However, it has been regarded as a very important region corresponding to the inactivating effect of disease-causing p94 (The skeletal-muscle-specific member of the CAPN family: CAPN3, the CAPN isoform, can be regarded as close to CAPN1 and CAPN2, at least with respect to 45% sequence identity) gene mutations in LGMD-2A (limb girdle muscular dystrophy type 2A) patients [9,40,41]. Here, CAPN1 is linked to autosomal recessive limb-girdle muscular dystrophy type 2A.