One of the main mechanisms by which elevated Ca2+ causes muscle wasting is due to the activation of calcium-dependent proteases, such as calpain 3, a calcium-dependent cysteine protease expressed in skeletal muscle, whose deficiency has been associated with Limb-girdle muscular dystrophy type 2A (LGMD2A). Here, CAPN3 is linked to autosomal recessive limb-girdle muscular dystrophy type 2A.