound is (2R, 3R,
          4R)-3,4-dihydroxy-2-hydroxymethyl)pyrrolidinum
          (2S,3S)-3-carboxy-2,3-dihydroxy-propanoate.

     2.1.7 "Effective Date" shall mean the date of the last signature to this
          Agreement.

     2.1.8 "Field" shall mean any and all human therapeutic or diagnostic
          indications.

     2.1.9 "Intellectual Property Rights" shall mean discoveries, know-how, data
          and technical information owned and controlled by NOVO NORDISK related
          to the NOVO NORDISK proprietary information, patents and patent
          applications delineated in Appendix A (and in respect of Patent
          Cooperation Treaty applications, European Patent Convention
          applications or applications under similar administrative
          international conventions, patent applications in the listed or
          designated countries), as well as any and all patents derived from
          these patents and patent applications, including selection patents,
          continuations, continuations-in-part, continued prosecutions
          applications, divisionals, reissues, re-examinations, renewals, or
          extensions, of the listed patent rights or any legal equivalent
          thereof which have been or may be filed in any country for the full
          term or terms for which the same may be granted. Extensions shall
          include: (a) extensions under U.S. Patent Term Restoration Act; (b)
          extensions under Japanese Patent Law; (c) Supplementary Protection
          Certifications (SPCs) according to Council Regulation (EEC) No 1768/92
          for members of the European Patent Convention and other countries in
          the European Economic Area, and (d) similar extensions under
          applicable law anywhere in the world.

     2.1.10 "Licensed Product(s)" shall mean any compound including but not
          limited to Compound, which is made, used, sold or offered for sale
          and/or imported in at least one country as a human therapeutic and
          that (a) is identified, discovered, made or developed, by AMICUS
          THERAPEUTICS for the benefit or on behalf of any Third Party, using a
          method covered by a Valid Claim of the Intellectual Property Rights,
          or (b) reasonably could not have been identified, discovered, made,
          used, developed, imported, offered for sale or been sold by AMICUS
          THERAPEUTICS but for the Intellectual Property Rights, or (c) is
          otherwise covered by a Valid Claim of the Intellectual Property Rights
          and would, in the absence of the License granted under this Agreement,
          infringe any Valid Claim. For the avoidance of doubt, Licensed Product
          includes compounds as described in the preceding sentence which are
          being made for and/or used in clinical trials in humans for the
          purpose of obtaining regulatory approval for use as an human
          therapeutic. Licensed Product also includes any Replacement Product(s)
          that may be developed under the Agreement.

     2.1.11 "NOVO NORDISK Data" as used herein, shall mean all NOVO NORDISK
          scientific and clinical/regulatory data relating to the use of
          Compound in humans.

     2.1.12 "Replacement Product" shall mean any Licensed Product which is a
          replacement for the Licensed Product or a potential Licensed Product.

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.


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<PAGE>

     2.1.13 "Territory" shall mean all countries in the world.

     2.1.14 "Third Party", as used herein, shall mean all individuals or
          entities other than NOVO NORDISK and AMICUS THERAPEUTICS and any of
          their respective Affiliates and/ or sublicensees.

     2.1.15 "Valid Claim" shall mean a claim of any unexpired patent or patent
          application within Intellectual Property Rights so long as such claim
          shall not have been held invalid or unenforceable in a final decision
          rendered by tribunal of competent jurisdiction from which no appeal
          has been or can be taken.

3.   CONSIDERATIONS AND GRANT OF RIGHTS

3.1  NOVO NORDISK hereby grants to AMICUS THERAPEUTICS and its Affiliates an
     exclusive, worldwide, royalty-bearing license, with right to sublicense
     without restriction (provided that AMICUS THERAPEUTICS and its Affiliates
     remain responsible for the performance of their sublicensees), under the
     Intellectual Property Rights, to use, develop, promote, manufacture, have
     manufactured, market, register, package, distribute, sell, offer for sale,
     have sold, import, export and otherwise commercialize Licensed Products in
     the Field throughout the Territory (the "License"). NOVO NORDISK hereby
     also grants to AMICUS THERAPEUTICS the exclusive right and license to use
     the NOVO NORDISK Data in connection with regulatory filings with the U.S.
     Food and Drug Administration and other comparable international regulatory
     bodies for approval of the Licensed Products.

3.2  If NOVO NORDISK determines, after consultation with AMICUS THERAPEUTICS,
     that NOVO NORDISK controls or owns other Intellectual Property Rights as of
     the Effective Date, that are necessary for the development, use or
     manufacture of Licensed Products, then NOVO NORDISK shall to the extent
     legally possible include such other Intellectual Property Rights in the
     License granted under Article 3.1. If any such other Intellectual Property
     Rights are included in the License after the Effective Date, these shall be
     added to Appendix A together with the date for addition of them.

3.3  In consideration of the License granted hereunder to AMICUS THERAPEUTICS
     and its Affiliates, AMICUS THERAPEUTICS, its Affiliates or its sublicenses
     agree to pay to NOVO NORDISK the milestone payments and royalties set forth
     in this Article 3.3 and Article 3.4.

     a)   A total of [***] USD [***] to be paid in full no later than fifteen
          business days after the Effective Date into an account in the bank
          defined in Article 3.5.

     b)   A total of [***] USD [***] to be paid in full no later than fifteen
          business days after the IND filing in the US for each indication.

     c)   A total of [***] USD [***] to be paid in full no later than fifteen
          business days after initiation of a Phase III clinical trial (the date
          of the Investigator's meeting) in the US for each indication.

     d)   A total of [***] USD [***] to be paid in full no later than fifteen
          business days after filing of an NDA in the US for each indication.

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       4
<PAGE>


     e)   A total of [***] USD [***] to be paid in full no later than fifteen
          business days after filing with EMEA for each indication.

     f)   A total of [***] USD [***] to be paid in full no later than fifteen
          business days after filing for regulatory approval in Japan for each
          indication.

     g)   A total of [***] USD [***] to be paid in full no later than fifteen
          business days after regulatory approval in the US for each indication.

     h)   A total of [***] USD [***] to be paid in full no later than fifteen
          business days after regulatory approval in EMEA for each indication.

     i)   A total of [***] USD [***] to be paid in full no later than fifteen
          business days after regulatory approval in Japan for each indication.

