Dystroglycanopathies are primarily linked mutations in glycosyltransferases that target α-dystroglycan, including fukutin-related protein (FKRP/LGMD2I) [139], fukutin (FKTN/LGMD2M), -like-acetylglucosaminyl transferase (LARGE), protein-O-mannosyltransferase 1 (POMT1/LGMD2K), protein-O-mannosyl transferase 2 (POMT2/LGMD2N), protein-O-mannose-1,2-N-acetylglucosaminyltransferase 1 (POMGNT1/LGMD2O), dolichyl-phosphate mannosyltransferase polypeptide 3 (DPM3), and isoprenoid synthase domain containing (ISPD/LGMD2U) [151,152,153,154,155,156]. This evidence concerns the gene DAG1 and neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.