Limb-girdle muscular dystrophy recessive type 1 (LGMDR1, previously LGMD2A), caused by calpain 3 (CAPN3) gene variants typically resulting in loss of function of calpain 3 (CAPN3), is the most common recessive LGMD worldwide (Zatz and Starling, 2005; Guglieri et al., 2008; Norwood et al., 2009). The gene discussed is CAPN3; the disease is limb-girdle muscular dystrophy.