To study the pathological mechanisms leading to dysferlinopathies and to investigate therapeutic strategies, we created the mouse model Dysf p.Y1159X/p.Y1159X by integrating the nonsense mutation NM_003494.3: c.3477C > A (p.Y1159X) which leads to dysferlin absence. The gene discussed is DYSF; the disease is neuromuscular disease caused by qualitative or quantitative defects of dysferlin.