Novel therapy for patients with muscular dystrophy identified

Researchers at the University of Nevada School of Medicine Current have suggested that a protein, which helps cells stick together, may lead to enhanced muscle repair in muscular dystrophy.

Muscular dystrophy is a group of inherited genetic diseases that cause progressive muscle weakness.

In one type of muscular dystrophy, patients with mutations in the adhesion molecule alpha 7 integrin experience delayed developmental milestones and impaired mobility.

Currently, there is no treatment or cure for alpha 7 integrin congenital myopathy.

Interactions of alpha 7 integrin with laminin, an extracellular protein found surrounding muscle fibers, promote muscle cell health and survival.

Alpha 7 integrin has also been implicated in muscle repair.

To find out if alpha 7 integrin is critical for muscle repair, Dr. Dean Burkin at the University of Nevada and colleagues examined the response to muscle damage in alpha 7 integrin-deficient mice.

They found that alpha 7 integrin-deficient muscle exhibited defective muscular regeneration.

However, injection of laminin-111 restored muscle repair and regeneration.

The data from Rooney et al “indicate a critical role for the alpha7beta1 integrin and laminin in muscle repair and suggest direct muscle injections of laminin may serve as an exciting novel therapy for patients with alpha 7 integrin congenital myopathy and other muscle diseases.”

The study appears in the January 2009 issue of The American Journal of Pathology. (ANI)

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