Glyko Biomedical Ltd. Announces its 36.2%-Owned Affiliate, BioMarin Pharmaceutical Inc., and Genzyme Form Joint Venture to Develop New Product to Treat Fatal Genetic Disease.

NOVATO, Calif.--(BW HealthWire)--Sept. 15, 1998--Glyko Biomedical Ltd. (OTC BB:GLYK)(TSE:GBL)(BVD -- Berlin:GLY) announces that its 36.2%-owned affiliate, BioMarin Pharmaceutical Inc., a privately held biopharmaceutical company and Genzyme General (Nasdaq:GENZ) have formed a joint venture to develop and commercialize BioMarin's lead product candidate, (alpha)-L-iduronidase, a recombinant enzyme to treat the lysosomal storage disorder mucopolysaccharidosis I (MPS I).

The companies announced in June that they intended to form the joint venture.

The agreement provides for Genzyme to make a $12.1 million milestone payment to BioMarin upon receipt of approval from the U.S. Food and Drug Administration (FDA) of a Biologics License Application for (alpha)-L-iduronidase to treat MPS I.

In addition, Genzyme has made an $8 million equity investment for a 5.6% interest in BioMarin and agreed to purchase an additional $10 million of BioMarin stock in a private placement in the event that BioMarin completes an initial public offering, at the price to the public.

As announced in January 1998, BioMarin initiated definitive clinical trials of (alpha)-L-iduronidase in patients with MPS I. In pre-clinical studies, scientists had demonstrated that enzyme replacement therapy with recombinant (alpha)-L-iduronidase may be effective in treating many of the symptoms of MPS I.

The trial is being conducted at the Harbor-UCLA Research and Education Institute in Torrance, Calif. Emil Kakkis, M.D., Ph.D., president of BioMarin Genetics and assistant professor, Division of Medical Genetics, Department of Pediatrics, Harbor-UCLA Medical Center, is the principal investigator of the study. Serving as advisor to the study is Elizabeth F. Neufeld, Ph.D., professor and chair of the Biological Chemistry Department at UCLA.

BioMarin received orphan drug designation for (alpha)-L-iduronidase in September 1997, potentially giving the product market exclusivity for seven years following FDA approval.

MPS I encompasses three related disorders, the most severe form of which is Hurler syndrome, a crippling and fatal disease that affects young children. Characterized by a halt in a patient's physical and mental development, MPS I is caused by the lack of an active enzyme, (alpha)-L-iduronidase, which results in a build-up of certain carbohydrate materials in all parts of the body.

The debilitating effects of MPS I can lead to early death often before the age of ten. They can include enlargement of the liver and spleen, skeletal deformity, vision impairment, stunted growth, hearing loss, obstruction of airways, mental retardation, and cardiomyopathy.

Approximately 2,000-3,000 people in the developed world have been diagnosed with MPS I. As with other lysosomal storage diseases, there are believed to be additional undiagnosed patients. There is currently no known effective treatment for MPS I.

The Glyko is focused in three areas: 1) the manufacture and sale of carbohydrate analysis tools for pharmaceutical and life sciences researchers, 2) the development of diagnostic tests for Lysosomal Storage Diseases, Osteoporosis and blood heparin measurements, and 3) its interest in the development of recombinant enzymes for pharmaceutical uses through BioMarin.

BioMarin is a privately held biopharmaceutical company specializing in the discovery, development and commercialization of carbohydrate enzyme therapeutics. Formed in 1996 by Glyko Biomedical Ltd., the lead investor in the company, BioMarin applies its proprietary enzyme technology to the treatment of numerous disease applications focused currently in five therapeutic areas: genetic diseases, fungal infections, burn and wound care, male pro-fertility compounds, and psoriasis.

Genzyme General develops and markets therapeutic and surgical products and diagnostic products and services. A division of the biotechnology company Genzyme Corp., Genzyme General has its own common stock intended to reflect its value and track its performance.

This press release contains forward-looking statements about potential therapeutic uses of (alpha)-L-iduronidase, the potential market exclusivity of (alpha)-L-iduronidase, the size of the potential market for (alpha)-L-iduronidase, the ability of the current clinical trial of (alpha)-L-iduronidase to serve as a pivotal trial, and the expected completion date of such clinical trial. Results may differ materially depending on the actual therapeutic effect of (alpha)-L-iduronidase, the actual timing and results of the clinical trial, the accuracy of information regarding the number of patients suffering from MPS I, and decisions made by the FDA.

Note to Editors: (alpha)-L-iduronidase is written with the Greek letter in place of (alpha).

CONTACT: Glyko Biomedical Ltd.

John C. Klock MD, 415/382-3500 (President)

www.glyko.com