SCHEDULE 14A INFORMATION

                PROXY STATEMENT PURSUANT TO SECTION 14(a) OF THE

                         SECURITIES EXCHANGE ACT OF 1934

Filed by the Registrant |X|
Filed by a Party other than the Registrant |_|

Check the appropriate box:
|_|      Preliminary Proxy Statement

|_|  Confidential,  for Use of the  Commission  Only (as  permitted  by Rule 14a
6(e)(2)) |X| Definitive Proxy Statement |_| Definitive  Additional Materials |_|
Soliciting Material Pursuant to Section 240.14a-11(c) or Section 240.14a 12

                          BioMarin Pharmaceutical Inc.

-- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
                (Name of Registrant as Specified In Its Charter)

-  -  -  - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
    (Name of Person(s) Filing Proxy Statement, if other than the Registrant)

Payment of Filing Fee (Check the appropriate box):

|X|      No fee required.
|_| Fee computed on table below per Exchange Act Rules 14a-6(i)(1) and 0-11.

(1)      Title of each class of securities to which transaction applies:

- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
(2)      Aggregate number of securities to which transaction applies:

- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
(3)      Per unit  price  or other  underlying  value  of  transaction  computed
         pursuant to  Exchange  Act Rule 0-11 (Set forth the amount on which the
         filing fee is calculated and state how it was determined)

-  -  -  - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
(4)      Proposed maximum aggregate value of transaction:

-  -  -  - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
(5)      Total fee paid:

- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
         -

|_| Fee paid previously with preliminary materials.

|_|      Check box if any part of the fee is offset as provided by Exchange  Act
         Rule  0-11(a)(2)  and identify the filing for which the  offsetting fee
         was paid  previously.  Identify  the  previous  filing by  registration
         statement number, or the Form or Schedule and the date of its filing.

(1)      Amount Previously Paid:

 -  -  - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
(2)      Form, Schedule or Registration Statement No.:

- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
(3)      Filing Party:

- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -
(4)      Date Filed:

-  -  -  - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -



                          BioMarin Pharmaceutical Inc.
                     371 Bel Marin Keys Boulevard, Suite 210
                                Novato, CA 94949

                    NOTICE OF ANNUAL MEETING OF STOCKHOLDERS
                           TO BE HELD ON MAY 24, 2001

TO THE STOCKHOLDERS:

         NOTICE IS HEREBY  GIVEN that the 2001  Annual  Meeting of  Stockholders
(the  "Annual  Meeting")  of BioMarin  Pharmaceutical  Inc.  ("BioMarin"  or the
"Company") will be held on Thursday,  May 24, 2001 at 10:00 a.m., local time, at
the Company's facility located at 46 Galli Drive,  Novato,  California 94949 for
the following purposes:

1.   To elect five directors of the Company;
2.   To ratify the selection by the Board of Directors of Arthur Andersen LLP as
     the Company's independent auditors for the year ending December 31, 2001;
3.   To  transact  such other  business as  properly  may be brought  before the
     Annual Meeting or any adjournment thereof.

         The foregoing  items of business are more fully  described in the Proxy
statement accompanying this notice (the "Proxy").

         The Board of Directors has fixed the close of business on April 3, 2001
as the record date for determining the  stockholders  entitled to receive notice
of, and to vote at, the Annual Meeting or any  adjournment  thereof.  A complete
list of such stockholders  will be available at the Company's  executive offices
at 371 Bel Marin Keys Boulevard,  Suite 210, Novato,  California  94949, for ten
days before the Annual Meeting.

         All stockholders are cordially invited to attend the Annual Meeting. To
ensure your  representation  at the Annual  Meeting,  however,  you are urged to
complete,  date,  sign and return the enclosed Proxy as promptly as possible.  A
postage-prepaid envelope is enclosed for that purpose. Any stockholder attending
the Annual  Meeting may vote in person even if that  stockholder  has returned a
Proxy.

By Order of the Board of Directors:



Raymond W. Anderson

Chief Operating Officer, Chief Financial Officer, Secretary
and Vice President, Finance and Administration

Novato, California
April 3, 2001

                             YOUR VOTE IS IMPORTANT

IN ORDER TO ASSURE YOUR REPRESENTATION AT THE ANNUAL MEETING,  YOU ARE REQUESTED
TO COMPLETE, SIGN AND DATE THE ENCLOSED PROXY AS PROMPTLY AS POSSIBLE AND RETURN
IT IN THE ENCLOSED ENVELOPE.








                          BioMarin Pharmaceutical Inc.
                     371 Bel Marin Keys Boulevard, Suite 210
                                Novato, CA 94949

                               PROXY STATEMENT FOR
                       2001 ANNUAL MEETING OF STOCKHOLDERS

INFORMATION CONCERNING SOLICITATION OF PROXIES AND VOTING

General

This Proxy is furnished in connection  with the  solicitation  of Proxies by the
Board of Directors of the Company for use at the Annual Meeting of  Stockholders
of the Company (the "Annual  Meeting") to be held on Thursday,  May 24, 2001, or
at any adjournment of the Annual Meeting,  for the purposes set forth herein and
in the foregoing  Notice of Annual Meeting of  Stockholders.  The Annual Meeting
will be held at the  Company's  facility  located  at 46  Galli  Drive,  Novato,
California  94949.  The phone  number is  415-884-6700.  Copies of  solicitation
material and the Company's Annual Report including the financial  statements and
financial statement schedules will be furnished to brokerage houses, fiduciaries
and  custodians to forward to  beneficial  owners of common stock of the Company
held in their names. The Annual Report to Stockholders for the fiscal year ended
December  31,  2000,   including  financial   statements,   will  be  mailed  to
stockholders entitled to vote at the Annual Meeting concurrently with this Proxy
Statement and accompanying Proxy on or about April 17, 2001.

Record Date; Outstanding Securities

The voting  securities  of the Company  entitled  to vote at the Annual  Meeting
consist of shares of common stock.  Only  stockholders of record at the close of
business  on April 3, 2001 are  entitled  to notice of and to vote at the Annual
Meeting.  On April 3, 2001,  there were 37,134,859  shares of common stock,  par
value $0.001 per share,  issued and  outstanding.  Each share of common stock is
entitled  to one vote.  As of the date of  record,  no  shares of the  Company's
Preferred Stock were outstanding.

Revocability of Proxies

A stockholder  who signs and returns a Proxy will have the power to revoke it at
any time  before it is voted.  A Proxy may be revoked by filing with the Company
(Attention:  Raymond W. Anderson, Chief Financial Officer) a written revocation,
or a duly  executed  Proxy  bearing a later date,  or by appearing at the Annual
Meeting and electing to vote in person.

Voting

Each stockholder is entitled to one vote for each share held.

Solicitation of Proxies

This  solicitation  of Proxies is made by the  Company,  and all related  costs,
including  expenses in connection with preparing and mailing this Proxy, will be
borne by the Company.  In addition,  the Company will reimburse  brokerage firms
and other persons representing beneficial owners of shares for their expenses in
forwarding  solicitation material to such beneficial owners. Proxies may also be
solicited by certain of the Company's directors, officers and regular employees,
without additional compensation, in person or by telephone or by facsimile or by
e-mail or by telegram.

Quorums; Abstentions; Broker Non-Votes

The  Company's  Bylaws  provide  that a majority of all the shares of the common
stock entitled to vote,  whether present in person or by Proxy, shall constitute
a quorum for the transaction of business at the Annual  Meeting.  If a quorum is
not present or  represented,  then either the chairman of the Annual  Meeting or
the  stockholders  entitled to vote at the Annual Meeting,  present in person or
represented  by Proxy,  will have the power to adjourn the Annual  Meeting  from
time to time,  without notice other than an  announcement at the Annual Meeting,
until a quorum is present.  At any adjourned Annual Meeting at which a quorum is
present,  any business may be transacted  that might have been transacted at the
Annual  Meeting as  originally  notified.  If the  adjournment  is for more than
thirty  days,  or if after that  adjournment  a new record date is fixed for the
adjourned  Annual  Meeting,  a notice of the adjourned  Annual  Meeting shall be
given to each  stockholder  of record  entitled to vote at the adjourned  Annual
Meeting.

All shares  represented  by valid Proxies  received  prior to the Annual Meeting
will be voted and, where a stockholder  specifies by means of the Proxy a choice
with  respect  to any  matter  to be acted  upon,  the  shares  will be voted in
accordance with the specification so made.


                                       1

Votes cast by Proxy or in person at the Annual  Meeting will be tabulated by the
Inspector  of  Elections  (the  "Inspector")  who  will  be an  employee  of the
Company's  transfer  agent.  The Inspector will also determine  whether or not a
quorum is present.  Except in certain  specific  circumstances,  the affirmative
vote of a majority of shares present in person or represented by Proxy at a duly
held Annual  Meeting at which a quorum is present is required under Delaware law
for  approval of  proposals  presented  to  stockholders.  An  exception to this
procedure relates to the election of directors.  The five nominees receiving the
highest  number of votes  "FOR" a director  will be elected as  directors.  This
number is called a  plurality.  In general,  Delaware law also  provides  that a
quorum  consists  of a majority  of shares  entitled to vote that are present or
represented by Proxy at the Annual Meeting.

The Inspector will treat shares that are voted  "WITHHELD" or "ABSTAIN" as being
present and  entitled  to vote for  purposes of  determining  the  presence of a
quorum but such  shares will not be treated as votes in favor of  approving  any
matter  submitted  to the  stockholders  for a vote.  When  Proxies are properly
dated,  executed and returned,  the shares  represented  by such Proxies will be
voted  at  the  Annual  Meeting  in  accordance  with  the  instructions  of the
stockholder. If no specific instructions are given, the shares will be voted (i)
for the election of the nominees for directors  set forth  herein;  (ii) for the
ratification of Arthur  Andersen LLP as independent  auditors of the Company for
the fiscal year ending  December 31, 2001;  and (iii) at the  discretion  of the
proxy  holder,  upon such other  business as may properly come before the Annual
Meeting or any adjournment thereof.

If a broker  indicates on the enclosed Proxy or its substitute  that such broker
does  not  have  discretionary  authority  as to  certain  shares  to  vote on a
particular matter ("Broker  Non-Votes"),  those shares will not be considered as
present with respect to that matter.  The Company  believes that the  tabulation
procedures  to be  followed by the  Inspector  are  consistent  with the general
statutory requirements in Delaware concerning voting of shares and determination
of a quorum.

