Mutations in the genes COL6A1, COL6A2, and COL6A3, coding for three α chains of collagen type VI, cause COL6-related myopathies (COL6-RM), including the severe Ullrich congenital muscular dystrophy (UCMD), the milder Bethlem myopathy (BM) (Bertini and Pepe, 2002; Allamand et al., 2010), and the Myosclerosis Myopathy in a single family (Merlini et al., 2008a,b). The gene discussed is COL6A2; the disease is Ullrich congenital muscular dystrophy.