Amicus Therapeutics Announces Positive Progress of Three Lead Clinical Programs and...

Amicus Therapeutics Announces Positive Progress of Three Lead Clinical
Programs and General Outlook for 2009

Company Expects to Deliver Multiple Clinical Milestones throughout the Year;
Amigal to Initiate Phase 3 Global Development in Second Quarter; Continued
Strong Financial Position

CRANBURY, N.J., Jan. 12 /PRNewswire-FirstCall/ -- Amicus Therapeutics (Nasdaq:
FOLD) today announced positive progress in its three lead clinical programs
for lysosomal storage disorders and presented a corporate outlook consisting
of multiple clinical milestones and financial guidance for 2009 at the 27th
Annual J.P. Morgan Healthcare Conference. The Company also provided an update
on its interactions with U.S. and European regulatory agencies that are
expected to lead to the commencement of Phase 3 clinical development for
Amigal(TM) (migalastat hydrochloride), an oral therapeutic drug candidate for
the treatment of Fabry disease. Amicus, along with its partner, Shire Human
Genetic Therapies, Inc. (Shire HGT), plans to initiate Phase 3 development of
Amigal for the treatment of Fabry disease in the second quarter of 2009.
Amicus also reviewed data on the potential for the combination of
pharmacological chaperones and enzyme replacement therapy (ERT).

"We continue to make significant advancements in our clinical programs and we
expect this to progress through several key milestones which we plan to
deliver in 2009. Combined with our financial strength, our partnership with
Shire HGT, and the growing breadth of our pharmacological chaperone technology
platform, we feel this progress sets the stage for a transformational year for
our company," said John F. Crowley, Amicus' President and CEO.

CLINICAL PROGRAM ADVANCEMENTS

Amigal (migalastat hydrochloride) for the treatment of Fabry Disease

Amicus previously announced in August 2008 that it had completed a successful
End of Phase 2 meeting with the U.S. Food and Drug Administration (FDA). At
that time, the FDA indicated that the data from the completed Phase 2 clinical
studies of Amigal support the initiation of Phase 3 development and that
Amigal was eligible for Accelerated Approval under Subpart H regulations. The
FDA further indicated at that time that it was not opposed to the use of a
surrogate primary endpoint, pending further discussions with the company.

Following this End of Phase 2 meeting, Amicus continued discussions with the
FDA in the second half of 2008. The Agency has indicated that it supports a
Phase 3 clinical trial comparing Amigal to placebo based on a surrogate
primary endpoint of the change in the amount of kidney GL-3, the substrate
that accumulates in the cells of Fabry patients. Amicus plans to continue
discussions with the FDA through a Special Protocol Assessment (SPA) procedure
that it commenced in the fourth quarter of 2008 to finalize how the primary
endpoint will be measured. Amicus expects the SPA process to be complete in
the second quarter of this year.

Based on discussions with the European Medicines Agency (EMEA), Amicus expects
to initiate a separate clinical study designed to evaluate the safety and
efficacy of Amigal versus ERT in Fabry patients. Amicus and its partner Shire
HGT plan additional discussions with the EMEA in the first half of 2009 to
finalize the design of this study.

In parallel with the Phase 3 regulatory discussions, 23 of the original 26
patients from the Phase 2 studies continue to be treated in a voluntary
extension study to characterize the long-term safety and efficacy of Amigal
and to evaluate additional doses and dose regimens. Data from this extension
study are expected to be available in the first quarter of 2009 and the
results will be used to finalize the dose and regimen for the Phase 3 studies.

Plicera(TM) (isofagomine tartrate) for the treatment of Gaucher Disease

A Phase 2 clinical trial of Plicera in Gaucher patients is ongoing. This 6
month study is designed to evaluate safety as well as to demonstrate trends of
efficacy as measured by the standard endpoints in Gaucher disease. Target
enrollment of 16 patients for this study is expected to be surpassed and the
results are expected to be available in the third quarter of 2009.

In addition, Amicus is working closely with its partner Shire HGT to prepare
for Phase 3 development of Plicera pending the results of the ongoing Phase 2
trial.

AT2220 (1-deoxynojirimycin HCl) for the treatment of Pompe Disease

A Phase 2 clinical trial of AT2220 in adult Pompe patients is ongoing. The
trial includes an 11 week treatment period with an optional extension study.
The objectives of the trial include the evaluation of the safety and
pharmacodynamics of multiple doses and regimens of AT2220. The results of this
study are expected to be available in the second half of 2009.

