Meanwhile, the heterozygous CAPN3 variants have been reported to cause autosomal dominant limb–girdle muscular dystrophy-4 (LGMDD4; MIM #618129; ORPHA # 102014), which has a later onset and milder phenotype than LGMDR1 [2, 4, 5]. Here, CAPN3 is linked to muscular dystrophy, limb-girdle, autosomal dominant 4.