Although it remains unknown whether dystroglycan interacts with specific dystrophins or utrophin paralogues during fetal brain development, genetic loss of dystroglycan recapitulates the brain abnormalities observed in these congenital muscular dystrophies called dystroglycanopathies (Satz et al., 2010). Here, DAG1 is linked to neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.