     The above milestone payments shall be payable once for the first Licensed
     Product achieving these milestones for an indication. AMICUS THERAPEUTICS
     shall also make milestone payments to NOVO NORDISK for each Replacement
     Product developed by AMICUS THERAPEUTICS and/or a sublicensee achieving
     milestones (d) through (i) for an indication, provided that each milestone
     payment amount shall be reduced by [***]. For the purposes of determining
     the satisfaction of these milestones, the category of diseases known as
     lysosomal storage diseases, and all classes of diseases within such
     category, shall be counted collectively as one indication (provided,
     however, that such disease is an orphan drug indication (US)), and all
     other human diseases shall each be counted individually as one indication.

3.4  Royalties will be payable by AMICUS THERAPEUTICS, its Affiliates or its
     sublicensees to NOVO NORDISK on a product to-by product and country by
     country basis until the last to expire of the NOVO NORDISK Intellectual
     Property Rights claiming the making, using, selling, offering to sell
     and/or import of such Licensed Product in such country. The Royalty rates
     shall be according to the following:

                           [table begins on next page]


[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       5
<PAGE>

Table 1

<TABLE>
<CAPTION>
LICENSED PRODUCT DESCRIPTION                                             ANNUAL NET SALES                ROYALTY
----------------------------                               -------------------------------------------   -------
<S>                                                        <C>                                           <C>
Has Compound as an active component and the indication                 $25 million or less                  [***]%
is a lysosomal storage disease or other orphan drug (US)   > $25 million but less than or equal to $50      [***]%
indication:                                                                  million
                                                             > $50 million but less than or equal to        [***]%
                                                                           $100 million
                                                                          > $100 million                    [***]%

Has Compound as an active component and the indication                 $25 million or less                  [***]%
is other than a lysosomal storage disease or other         > $25 million but less than or equal to $50      [***]%
orphan drug (US) indication:                                                 million
                                                             > $50 million but less than or equal to        [***]%
                                                                           $100 million
                                                                          > $100 million                    [***]%

Has an Analogue of the Compound as an active component                 $25 million or less                  [***]%
and the indication is a lysosomal storage disease or       > $25 million but less than or equal to $50      [***]%
other orphan drug (US) indication:                                           million
                                                             > $50 million but less than or equal to        [***]%
                                                                           $100 million
                                                                          > $100 million                    [***]%

Has an Analogue of the Compound as an active component                 $25 million or less                  [***]%
and the indication is other than a lysosomal storage       > $25 million but less than or equal to $50      [***]%
disease or other orphan drug (US) indication:                                million
                                                             > $50 million but less than or equal to        [***]%
                                                                           $100 million
                                                                          > $100 million                    [***]%

Has neither the Compound nor an Analogue thereof as an                 $100 million or less                 [***]%
active component:                                                         > $100 million                    [***]%
</TABLE>

     Notwithstanding the foregoing, if (a) AMICUS THERAPEUTICS and/or its
     Affiliates (and/or appertaining sublicensees, as the case may be) is
     required to obtain from any Third Party that is not an Affiliate or a
     sublicensee any licenses and/or sublicenses for patent rights in order to
     practice NOVO NORDISK Intellectual Property Rights in the Field or in order
     to develop, make, have made, use, import, offer for sale, sell, import,
     export or provide Licensed Products (including, without limitation, as a
     result of any claim referred to in subsection (b)), or (b) any claim is
     made against AMICUS THERAPEUTICS and/or its Affiliates (and/or appertaining
     sublicensees, as the case may be) alleging that the practice of the NOVO
     NORDISK Intellectual Property Rights in the Field infringes any Third Party
     patent rights, then AMICUS THERAPEUTICS and/or its Affiliates (and/or
     appertaining sublicensees, as the case may be) shall be entitled to credit,
     in the case of subsection (a), any payment by AMICUS THERAPEUTICS and/or
     its Affiliates (and/or appertaining sublicensees, as the case may be) of
     additional running royalties to such Third Party(ies), if any, on Licensed
     Products, and, in the case of subsection (b), [***] of any
     reasonable costs and expenses


[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       6
<PAGE>

     (including, without limitation, attorneys' fees, but excluding any
     judgments or any settlements in connection with such claims) incurred by
     AMICUS THERAPEUTICS and/or its Affiliates (and/or appertaining
     sublicensees, as the case may be) in connection with any such infringement
     claim against the running royalty for the subject Licensed Products, in the
     appertaining country(ies) during the appertaining time period. However, not
     withstanding the above the minimum royalty payable by AMICUS THERAPEUTICS
     and its Affiliates and sublicensees to NOVO NORDISK shall never be reduced
     below [***] of the royalties set forth in this Article 3.4, Table 1 and
     which are payable for Licensed Product in the specific country or countries
     in question.

3.5  All payments required under this Agreement shall be made in US Dollars to
     the following bank account or to such account as NOVO NORDISK may, from
     time to time, notify AMICUS THERAPEUTICS in writing:

     Danske Bank,
     Copenhagen
     Account number: [***] send via the correspondent bank:

     Bank of America N.A.
     New York
     SWIFT code: BOFAUS3N.

3.6  Royalty Accounting. The tiered royalties under this Agreement shall be paid
     quarterly but calculated on an annual basis. Only a single royalty rate
     shall be applicable in any given year and that rate will be determined by
     the total Annual Net Sales. An adjustment to prior quarters in any given
     year shall be made in any subsequent quarter of the same year in which a
     threshold in a higher royalty bracket has exceeded. A yearend adjustment
     will be made, if a royalty threshold is exceeded in the fourth quarter.

