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Attenuated Ca(2+) release in a mouse model of limb girdle muscular dystrophy 2A

BACKGROUND: Mutations in CAPN3 cause limb girdle muscular dystrophy type 2A (LGMD2A), a progressive muscle wasting disease. CAPN3 is a non-lysosomal, Ca-dependent, muscle-specific proteinase. Ablation of CAPN3 (calpain-3 knockout (C3KO) mice) leads to reduced ryanodine receptor (RyR1) expression and...

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Détails bibliographiques
Publié dans:Skelet Muscle
Auteurs principaux: DiFranco, Marino, Kramerova, Irina, Vergara, Julio L., Spencer, Melissa Jan
Format: Artigo
Langue:Inglês
Publié: BioMed Central 2016
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Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC4765215/
https://ncbi.nlm.nih.gov/pubmed/26913171
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s13395-016-0081-y
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