Small interference RNA to silence the COL6A3 allele carrying an in-frame deletion in exon 16 [10] or to specifically silence the p.G284R or p.G293R point mutations in the COL6A1 gene [11,12] have been demonstrated to considerably improve the quantity and quality of the collagen VI matrix in transfected skin fibroblasts from UCMD patients. The gene discussed is COL6A3; the disease is Ullrich congenital muscular dystrophy.