3.7  Payments and Reports. Royalties payable pursuant to this agreement shall be
     due quarterly within forty five (45) days following the end of each
     calendar quarter for Annual Net Sales in such calendar quarter. All sales
     in foreign currencies shall be converted into United States dollars using
     the rate of exchange quoted by Bank of America and its successor(s) on the
     last business day of the calendar quarter in which the sales were made.
     Each such payment shall be accompanied by a statement of Annual Net Sales
     for the quarter (including number of units), applicable exchange rates and
     the calculation of royalty payable hereunder by Licensed Product and
     country. AMICUS THERAPEUTICS shall keep and shall cause its Affiliates and
     sublicensees to keep complete, true and accurate records for at least five
     (5) years for the purpose of showing the derivation of all milestone
     payments and royalties payable under this Agreement.

     3.7.1 NOVO NORDISK duly accredited representatives, which are reasonably
          acceptable to AMICUS THERAPEUTICS, shall have the rights to inspect
          and audit such records at any time with reasonable prior notice to
          AMICUS THERAPEUTICS or any of its Affiliates or sublicensees, but such
          right will not be exercised more often than once a year.

     3.7.2 Any adjustment required as a result of an audit conducted under this
          Article shall be made within thirty (30) days after the date on which
          NOVO NORDISK completed the audit. In the event of an underpayment by
          AMICUS THERAPEUTICS, its Affiliates and/or sublicensees, AMICUS
          THERAPEUTICS shall pay to NOVO NORDISK the amount underpaid plus
          interest (calculated on a daily basis) on the overdue payment from the
          date such payment was due to the date of actual payment an annual rate

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       7
<PAGE>

          equal to the discount rate ("diskontoen") of the Danish National Bank
          plus 2% (two percent). In case of overpayment by AMICUS THERAPEUTICS,
          ifs Affiliates and/or sublicensees, AMICUS THERAPEUTICS may, at its
          option, offset any future royalty payments payable to NOVO NORDISK by
          the amount of the overpayment. Each Party shall have five (5) years
          after receipt by NOVO NORDISK of any royalty paid by AMICUS
          THERAPEUTICS, its Affiliates and/or sublicenses pursuant to this
          Agreement to dispute the amount of any such royalty payment.

3.8  Transfer of NOVO NORDISK Data. NOVO NORDISK will transfer, and will
     instruct its contractors about transfer, of NOVO NORDISK Data to AMICUS
     THERAPEUTICS after AMICUS THERAPEUTICS has given NOVO NORDISK a written
     notice that AMICUS wishes to receive such NOVO NORDISK Data. NOVO NORDISK's
     obligations on transfer of data will cease six (6) months after the
     Effective Date. After this date NOVO NORDISK will in good faith consider
     fulfilling requests from AMICUS THERAPEUTICS regarding additional
     information. NOVO NORDISK will charge AMICUS THERAPEUTICS the costs
     associated with such requests at a cost basis. The contact person at NOVO
     NORDISK will be head of Scientific Licensing, Pierre Honore
     (pfh@novonordisk.com).

3.9  AMICUS THERAPEUTICS shall deliver a written annual report on each
     anniversary of the Effective Date covering the preceding year regarding the
     status of the NOVO NORDISK Intellectual Property Rights and the Licensed
     Products identified, discovered or developed fully or partly through the
     use of Intellectual Property Rights by AMICUS THERAPEUTICS. Such annual
     report shall include, as a minimum; (a) identification by code number of
     Licensed Products identified, discovered or developed, using a method
     covered in whole or in part by the Intellectual Property Rights, or which
     reasonably could not have been identified, discovered or developed but for
     the Intellectual Property Rights or which are otherwise covered by the
     Intellectual Property Rights, unless AMICUS THERAPEUTICS provides
     contemporaneous written evidence to NOVO NORDISK that such identification,
     discovery or development took place before the date of issue or grant of
     relevant Intellectual Property Rights; (b) the status of any submissions to
     a regulatory agency in any country concerning Licensed Product; the
     identity of Third Parties that AMICUS THERAPEUTICS has granted sublicensees
     to under this agreement to; and, (c) such additional material as NOVO
     NORDISK may reasonably request. NOVO NORDISK shall maintain the
     confidentiality of all such reports and shall not use the information
     therein for any purpose other than determining compliance of AMICUS
     THERAPEUTICS with the terms of this Agreement.

3.10 The AMICUS THERAPEUTICS contact person responsible for communicating with
     the NOVO NORDISK under the reporting requirements of this Agreement shall
     be the same as is given in Article 16 (NOTICES), unless AMICUS THERAPEUTICS
     designates otherwise to NOVO NORDISK in writing.

3.11 All payments due under this Agreement shall be made without deduction other
     than such amount as AMICUS THERAPEUTICS is required to deduct or withhold
     by law. When making any payment due under this Agreement, AMICUS
     THERAPEUTICS shall also pay any value added (or similar) tax which is
     payable. The sums payable by AMICUS THERAPEUTICS are non-creditable and
     non-refundable. The previous sentences of this Article notwithstanding,
     each Party undertakes to cooperate with the other Party to achieve the tax
     arrangements that are most favourable for both Parties.

3.12 In the event of any delay in effecting the payments due under this
     Agreement by the due date, AMICUS THERAPEUTICS, its Affiliates and
     sublicensees agree to pay

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       8
<PAGE>

     to NOVO NORDISK interest (calculated on a daily basis) on the overdue
     payment from the date such payment was due to the date of actual payment an
     annual rate equal to the discount rate ("diskontoen") of the Danish
     National Bank plus 2% (two percent).

3.13 Under a purchase order separate from this Agreement, AMICUS THERAPEUTICS
     shall purchase and NOVO NORDISK shall sell and deliver to AMICUS
     THERAPEUTICS, [***] kg of the Compound, at a price of $[***] per gram. Such
     delivery shall be shipped no later than fifteen (15) calendar days after
     receipt of the purchase order. Payment terms and other terms for use of the
     Compound shall be established in such purchase order. NOVO NORDISK may
     require that a Materials Transfer Agreement is entered into in connection
     with such purchase.

