CAPN3 and autosomal recessive limb-girdle muscular dystrophy type 2A: We also describe a group of people affected by calpainopathy (LGMD-R1), the most frequent form of girdle dystrophy, which in European countries would represent about 40–50% of total cases of LGMD [15,16,17,18,19], a recessive form, due to mutations in the gene coding for calpain-3 (CAPN3), a muscle specific family’s member of Ca++ activated neutral protease [20].