Submission of Stockholder Proposals for 2002 Annual Meeting

Stockholders,  who intend to submit a proposal for  inclusion  in the  Company's
Proxy  materials for the 2002 Annual  Meeting of  Stockholders,  must submit the
proposal to the Company no later than December 20, 2001. Stockholders who intend
to  present a  proposal  at the 2002  Annual  Meeting  of  Stockholders  without
inclusion of such proposal in the Company's  proxy materials for the 2002 Annual
Meeting are required to provide  notice of such proposal to the Company no later
than March 29,  2002.  The  Company  reserves  the right to reject,  rule out of
order, or take other  appropriate  action with respect to any proposal that does
not comply with these and other applicable requirements.










































                                       2




SECURITY OWNERSHIP OF CERTAIN BENEFICIAL OWNERS

The  following  table  sets  forth  certain  information  with  respect  to  the
beneficial  ownership  of our  common  stock as of April 3,  2001 as to (i) each
person, or group of affiliated  persons,  who is known by us to own beneficially
more than 5% of our common stock; (ii) each of our directors;  (iii) each of our
executive  officers  named in the Summary  Compensation  Table set forth  herein
under the caption  "Executive  Compensation"  (we refer to all these officers as
the "Named Executive Officers"); (iv) all of our directors and current executive
officers as a group.

Except as  otherwise  noted,  the  persons or  entities  in this table have sole
voting  and  investing  power with  respect  to all the  shares of common  stock
beneficially owned by them subject to community property laws, where applicable.
The  information  with  respect  to each  person  specified  is as  supplied  or
confirmed by such person,  based upon  statements  filed with the Securities and
Exchange Commission ("SEC"), or based upon the actual knowledge of the Company.

                               Number of Number of
                                  Shares Shares


                                                    Beneficially             Subject           Percentage of
           Name of Beneficial Owner                  Owned (1)             To Options           Common Stock
           ------------------------                  ----------            -----------          ------------
                                                                                           
Glyko Biomedical Ltd.                                11,367,617                  0                  30.6%
    Box 25, Commerce Court West
    199 Bay Street
    Toronto, Ontario
    Canada M5L 1A9

BB BioVentures, L.P.(2)                               2,725,787                  0                  7.3%
   One Cambridge Center, 9th Floor
   Cambridge, MA 02142

Genzyme Corporation                                   2,102,563                  0                  5.7%
   One Kendall Square
   Cambridge, MA 02139

Fredric D. Price (3)                                   122,836                 97,836                 *

Grant W. Denison, Jr. (3)                               1,346,939              46,322               3.6%

John C. Klock, M.D. (3)                                815,513                   0                  2.2%

Christopher M. Starr, Ph.D. (3)                        615,722                253,105               1.7%

Emil D. Kakkis, M.D., Ph.D. (3)                        173,986                171,185                 *

Raymond W. Anderson (3)                                200,237                197,549                 *


































                                     3





                               Number of Number of
                                  Shares Shares


                                                    Beneficially             Subject           Percentage of
           Name of Beneficial Owner                  Owned (1)             To Options           Common Stock
           ------------------------                  ----------            -----------          ------------
                                                                                          
John L. Jost, Ph.D. (3)                                109,453                107,260                 *

Ansbert S. Gadicke, M.D., Ph.D. (3)(4)                2,821,412                95,625               7.6%

Erich Sager (3)(5)                                   11,525,892                79,375               31.0%

Gwynn R. Williams (3)                                  57,500                  57,500                 *

All current executive officers and directors
as a group (13 persons)(6)                           18,004,156              1,309,794              48.4%


* Represents less than 1% of the Company's outstanding common stock.

(1)  The "Number of Shares  Beneficially  Owned"  column is based on  37,134,859
     shares of common stock outstanding at April 3, 2001. Shares of common stock
     subject  to  options  or  warrants  that  are  currently   exercisable   or
     exercisable  within  60 days of  April  3,  2001  (the  "Number  of  Shares
     Beneficially  Owned") are deemed to be outstanding  and to be  beneficially
     owned by the person  holding  the  options or  warrants  for the purpose of
     computing  the  percentage  ownership  of the person but are not treated as
     outstanding  for the purpose of computing the  percentage  ownership of any
     other person. The "Number of Shares Subject to Options" enumerates for each
     principal  stockholder,  director and Named  Executive  Officer and for all
     officers and directors in the aggregate, the shares of common stock subject
     to options  exercisable  within 60 days of April 3, 2001.  These shares are
     included in the calculation of the "Number of Shares Beneficially Owned."

(2)  Includes shares held by MPM Asset  Management 1998 Investors L.L.C. and MPM
     BioVentures  Parallel  Fund  L.P.,  both of which  are  affiliated  with BB
     BioVentures L.P.

(3)  The mailing  address for such  stockholder  is c/o BioMarin  Pharmaceutical
     Inc., 371 Bel Marin Keys Blvd., Suite 210, Novato, California 94949

(4)  Includes  2,725,787 shares currently held by BB BioVentures L.P., MPM Asset
     Management 1998 Investors L.L.C. and MPM BioVenture  Parallel Fund L.P., of
     which  Dr.  Gadicke  is an  affiliate.  Dr.  Gadicke  disclaims  beneficial
     ownership in these shares except to the extent of his pecuniary interest.

(5)  Includes 11,367,617 shares held by Glyko Biomedical Ltd. of which Mr. Sager
     is an officer.  Mr. Sager disclaims  beneficial  ownership of these shares,
     except to the extent of his pecuniary interest.

(6)  See footnotes 2, 4 and 5.































                                     4



PROPOSAL ONE:  ELECTION OF DIRECTORS

The Company has a Board of Directors,  currently  consisting of five  directors,
which will be elected at the Annual Meeting.  The Proxy holders may not vote the
Proxies  for a greater  number of  persons  than the number of  nominees  named.
Unless otherwise instructed, the Proxy holders will vote the Proxies received by
them for the five nominees named below,  all of whom are presently  directors of
the Company.  If any nominee is unable or declines to serve as a director at the
time of the Annual Meeting, the Proxies will be voted for any nominees who shall
be designated  by the present Board of Directors to fill the vacancy.  It is not
expected  that any  nominee  will be  unable  to or will  decline  to serve as a
Director. If stockholders nominate additional persons for election as directors,
the Proxy holder will vote all Proxies received by him to assure the election of
as many of the Board of  Directors'  nominees as possible  with the Proxy holder
making any required  selection of specific nominees to be voted for. The term of
office of each person elected as a director shall continue until the next Annual
Meeting of Stockholders or until that person's successor has been elected.

If a quorum is  present,  the five  nominees  receiving  the  highest  number of
affirmative votes of the votes cast shall be elected as directors.

NOMINEES FOR DIRECTOR

Set forth below is certain information regarding our directors:


                    Name                     Age         Position with BioMarin               Director Since

                                                                                      
Fredric D. Price                              55       Chief Executive Officer and             October 2000
                                                       Chairman of the Board
Grant W. Denison, Jr.                         51                Director                       October 1996
Ansbert S. Gadicke, M.D., Ph.D.(1)(2)         43                Director                      December 1997
Erich Sager(1)(2)                             43                Director                      November 1997
Gwynn R. Williams(1)(2)                       67                Director                       October 1996


    (1)           Member of the Compensation Committee.
    (2)           Member of the Audit Committee.

There is no family  relationship  between any director and any executive officer
of the Company.

Fredric  D. Price has  served as  Chairman  and Chief  Executive  Officer  since
November  2000.  From  September  1994  until  September  2000,  Mr.  Price  was
President,  Chief  Executive  Officer and a member of the Board of  Directors of
AMBI Inc., formerly Applied Microbiology,  Inc., (Nasdaq: AMBI), a biotechnology
and  nutrition  company.  From July 1991 to  September  1994,  he served as Vice
President,  Finance &  Administration  and Chief Financial  Officer of Regeneron
Pharmaceuticals,  Inc.  (Nasdaq:  REGN),  and  prior  to  that  was  a  strategy
consultant for biopharmaceutical CEOs and investment groups. Mr. Price began his
career at Pfizer  Pharmaceuticals,  where he simultaneously  held line and staff
positions  as Vice  President,  reporting  directly  to a member of the board of
Pfizer  Inc.  Mr.  Price  received  an  M.B.A.  from the  Wharton  School of the
University of  Pennsylvania  and a B.A.  from  Dartmouth  College.  He is also a
member of the Advisory Board of equity4life AG, a health care investment company
based in Zurich, Switzerland.

Grant W.  Denison,  Jr.  has  served  as a  director  of the  Company  since its
inception.  Mr.  Denison served as Chief  Executive  Officer of the Company from
inception  until his  resignation  in October 2000.  Mr.  Denison also served as
Chairman of the Board of the Company from April 1997 until  October  2000.  From
July 1993 to April 1997, Mr. Denison served as President,  Consumer Products and
Corporate   Senior  Vice   President,   Business   Development   at  Searle,   a
pharmaceutical  company. From July 1989 to June 1993, Mr. Denison served as Vice
President  Corporate  Planning at Monsanto Company,  a diversified life sciences
company.  From April 1986 to June 1989,  Mr.  Denison  served as Vice  President
Corporate  Planning and President,  U.S.  Operations at Searle, a pharmaceutical
company. From 1985 to 1986, Mr. Denison served as Vice President,  International
Operations at Squibb Medical Systems,  a medical devices  company.  From 1980 to
1985, Mr. Denison served as Vice President, Planning and Business Development at
Pfizer,  Inc., a  pharmaceutical  company.  Mr.  Denison serves as a director of
Nastech  Pharmaceutical  Company Inc. and  Genetronics.  Mr. Denison received an
A.B. in  Mathematical  Economics  from  Colgate  University  and an M.B.A.  from
Harvard Graduate School of Business Administration.

Ansbert S. Gadicke,  M.D.,  Ph.D.  has served as a director of the Company since
December  1997.  Since July 1992,  Dr. Gadicke has served as the Chairman of the
Board  and  Managing  Director  of MPM  Capital,  L.P.,  an  investment  company
specializing  in the healthcare  industry.  From 1989 to 1992, Dr. Gadicke was a
consultant with Boston  Consulting  Group.  Dr. Gadicke  currently serves on the
boards of DoubleTwist,  Inc.,  Endeavor,  LXN Corporation,  MediGene AG, Novirio
Pharmaceuticals Limited, Omrix Pharmaceuticals,  Inc., Pharmasset Ltd., ViaCell,
Inc. and  Transform,  Inc. Dr.  Gadicke  received a Ph.D.  and an M.D. from J.W.
Goethe Universitat, Frankfurt, Germany.

                                     5

Erich Sager has served as a director of the Company since November  1997.  Since
September 1996, Mr. Sager has served as the Chairman of LaMont Asset  Management
SA, a private  investment  management  firm. From April 1994 to August 1996, Mr.
Sager served as Senior Vice President, Head of Private Banking for Dresdner Bank
(Switzerland)  Ltd. From  September 1991 to March 1994, Mr. Sager served as Vice
President, Private Banking-Head German Desk for Deutsche Bank (Switzerland) Ltd.
From 1981 to 1989,  Mr.  Sager held  various  positions  at a number of banks in
Switzerland.  Mr. Sager serves as a director of BioNebraska,  Inc.,  Dentalview,
Inc., Kimsa Holding,  LaMont Asset Management,  SA and Sermont Asset Management,
SA. Mr.  Sager  received  a Business  Degree  from the School of  Economics  and
Business Administration in Zurich, Switzerland.

