Alpha-dystroglycanopathies are a clinically and genetically heterogenous group of muscular dystrophies characterized by the reduced or absent glycosylation of alpha-dystroglycan (Muntoni et al., 2002). The hypoglycosylation of alpha-dystroglycan leads to decreased binding of its ligands, including laminin, agrin and perlecan in skeletal muscle and neurexin in the brain. The only known target fo...