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Pathogenity of some limb girdle muscular dystrophy mutations can result from reduced anchorage to myofibrils and altered stability of calpain 3

Calpain 3 (CAPN3) is a muscle-specific, calcium-dependent proteinase that is mutated in Limb Girdle Muscle Dystrophy type 2A. Most pathogenic missense mutations in LGMD2A affect CAPN3's proteolytic activity; however, two mutations, D705G and R448H, retain activity but nevertheless cause muscula...

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Bibliographic Details
Main Authors: Ermolova, Natalia, Kudryashova, Elena, DiFranco, Marino, Vergara, Julio, Kramerova, Irina, Spencer, Melissa J.
Format: Artigo
Language:Inglês
Published: Oxford University Press 2011
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Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC3153300/
https://ncbi.nlm.nih.gov/pubmed/21624972
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddr239
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