Gwynn R. Williams has served as a director of the Company  since its  inception.
Mr. Williams founded AstroMed Limited and Astroscan Limited, UK manufacturers of
scientific  equipment,  in March 1984, which entities,  in December 1997, merged
into Life  Science  Resources  Ltd.  Previously,  Mr.  Williams was a partner of
Arthur  Andersen & Co., a  mathematician  with General  Motors  Research,  and a
mathematician with British Steel. Mr. Williams was a founder of Glyko Biomedical
Ltd. and its predecessor Glyko, Inc. Mr. Williams received a B.S. in Theoretical
Physics from the University of Wales.

Board Meetings and Board Committees

The Board of  Directors  manages the  business of the  Company.  It  establishes
overall  policies and standards for the Company and reviews the  performance  of
management.  In addition,  the Board has  established  an Audit  Committee and a
Compensation  Committee whose functions are briefly  described  below. The Board
has not established a Nominating Committee.

The Board of Directors of the Company held a total of seven meetings  during the
year ended December 31, 2000 and took action by Unanimous  Written Consent on 11
occasions.  No director  participated in fewer than 75% of all such meetings and
actions of the Board of Directors and the committees thereof, held during fiscal
2000, if any, upon which such director served.

Audit  Committee.  The Audit Committee  provides  oversight of the (a) financial
reporting process,  the system of internal controls and the audit process of the
Company and (b) the Company's  independent  auditors.  The Audit  Committee also
recommends  to  the  Board  of  Directors  the   appointment  of  the  Company's
independent  auditors.  On June 15, 2000,  the Board adopted a new written Audit
Committee Charter (the "Audit  Charter"),  a copy of which is included with this
Proxy.  As required by the Audit  Charter,  Mr. Sager was appointed to the Audit
Committee as the third member.  As also required by the Audit  Charter,  each of
the members of the Audit Committee is an independent director as defined by Rule
4200(a)(15) of the National  Association of Securities  Dealers.  The members of
the Audit Committee are Dr. Gadicke,  Mr. Sager,  and Mr.  Williams.  During the
fiscal year 2000, the Audit  Committee met on two (2)  occasions.  Additionally,
prior to each financial press release, Mr. Williams,  as a representative of the
Audit  Committee,  discussed the  information  included in such release with our
independent auditors.

Compensation  Committee.  The  Compensation  Committee,  which  consists  of Dr.
Gadicke,  Mr. Sager and Mr. Williams,  sets general  compensation policy for the
Company and has final approval power over  compensation  of executive  officers.
The  Compensation  Committee also has final  approval power over  guidelines and
criteria for employees'  bonuses and  administers  the Company's 1997 Stock Plan
and 1998 Director Option Plan. The Compensation Committee met twice in 2000.
Compensation Committee Interlocks and Insider Participation

None of the members of the  Compensation  Committee is currently or has been, at
any time since the  formation  of the  Company,  an officer or  employee  of the
Company. No member of the Compensation Committee serves as a member of the Board
of  Directors  or  compensation  committee  of any  entity  that has one or more
executive  officers  serving as a member of the Company's  Board of Directors or
Compensation Committee.

The Board unanimously recommends voting "For" each of the foregoing nominees for
director.




















                                      6

PROPOSAL TWO:  RATIFICATION OF APPOINTMENT OF INDEPENDENT AUDITORS

The Board of Directors has selected Arthur Andersen LLP,  independent  auditors,
to audit the  financial  statements  of the  Company  for the fiscal year ending
December 31, 2001, and recommends  that  stockholders  vote for  ratification of
such  appointment.  Although  action by stockholders is not required by law, the
Board of Directors has  determined  that it is desirable to request  approval of
this selection by the stockholders.  Notwithstanding the selection, the Board of
Directors,  in its  discretion,  may direct the  appointment of new  independent
auditors at any time during the year, if the Board of Directors  feels that such
a change would be in the best interest of the Company and its  stockholders.  In
the  event of a  negative  vote on  ratification,  the Board of  Directors  will
reconsider its selection.

Arthur  Andersen LLP has audited the  Company's  financial  statements  annually
since  inception on March 21, 1997.  Representatives  of Arthur Andersen LLP are
expected  to be present at the Annual  Meeting  with the  opportunity  to make a
statement if they desire to do so and are expected to be available to respond to
appropriate questions.

For the year ended December 31, 2000, Arthur Andersen LLP billed the Company the
following amounts for the respective professional services:

Audit Fees:                                           $125,000
Financial Information Systems Design and
Implementation Fees:                                  $      -
All Other Fees:                                       $ 32,550


The Audit  Committee  has  considered  the  nature  and amount of these fees and
believes  that the provision of the services  relating to Financial  Information
Systems and All Other Fees is compatible with maintaining  Arthur Andersen LLP's
independence.

The  Board   unanimously   recommends  voting  "For"  the  ratification  of  the
appointment of Arthur  Andersen LLP as  independent  auditors of the Company for
the fiscal year ending December 31, 2001.



















































                                      7

EXECUTIVE OFFICERS

In  addition  to Fredric D. Price the  Company's  Chief  Executive  Officer  and
Chairman of the Board,  whose biography  appears above, the following table sets
forth as of April 3, 2001  information  concerning the Company's other executive
officers:


                  Name                       Age                         Position with BioMarin

                                          
Raymond W. Anderson                          59     Chief Operating Officer, Chief Financial Officer, Secretary and
                                                    Vice President, Finance and Administration
Christopher M. Starr, Ph.D.                  48     Vice President, Research and Development
John L. Jost, Ph.D.                          56     Vice President, Manufacturing
Robert A. Baffi, Ph.D.                       46     Vice President, Quality Assurance/Quality Control
Emil D. Kakkis, M.D., Ph.D.                  40     Vice President, Business Development
Stuart J. Swiedler, M.D., Ph.D.              45     Vice President, Scientific and Clinical Affairs
Brian K. Brandley, Ph.D.                     44     Vice President of BioMarin and Managing
                                                    Director, Glyko, Inc., a wholly-owned subsidiary of BioMarin



Raymond W. Anderson has served as Chief  Financial  Officer and Vice  President,
Finance  and  Administration  since June 1998 and has served as Chief  Operating
Officer  since April 2000.  Mr.  Anderson has served as  Secretary  since August
2000. Mr.  Anderson  served as the Vice  President,  Finance and Chief Financial
Officer at Fusion  Medical  Technologies,  Inc.,  a medical  technology  company
developing  drug delivery  systems,  from July 1997 to June 1998.  Mr.  Anderson
served as the Vice  President,  Finance  and Chief  Financial  Officer  at Fidus
Medical Technology,  Inc., a medical technology company  specializing in cardiac
arrhythmias,  from October 1996 to July 1997. Mr.  Anderson served as a director
of Recombinant  Capital,  a consulting firm, from July 1994 to October 1996. Mr.
Anderson  served as the Vice President,  Finance and Chief Financial  Officer of
Glycomed  Incorporated,  a biotechnology  company, from April 1989 to July 1994.
Mr.  Anderson  was  the  Chief  Financial  Officer  at  Chiron  Corporation,   a
biotechnology  company,  from 1985 to 1989.  Mr.  Anderson was a Controller  and
Director  of  Financial  Planning  and  Analysis  at  Syntex   Laboratories,   a
pharmaceutical  company, from 1981 to 1985. Mr. Anderson served as a director of
Glyko  Biomedical  Ltd.  and  its  predecessor,   Glyko,  Inc.,  a  carbohydrate
analytical and diagnostic company,  from October 1989 to June 2000. Mr. Anderson
received a B.S. in Engineering from the United States Military  Academy,  a M.S.
in  Administration  from  George  Washington  University  and a M.B.A.  from the
Harvard Graduate School of Business Administration.

Christopher  M.  Starr,  Ph.D.  has  served  as  Vice  President,  Research  and
Development  since the Company's  inception.  From July 1991 to April 1998,  Dr.
Starr served as the Vice President, Research and Development for Glyko, Inc. Dr.
Starr  held  the  position  as  National  Research  Associate  at  the  National
Institutes of Health in Bethesda,  Maryland  from August 1986 to June 1991.  Dr.
Starr  received a B.S.  in  Biology  from  Syracuse  University  and a Ph.D.  in
Biochemistry and Molecular Biology from the State University of New York, Health
Science Center, Syracuse, NY.

John L. Jost, Ph.D. has served as Vice President, Manufacturing since June 1999.
Dr. Jost devoted his time from November  1997 to June 1999 to personal  affairs.
Dr.  Jost  served in several  positions  at  Genentech,  Inc.,  a  biotechnology
company,  from February 1983 to November 1997.  From June 1992 to November 1997,
he was Director of  Manufacturing  Sciences at Genentech.  From 1971 to 1983, he
served in various  scientific  positions  in process  development  at The Upjohn
Company, a pharmaceutical  company,  ending as a Senior Research Scientist.  Dr.
Jost received a B.S. and a Ph.D. in Chemical  Engineering from the University of
Minnesota.

Emil D. Kakkis,  M.D.,  Ph.D.  has served as Vice  President  of BioMarin  since
September  1998.  From July 1994 to August  1998,  Dr.  Kakkis  was a  Physician
Specialist at the Department of Pediatrics at Harbor-UCLA  Medical Center.  From
July 1991 to June 1994,  Dr.  Kakkis  completed a Fellowship  in Genetics at the
UCLA Intercampus  Medical Genetics Training Program.  Dr. Kakkis received a B.A.
in Biology from Pomona College and received a Ph.D. in Biological Chemistry from
UCLA. Dr. Kakkis received an M.D. from UCLA.

Stuart J. Swiedler,  M.D.,  Ph.D. has served as Vice President of Scientific and
Clinical  Affairs since June 1998. From November 1997 to June 1998, Dr. Swiedler
was as an independent biotechnology  consultant.  From February 1993 to November
1997, Dr. Swiedler served, in chronological order, with Glycomed Incorporated, a
biotechnology  company, as Assistant Vice President,  Biology from February 1993
to July 1994, as Assistant Vice President,  Research from July 1994 to May 1995,
and as Vice  President,  Research from May 1995 to November 1997.  Dr.  Swiedler
received a B.S. in Biology from the State University of New York at Albany.  Dr.
Swiedler  received an M.D. and a Ph.D.  in  Biochemistry  from the Johns Hopkins
University School of Medicine.





                                       8

Brian K. Brandley,  Ph.D. has served as Vice President of BioMarin since October
1998.  Dr.  Brandley has served as the Managing  Director of Glyko,  Inc.  since
April 1998. He was an Assistant  Professor at Rush  University from July 1995 to
April  1998,  and was the Senior  Scientist,  Head of Cell  Biology at  Glycomed
Incorporated, a biotechnology company, from July 1988 to July 1995. Dr. Brandley
received a B.S. and a M.S. in Biology from the University of Miami. Dr. Brandley
received a Ph.D. in Biology from the University of Sydney, Australia.