4.   CONFIDENTIALITY

4.1  Neither Party shall publish, disclose or commit to any Third Party any
     information in whatever form concerning this Agreement and the license
     granted hereunder, nor shall it make any reference to this Agreement to any
     Third Party for five (5) years from the date of termination or expiration
     of this Agreement without the prior written consent of the other Party.

4.2  All information disclosed by one Party ("Disclosing Party") to the other
     Party ("Recipient") in oral, visual, written, or electronic form hereunder,
     including but not limited to, any technical or non-technical information
     concerning technical processes, specifications, instrumentation, chemical
     formulae, assays, techniques, sales and marketing information, material, or
     data related to this Agreement, ("Information"), shall be kept strictly
     confidential and shall not be disclosed by Recipient to any Third Party
     without the prior written and express consent of the Disclosing Party.
     Information disclosed in oral form shall be deemed Confidential Information
     only to the extent that it has been confirmed in writing to Disclosing
     Party and marked "confidential" within 30 (thirty) days after the date of
     oral disclosure.

4.3  Recipient shall not use the Information for any other purpose than
     performing its obligations under this Agreement; however AMICUS
     THERAPEUTICS shall be entitled to use Information for any regulatory
     purposes, including clinical trials.

4.4  The obligations of confidentiality described above in Articles 4.1 - 4.3
     shall not apply to

     a)   Information, which at the time of disclosure is already in the public
          domain;

     b)   Information, which, after disclosure, becomes part of the public
          domain through no violation of this Agreement;

     c)   Information, which Disclosing Party is able to prove has been
          disclosed to Recipient and which Recipient is able to prove has been
          in its possession of prior to disclosure. In this case, Recipient
          shall, in writing and within forty-five (45) days from the date of
          disclosure, demonstrate to the satisfaction of the Disclosing Party
          that it was in possession of such Information;

     d)   Information, which is hereafter lawfully disclosed by a Third Party to
          the Recipient, which Information such Third Party did not acquire
          under a still effective obligation of confidentiality to the
          disclosing Party;

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       9
<PAGE>

     e)   Information, which can be demonstrated as independently developed or
          acquired by Recipient without reference to or reliance upon
          confidential Information defined in this Agreement, and as evidenced
          by Recipient's written records;

     f)   Information disclosed to the extent required by law or regulation
          provided that Recipient shall give the Disclosing Party prompt written
          notice and sufficient opportunity to object, time permitting, to such
          disclosure.

4.5  Notwithstanding the foregoing, Recipient may disclose Information of the
     Disclosing Party to reliable employees, consultants and agents if necessary
     for exploiting the license granted under this Agreement or for a Party in
     order to fulfil its obligations under this Agreement, provided that such
     persons are bound by obligations of confidentiality and non-use to
     Recipient which are equal to the terms of this Agreement. Recipient shall
     ensure that such employees, consultants and agents be fully aware of the
     obligations of this Agreement and shall be responsible for any breach of
     these provisions by its employees, consultants and agents. Further, AMICUS
     THERAPEUTICS may disclose information relating to, or embodied by, NOVO
     NORDISK Intellectual Property Rights, as well as NOVO NORDISK Data to:
     manufacturing, distribution, marketing, co-development or other strategic
     or corporate partners or vendors, potential sublicensees, investors, board
     members, investment bankers, provided that such persons or entities are
     bound by obligations of confidentiality and non-use to AMICUS THERAPEUTICS
     which are equal to the terms of this Agreement. AMICUS THERAPEUTICS shall
     ensure that such persons or entities are fully aware of the obligations of
     this Agreement and shall be responsible for any breach of these provisions
     by such persons or entities.

5.   PUBLIC ANNOUNCEMENTS AND PUBLICATIONS

5.1  The Parties agree not to make, issue or release any public announcement,
     statement or acknowledgement of the existence of this Agreement without the
     prior written approval of the other Party. Such approval shall not be
     unreasonably withheld or delayed. NOVO NORDISK needs fourteen (14) calendar
     days for such approval.

5.2  The Parties agree that NOVO NORDISK has the rights to publish the papers as
     indicated in APPENDIX B.

6    PATENT FILING AND MAINTENANCE

6.1  NOVO NORDISK agrees to execute any and all papers necessary in connection
     with the applications set forth in Appendix A and any continuing
     divisional, reissue, reexamination or corresponding application thereof.

6.2  NNOVO NORDISK agrees to execute all papers necessary in connection with any
     interference which may be declared concerning the application or any
     continuing divisional, reissue, reexamination or corresponding application
     thereof and to cooperate with AMICUS THERAPEUTICS, in every reasonable way
     to obtain evidence and go forward with such interference.

6.3  AMICUS THERAPEUTICS shall be obliged at AMICUS THERAPEUTICS costs and
     expense to maintain and prosecute NOVO NORDISK Intellectual Property Rights
     until their expiry and AMICUS THERAPEUTICS shall have sole responsibility
     for the preparation, filing, prosecution, and maintenance of the
     Intellectual Property

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       10
<PAGE>

     Rights. AMICUS THERAPEUTICS shall in good faith consider input from NOVO
     NORDISK with respect to prosecution and maintenance. For the avoidance of
     doubt, AMICUS THERAPEUTICS will pay for the continued filing of
     Intellectual Property Rights concerning Licensed Products.

7    ENFORCEMENT OF INTELLECTUAL PROPERTY RIGHTS

7.1  Notification. Should NOVO NORDISK become aware that a Third Party has been
     or is threatening to infringe any of the Intellectual Property Rights, or
     that a Third Party is challenging the validity of any Intellectual Property
     Rights, NOVO NORDISK shall give AMICUS THERAPEUTICS prompt written notice
     detailing as many facts as possible concerning such infringement or
     potential infringement or challenge to validity.

