UPDATE 1-Amicus Therapeutics signs licensing deal with Shire

(Updates with details)

By Toni Clarke

BOSTON, Nov. 8 (Reuters) - Amicus Therapeutics Inc (FOLD.O) said on Thursday it granted British drugmaker Shire Plc (SHP.L) marketing rights outside the United States to three drugs Amicus is developing to treat rare genetic disorders.

Under an agreement with Shire, Amicus, a small biotechnology company based in Cranbury, New Jersey, will receive $50 million in cash and up to $150 million more if certain milestones are met. If the drugs are commercialized, Amicus will receive up to $240 million in sales milestone payments and double-digit royalties.

The companies will equally split the cost of developing and bringing the drugs to the market, and Amicus will retain rights to the lucrative U.S. market.

The agreement gives Shire an additional plank in its effort to build a stable of drugs for human genetic disorders. In 2005, it paid $1.6 billion for Transkaryotic Therapies Inc., a company that developed a drug called Replagal to treat the genetic disorder Fabry disease. Shire also sells Elaprase, a drug for Hunter Syndrome.

Shares of Amicus have risen 6.6 percent since its initial public offering at the end of May. If successful, the company would compete with Genzyme Corp. (GENZ.O), one of the world's biggest biotechnology companies and a leader in the development of drugs for rare inherited disorders.

The agreement with Shire covers Amicus's experimental drug Amigal, which is currently in mid-stage trials to treat Fabry disease, a disorder in which deficient activity of a certain enzyme can cause kidney failure and increased risk of heart attack and stroke.

It also covers Plicera, a drug in mid-stage trials to treat Gaucher disease, a condition that can cause enlarged liver and spleen, low levels of red blood cells and central nervous system impairment.

A third drug that is part of the agreement with Shire, known as AT2220, is in early stage trials for the rare muscle disorder Pompe disease.

Current sales of drugs for these three diseases, known as lysosomal storage disorders, come to roughly $1.7 billion, with the vast majority going to Genzyme.

John Crowley, the chief executive of Amicus, said roughly 60 percent of the market is outside the United States, and that while he wouldn't rule out a separate partnership for the U.S. market, right now the company plans to build itself into a fully-fledged biotech company.

At a time when many biotechnology companies are seeking to wait as long as possible before signing partnerships, hoping that they will get a better deal the further their products are along in the pipeline, Crowley said the terms of the Shire deal were too good to pass up.

"For us, the economics of this deal were just so strong," he said.

(Reporting by Toni Clarke, editing by Steve Orlofsky)

((toni.clarke@reuters.com, 617-367-4165)) Keywords: AMICUS/

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