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Summit Corp plc - BioMarin and Summit sign worldwide licensing agreement for Duchenne Muscular Dystrophy program

22 Jul 2008

BioMarin  Pharmaceutical Inc. (Nasdaq and SWX:  BMRN) and Summit  Corporation plc (AIM:  SUMM) announced today that  they have entered  into an exclusive  worldwide
licensing agreement  for  Summit's novel  preclinical  candidate  SMT C1100 and all follow-on molecules, which are being developed to treat the fatal genetic disorder Duchenne muscular dystrophy (DMD).

Under the terms of  the licensing agreement,  Summit will receive  an upfront payment of $7 million in the form of an equity investment  in Summit shares, future development and regulatory milestones  totaling $51 million, tiered royalties rising to low teens, depending on sales and product sales milestones giving a total deal value of up to  $143 million.

Summit will be responsible for completing the preclinical development of  SMT  C1100.   BioMarin  will  be  responsible  for  the  clinical development, regulatory filing and  commercialization of the  product candidate.

"We are  pleased  to  work  with  Summit  on  the  Duchenne  muscular dystrophy  program.  SMT  C1100,  an  oral  small  molecule  utrophin upregulator, has shown promise in animal  models of DMD and may  have the potential for treating the  entire spectrum of DMD patients,  not just those with  a particular  type of  mutation," said  Jean-Jacques Bienaim, Chief Executive Officer  of BioMarin. "The DMD  indication aligns well with our growing product development pipeline as it is a genetic disorder with  no approved  treatments. IND-enabling studies with SMT C1100 are underway, and we plan on entering the clinic in 2009.  By  leveraging  our   expertise  in  rapidly  developing   and commercializing products for focused patient populations, we hope  to soon provide a new treatment option for all DMD patients."

Steven Lee  Ph.D.,  Chief Executive  of  Summit, said,  "We  are  very pleased that BioMarin  has become  our partner for  the DMD  program. BioMarin has an unparalleled track record in developing orphan  drugs to market  and has  developed and  launched successfully  three  such drugs in record time.  The expertise and  commitment of the  BioMarin team gives me great confidence that they are an excellent partner for this program.  I believe they will  help to deliver SMT C1100 into  a medicine in the shortest  timeframe possible for  the benefit of  all DMD patients.

"For Summit, this deal is important as  it is the first of many  that we anticipate signing from our broad pipeline of assets including two clinical and two preclinical programs with future research driven  by our world  leadership in  two  innovative technology  platforms.  Our
business  strategy  is   focused  on   out-licensing  or   partnering candidates at a preclinical or early clinical stage, where there is a strong demand from  pharma and biotech  companies looking to  enhance their own pipelines, and this  deal provides important validation  of this strategy."

The equity investment of $7.0 million will be made at 69 pence per share, calculated  from the 60-day trailing share  price at the time issue, upon which BioMarin will hold approximately  9.16% of the enlarged share capital of Summit.

About Duchenne muscular dystrophy (DMD) and SMT C1100

Duchenne muscular dystrophy  is a fatal  neuromuscular disorder  that affects 1 in 3,500 boys with an estimated patient population of  over 40,000 in the developed world.

DMD is  caused by  a  genetic defect  meaning  DMD patients  lack  an important protein called dystrophin, which is crucial to  maintaining muscle integrity and function. The  absence of dystrophin results  in extensive muscle  wasting in  all voluntary  muscles as  well as  the heart and  breathing muscles  and causes  severe restriction  in  the mobility of  DMD patients  by  their early  teens and  is  ultimately fatal, generally in their  twenties. Currently there  is no cure  for DMD; corticosteroid treatment is the only frontline therapy and  acts to only delay the progression of the disease.

Summit has  identified SMT  C1100,  a proprietary,  orally  available small molecule with a  novel mechanism of action  for DMD. SMT  C1100 acts to modify the progression of DMD by replacing dystrophin with an endogenous, functionally  similar  protein  called  utrophin.  Summit believes the  primary advantage  of SMTC1100  is that  it offers  the
potential  to  treat  the  entire  DMD  patient  population.   Summit recently  presented  important   preclinical  data   for  SMT   C1100 demonstrating significantly improvements in the strength and function of muscles in in vivo models.

Due to the low patient population and high unmet medical need, DMD is designated as an orphan indication by the regulatory agencies. Orphan products can  expect to  receive  additional regulatory  support  and accelerated approval in  addition to  seven and ten  years of  market exclusivity in the US and EU respectively upon designation by the FDA
and the EMEA.

