Dystroglycanopathy patients with moderate severity can exhibit cognitive impairments even in the absence of identifiable brain malformations, suggesting that Dystroglycan functions at later stages of neural circuit formation such as synapse formation and/or maintenance (Clement et al., 2008; Godfrey et al., 2007). The gene discussed is DAG1; the disease is neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.