Glial fibrillary acidic protein (GFAP)-Cre- or Nestin-Cre-mediated deletion of dystroglycan in the central nervous system recapitulated the structural brain defects found in patients with dystroglycanopathy, such as basement membrane disruption, neuronal migration defects, and neuronal heterotopia formation20, 23. This evidence concerns the gene NES and neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.