Defects of α-dystroglycan (α-DG) O-glycosylation are associated with several forms of inheritable muscular dystrophies (Limb Girdle Muscular Dystrophy type 2I; LGMD2I), of which some are congenital (Congenital Muscular Dystrophy type 1C; MDC1C), and some are associated with brain (Fukyama Congenital Muscular Dystrophy; FCMD, Walker-Warburg Syndrome; WWS) and eye abnormalities (Muscle Eye Brain disease; MEB) [1]. The gene discussed is DAG1; the disease is muscle-eye-brain disease.