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Article

Muscle atrophy in L imb G irdle M uscular D ystrophy 2 A : a morphometric and molecular study

19 Feb 2013

Abstract excerpt

AIMS: The peculiar clinical features and the pathogenic mechanism related to calpain-3 deficiency (impaired sarcomere remodelling) suggest that the ubiquitin-proteasome degradation pathway may have a crucial role in Limb Girdle Muscular Dystrophy 2A (LGMD2A). We therefore investigated muscle atrophy and the role of the ubiquitin-proteasome and lysosomal-autophagic degradation pathways. METHODS: We selected 25...

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PMID: 23414389
DOI: 10.1111/nan.12034

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