As we recently reported, quadriceps and gastrocnemius muscles of dysferlin-deficient Bla/J mice, another model of dysferlinopathy, exhibit high levels of protein oxidation and lipid peroxidation and altered activity of the antioxidant enzymes superoxide dismutase and catalase [33]. Here, DYSF is linked to neuromuscular disease caused by qualitative or quantitative defects of dysferlin.