Loading...
The transmembrane protein dystroglycan, which cleaves to form an outer (α) component and an inner (β) component, links the cytoskeleton to extracellular proteins. The α-dystroglycan serves as a matrix receptor and requires extensive O-glycosylation to function. Maturation of α-dystroglycan to its proper laminin-binding form requires phosphorylation on O-mannosyl glycans and the glycosyltransferase protein, like-acetylglucosaminyltransferase (LARGE), facilitates this action. Hypoglycosylation of α-dystroglycan can cause several types of muscular dystrophy, including congenital forms (with varying phenotypes) and limb-girdle muscular dystrophy. Approximately half of patients with these diseases, which are known as secondary dystroglycanopathi…