α-DYSTROGLYCAN hypoglycosylation is associated with dystroglycanopathies that are classified depending on whether the causative mutation affects α-DYSTROGLYCAN itself (primary) or genes encoding for α-DYSTROGLYCAN-glycosylating enzymes (secondary or tertiary) [182]. This evidence concerns the gene DAG1 and neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.