While the spontaneous occurrence of RMS has been previously reported in mdx mice [20] and in addition in mice deficient of α-sarcoglycan [21] (Sgca −/−, a model for the human LGMD2D), this is the first report of sarcomas in mice lacking dysferlin, calpain 3, or Large. Here, CAPN3 is linked to autosomal recessive limb-girdle muscular dystrophy type 2D.