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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...
Autores principales: | DiFranco, Marino, Kramerova, Irina, Vergara, Julio L., Spencer, Melissa Jan |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4765215/ https://www.ncbi.nlm.nih.gov/pubmed/26913171 http://dx.doi.org/10.1186/s13395-016-0081-y |
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