Halting cell death heals muscular dystrophy

Main Category: Muscular Dystrophy / ALS
Article Date: 02 Dec 2004 - 10:00 PDT


The most common form of muscular dystrophy present at birth is caused by mutation of the LAMA2 gene that encodes laminin-alpha2. Loss of laminin-alpha2 function leads to neuromuscular dysfunction and often early death. Researchers have previously proposed that programmed cell suicide, known as apoptosis, in skeletal muscles and nerves at inappropriate times may be one way in which laminin-alpha2 loss results in muscular dystrophy.

In the December 1 issue of the Journal of Clinical Investigation, Jeffrey Boone Miller and colleagues from Boston Biomedical Research Institute investigated whether the symptoms of muscular dystrophy in laminin-alpha2-deficient mice could be relieved by inhibiting apoptosis in muscle via 2 mechanisms: (i) inactivation of the proapoptotic protein Bax, or (ii) overexpression of the antiapoptosis protein Bcl-2.

The authors found that both interventions significantly increased the lifespan of these mice and improved neuromuscular dysfunction. The authors suggest that antiapoptosis therapy may be a possible way in which to relieve neuromuscular dysfunction due to laminin-alpha2 deficiency in humans with muscular dystrophy.

TITLE: Inhibition of apoptosis improves outcome in a model of congenital muscular dystrophy

AUTHOR CONTACT:
Jeffrey Boone Miller
Boston Biomedical Research Institute
Watertown, Massachusetts, USA
Phone: 617-658-7737
Fax: 617-972-1761
E-mail: miller@bbri.org.
View the PDF of this article at: https://www.the-jci.org/press/22928.pdf

Contact: Brooke Grindlinger
press_releases@the-jci.org
212-342-0497
Journal of Clinical Investigation

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