Article
Therapeutic potential of proteasome inhibition in Duchenne and Becker muscular dystrophies.
The American journal of pathology - 1 Apr 2010
Gazzerro Elisabetta, Assereto Stefania, Bonetto Andrea, Sotgia Federica, Scarfì Sonia, Pistorio Angela, Bonuccelli Gloria, Cilli Michele, Bruno Claudio, Zara Federico, Lisanti Michael P, Minetti Carlo
Abstract excerpt
Duchenne muscular dystrophy (DMD) and its milder allelic variant, Becker muscular dystrophy (BMD), result from mutations of the dystrophin gene and lead to progressive muscle deterioration. Enhanced activation of proteasomal degradation underlies critical steps in the pathogenesis of the DMD/BMD dystrophic process. Previously, we demonstrated that treatment with the proteasome inhibitor MG-132 rescues the cell...
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