Article
Attenuated Ca(2+) release in a mouse model of limb girdle muscular dystrophy 2A.
Skeletal muscle - 1 Jan 2016
DiFranco Marino, Kramerova Irina, Vergara Julio L, Spencer Melissa Jan
Abstract excerpt
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 abnormal Ca2+/calmodulin-dependent protein kinase II (Ca-CaMKII)-mediated signaling. We previously reported that...
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