Robert A. Baffi,  Ph.D.,  has served as Vice President of Quality  Assurance and
Quality  Control since May 2000. From 1986 to 2000, Dr. Baffi served in a number
of progressively more responsible positions at Genentech, Inc., primarily in the
functional  area of quality  control.  Dr.  Baffi's  most recent role within the
quality  organization at Genentech was Director of  Collaborations  where he was
responsible  for  providing  coordination  and  support  in all areas of quality
assurance and control to over 14 Genentech  collaborative  partners. He was also
responsible   for   assessments  of  the  quality   capabilities   of  potential
collaborative  partners.  Prior  to  Genentech,  Dr.  Baffi  worked  for  Cooper
BioMedical as a research  scientist and at Becton Dickinson Research Center as a
post-doctoral fellow. Dr. Baffi received a Ph.D. in Biochemistry,  a M.Phil. and
a B.S. in Biochemistry from the City University of New York.


EXECUTIVE COMPENSATION

Summary Compensation Table.

We are required by the SEC to disclose  compensation  awarded to,  earned by, or
paid for  services  rendered  to the Company in all  capacities  during the last
three  fiscal  years  to (a) the  Company's  Chief  Executive  Officer;  (b) the
Company's four most highly compensated executive officers,  other than the Chief
Executive  Officer,  who were serving as executive officers at the end of fiscal
year 2000; and (c) up to two  additional  individuals  for whom such  disclosure
would have been  provided  under  clause (a) and (b) above but for the fact that
the individual was not serving as an executive officer of the Company at the end
of fiscal year 2000; provided,  however, that no disclosure need be provided for
any executive  officer,  other than the CEO, whose total annual salary and bonus
does not exceed $100,000.

Accordingly,  the following  table  discloses  compensation  paid by the Company
during the last three fiscal years to (a) Fredric D. Price,  the Company's Chief
Executive Officer; (b) Raymond W. Anderson, Christopher M. Starr, Ph.D., Emil D.
Kakkis,  M.D., Ph.D. and John L. Jost,  Ph.D., the four most  highly-compensated
executive officers,  other than the Chief Executive Officer, who were serving as
executive  officers  at the end of fiscal  year 2000 and whose  salary and bonus
exceeded  $100,000;  and (c) Grant W. Denison,  Jr. and John C. Klock,  M.D. our
former Chief Executive Officer and former President,  respectively.  We refer to
all of these officers as the "Named Executive Officers."

The entries under the column "Other Annual  Compensation" for Mr. Price, include
the value of his restricted stock grant issued upon his employment commencement,
a tax gross-up benefit  relating to the compensation  earned from the restricted
stock grant and payments for temporary  housing and for Mr.  Denison,  relate to
payments  for  temporary  housing.  Each of these  amounts  paid to Mr. Price is
described in more detail in the section captioned  "Employment  Agreements." The
entries  under the column "All Other  Compensation"  in the table  represent the
premiums paid for life  insurance  benefits and vested 401(k)  matching for each
Named Executive Officer.
































                                     9



                           SUMMARY COMPENSATION TABLE

                                                                                        LONG-TERM
                                                                                      COMPENSATION

                                                  ANNUAL COMPENSATION (1)                AWARDS
                                         ------------------------------------------ ------------------
                                                                                       SECURITIES
                                                                   OTHER ANNUAL        UNDERLYING          ALL OTHER
NAME AND PRINCIPAL POSITION     YEAR     SALARY ($)   BONUS ($)  COMPENSATION ($)    OPTIONS/SARS(#)   COMPENSATION ($)
------------------------------- -------- ------------ ---------- ------------------ ------------------ ------------------

                                                                            
Fredric D. Price (1)            2000          99,487    100,000            578,424            523,288                 --
Chairman and Chief Executive    1999              --         --                 --                 --                 --
Officer                         1998              --         --                 --                 --                 --

Christopher M. Starr, Ph.D.     2000         190,000     75,000                 --             75,194              1,614
(2)                             1999         150,000    150,000                 --             94,189                876
Vice President, Research and    1998         139,560     87,550                 --            200,000                876
Development

Emil D. Kakkis, M.D., Ph.D.     2000         229,327     50,000                 --             50,000              1,009
(2)                             1999         225,000         --                 --             64,502                336
Vice President, Business        1998          75,000     50,000                 --            200,000                 18
Development

Raymond W. Anderson (2)         2000         222,466     25,000                 --             58,469              1,893
Chief Operating Officer,        1999         189,625    100,000                 --             72,658              2,352
Chief Financial Officer,        1998          90,484         --                 --            200,000                 --
Secretary and Vice President,
Finance and Administration

John L. Jost, Ph.D. (2)         2000         200,000     50,000                 --             17,365              1,681
Vice President, Manufacturing   1999         106,154         --                 --            200,000                645
                                1998              --         --                 --                 --                 --

Grant W. Denison, Jr. (3)       2000         214,615    128,572                 --             32,582                447
Former Chief Executive Officer  1999         257,143    257,143             32,805            154,574                240
                                1998         202,500     87,550                 --            400,000                 --

John C. Klock, M.D. (4)         2000         195,769    125,000                 --             32,807                875
Former President                1999         250,000    250,000                 --            121,246              2,142
                                1998         222,450     87,550                 --            300,000              2,142
- - - - - - - - - - - - - - - -



(1)  Mr. Price was appointed as the Chief Executive  Officer  effective  October
     31, 2000.

(2)  Options  were earned for the fiscal  year 2000 and granted on December  29,
     2000. Long-term options vest 6/48ths at June 30, 2001 and 1/48ths per month
     thereafter.

     Short-term  options vest 25% per 2001 calendar  quarter for options granted
     on December 29, 2000.  Short-term  options  earned on June 30, 2000 for Mr.
     Denison,  Dr.  Klock and Dr.  Starr in the  amounts of  17,582,  17,807 and
     20,959, respectively, vested 100% on June 30, 2000.

     Short-term  options  include  options to purchase  15,000 shares granted to
     each of Mr. Denison and Dr. Klock under the 1998 Director  Option Plan upon
     termination of their respective employment with the Company.

(3)  Mr. Denison resigned as Chief Executive Officer and Chairman on October 31,
     2000, but remains a member of the Board.

(4)  Dr.  Klock  resigned as President on July 31, 2000 and resigned as Director
     on November 1, 2000.

















                                     10

Stock Option Grants Table.  The following  table sets forth certain  information
for each grant of options to purchase the  Company's  common stock during fiscal
2000 to each of the Named Executive  Officers.  Except as otherwise  noted,  all
these  options  were  granted  under the 1997  Stock Plan and have a term of ten
years subject to early  termination  in the event the officer's  services to the
Company cease.

                         Fiscal 2000 Stock Option Grants



                                 Number of     Percent of
                                Securities   Total Options                    Option         Potential Realizable Value at
                                Underlying     Granted to     Exercise         Term          Assumed Annual Rates of Stock
                                  Options      Employees     Price Per      Expiration       Price Appreciation for Option
                                 Granted      in 2000 (1)   Share($) (2)       Date                  Term ($) (3)
                               -------------  -----------     -----------   ----------         ------------------------------
Name                                                                                               5%                    10%
------------------                                                                             -------------       -----------
                                                                                                   
Fredric D. Price                      500,000     18%               12.50    10/30/10             3,930,591          9,960,890
                                       23,288                        9.50    01/01/11               139,134            352,593
Grant W. Denison, Jr.                  17,582      1%               17.00    06/30/10               187,973            476,360
                                       15,000 (5)                   13.00    10/31/10               122,634            310,780
John C. Klock, M.D. (4)                17,807      1%               17.00    01/30/01                     -                  -
                                       15,000 (5)                   19.00    01/30/01                     -                  -
Christopher M. Starr, Ph.D.            20,959      3%               17.00    06/30/10               224,077            567,855
                                       54,235                        9.50    01/01/11               324,027            821,148
Emil D. Kakkis, M.D., Ph.D.            50,000      2%                9.50    01/01/11               298,725            757,028
Raymond W. Anderson                    58,469      2%                9.50    01/01/11               349,323            885,253
John L. Jost, Ph.D.                    17,365      1%                9.50    01/01/11               103,747            262,916


(1)  Based on an aggregate of 2,923,835 shares subject to options granted by the
     Company  during  fiscal year 2000 to employees,  consultants  and the Named
     Executive Officers.

(2)  Options were granted at an exercise price equal to the fair market value of
     the common stock,  as determined by the Board of Directors,  on the date of
     grant or by the closing price on the Nasdaq National Market.

(3)  The 5% and 10% assumed annual rates of compounded stock price  appreciation
     are  mandated  by rules of the  Securities  and  Exchange  Commission.  The
     Company  cannot  assure any  executive  officer or any other  holder of its
     securities  that the actual stock price  appreciation  over the option term
     will be at the  assumed  5% and 10% levels or at any other  defined  level.
     Unless the market  price of its common  stock  appreciates  over the option
     term,  no  value  will be  realized  from  the  option  grants  made to the
     executive  officers.  The  potential  realizable  value  is  calculated  by
     assuming that the closing price per share on the date of grant  appreciates
     at the indicated rate for the entire term of the option and that the option
     is exercised at the exercise  price and sold on the last day of its term at
     the appreciated price. The potential realizable value computation is net of
     the applicable  exercise price,  but does not take into account  applicable
     federal  or state  income tax  consequences  and other  expenses  of option
     exercises or sales of appreciated  stock.  The values shown do not consider
     non-transferability  or termination of the options upon termination of such
     employee's employment with the Company.

(4)  Dr. Klock's  options  terminated on January 30, 2001 due to his resignation
     as  an  officer  and  director  of  the  Company.   These  options  expired
     unexercised.

(5)  These options were granted  under the 1998 Director  Option Plan and have a
     term of ten years subject to early  termination in the event the Director's
     services to the Company cease.




















                                       11

Fiscal Year-End Option Value Table. The following table sets forth the number of
shares covered by both exercisable and unexercisable  stock options held by each
of the Named Executive Officers at December 31, 2000.


                                             Fiscal Year-End Option Value Table

                                             Number of Securities              Value of Unexercised
                                            Underlying Unexercised             In-the-Money Options
                                           Options at Year-End                    at Year-End (1)
                                       ------------------------------     --------------------------------
Name                                   Exercisable      Unexercisable     Exercisable     Unexercisable
--------------------------             -----------      -------------     -------------   ----------------
                                                                                      
Fredric D. Price                           27,778          495,510            $     -            $  4,367
Grant W. Denison, Jr.                      89,518          185,833            $ 293,278          $ 608,331
John C. Klock, M.D.                        42,803           81,563             $  8,398          $ 113,380
Christopher M. Starr, Ph.D.               209,731          139,652            $ 853,956          $ 314,338
Emil D. Kakkis, M.D., Ph.D.               144,710          169,792            $ 695,904          $ 505,729
Raymond W. Anderson                       175,032          149,095            $ 859,194          $ 328,934
John L. Jost, Ph.D.                        82,633          156,427            $     -            $   3,256


(1)  Based on closing  price on  December  31,  2000 of  $9.6875  per share less
     exercise price per share.




