Defect in glycosylation that causes muscular dystrophy

PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Defect in glycosylation that causes muscular dystrophy (Methods Enzymol. 2006;417:137-52.). Authors are Endo T Manya H from the Glycobiology Research Group, Tokyo Metropolitan Institute of Gerontology Foundation for Research on Aging and Promotion of Human Welfare, Tokyo, Japan. Muscular dystrophies are a diverse group of inherited disorders characterized by progressive muscle weakness and wasting. The dystrophin-glycoprotein complex is composed of alpha-, beta-dystroglycan (DG), dystrophin and some other molecules.New findings indicate that disrupted glycosylation of alpha-DG results in a loss of ligand binding, giving rise to both progressive muscle degeneration and abnormal neuronal migration in the brain. This article discusses methods, including purification of alpha-DG and glycosyltransferase assays involved in alpha-DG glycosylation. To access the full abstract of the article, click here.