Dysregulated Ca handling linked to RyR dysfunction is reported in several muscular dystrophies, including Duchennes [9], dysferlin-related LGMD R2 [10], β-sarcoglycan-related LGMD R4 [11], and calpain 3-related LGMD R1 [12]. This evidence concerns the gene CAPN3 and autosomal recessive limb-girdle muscular dystrophy type 2E.