Employment Agreements

We are party to employment  agreements with each executive  officer on the terms
enumerated on the chart below. Each of these employment agreements is terminable
without  cause by the  Company  upon six  months  prior  written  notice  to the
officer,  or by the  officer  upon  three  months  prior  written  notice to the
Company. The Company is obligated to pay the officer's salary and benefits until
this termination. Except in the case of Mr. Price, in the event that the officer
is  involuntarily  terminated  within  one year of a change  of  control  of the
Company,  he will  receive  (i) a severance  payment  equal to six months of his
annual  salary;  (ii) a bonus  equal to 50% of the  annual  bonus  that he would
otherwise  be entitled  to, and (iii)  immediate  vesting of 50% of the unvested
portion of his outstanding options to purchase Company capital stock.















































                                       12




                                      2000                                      Initial Grant of Right            Agreement
                                     Annual                                          to Purchase                 Termination
Name of Executive Officer       Salary Rate (1)         Annual Bonus              Equity Securities                 Date
-------------------------       ---------------         ------------              -----------------                 ----

                                                                                                  
Fredric D. Price                    $400,000     Annual bonus, payable     Option to purchase up to           October 30, 2003
                                                 in cash based on          500,000 shares of
                                                 performance.              our common stock at a purchase
                                                                           price of $12.50 per share.

Christopher M. Starr, Ph.D.         $230,000     Annual bonus, payable     400,000 shares of                        None
                                                 in cash or stock.         our common stock at a purchase
                                                                           price of $1.00 per share.

Brian K. Brandley, Ph.D.            $139,500     Annual bonus, payable     Option to purchase up                    None
                                                 in cash or stock.         to 150,000 shares of
                                                                           Glyko Biomedical Ltd.'s
                                                                           common stock. Now
                                                                           exercisable for 65,415
                                                                           shares of our common
                                                                           stock at a purchase price
                                                                           of $5.27 per share.

Raymond W. Anderson                 $230,000     Annual bonus, payable     Option to purchase up to                 None
                                                 in cash or stock.         200,000 shares of our
                                                                           common stock at a purchase
                                                                           price of $4.00 per share.

Emil D. Kakkis, M.D., Ph.D.         $225,000     Eligible to receive a     Option to purchase up to                 None
                                                 cash bonus based on       200,000 shares of our
                                                 achievement of            common stock at a purchase
                                                 milestones.               price of $4.00 per share.

John L. Jost, Ph.D.                 $200,000     Annual bonus, payable     Option to purchase up to                 None
                                                 in cash or stock.         200,000 shares of our
                                                                           common stock at a purchase
                                                                           price of $13.00 per share.

Stuart Swiedler, M.D., Ph.D.        $210,000     Annual bonus, payable     Option to purchase up to                 None
                                                 in cash or stock.         150,000 shares of our
                                                                           common stock at a purchase
                                                                           price of $4.00 per share.

Robert A. Baffi, Ph.D.              $200,000     Annual bonus, payable     Option to purchase up to                 None
  (hired in May 2000)                            in cash or stock.         200,000 shares of our
                                                                           common stock at a purchase
                                                                           price of $22.00 per share.


(1)  2000 Annual Salary Rate  reflected in the above table  reflects 2000 annual
     salary  rate as of  December  31,  2000.  Actual  salaries  paid  in  2000,
     reflected  in the  table  entitled  "SUMMARY  COMPENSATION  TABLE"  include
     mid-year salary adjustments.































                                       13

Effective  October 31, 2000,  in  connection  with Mr.  Price's  employment,  we
entered  into an  employment  agreement  with him which  provides for an initial
payment to Mr. Price of $357,624, an annual base salary of $400,000 in the first
year,  $450,000 in the second year,  and $500,000 in the third year, and a bonus
of $200,000 in the first year and a performance bonus of between 25% and 100% of
his  respective  annual base salary for each of the second and third  years.  In
addition,  we granted Mr.  Price  25,000  restricted  shares of our common stock
(which vest in 3 equal annual  installments  commencing on January 1, 2001),  we
granted Mr. Price an option to purchase 500,000 shares of our common stock, at a
purchase  price of  $12.50  per  share  (which  vests  monthly  over 36  months,
commencing October 30, 2000) and we further agreed to grant Mr. Price additional
options on each  anniversary of the agreement,  in an amount to be determined by
the Board.  We also agreed to reimburse  Mr. Price for all expenses  incurred in
relocating  to the area  with a tax  gross-up  adjustment  and to  extend  him a
$1,500,000 interest-deferred loan for the purchase of a house.

The  agreement  has a three-year  term,  which will  automatically  renew for an
additional three-year period unless either Mr. Price or we give the other notice
of our  intent  not to renew  the  agreement.  If we  decide  not to  renew  the
agreement,  we must pay Mr.  Price an  amount  such that our net  payment  after
deduction  of all payroll  taxes and all income  taxes at the  highest  marginal
rates  applicable to him will equal the base salary and bonus we paid him in the
third year of the agreement. Additionally, the expiration for any vested options
will be one year from the termination of the agreement and all unvested  options
will remain unvested and unexercisable.

Either party can terminate the agreement on sixty days' notice.  However, in the
event  there is a change in  control  which  results  in Mr.  Price's  actual or
constructive  termination,  he is entitled to a severance payment equal to twice
the  aggregate of his annual base salary and bonus  payable in the year in which
termination occurs, forgiveness of all outstanding principal and interest on the
interest-deferred  loan, acceleration of the full unvested portion of his 25,000
share  restricted  stock grant and all stock options and an  additional  payment
equal to Mr. Price's maximum total income tax liability  applicable to the total
severance  package.  If Mr.  Price is  terminated  other than for  cause,  he is
entitled to receive a severance  payment  equal to twice his  applicable  annual
base salary and bonus if he is terminated in the first year of the agreement and
equal to his  applicable  annual base salary and bonus of he is  terminated in a
subsequent  year,  forgiveness of all outstanding  principal and interest on the
interest  deferred-loan  and  acceleration  of the full unvested  portion of his
25,000 share restricted stock grant and all stock options.  Additionally,  if he
is  terminated  other  than for cause  prior to the  second  anniversary  of the
agreement,  he is entitled to an additional payment equal the maximum income tax
liability associated with forgiveness of the loan and such additional payment.

We  provided  a  three-year  loan to Dr.  Starr for the  purchase  of his shares
referenced in column four of the table above and to each of Mr.  Denison and Dr.
Klock for the purchase of 1,300,000 and 800,000  shares of the Company's  common
stock all of which were  purchased  pursuant  to the  Founders'  Stock  Purchase
Agreement described below. Each loan bears interest at a rate of 6%. For each of
them, respectively, if his employment is terminated by us for any reason, he has
the right to sell any or all of these  shares  of common  stock to us at a price
per share equal to the lesser of the  then-current per share market price of the
shares or the  original  per share  purchase  price of $1.00.  Upon Dr.  Klock's
resignation  as  President,  we  repurchased  33,333  shares of common  stock in
consideration for reducing his note obligation by $33,333 and Dr. Klock paid the
remainder of his note including  interest.  The repurchase right was pursuant to
provisions contained in the Founders' Stock Purchase Agreement. These repurchase
rights have since lapsed with respect to all shares held by Mr.  Denison and Dr.
Starr.

Dr.  Brandley's  employment  agreement was originally  with Glyko,  Inc. but was
assigned to us in connection with our acquisition of Glyko, Inc.

Through June 30, 2000,  the founder's  cash bonus for each of Mr.  Denison,  Dr.
Klock  and Dr.  Starr  was based on the  difference  between  a  minimum  market
capitalization  and our  quarterly  market  capitalization.  The  cash  bonus is
calculated as follows:

The Board of Directors  established  a minimum  market  capitalization  of $20.0
million  for the  first  quarter  of 1998.  The  minimum  market  capitalization
increases  by $1.0  million per quarter  until the end of the  agreement  in the
second quarter of 2000. Our quarterly market capitalization is calculated at the
end of each  calendar  quarter by  multiplying  the number of our common  shares
outstanding times the average closing price of our common stock for the last ten
trading  days of the quarter.  If our common  stock is not  publicly  traded the
quarterly market  capitalization  is determined by multiplying the shares of our
common stock  outstanding  by the price at which of our common stock was sold in
the latest significant  investment by an independent  third-party investor.  For
each full $5.0  million that the  quarterly  market  capitalization  exceeds the
minimum market capitalization,  the founders each receive a cash bonus of $1,200
in the first calendar  quarter and $1,250 in the second quarter.  Each founder's
cash bonus is the sum of the two quarterly bonuses for 2000.





                                       14

Each founder's  total cash bonus may not exceed 100% of base salary in any year.
Additional  amounts  beyond  the cash limit that may be earned in a year will be
paid in stock options using the Black-Scholes  option pricing model to calculate
the value of the stock option based on period-end parameters.

There are no adjustments in the founders'  annual base salaries that result from
increases in market capitalization.

In December 1998, the Board approved a form of  indemnification  agreement to be
entered  into  between  us  and  each  of  our  officers  and  directors.   This
indemnification agreement requires us, among other things, to indemnify officers
and directors  against  liabilities  that may arise by reason of their status or
performance  of their  duties as  officers  or  directors  and to advance  their
expenses  incurred as a result of any  proceeding  against them as to which they
could be indemnified.

For a description  of other  transactions  between the Company and affiliates of
the Company, see "Certain Relationships and Related Transactions".

Section 162(m)

The Company has considered the potential future effects of Section 162(m) of the
Internal  Revenue  Code  on the  compensation  paid to the  Company's  executive
officers.  Section  162(m)  disallows  a tax  deduction  for any  publicly  held
corporation  for individual  compensation  exceeding $1.0 million in any taxable
year  for  any  of  the  Named  Executive   Officers,   unless  compensation  is
performance-based.  The  Company  has adopted a policy  that,  where  reasonably
practicable,  the Company will seek to qualify the variable compensation paid to
its executive  officers for an exemption from the  deductibility  limitations of
Section 162(m).

Director Compensation

Directors do not receive cash  compensation  for their  services as directors of
the Company  but are  reimbursed  for their  reasonable  expenses  in  attending
meetings of the Board and while  performing  services for the  Company.  In July
2000,  under the Company's  1997 Stock Plan, Mr. Sager and Dr. Gadicke were each
issued an option to purchase an additional  50,000 shares of common stock of the
Company at an exercise  price set at the fair market  value on the date of grant
(closing  price of  common  stock on the  Nasdaq  National  Market),  which  was
$20.375,  as  consideration  for  services  rendered  by them to the  Company in
connection with the search for a new senior executive  officer.  In August 2000,
under the Company's  1998 Director  Option Plan, the Company issued to Dr. Klock
an option to purchase  15,000 shares of common stock with an exercise  price set
at the fair market value (closing  price of common stock on the Nasdaq  National
Market) on the date of grant, which was $19.00, as consideration for his ongoing
services to the Company as a director.  In October 2000, under the 1998 Director
Option  Plan,  the Company  issued to Mr.  Denison an option to purchase  15,000
shares of common  stock  with an  exercise  price set at the fair  market  value
(closing  price of common  stock on the Nasdaq  National  Market) on the date of
grant,  which was  $13.00,  as  consideration  for his  ongoing  services to the
Company as a director.  In November 2000,  under the 1998 Director  Option Plan,
the Company  issued to Mr.  Sager and Mr.  Williams  options to purchase  15,000
shares of common  stock  with an  exercise  price set at the fair  market  value
(closing  price of common  stock on the Nasdaq  National  Market) on the date of
grant,  which was $9.6875 as  consideration  for their  ongoing  services to the
Company as directors. In December 2000, under the 1998 Director Option Plan, the
Company  issued to Dr.  Gadicke an option to  purchase  15,000  shares of common
stock with an exercise  price set at the fair  market  value  (closing  price of
common  stock on the Nasdaq  National  Market)  on the date of grant,  which was
$9.6875, as consideration for his ongoing services to the Company as a director.

