Limb-girdle muscular dystrophy type 2A (LGMD2A) is caused by a loss of the protease activity of the skeletal-muscle-specific calpain p94/calpain 3, which is caused by a pathogenic mutation of the p94 gene, CAPN3 [17, 18]. Here, CAPN3 is linked to autosomal recessive limb-girdle muscular dystrophy type 2A.