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Potential Therapy For Congenital Muscular Dystrophy

Date:
January 1, 2009
Source:
American Journal of Pathology
Summary:
Current research suggests laminin, a protein that helps cells stick together, may lead to enhanced muscle repair in muscular dystrophy.
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Current research suggests laminin, a protein that 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. There is currently 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 determine if alpha 7 integrin is critical for muscle repair, researchers led by Dr. Dean Burkin at The University of Nevada School of Medicine examined the response to muscle damage in alpha 7 integrin-deficient mice. They found that alpha 7 integrin-deficient muscle exhibited defective muscular regeneration. Injection of laminin-111, however, restored muscle repair and regeneration.

The data from the new study by 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." Dr. Burkin's group is "currently investigating the potential of this technology to treat Duchenne and other forms of muscular dystrophy. This work opens a whole new modality in therapeutics, of injecting extracellular matrix proteins to treat genetic diseases."

This work was supported by grants from the National Institutes of Health.


Story Source:

The above post is reprinted from materials provided by American Journal of Pathology. Note: Materials may be edited for content and length.


Journal Reference:

  1. Rooney JE, Gurpur PB, Yablonka-Reuveni Z, and Burkin DJ. Laminin-111 restores regenerative capacity in a mouse model for alpha 7 integrin congenital myopathy. Am J Pathol, 2009 174: 256-264

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American Journal of Pathology. "Potential Therapy For Congenital Muscular Dystrophy." ScienceDaily. ScienceDaily, 1 January 2009. <www.sciencedaily.com/releases/2008/12/081230072244.htm>.
American Journal of Pathology. (2009, January 1). Potential Therapy For Congenital Muscular Dystrophy. ScienceDaily. Retrieved August 2, 2015 from www.sciencedaily.com/releases/2008/12/081230072244.htm
American Journal of Pathology. "Potential Therapy For Congenital Muscular Dystrophy." ScienceDaily. www.sciencedaily.com/releases/2008/12/081230072244.htm (accessed August 2, 2015).

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