1998 Director Option Plan

The 1998 Director  Option Plan was adopted by the Board in December 1998. It was
approved by the  stockholders  as of January 15, 1999. The plan provides for the
grant of  nonstatutory  stock  options  to  non-employee  directors.  A total of
200,000 shares of Company common stock have been reserved for issuance under the
plan.  The plan also  provides  for an annual  increase in this number of shares
equal to the lesser of: (1) 0.5% of the Company's outstanding capital stock, (2)
200,000 shares, or (3) a lesser amount determined by the Board.

In fiscal year 2000, options to purchase,  in the aggregate,  75,000 shares were
issued to directors.

The 1998 Director  Option Plan provides that each  non-employee  director  shall
automatically  be granted an option to purchase  20,000 shares of Company common
stock on the date which that person first becomes a non-employee director.  This
option shall have a term of ten years. The shares subject to this initial option
shall vest over one year. Each  non-employee  director shall  thereafter also be
automatically  granted an option to  purchase  15,000  shares of Company  common
stock  on the  first  anniversary  of  the  date  of  their  respective  initial
appointments to the Board and each anniversary  thereafter,  provided that he or
she retains the Board seat on his or her anniversary date. The shares subject to
this annual  option shall vest in full one year from the date of grant and shall
have a term of ten years.  These options  shall  continue to vest only while the
director serves.  The exercise price per share of each of these options shall be
100% of the fair market value of a share of Company  common stock at the date of
the grant of the option.

                                       15

In the event of a merger or the sale of  substantially  all of the assets of the
Company, each option may be assumed or substituted by the successor corporation.
If an option is assumed or substituted, it shall continue to vest as provided in
the plan.  However,  if a  non-employee  director's  status as a director of the
Company or the successor corporation,  as applicable, is terminated,  other than
upon a voluntary  resignation  by the  non-employee  director,  the option shall
immediately  become fully vested and exercisable.  If the successor  corporation
does not agree to assume or  substitute  the option,  each option  shall  become
fully  vested  and  exercisable  for a period of 30 days from the date the Board
notifies the optionee of the option's  full  exercisability,  after which period
the option shall terminate.

Options granted under the plan must be exercised  within three months of the end
of the optionee's tenure as a director, or within 12 months after termination by
death or  disability,  but in no event later than the expiration of the option's
ten-year term. No option granted under the plan is  transferable by the optionee
other  than by will or the laws of  descent  or  distribution.  Each  option  is
exercisable,  during the lifetime of the optionee, only by the optionee.  Unless
sooner terminated by the Board, the plan will terminate  automatically ten years
from the effective date of the plan.

The information  contained below under the captions "Performance Graph," "Report
of the  Compensation  Committee  on Executive  Compensation"  and "Report of the
Audit  Committee"  shall  not be  deemed to be  "soliciting  material"  or to be
"filed" with the Securities and Exchange Commission,  nor shall such information
be  incorporated by reference into any future filing under the Securities Act of
1933, as amended, or the Securities Exchange Act of 1934, as amended,  except to
the extent that the Company specifically  incorporates it by reference into such
filing.

PERFORMANCE GRAPH

The following graph compares the Company's  cumulative total stockholder  return
with the  cumulative  total  return of the Nasdaq  Stock  Market  (U.S.) and the
Nasdaq  Pharmaceutical  Index of stocks in  Standard  Industry  Code  (SIC) 283,
encompassing  primarily  biotechnology,  pharmaceutical  and  medical  specialty
companies,  assuming  a $100  investment  in common  stock on July 31,  1999 and
reinvestment  of dividends  during the period.  The period  covered by the graph
includes  that  portion of the fiscal year ended  December 31, 1999 during which
the Company was publicly traded.

[OBJECT OMITTED]













































                                       16

REPORT OF THE COMPENSATION COMMITTEE ON EXECUTIVE COMPENSATION

The Compensation Committee is responsible for setting general compensation goals
and operational  guidelines for Company  personnel,  for setting all elements of
the  compensation  of the executive  officers of the Company,  and for approving
grants of stock options for the Company. In 2000, the Compensation Committee was
composed of the three outside, non-management members of the Board of Directors.

Compensation Goals and Policies

The goal of the  Company's  compensation  policies  is to  provide  compensation
sufficient to attract,  motivate and retain  executives and staff of outstanding
ability and  potential.  Compensation  policies  are  intended to  establish  an
appropriate  relationship  between  executive  compensation  and the creation of
stockholder  value as  measured  by the equity  markets.  The  Company  uses the
following principles to help achieve that goal:

(1.) The Company provides competitive  compensation  packages  incorporating all
     compensation  elements for executives and staff based upon internal Company
     policies and compensation packages at similarly situated pharmaceutical and
     biotechnology companies in the San Francisco Bay Area.

(2.) The Company rewards executives and senior staff for outstanding performance
     by the individual and by the Company.
(3.) The Company seeks to align the long-term  interests of the  stockholder and
     the  executives  and the senior  staff  through the use of  employee  stock
     options and other stock priced related  compensation,  such as our Employee
     Stock Purchase Plan.

Considerations for 2000 Compensation

Increases in base salary for 2000 were made  effective  January  2000  primarily
based  on  the  progress  and  achievements  of  the  Company  during  1999  and
competitive conditions in the job marketplace for biotechnology expertise in the
San Francisco Bay Area marketplace.

The Compensation Committee took particular note of 1999 achievements including a
successful  initial public  offering,  continued  progress on the Company's lead
programs:   Aldurazyme   for   MPS-I,   rhASB   (then   known   as  BM   102  or
N-acetylgalactosamine-4 sulfatase) for MPS-VI, and Vibriolysin (then known as BM
202) for severe burn debridement. However, the Compensation Committee also noted
that, in November 1999, the FDA had requested a confirmatory  Phase III clinical
trial for Aldurazyme which had the effect of significantly delaying the approval
of that product and delaying the potential growth of stockholder value.

Based on the Compensation  Committee's  judgment as to the value of these events
and other less visible internal developments, the Compensation Committee awarded
long-term  compensation  stock option grants to the  executives and staff of the
Company.  Except for the  Company  founders  and others who were  covered  under
separate employment agreements,  the Committee also granted short-term incentive
stock  options as a reward for the  Company's  progress in comparison to plan in
1999. Grants under both programs were pro-rated for the portion of the year that
the  employee  was in the service of the Company.  Salary  compensation  for the
staff below the rank of officer  was  generally  increased  by an average of 5%,
which  approximated  the reported  average salary increase in the  biotechnology
industry in the San Francisco Bay Area and which was also  pro-rated for time in
service  during  the year.  Benefits  were  improved  primarily  in the  Company
matching  payments  under the 401(k)  plan,  a benefit  feature that was weak in
comparison to competitive peer companies. The Compensation Committee deemed that
these compensation actions were appropriate for the progress made by the Company
in 1999 and  maintained a competitive  balance with  biotechnology  companies of
similar size and state of development in the region.

With  respect  to officer  compensation,  and based on an  overall  analysis  of
performance  factors and in particular  the delays  resulting from an Aldurazyme
Phase III  clinical  trial,  the  Compensation  Committee  determined  that,  in
January,  officers would not receive salary increases and would receive only 50%
of normal long term  compensation  stock option grants  (compared to the program
algorithms used for all staff in 1999 and used for lower ranks in 2000).  During
the course of 2000, one officer was given a raise as a result of a promotion and
one officer  received an  increase  in base salary to a  competitive  level as a
result of the expiration of his founders' employment agreement.
















                                     17

Chief Executive Officer Compensation

 In the first half of 2000, the  compensation  of the three  employee  founders,
including the then Chief Executive  Officer,  Mr. Grant W. Denison Jr., for 2000
was set by compensation  and bonus formulas in their employment  agreements,  as
amended,  which were effective  until June 30, 2000. The primary  determinant of
the founders' bonus for the first six months of 2000 was based on the difference
between  a  minimum  target  market  capitalization  and  the  Company's  market
capitalization as measured quarterly.

Based on the  application of the bonus formula for the first six months of 2000,
the  founders  reached the limit for cash bonus,  which was 100% of base salary.
Additional  bonus amounts beyond the cash limit were paid in stock options using
the Black-Scholes option- pricing model based on the period-end market price and
other market parameters.

As Chairman and Chief Executive Officer,  Mr. Denison continued at his 1999 base
salary from  January 1, 2000 until  October 31, 2000 when he resigned  from both
positions but remained as a member of the Board of Directors.  No bonus was paid
for the period from the end of his  founders'  employment  agreement at June 30,
2000 until October 31, 2000.

As of  October  31,  2000,  Mr.  Fredric  D.  Price was  elected by the Board of
Directors to the position of Chief Executive  Officer and Chairman of the Board.
The  Compensation  Committee  negotiated  a  competitive   compensation  package
appropriate   to  Mr.   Price's   demonstrated   leadership   capabilities   and
achievements.  In negotiating this package, the Compensation Committee took into
consideration the following factors:

(1.) Mr.  Price's six years of  experience as the Chief  Executive  Officer of a
     biotechnology/nutrition   company  during  which  time  he  demonstrated  a
     dedication to the successful  commercialization  of products and the vision
     to re-orient the business away from research  programs which had challenges
     beyond the resources of that company.
(2.) Mr. Price's three years of experience as the Chief  Financial  Officer of a
     biotechnology  company.  This period  demonstrated the proven capability to
     develop and maintain  strong,  positive  relationships  with  investors and
     analysts and to help secure a strong financial position for that company.
(3.) Mr.  Price's  five  years of  experience  as a strategy  consultant  to the
     biopharmaceutical  industry  and to investor  groups  during  which time he
     developed a capacity for concise,  insightful  and  realistic  analysis and
     actionable  recommendations and an understanding of the success factors and
     risks of biopharmaceutical development and commercialization.
(4.) Mr. Price's record of advancement  and achievement in the earlier phases of
     his career in  progressively  more  responsible  positions in both line and
     staff assignments at Pfizer Pharmaceuticals.
(5.) Mr. Price's demonstrated problem solving and analytical  capabilities while
     serving as a paid  consultant to BioMarin in the one-month  period prior to
     his election as Chief Executive Officer.