7.2  Enforcement. AMICUS THERAPEUTICS shall at its own cost and expense be
     responsible for taking action as AMICUS THERAPEUTICS - in any event after
     consulting with NOVO NORDISK - may deem necessary to prevent an
     infringement of the Intellectual Property Rights; to enforce the
     Intellectual Property Rights and to defend the NOVO NORDISK Intellecutal
     Property Rights against any action challenging the validity of the NOVO
     NORDISK Patent Rights. No settlement shall be made unless with the prior
     written approval of NOVO NORDISK. Any sums recovered in a suit or
     settlement shall belong to AMICUS THERAPEUTICS. However, AMICUS
     THERAPEUTICES shall not name NOVO NORDISK as a coparty in the enforcement
     and defense of the Intellectual Property Rights without the express written
     consent of NOVO NORDISK and AMICUS THERAPEUTICS shall hold harmless NOVO
     NORDISK from all reasonable costs and expenses of such litigation,
     including reasonable attorney's fees. In the event NOVO NORDISK as the
     owner of the Intellectual Property Rights has to be joined in a suit, NOVO
     NORDISK shall have the right to be represented by a counsel of its own
     choice.

7.3  Obligations. AMICUS THERAPEUTICS shall be obligated to enforce any of the
     Intellectual Property Rights covered by this Agreement at its own expense.
     AMICUS THERAPEUTICS can partially be released from such obligation
     according to Article 12.2.

8    PATENT VALIDITY

8.1  If any claim challenging the validity or enforceability of any Intellectual
     Property Rights shall be brought against NOVO NORDISK, NOVO NORDISK shall
     promptly notify AMICUS THERAPEUTICS. Article 9.2 shall govern the
     disposition of any such claim.

8.2  If any Third Party challenges the validity or enforceability of any of the
     Intellectual Property Rights, AMICUS THERAPEUTICS agrees not to suspend any
     payments due to NOVO NORDISK until such time as that patent in Intellectual
     Property Rights is determined to be invalid or unenforceable by final
     judgement of a governmental agency or a court of competent jurisdiction
     from which no appeal can be or has been taken.

9    REPRESENTATIONS AND WARRANTIES

9.1  NOVO NORDISK represents and warrants that, to the best of its knowledge, it
     has the right to grant the license in and to Intellectual Property Rights
     set forth in this

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       11
<PAGE>

     Agreement, that the rights granted to AMICUS THERAPEUTICS, hereunder do not
     conflict with rights previously granted to any Third Party or any agreement
     to which NOVO NORDISK is bound, and that, to the best of its knowledge,
     there is no litigation pending or threatened with respect to the
     Intellectual Property Rights.

9.2  Nothing in this Agreement shall be construed as:

     9.2.1 A representation or warranty by NOVO NORDISK as to the patentability,
          validity, scope, or usefulness of Intellectual Property Rights; or

     9.2.2 A representation or warranty by NOVO NORDISK that anything made,
          used, sold, or otherwise disposed of under any license granted in this
          Agreement is or will be free from infringement of patents or other
          proprietary rights not included in Intellectual Property Rights.

9.3  EXCEPT AS EXPRESSLY SET FORTH ABOVE, NOVO NORDISK EXPRESSLY DISCLAIMS ANY
     AND ALL WARRANTIES, WHETHER EXPRESS OR IMPLIED, PERTAINING TO THE
     MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE OF THE INTELLECTUAL
     PROPERTY RIGHTS, LICENSED PRODUCTS, OR ANYTHING ELSE LICENSED, DISCLOSED,
     OR OTHERWISE PROVIDED TO AMICUS THERAPEUTICS UNDER THIS AGREEMENT. NOVO
     NORDISKS' TOTAL LIABILITY UNDER THIS AGREEMENT IS LIMITED TO THE COSTS AND
     FEES PAID BY AMICUS THERAPEUTICS TO NOVO NORDISK UNDER THIS AGREEMENT.

9.4  NOVO NORDISK warrants that NOVO NORDISK Data is transferred as is, i.e.
     AMICUS THERAPEUTICS will be responsible for finalising reports or document
     studies for the regulatory authorities. NOVO NORDISK will assist in tracing
     existing documents, data and or information available which are requested
     by such authorities under the terms and conditions described in Article
     3.8.

10   GOVERNING LAW AND DISPUTES

10.1 Both Parties will use their best efforts to settle all matters in dispute
     amicably. All disputes and differences of any kind related to this
     Agreement, which cannot be solved amicably by the Parties, shall be
     referred to arbitration as described below.

10.2 All disputes arising out of or in connection with the present contract
     shall be finally settled under the Rules of Arbitration of the
     International Chamber of Commerce by one or more arbitrators appointed in
     accordance with the said Rules.

10.3 The arbitration shall take place in London, England, and shall be conducted
     in the English language. The award of the arbitrators shall be final and
     binding on both Parties. The Parties bind themselves to carry out the
     awards of the arbitrators.

10.4 This contract shall be construed and interpreted pursuant to the laws of
     Denmark to the exclusion of any rule that would refer the subject matter to
     another forum. The English wording in this Agreement shall prevail.

11   TERM AND TERMINATION

11.1 This Agreement shall be in full force and effect from the Effective Date
     and shall remain in effect until expiry of the last to expire patent of
     Intellectual Property Rights, unless otherwise terminated by operation of
     law or pursuant to the terms and conditions of this Agreement.

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       12
<PAGE>

11.2 Either Party may terminate this Agreement on thirty (30) days written
     notice to the other Party ("the Notified Party") if any of the following
     events occur:

     (a)  If the Notified Party is in breach of any of the material terms or
          obligations of this Agreement and such breach remains uncured for
          sixty (60) days following receipt by the Notified Party of written
          notice of such breach (if such default is cured within the cure
          period, such written notice shall be null and void), provided that, if
          the Notified Party can establish to the reasonable satisfaction of the
          other Party that it is diligently and actively pursuing a cure at the
          expiration of the cure period, and that the default is reasonably
          capable of being cured, then the cure period shall be extended for up
          to ninety (90) days from the date of receipt of the written notice of
          breach by the Notified Party. For the avoidance of doubt, in the event
          of a dispute whether a Party is in breach of the material terms and
          obligations of the Agreement and/or whether the cure period shall be
          extended, the dispute shall be resolved under Article 10. The
          Agreement shall not terminate until a final decision has been reached
          either by the Parties or under Arbitration as set forth in Article 10.