For further information:

BioMarin Pharmaceutical, Inc.
Eugenia Shen, Investor Relations
Tel: +1 (415) 506-6570
Susan Berg, Media Relations
Tel: +1 (415) 506-6594

Summit plc
Steven Lee, PhD, Chief Executive Officer
Darren Millington, ACMA, Chief Financial Officer
Richard Pye, PhD, Investor Relations
Tel: +44 (0)1235 443951

Citigate Dewe Rogerson
Mark Swallow, PhD / David Dible / Sylvie Berrebi / Emma Palmer
Tel: +44 (0)207 638 9571

Panmure Gordon
Andrew Burnett / Rakesh Sharma (Corporate Finance)
Ashton Clanfield (Corporate Broking)
Tel: +44 (0)207 459 3600

About BioMarin

BioMarin develops  and commercializes  innovative  biopharmaceuticals for serious diseases  and medical conditions.  The company's  product portfolio comprises three approved products and multiple clinical and preclinical product candidates. Approved products include  Naglazyme (galsulfase) for mucopolysaccharidosis VI (MPS VI), a product  wholly developed and  commercialized by  BioMarin; Aldurazyme  (laronidase)
for  mucopolysaccharidosis  I  (MPS  I),  a  product  which  BioMarin developed through a 50/50 joint venture with Genzyme Corporation; and Kuvan (sapropterin  dihydrochloride)  Tablets,  a  product  for  the treatment of  phenylketonuria (PKU),  developed in  partnership  with Merck Serono, a division of Merck KGaA of Darmstadt, Germany.   Other product candidates  include  6R-BH4 for  cardiovascular  indications,
which is currently in Phase 2 clinical development for the  treatment of peripheral arterial disease and  sickle cell disease, and  PEG-PAL (PEGylated  recombinant  phenylalanine   ammonia  lyase),  which   is currently in Phase 1 clinical development for the treatment of  PKU.

For additional information, please visit www.BMRN.com. Information on BioMarin's website is not incorporated  by reference into this  press release.

About Summit plc

Summit plc is a leading UK biotechnology company with a broad preclinical and clinical pipeline, two world-leading technology platforms and an innovative business model that is expected to generate sustainable value for investors.  Summit is developing many
drug programmes that target unmet medical needs from which it intends to generate value by out-licensing attractive late preclinical or early clinical stage programmes in return for upfront, milestone and royalty payments. Summit uses its scientific expertise to target
orphan indications, neuro-disorders and infectious diseases. Summit's drug pipeline is supported by its world leadership in two innovative technology platforms: carbohydrate chemistry and zebrafish biology. These platforms support existing programmes and also will be the source of future programmes to refuel Summit's drug pipeline. These platform technologies also form the basis of the Company's revenue generating service business.  The company listed on the alternative investment market (AIM) of the London Stock Exchange in October 2004 - symbol: SUMM.  Further information about the company
is available at www.summitplc.com.

Forward-Looking Statements

For BioMarin

This press  release  contains forward-looking  statements  about  the
business  prospects  of  BioMarin  Pharmaceutical  Inc.,   including,
without limitation, statements about: the expected development of SMT
C1100, the continued  clinical development  and commercialization  of
BioMarin's products  and  other  product candidates  and  actions  by
regulatory  authorities.   These   forward-looking   statements   are
predictions and  involve risks  and  uncertainties such  that  actual
results may differ materially from these statements. These risks  and
uncertainties include, among others:  results of planned and  ongoing
preclinical and clinical trials, including preclinical trials of  SMT
C1100; the content and timing of decisions by the U.S. Food and  Drug
Administration,  the   European  Commission   and  other   regulatory
authorities concerning  each of  the described  products and  product
candidates; and those factors detailed in BioMarin's filings with the
Securities and  Exchange Commission,  including, without  limitation,
the factors contained under the caption "Risk Factors" in  BioMarin's
2007 Annual Report on Form 10-K. Stockholders are urged not to  place
undue reliance on forward-looking statements, which speak only as  of
the date  hereof.  BioMarin is  under  no obligation,  and  expressly
disclaims any  obligation  to  update or  alter  any  forward-looking
statement, whether as a result  of new information, future events  or
otherwise.

For Summit plc

This press release contains "forward-looking statements" within the
meaning of the U.S. Private Securities Litigation Reform Act of 1995.
Forward-looking statements can be identified by words such as
"anticipates", "intends", "plans", "seeks", "believes", "estimates",
"expects" and similar references to future periods, or by the
inclusion of forecasts or projections.

Forward-looking statements are based on Summit Corporation plc's
 current expectations and assumptions regarding our business, the
economy and other future conditions. Because forward-looking
statements relate to the future, by their nature, they are subject to
inherent uncertainties, risks and changes in circumstances that are
difficult to predict. Summit's actual results may differ materially
from those contemplated by the forward-looking statements. Summit
cautions you therefore that you should not rely on any of these
forward-looking statements as statements of historical fact or as
guarantees or assurances of future performance. Important factors
that could cause actual results to differ materially from those in
the forward-looking statements include regional, national, global
political, economic, business, competitive, market and regulatory
conditions.



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