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Article

Genetic overexpression of Serpina3n attenuates muscular dystrophy in mice

6 Jan 2016

Abstract excerpt

Muscular dystrophy (MD) is associated with mutations in genes that stabilize the myofiber plasma membrane, such as through the dystrophin-glycoprotein complex (DGC). Instability of this complex or defects in membrane repair/integrity leads to calcium influx and myofiber necrosis leading to progressive dystrophic disease. MD pathogenesis is also associated with increased skeletal muscle protease levels and...

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PMID: 26744329
DOI: 10.1093/hmg/ddw005

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