Mutations in CAV3 have been associated with several phenotypes, including rippling muscle disease, LGMD and hyperCKemia, and are thought to cause disease via a dominant-negative mechanism involving retention and aggregation of normal caveolin-3 within the Golgi complex (Galbiati et al., 1999; Herrmann et al., 2000). The gene discussed is CAV3; the disease is limb-girdle muscular dystrophy.