Our current findings indicate that gene deletion starting at embryonic day 7 driven by Pax7-Cre led to a severe phenotype, with early onset of skeletal muscle pathology, reduced body size and weight, and a shortened lifespan, thus presenting a similar phenotype to that of dystroglycanopathy patients with mutations in Pomt1. The gene discussed is PAX7; the disease is neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.