The  Compensation  Committee also  considered Mr.  Price's  compensation  in his
previous position,  competitive compensation levels for Chief Executive Officers
in the  biotechnology  industry  especially  in the San  Francisco  Bay Area (as
recorded in a well-regarded  salary and  compensation  survey),  and Mr. Price's
qualifications in a favorable  comparison to the  qualifications of the slate of
candidates developed for the Committee by an executive search firm.

Based on the above and additional  considerations,  the  Compensation  Committee
signed a  three-year  employment  agreement  with Mr.  Price that  includes  the
following major compensation elements:

(1.) A base  annual  salary in the first year of  $400,000,  with  increases  to
     $450,000 in the second year and to $500,000 in the third year.
(2.) A  sign-on  bonus  of  $100,000  and  annual  cash  bonuses  based  on  the
     achievement of pre-set goals set by the Board of Directors.  The cash bonus
     for the first year will be $200,000  and the minimum cash bonus for each of
     the second and third years will be 25% of the base  salary in those  years.
     The  maximum  cash bonus in the second and third years will be 100% of base
     salary in those years.
(3.) A stock  grant of 25,000  restricted  shares  that will vest in three equal
     parts on January 1, 2001, January 1, 2002 and January 1, 2003. We also made
     a payment to Mr. Price of $257,624  related to his tax liability  resulting
     from this initial stock grant.
(4.) A stock option to purchase  500,000 shares of the Company's common stock at
     the closing price on the Nasdaq  National  Market on October 30, 2000 which
     was $12.50 per share.  Subsequent  option grants to purchase 125,000 shares
     will be granted on each  anniversary date (October 31st) during the term of
     the agreement. The options vest monthly over a three- year period.
(5.) A loan of up to  $1,500,000  with  interest  deferred for the purchase of a
     residence in connection with a relocation closer to the Company. Payment of
     relocation expenses including an appropriate gross-up to compensate for any
     tax liability incurred is also included.
(6.) Significant  severance  and  change  of  control  benefits,  including  the
     following maximum benefits if he is actually or  constructively  terminated
     following a change of control or if he is terminated  other than for cause:
     the payment of twice his annual base salary and bonus,  full vesting of his
     restricted  stock  grant,  forgiveness  of his loan,  and the  payment of a
     gross- up bonus based upon Mr. Price's maximum tax liability related to the
     total severance package.
                                       18

(7.) If we  elect  not to  renew  the  employment  agreement  for an  additional
     three-year  term,  we will pay Mr. Price an amount equal to his base salary
     and bonus paid in the third year of the  agreement  and a payment  equal to
     his maximum tax liability related the total severance package.

 The  Compensation  Committee  believes that the above  authorized  compensation
actions based upon Company achievements and competitive compensation levels will
serve to help retain a highly  qualified  and  motivated  staff led by excellent
senior  management  that is a requirement  for the prosperity of the Company and
the creation of stockholder value.

Respectfully  submitted  on April 3,  2001 by the  members  of the  Compensation
Committee of the Board of Directors:

Erich Sager
Ansbert S. Gadicke, M.D., Ph.D.
Gwynn R. Williams

REPORT OF THE AUDIT COMMITTEE

The Audit Committee reviews the Company's  financial reporting process on behalf
of the Board of Directors.  Management  has the primary  responsibility  for the
financial statements and the reporting process, including the system of internal
controls.

In this context,  the Committee has met and held discussions with management and
the  independent  auditors.  Management  represented  to the Committee  that the
Company's  consolidated  financial  statements  were prepared in accordance with
generally  accepted  accounting  principles,  and the Committee has reviewed and
discussed  the  consolidated  financials  statements  with  management  and  the
independent  auditors.  The Committee  discussed with the  independent  auditors
matters  required to be  discussed by  Statement  on Auditing  Standards  No. 61
(Communication With Audit Committees).

In addition,  the Committee has discussed  with the  independent  auditors,  the
auditor's  independence  from the  Company  and its  management,  including  the
matters in the written disclosures required by the Independence  Standards Board
Standard No. 1 (Independent Discussions With Audit Committees).

The Committee discussed with management and the independent auditors the overall
scope and plans for the 2000 annual audit.  The Committee  meets with management
and the independent  auditors,  with and without management  present, to discuss
the results of their  examination,  the  evaluations  of the Company's  internal
controls, and the overall quality of the Company's financial reporting

In  reliance on the reviews and  discussions  referred to above,  the  Committee
recommended  to the Board of  Directors,  and the Board has  approved,  that the
audited financial  statements be included in the Company's Annual Report on Form
10-K for the year ended December 31, 2000,  for filing with the U.S.  Securities
and Exchange  Commission  and the Swiss SWX New Market.  The  Committee  and the
Board also have recommended,  subject to stockholder ratification, the selection
of the Company's independent auditors for the year ending December 31, 2001.

Respectfully submitted on April 3, 2001 by the members of the Audit Committee of
the Board of Directors:

Gwynn R. Williams
Ansbert S. Gadicke, M.D., Ph.D.
Erich Sager




























                                     19

CERTAIN RELATIONSHIPS AND RELATED TRANSACTIONS

Since January 1, 2000,  there has not been nor is there  currently  proposed any
transaction or series of similar  transactions to which the Company was or is to
be a party in  which  the  amount  involved  exceeds  $60,000  and in which  any
director,  executive officer,  holder of more than 5% of the common stock of the
Company or any member of the immediate  family of any of the  foregoing  persons
had or  will  have a  direct  or  indirect  material  interest  other  than  (1)
compensation agreements and other arrangements, which are described elsewhere in
this Proxy and (2) the transaction described below.

Transactions with Directors, Executive Officers and 5% Stockholders

Following Dr. Klock's  resignation as our President and continuing until October
31, 2000, Mr. Sager performed certain consulting  services for which we paid him
a total of $115,479.  These  services  included  assisting  us in the  executive
search for and  selection  of a senior  executive  officer and, as Chairman of a
Board management  subcommittee,  coordinating  with and aiding the management of
the Company in a wide variety of senior level tasks.

During 2000,  Glyko  Biomedical Ltd. paid us $7,700 per month in management fees
for accounting and related governmental reporting services.
Indebtedness Of Directors And Executive Officers

Other than as described  below, no director or executive  officer of the Company
or associate  of any director or executive  officer is, or at any time since the
beginning of the most recently  completed fiscal year has been,  indebted to the
Company.

Pursuant to the Stock  Purchase  Agreements  under which the Company  sold stock
("Founders'  Shares") to the three founding officers of the Company, the Company
has loaned $1,300,000,  $800,000, and $400,000 to Mr. Denison, Dr. Klock and Dr.
Starr,  respectively,  to purchase  common stock of the  Company.  The loans are
evidenced  by  interest  bearing  promissory  notes due on demand  and are fully
recourse. In August 2000, Dr. Klock paid-off the balance, including interest, of
his note. In September,  the Compensation  Committee  extended Mr. Denison's and
Dr. Starr's notes until March 31, 2001.

The  following  table sets forth any  indebtedness  of  directors  or  executive
officers  of the  Company  entered  into in  connection  with  the  purchase  of
securities of the Company.



                                                                             Outstanding                          Security for
                                                   Largest Amount of         Indebtedness        Number of       Indebtedness
                                                      Outstanding          as of December 31,   Common Shares   Number of Shares
Name of Borrower                      Lender        Indebtedness (1)            2000 (1)          Purchased      of Common Stock
---------------------------------------------------------------------------------------------------------------------------------
                                                                                                           
Grant W. Denison, Jr.                   Company            $1,553,660             $1,553,660       1,300,000           1,300,000
John C. Klock, M.D.                     Company              $780,335                     --         800,000                  --
Christopher M. Starr, Ph.D.             Company              $428,288               $409,875         400,000             400,000


     (1)  Includes accrued interest at 6% per annum.

SECTION 16(a) BENEFICAL OWNERSHIP REPORTING COMPLIANCE

Section 16(a) of the Securities  Exchange Act of 1934, as amended (the "Exchange
Act"),  requires the  Company's  directors and officers and persons who own more
that  10% of a  registered  class of the  Company's  equity  securities  to file
reports of ownership  and reports of changes in the  ownership  with the SEC and
the  National  Association  of  Securities  Dealers,  Inc.  Executive  officers,
directors,  and greater than 10% stockholders are required by the SEC to furnish
the Company with copies of all Section 16(a) forms they file.

Except for the following  transaction,  to the best of the Company's  knowledge,
based solely on review of the copies of such reports  furnished to us or written
representations  that no other  reports  were  required,  during the fiscal year
ended  December  31,  2000,  all  officers,  directors,  and  greater  than  10%
stockholders complied with all Section 16(a) filing requirements.

The Company is aware that (i) the Change of  Beneficial  Ownership of Securities
on Form 4 for Mr. Sager for the months of  February,  March and April 2000 which
related to 14 transactions was filed in June 2000 and for the month of September
2000 which related to 3 transactions was filed in January 2001, for Dr. Brandley
for the month of March 2000 which related to 2 transactions was filed in October
2000, for Dr. Starr for the month of May 2000 which related to 1 transaction was
filed in July 2000, for Mr. Denison for the month of March 2000 which related to
2  transactions  was filed in July 2000, for Dr. Klock for the month of May 2000
which related to 1 transaction  was filed in July 2000, for Dr. Swiedler for the
month of May 2000 which related to 2  transactions  was filed in July 2000,  and
(ii) the Initial  Statement of Beneficial  Ownership of Securities on Form 3 for
Dr. Baffi for the month of May 2000 was filed in November 2000,  were each filed
late.

                                       20

OTHER MATTERS

Except as otherwise indicated, information contained herein is given as of April
3, 2001. The Company's  management knows of no matters to come before the Annual
Meeting  other than the matters  referred to in the Notice of Annual  Meeting of
Stockholders.  However,  if any  other  matters  which  are not now known to the
Company's  management should come properly before the Annual Meeting,  the Proxy
will be voted on such matters in accordance with the best judgment of the person
voting it.

APPROVAL

The contents of this Proxy and the sending thereof to the stockholders have been
authorized by the Board of Directors of the Company.

DATED at Novato, California this 3rd day of April, 2001




Raymond W. Anderson

Chief Operating Officer, Chief Financial Officer, Secretary and
Vice President, Finance and Administration































































                                       21

                          BioMarin Pharmaceutical Inc.

                             Audit Committee Charter

Introduction

BioMarin Pharmaceutical Inc.'s executive management is primarily responsible for
the completeness and accuracy of its financial reporting and the adequacy of its
internal  financial  and  operating   controls.   Its  Board  of  Directors  has
responsibility to oversee management's exercise of these  responsibilities.  The
Board  has  determined  that  it is in the  best  interests  of the  Company  to
establish an Audit Committee whose authority and  responsibilities are described
by this Charter.

