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PLoS Curr. 2012 May 29;e4f84a944d8930. doi: 10.1371/4f84a944d8930.

A proteasome inhibitor fails to attenuate dystrophic pathology in mdx mice.

PLoS currents

Joshua Selsby, Carl Morris, Linda Morris, Lee Sweeney

PMID: 22866241 PMCID: PMC3392143.1 DOI: 10.1371/4f84a944d8930

Abstract

Dystrophin deficiency leads to increased proteasome activity in skeletal muscle. Previous observations suggest short-term inhibition of the proteasome restores dystrophin expression. Contrary to our hypothesis, eight days of MG-132 administration to mdx mice increased susceptibility to contraction induced injury and Evan's blue dye penetration compared to controls. Following six weeks of MG-132 administration muscle function was similar to control animals. These data suggest that proteasome inhibition does not reduce the severity of muscle dysfunction caused by dystrophin-deficiency.

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