So far, 18 mutations in Cav3 (Figure 1) have been associated with human dystrophies such as limb-girdle muscular dystrophy 1C (LGMD-1C), hereditary rippling muscle disease (RMD), hyperCKemia, and distal myopathy [13], whereas Cav3 overexpression is observed in Duchenne muscular dystrophy. Here, CAV3 is linked to distal myopathy.