     (b)  In the event the Notified Party shall have become bankrupt, or shall
          have made an assignment for the benefit of its creditors or there
          shall have been appointed a trustee or receiver of the Notified Party
          or for all or a substantial part of its property or any case or
          proceeding shall have been commenced or other action taken by or
          against the Notified Party in bankruptcy or seeking reorganization,
          liquidation, dissolution, winding-up, arrangement, composition or
          readjustment of its debts or any other relief under any bankruptcy,
          insolvency, reorganization, or other similar act or law of any
          jurisdiction now or hereafter in effect and any such event shall have
          continued for ninety (90) days undismissed, unbonded and/or
          undischarged. All rights and license granted under this Agreement by
          one Party to the other Party are, and shall otherwise be deemed to be,
          for purposes of Section 365(n) of the Bankruptcy Code, license of
          rights to "intellectual property" as defined under Section 101 (56) of
          the Bankruptcy Code. The Parties agree that the licensor under this
          Agreement shall retain and may fully exercise all of its rights and
          elections under the Bankruptcy Code in the event of a bankruptcy by
          the other Party. The Parties further agree that in the event of the
          commencement of a bankruptcy proceeding by or against one Party under
          the Bankruptcy Code of their respective countries, the other Party
          shall be entitled to complete access to any such intellectual property
          pertaining to the rights granted in the licenses hereunder of the
          Party by or against whom a bankruptcy proceeding has been commenced
          and all embodiments of such intellectual property. However, if NOVO
          NORDISK is the bankrupt party, this above shall only apply to the
          extent this is allowed under the Danish Bankruptcy Code
          ("Konkursloven").

11.3 Lack of payments due to NOVO NORDISK under this License Agreement or in
     relation to maintenance, defending and enforcing the Intellectual Property
     Rights shall always be considered material breach.

11.4 AMICUS THERAPEUTICS may terminate this License Agreement in its entirety at
     any time upon one hundred and eighty (180) days written notice to NOVO
     NORDISK.

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       13
<PAGE>

11.5 This Agreement, and the license granted to AMICUS THERAPEUTICS, may be
     terminated with effect immediately by NOVO NORDISK in the event that AMICUS
     THERAPEUTICS either directly or indirectly opposes, or assists any Third
     Party to oppose, the grant of any of the Intellectual Property Rights or
     disputes, or directly or indirectly assists any Third Party to dispute, the
     validity of any of the NOVO NORDISK Intellectual Property Rights.

11.6 The provisions under which this Agreement may be terminated shall be in
     addition to any and all other legal remedies which either Party may have
     for the enforcement of any and all terms hereof, and do not in any way
     limit any other legal remedy such Party may have.

11.7 Termination of this Agreement shall terminate all rights and licenses
     granted to AMICUS THERAPEUTICS relating to the Intellectual Property
     Rights.

12   TERMINATION OF CERTAIN INTELLECTUAL PROPERTY RIGHTS

12.1 AMICUS THERAPEUTICS shall be entitled to give NOVO NORDISK a written notice
     of ninety (90) days that AMICUS THERAPEUTICS wishes to exclude certain NOVO
     NORDISK Intellectual Property Rights from the license granted under this
     Agreement. In this case those NOVO NORDISK Intellectual Property Rights
     excluded from the license granted under this Agreement shall revert to NOVO
     NORDISK. The Parties shall enter into an amendment to this Agreement
     stating that Intellectual Property Rights continue to be included in the
     Agreement. The license granted under this Agreement shall continue to be in
     full force and effect with respect to this remaining part of the
     Intellectual Property Rights.

13   RIGHTS AND DUTIES UPON EXPIRATION OR TERMINATION

13.1 Upon termination of this Agreement, NOVO NORDISK shall have the right to
     retain any sums already paid by AMICUS THERAPEUTICS hereunder.

13.2 Expiration or termination of this Agreement shall terminate all outstanding
     grants, obligations and liabilities between the Parties arising from this
     Agreement, except those described in Articles 4, 5, 11.6, 11.7, 13, 14, 15
     and 17 which shall survive expiration or any termination of the Agreement

13.3 The grant under Article 3 of this Agreement shall cease by termination of
     this Agreement and AMICUS THERAPEUTICS shall return its rights to NOVO
     NORDISK Intellectual Property Rights to NOVO NORDISK.

14   USE OF NAMES

14.1 Nothing contained in this Agreement shall be construed as conferring any
     rights to use in advertising, publicity or other promotional activities any
     name, trade name, trademark, or other designation of a party hereto,
     including any contraction, abbreviation, or simulation of any of the
     foregoing, unless the express written permission of the other party has
     been obtained. Each party hereby agrees not to use the names of the other
     party without prior written approval from such other party.

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       14
<PAGE>

15   INDEMNIFICATION

15.1 AMICUS THERAPEUTICS agrees to indemnify, hold harmless, and defend NOVO
     NORDISK, its officers, employees and agents against any and all claims,
     suits, losses, damages, costs, fees and expenses resulting from or arising
     out of exercise of this Agreement, including, but not limited to, any
     damages, losses, or liabilities whatsoever with respect to death or injury
     to any person and damage to any property arising from the possession, use,
     or operation of Intellectual Property Rights by AMICUS THERAPEUTICS,
     including any infringement by AMICUS THERAPEUTICS of the intellectual
     property of a Third Party through AMICUS THERAPEUTICS' use or operation of
     Intellectual Property Rights. NOVO NORDISK shall indemnify AMICUS
     THERAPEUTICS in like manner with respect to any breach of the
     representations and warranties set forth in Article 9.