In addition, Amicus is continuing to conduct preclinical animal studies to
evaluate the effects of administering AT2220 in combination with ERT.
Encouraging preliminary results were announced at the American Society of
Human Genetics conference in November 2008. These results indicated that
AT2220 in combination with ERT significantly increases the stability and
tissue uptake of ERT. Amicus is conducting additional preclinical
proof-of-concept studies to determine the feasibility of a combination that
may be appropriate for Pompe patients who are not amenable to chaperone
monotherapy. Amicus expects to announce additional results in the first
quarter of 2009.

2009 FINANCIAL GUIDANCE

Amicus expects to spend a total of approximately $70 million on 2009 cash
operating expenses, to be offset by $50 million in program cost-sharing
reimbursements and clinical milestone payments from Shire. Amicus anticipates
ending 2009 with approximately $100 million in cash.

About Amicus Therapeutics

Amicus Therapeutics is a biopharmaceutical company developing novel, oral
therapeutics known as pharmacological chaperones for the treatment of a range
of human genetic diseases. Pharmacological chaperone technology involves the
use of small molecules that selectively bind to and stabilize proteins in
cells, leading to improved protein folding and trafficking, and increased
activity. Amicus is initially targeting lysosomal storage disorders, which are
severe, chronic genetic diseases with unmet medical needs. Amicus has
completed Phase 2 clinical trials of Amigal for the treatment of Fabry disease
and is conducting Phase 2 clinical trials of Plicera for the treatment of
Gaucher disease and AT2220 for the treatment of Pompe disease.

Amicus has a strategic collaboration with Shire Human Genetic Therapies, Inc.,
a wholly-owned subsidiary of Shire Limited, to develop and commercialize
Amicus' three lead pharmacological chaperone compounds for lysosomal storage
disorders. Under the agreement, Shire received commercial rights outside of
the United States. Amicus retains all U.S. rights.

About Shire plc

Shire's strategic goal is to become the leading specialty biopharmaceutical
company that focuses on meeting the needs of the specialist physician. Shire
focuses its business on attention deficit and hyperactivity disorder (ADHD),
human genetic therapies (HGT) and gastrointestinal (GI) diseases as well as
opportunities in other therapeutic areas to the extent they arise through
acquisitions. Shire's in-licensing, merger and acquisition efforts are focused
on products in specialist markets with strong intellectual property protection
and global rights. Shire believes that a carefully selected and balanced
portfolio of products with strategically aligned and relatively small-scale
sales forces will deliver strong results.

For further information on Shire, please visit the Company's website:
www.shire.com.

Forward-Looking Statements

This press release contains "forward-looking statements" within the meaning of
the Private Securities Litigation Reform Act of 1995. Words such as, but not
limited to, "look forward to," "believe," "expect," "anticipate," "estimate,"
"intend," "plan," "targets," "likely," "will," "would," "should" and "could,"
and similar expressions or words identify forward-looking statements. Such
forward-looking statements are based upon current expectations that involve
risks, changes in circumstances, assumptions and uncertainties. The inclusion
of forward-looking statements should not be regarded as a representation by
Amicus that any of its plans will be achieved. Any or all of the
forward-looking statements in this press release may turn out to be wrong.
They can be affected by inaccurate assumptions Amicus might make or by known
or unknown risks and uncertainties. For example, with respect to statements
regarding the goals, progress, timing and outcomes of ongoing discussions with
regulatory authorities and the potential goals, progress, timing and results
of clinical trials, actual results may differ materially from those set forth
in this release due to the risks and uncertainties inherent in the business of
Amicus, including, without limitation: the potential inability to reach final
agreement with regulatory agencies on the use of a surrogate endpoint and
phase 3 trial design for Amigal; the potential that results of clinical or
pre-clinical studies indicate that the product candidates are unsafe or
ineffective; and, our dependence on third parties in the conduct of our
clinical studies. Further, the results of earlier clinical trials may not be
predictive of future results. Additionally, with respect to statements
regarding projections of the Company's cash position and expected use of cash
during 2009, actual results may differ based on market factors, the company's
ability to execute its operational and budget plans, and its achievement of
milestones and receipt of milestone payments from Shire. Additionally, all
forward looking statements are subject to other risks detailed in our Annual
Report on Form 10-K for the year ended December 31, 2007, and our other public
filings with the Securities and Exchange Commission. You are cautioned not to
place undue reliance on these forward-looking statements, which speak only as
of the date hereof. All forward-looking statements are qualified in their
entirety by this cautionary statement, and Amicus undertakes no obligation to
revise or update this news release to reflect events or circumstances after
the date hereof. This caution is made under the safe harbor provisions of
Section 21E of the Private Securities Litigation Reform Act of 1995.

FOLD-G

SOURCE Amicus Therapeutics

Investors, John Quirk, +1-212-601-8296, or Media, Amy Speak, +1-617- 897-8262,
both of Porter Novelli Life Sciences, for Amicus Therapeutics