Organization

The full Board of  Directors  shall  designate  a  committee  of at least  three
independent  directors  to be an audit  committee  for the  Company  (the "Audit
Committee" or  "Committee").  The Audit Committee shall be composed of directors
who are  independent  of the  management  of the  Company  and  are  free of any
relationship  that,  in the opinion of the Board of Directors,  would  interfere
with their exercise of independent  judgement as a Committee member. Each member
shall be financially  literate and at least one member shall have  accounting or
related financial  management  expertise.  Each of the foregoing  qualifications
shall be in compliance  with all applicable  rules  promulgated by the Financial
Accounting  Standards Board ("FASB"),  U.S.  Securities and Exchange  Commission
("SEC"),  and the National  Association of Securities  Dealers ("NASD") or other
regulatory body.  Unless a chairperson is elected by the full Board, the members
of the  Committee  may  designate a  chairperson  by  majority  vote of the full
Committee membership.

Statement of Policy

The Audit  Committee  shall  provide  assistance  to the Board of  Directors  in
fulfilling their responsibilities to the stockholders,  potential  stockholders,
and  the  investment  community  relating  to  corporate  accounting,  reporting
practices, and the quality and integrity of the financial reports of Company. In
so doing, it is the  responsibility  of the Audit Committee to maintain free and
open means of communication  between directors,  the independent  auditors,  the
internal  auditors  (when  BioMarin is of sufficient  size to make this function
appropriate),  and the financial  management of the Company. The Audit Committee
and the Board of  Directors  will review and assess the adequacy of this Charter
annually.

Responsibilities

In carrying out its  responsibilities,  the Audit  Committee  believes  that its
policies and procedures  should remain  flexible,  in order to react to changing
conditions  and to ensure to the directors and  stockholders  that the corporate
accounting  and reporting  practices of the Company are in  accordance  with all
requirements and are of the highest quality.

In meeting its responsibilities:

1.   The Committee will meet no less frequently than on a quarterly basis, prior
     to the  release  of  earnings,  and  special  meetings  may be called  when
     circumstances  require and shall approve and maintain minutes for each such
     meeting.  The  Committee  may ask members of management or others to attend
     the meeting and provide pertinent information as necessary.

2.   The Committee,  as  representatives  of the stockholders,  has the ultimate
     authority  to  select,   evaluate  and,  where  appropriate,   replace  the
     independent  auditors,  or  to  nominate  the  independent  auditors  to be
     proposed for  stockholder  approval in the proxy  statement.  The Committee
     will review with management the performance, appointment and/or termination
     of the independent auditors and approve the compensation of the independent
     auditors.  The  Committee  will  recommend  to the Board of  Directors  the
     independent  auditors to be nominated to audit the financial  statements of
     the Company and its divisions and subsidiaries and (if deemed  appropriate)
     approve the discharge of the incumbent independent auditors.

3.   The  Committee  will  meet  with the  independent  auditors  and  financial
     management of the Company to review the scope of the proposed audit for the
     current year and the audit procedures to be utilized, and at the conclusion
     of the audit,  review the audit to include any comments and recommendations
     of the independent auditors.

4.   The Committee  will:  review with the independent  auditors,  the Company's
     financial and accounting  staff,  and the internal auditor the adequacy and
     effectiveness  of the internal  accounting  and  financial  controls of the
     Company including  computerized  information  system controls and security;
     elicit any  recommendations  for the  improvement  of any internal  control
     procedures  or  particular  areas  where new or more  detailed  controls or
     procedures are desirable;  and review the adequacy of internal  controls to
     expose  any  payments,  transactions,  or  procedures  that might be deemed
     illegal or otherwise improper.

5.   The Committee will confirm and assure the  independence  of the independent
     auditors and the internal auditor and require that the independent auditors
     provide a formal  written  statement  to the  Committee  setting  forth all
     relationships between the independent auditors and the Company,  consistent
     with the  Independence  Standards  Board Standard No. 1. The Committee will
     discuss  with the  independent  auditors  any  disclosed  relationships  or
     services  which  may  impact  the  objectivity  and   independence  of  the
     independent  auditors.  The Committee will take, or recommend that the full
     Board of Directors take,  appropriate  action to ensure the independence of
     the independent auditors.

6.   The Committee will inquire of management,  the director of internal  audit,
     and  the  independent   auditors  and  others  as  deemed  necessary  about
     significant  risks or exposures and assess the actions that  management has
     taken to minimize these significant risks to the Company.

7.   The Committee will review with management and the  independent  auditors at
     the completion of the annual audit:

(a)  The Company's annual financial statements and related footnotes
(b)  The independent  auditor's audit of the financial statements and his or her
     report thereon.
(c)  Any significant changes in the independent auditor's audit plan.
(d)  Any serious difficulties or disputes with management encountered during the
     course of the audit.
(e)  Other  matters  related  to the  conduct  of  the  audit,  which  are to be
     communicated to the Committee under generally accepted auditing standards.
8.   The Committee will review policies and procedures with respect to officers'
     expense reports and perquisites,  including the use of Company assets,  and
     consider  the  results  of any  review  of these  areas by the  independent
     auditors or by the internal auditor.

9.   The  Committee  will review legal and  regulatory  matters,  including  any
     litigation  contingencies  claims or assessments,  that may have a material
     impact on the financial statements, related company compliance policies and
     programs, and reports received from regulators.

10.  The  Committee  will meet with the  independent  auditors,  the director of
     internal auditing, and management in separate executive sessions to discuss
     any matters that the Committee or these groups  believe should be discussed
     privately with the Audit Committee.

11.  The  Committee  will conduct or authorize  investigations  into any matters
     within the Committee's scope of responsibilities. The Audit Committee shall
     be  empowered  to retain  independent  counsel,  accountants,  or others to
     assist it in the conduct of its investigations.

12.  The Committee will review accounting and financial  succession planning and
     depth of staff within the Company.

13.  The Committee will report Committee  actions to the Board of Directors with
     such  recommendations as the Committee may deem appropriate and will submit
     the minutes of all  meetings  to, or discuss  the matters  reviewed at each
     Committee meeting with, the Board of Directors.

14.  The Committee will review with management and the independent  auditors the
     Company's interim and year-end financial statements, including management's
     discussion  and analysis,  and audit findings  (including  any  significant
     suggestions for  improvements  provided to management by the internal audit
     director, if any, and the independent auditors). Such review will include a
     discussion of significant  adjustments  recorded or adjustments  passed and
     will conform with the  requirements of SAS 61.  Following such review,  the
     Committee  will  recommend  to the Board of  Directors  whether the audited
     financial  statements  should be included in the Company's annual report on
     Form 10-K and shall prepare a letter for the annual  report that  describes
     the  Committee's   composition  and  responsibilities  and  how  they  were
     discharged.

15.  The Committee will review with the independent  auditors or the director of
     internal  auditing the results of their review of the Company's  monitoring
     of compliance with the Company's code of conduct.

16.  With respect to the internal audit function, the Committee will:

(a)  Review  and  concur  in  the  appointment,  replacement,  reassignment,  or
     dismissal of the director of internal auditing.
(b)  Consider, in consultation with the independent auditors and the director of
     internal  auditing,  the audit scope and plan of the internal  auditors and
     the independent auditors.
(c)  Review with the  independent  auditors and the director of internal  audit,
     the  coordination  of audit  effort to  ensure  completeness  of  coverage,
     reduction of redundant efforts, and the effective use of audit resources.
(d)  Review   significant   internal   audit's  findings  during  the  year  and
     management's responses thereto.
(e)  Inquire  as to any  difficulties  encountered  in the  course  of  internal
     audits,  including any restrictions on the scope of their work or access to
     required information.
(f)  Review any changes in the scope of the audit plan.
(g)  Review the internal auditing department budget and staffing.
(h)  Approve the internal audit charter.
(i)  Review  internal  audit's  compliance  with  The  IIA's  Standards  for the
     Professional Practice of Internal Auditing (Standards).

17.  The  Committee  will  perform  other  functions  as  assigned  by law,  any
     applicable  regulatory body,  including the NASD, the company's  charter or
     by-laws, or the Board of Directors.


            PROXY

                          BIOMARIN PHARMACEUTICAL INC.

                       2001 ANNUAL MEETING OF STOCKHOLDERS

                                  MAY 24, 2001

              THIS PROXY IS SOLICITED ON BEHALF OF THE BOARD OF DIRECTORS

The  undersigned   stockholder  of  BIOMARIN  PHARMACEUTICAL  INC.,  a  Delaware
corporation,  hereby  acknowledges  receipt of the  Notice of Annual  Meeting of
Stockholders and Proxy  Statement,  each dated April 3, 2001 and hereby appoints
Fredric D.  Price,  Raymond W.  Anderson,  and Kim R.  Tsuchimoto,  as proxy and
attorney-in-fact,  with full power of substitution, on behalf and in the name of
the  undersigned,  to represent the  undersigned  at the 2001 Annual  Meeting of
Stockholders of BIOMARIN  PHARMACEUTICAL INC. to be held on May 24, 2001 at 10am
local time, at 46 Galli Drive,  Novato,  California 94949 and at any adjournment
or  adjournments  thereof,  and to vote all  shares  of common  stock  which the
undersigned would be entitled to vote, if then and there personally  present, on
the matters set forth on the reverse side.

                                 (Continued, and to be signed on the other side)










                                                                            
1.       ELECTION OF DIRECTORS:
         NOMINEES:

           Fredric D. Price, Grant W. Denison, Jr.,
           Ansbert S. Gadicke, M.D., Ph.D., Erich Sager
           and Gwynn R. Williams                                       FOR [   ]     WITHHOLD FOR ALL [   ]


         INSTRUCTION:  To withhold authority to vote for any
         individual nominee, write that nominee's name in the space
         provided below:

         --------------------------------------------

2.       Appointment of Arthur Andersen LLP as independent auditors
         of BIOMARIN PHARMACEUTICAL INC. for the fiscal year
         ending December 31, 2001                                      FOR [   ]     AGAINST [   ]   ABSTAIN [   ]


3.       In their discretion, the proxies are authorized to vote upon
         such matters as may properly come before the Annual
         Meeting, or any adjournments thereof.                         FOR [   ]           WITHHOLD [   ]



THIS PROXY WILL BE VOTED AS DIRECTED OR, IF NO CONTRARY  DIRECTION IS INDICATED,
WILL BE VOTED FOR THE COMPANY'S NOMINEES FOR ELECTION TO THE BOARD OF DIRECTORS,
THE APPOINTMENT OF ARTHUR  ANDERSEN LLP OR, AS SAID PROXIES DEEM  ADVISABLE,  ON
SUCH OTHER  MATTERS AS MAY PROPERLY COME BEFORE THE ANNUAL  MEETING,  INCLUDING,
AMONG OTHER  THINGS,  CONSIDERATION  OF ANY MOTION MADE FOR  ADJOURNMENT  OF THE
MEETING.

Signature(s)  _______________________________          Date:  _______________________



This proxy should be marked,  dated and signed by the stockholder(s)  exactly as
his or her name appears hereon,  and returned promptly in the enclosed envelope.
Persons signing in a fiduciary  capacity should so indicate.  If shares are held
by joint tenants or as community property, both should sign.