16   NOTICES

16.1 Any notice or other communication required or permitted to be given by
     either Party hereto shall be deemed to have been properly given and be
     effective upon the date of delivery if delivered in writing to the
     respective addresses set forth below, or to such other address as either
     party shall designate by written notice given to the other Party. If notice
     or other communication is given by facsimile transmission, said notice
     shall be confirmed by prompt delivery of the hard-copy original.

     If to NOVO NORDISK:        Attn: Pierre Honore,
                                Vice President Scientific Licensing
                                NOVO NORDISK A/S
                                Novo Alle
                                DK-2880 Bagsvaerd
                                Denmark
                                Fax: +45 44 42 13 13
                                Phone: +45 44 42 71 44

     with a copy to:            NOVO NORDISK A/S
                                Corporate Legal

                                Attn.: General Counsel
                                NOVO NORDISK A/S
                                Novo Alle
                                DK-2880 Bagsvaerd
                                Denmark
                                Fax: +45 44 98 06 70

     If to AMICUS THERAPEUTICS:

                                Attn.: John F. Crowley
                                Amicus Therapeutics, Inc.
                                President and CEO
                                675 U.S. Highway One
                                No. Brunswick, New Jersey USA 08902

     with a copy to:

                                Att.: Douglas A. Branch
                                Biotech Law Associates, P.C.
                                800 Research Parkway, Suite 310
                                Oklahoma City, Oklahoma USA 73104

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       15
<PAGE>

     Or such other address as either Party may request in writing.

17   INSURANCE REQUIREMENTS

17.1 AMICUS THERAPEUTICS shall maintain general liability insurance including
     product liability and contractual liability coverage within the limits tied
     to the risks inherent in use of the Intellectual Property Rights. AMICUS
     THERAPEUTICS must declare whether the insurance is provided on a claims
     made form and must notify NOVO NORDISK if coverage is cancelled. If
     coverage is maintained by AMICUS THERAPEUTICS on Licensed Product(s) after
     termination or expiration of this Agreement, such coverage must continue to
     name NOVO NORDISK.

17.2 AMICUS THERAPEUTICS shall list NOVO NORDISK as an additional insured under
     each liability policy that AMICUS THERAPEUTICS shall have or obtain that
     includes coverage of claims relating to products or processes used, made or
     sold as a result of AMICUS THERAPEUTICS'exercise of the Intellectual
     Property Rights. This insurance clause shall survive the termination of
     this Agreement.

18   GENERAL

18.1 Assignment. The License Agreement may not be assigned by either party
     without the other party's consent. In the event a party gives its consent
     to an assignment of the License Agreement, the assignee shall not be
     entitled to exercise any rights or receive any benefits under the License
     Agreement until it has expressly assumed in writing to the other party the
     performance and obligations of all the assigning party's duties and
     obligations as set forth in the License Agreement. No such consent of the
     other party will be required for assignment of the License Agreement (a) in
     connection with the transfer or sale of all or substantially all of the
     business of such party to which the agreement relates to a Third Party,
     whether by merger, sale of stock, sale of assets or otherwise, or (b) to
     any Affiliate. However, in the event of assignment to a successor by merger
     or by sale of all or substantially all of a party's assets, such successor
     shall not be entitled to exercise any rights or receive any benefits from
     this License Agreement until it has expressly assumed in writing to the
     other party the performance and obligations of all the assigning party's
     duties and obligations as set forth in the License Agreement. Any
     assignment of the License Agreement which is not in accordance with the
     aforementioned shall be void.

18.2 AMICUS THERAPEUTICS shall have the rights to assign or transfer any or all
     of its rights or obligations under this Agreement at any time after AMICUS
     THERAPEUTICS has paid the consideration set forth in Article 3.3.a, so long
     as the obligations in Article 18.1. are fulfilled.

18.3 Article headings in this Agreement are for convenience only and do not
     affect its interpretation.

18.4 In the event that one or more provisions of this Agreement are invalid for
     any reason, the validity of the remaining provisions of this Agreement
     shall not be affected. The Parties agree to replace such invalid provisions
     or any gaps in the Agreement that might become evident, by new, valid
     provisions that correspond as closely as possible to the intended purpose
     of this Agreement.

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       16
<PAGE>

18.5 The Confidentiality Agreement dated March 1st, 2005, the Stand Still
     Agreement dated April 1st, 2005 and the Material Transfer Agreement dated
     4th of April, 2005 shall continue to be in full force and effect until the
     Effective Date of this Agreement on which date those agreements shall be
     terminated. Provisions of such agreements which according to the wording of
     the agreements survive termination shall continue to be in full force and
     effect notwithstanding the aforementioned termination.

18.6 No Waiver. The failure of any Party to enforce at any time any provision of
     this Agreement, or any right with respect thereto, or to exercise any
     election herein provided, shall in no way be considered to be a waiver of
     such provision, right or election, or in any way affect the validity of
     this Agreement. The exercise by any Party of any right or election under
     the terms or covenants herein shall not preclude or prejudice any party
     from exercising the same or any other right it may have under this
     Agreement, irrespective of any previous action or proceeding taken by the
     Parties hereunder.

18.7 Severability. Should a court of competent jurisdiction later consider any
     provision of this Agreement to be invalid, illegal, or unenforceable, it
     shall be considered severed from this Agreement. All other provisions,
     rights, and obligations shall continue without regard to the severed
     revision, provided that the remaining provisions of this Agreement are in
     accordance with the intention of the parties.

18.8 Amendment. This Agreement may only be amended in writing signed by duly
     authorised representatives of AMICUS THERAPEUTICS and NOVO NORDISK.

18.9 Interpretation. In this Agreement the headings are used for convenience
     only and shall not affect its interpretation. References to the singular
     include the plural and vice versa.

18.10 Further Action. Each party agrees to execute, acknowledge and deliver such
     further instruments, and do all further similar acts, as may be necessary
     or appropriate to carry out the purposes and intent of this Agreement.

