Loss of protein leads to limb-girdle muscular dystrophy

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Washington, May 1 : A team of French and German researchers have discovered that a protein called c-FLIP, which the body uses to prevent the loss of muscle tissue, is missing in people with limb-girdle muscular dystrophy.

They said that targeting the cellular and molecular mechanisms responsible for creating this protein could pave the way for developing new drugs to stop muscle wasting from limb-girdle muscular dystrophy and other conditions.

"Unfortunately, rare diseases like limb-girdle muscular dystrophy don't get the attention or funding they deserve," said Gerald Weissmann, M.D., Editor-in-Chief of The FASEB Journal.

"I hope that the breakthrough described in this study-the discovery of what regulates a protein that determines which muscle tissue stays and goes in our bodies-will lead to a range of new drugs for this form of muscular dystrophy and many others," Weissmann added.

To identify the protein as the cause for limb-girdle muscular dystrophy, the researchers used tissue from human biopsies to analyse the molecular pathways involved at each step of the disorder's progression.

They found that c-FLIP, which blocks the death of muscle cells, is not produced as it should in people with limb-girdle muscular dystrophy, and that the creation of the protein is controlled by another protein called calpain-3.

Researchers believe that this finding might have implications for other types of muscular dystrophy and other situations that cause the death of muscle fibres, such as long-term immobilization, denervation, aging, or cachexia.

"Limb-girdle muscular dystrophy is a rare and devastating condition that robs people of movements that the rest of us take for granted. Fortunately, this study should provide researchers with a much-needed target for developing drugs to treat at least one of these conditions," Weissmann added.

The study is published in the May 2008 print issue of The FASEB Journal.

ANI

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