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Amicus Therapeutics Announces Second Quarter 2008 Financial Results
Date:8/7/2008

ion, the company no longer plans to conduct a longer-term Phase 2 study in patients switching from ERT to Plicera as previously disclosed. The design of the pharmacokinetics study is currently under development.

Pompe Disease:

AT2220 (1-deoxynojirimycin HCl) is an investigational, oral therapeutic being developed for the treatment of Pompe disease. In the second quarter of 2008, Amicus announced that it had initiated a Phase 2 clinical trial of AT2220 (1-deoxynojirimycin HCl). Amicus is conducting the study in adult Pompe patients in clinical centers throughout North America and Europe. AT2220 is the third compound based on Amicus' pharmacological chaperone technology platform to enter Phase 2 clinical development.

In addition, Amicus is conducting preclinical animal studies to evaluate the effects of administering AT2220 in combination with enzyme replacement therapy. Based on these results, Amicus will consider initiating a clinical trial of the AT2220-ERT combination treatment in Pompe patients.

Other Chaperone Programs:

Amicus is accelerating its investment in research and development to assess the potential for using pharmacological chaperones to treat a broader range of human genetic diseases beyond lysosomal storage diseases. As part of this effort, Amicus continues to conduct preclinical studies in Parkinson's disease, funded in part by a grant from the Michael J. Fox Foundation. In addition to the work in Parkinson's, Amicus is investing in new research aimed at evaluating disease targets for other neurodegenerative disorders and metabolic disorders.

Shire Collaboration:

In November 2007, Amicus entered into a strategic collaboration with Shire Human Genetic Therapies, Inc., a wholly-owned subsidiary of Shire Limited, to jointly develop Amicus' three lead pharmacological chaperone compounds for lysosomal storage disorders, Amigal, Plicera and AT2220. In this collaboration, valued at up to $440 million includi
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