18.11 Entire Agreement. Subject to Article 18.5 this Agreement sets out the
     entire agreement between the Parties relating to its subject matter and
     supersedes all prior oral or written agreements, arrangements or
     understandings between them relating to such subject matter. The
     confidentiality agreement, this exclusive license agreement and any
     amendments hereto are signed by AMICUS THERAPEUTICS on behalf of the
     company itself and its US based Affiliates.

18.12 Costs. Each party shall pay their own costs in connection with entering
     into this Agreement.

18.13 Counterparts. This Agreement may be executed in two or more counterparts,
     each of which shall be deemed an original, but all of which together shall
     constitute one and the same instrument.

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.


                                       17
<PAGE>

     IN WITNESS WHEREOF, the Parties hereto have caused this instrument to be
     executed in duplicate by their duly authorized representatives as of the
     date stated below.

Date: 5/26/05                           Date: 6/8/2005
AMICUS THERAPEUTICS, INC.               NOVO NORDISK A/S


/s/ John F. Crowley                     /s/ Peter Kurtzhals
-------------------------------------   ----------------------------------------
By: John F. Crowley                     By: Peter Kurtzhals
Title: Chairman and Chief Executive     Title: Senior Vice President and
       Officer                                 Head of Discovery


                                        /s/ Pierre Honore
                                        ----------------------------------------
                                        By: Pierre Honore
                                        Title: Vice President, Scientific
                                               Licensing


                                                                              18
<PAGE>

                                   APPENDIX A
                    ACTIVE PATENT FAMILIES RELATING TO NN4201

<TABLE>
<CAPTION>
CASE NO.      PRIORITY DATE                           SCOPE                               ACTIVE MEMBERS
--------   ------------------   -------------------------------------------------   --------------------------
<S>        <C>                  <C>                                                 <C>
4172       March 9, 1994        EPO: Compounds of the formula (CHEMICAL FORMULA)    EP patent: CH, DE, FR, GB,
                                US: Pharmaceutical compositions comprising          SE (EP 749423)
                                (CHEMICAL FORMULA) or (CHEMICAL FORMULA)            US 5,863,903
                                Methods of treating diabetes or reducing liver      JP application
                                glucose production using said compositions

4573       September 8, 1995    EPO: Use of a compound of the formula (CHEMICAL     EP 858335 in force in CH,
                                FORMULA) in the manufacture of medicaments for      DE, FR, GB, SE.
                                inhibiting liver glucose production or inhibiting
                                liver glycogen phosphorylase. 42-1001               US 5,854,272
                                specifically for manufacturing of medicaments for   US 6,541,836
                                diabetes
                                                                                    JP 3043430

                                US 5,854,272: A method of treating diabetes or
                                inhibiting liver glucose production by
                                administration of a (CHEMICAL FORMULA) compound
                                of the formula

                                US 6,541,836: A method or inhibiting liver
                                glycogen phosphorylase by administration of a
                                compound of the formula
                                (CHEMICAL FORMULA)
5243       May 6, 1997          Compounds of the formula                            US 6,046,214
                                (CHEMICAL FORMULA)
5841       March 15, 1999       Tatrate salt of 42-1001                             EP, JP applications
                                                                                    US 6,316,489
</TABLE>

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       19
<PAGE>

<TABLE>
<CAPTION>
CASE NO.      PRIORITY DATE                           SCOPE                               ACTIVE MEMBERS
--------   ------------------   -------------------------------------------------   --------------------------
<S>        <C>                  <C>                                                 <C>
5842       March 15,1999        Napsylate salt of 42-1001                           EP, JP applications
                                                                                    US 6,239,163

5941       September 29, 1999   Compounds of the formula (CHEMICAL FORMULA)         EP, JP and US applications
                                                                                    US 6,590,118

6261       November 8, 2000     Compositions comprising 41-1001 and other           Only in Denmark
                                anti-diabetica

                                NB Utility model

6474       October 28, 2002     The use of compounds of formula                     Applications in US and PCT
                                (CHEMICAL FORMULA), (CHEMICAL FORMULA), or          WO 04/037233
                                (CHEMICAL FORMULA) in the treatment of early
                                cardiac diseases
</TABLE>

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       20
<PAGE>

APPENDIX B

<TABLE>
<CAPTION>
NN4201 PUBLICATIONS    Responsible         Data on Compound       Authors (Initials) in random order       Journal
-------------------   -------------   -------------------------   ----------------------------------   ----------------
<S>                   <C>             <C>                         <C>                                  <C>
Safety package        Klaus Ryetved   All safety data on          KRYT, BEKI, NILD                     Arzneimittel /
                                      Compound                                                         Drug research

In vitro              Klaus Ryetved   VF/ECG data on Pathology,   KRYT, INSJ, NCBN, KF, (?)            Br. J. Pharmacol
                                      glycogen, and enzyme

In vivo/AMI           Niels C Berg    Biotrial report             BEKI, KF, KRYT, NCBN                 Circulation
                      Nyborg                                      and study director from Biotrial

Comparison to other   Keld Fosgerau   Comparison of data from     KRYT, NCBN, KF, BFH+ ?               Br. J. Pharmacol
compounds                             heart, including isolated
                                      muscle data.
</TABLE>

<TABLE>
<CAPTION>
                                                            Authors (Initials) in
NN4201 ABSTRACTS       Responsible       Data on Compound        random order        Journal
----------------   -------------------   ----------------   ---------------------   --------
<S>                <C>                   <C>                <C>                     <C>
In vitro           Keld Fosgerau         In vitro           KF, KRYT, NCBN?         ADA

In vivo            Niels C Berg Nyborg   In vivo            KF, KRYT                ADA
                                                            NCBN+?
</TABLE>

[***] INDICATES MATERIAL THAT HAS BEEN OMITTED AND FOR WHICH CONFIDENTIAL
      TREATMENT HAS BEEN REQUESTED. ALL SUCH OMITTED MATERIAL HAS BEEN FILED
      WITH THE SECURITIES AND EXCHANGE COMMISSION PURSUANT TO RULE 24b-2
      PROMULGATED UNDER THE SECURITIES EXCHANGE ACT OF 1934, AS AMENDED.

                                       21