Together, the increased lipid peroxidation in muscles of dysferlinopathy patients (Renjini et al., 2012), the progressive oxidation of muscle of dysferlin deficient A/J mice (Terrill et al., 2013), and the increase in X-ROS in dysferlin-deficient muscle (Prosser et al., 2013) all indirectly support a decline in Nrf2 activity as a factor in the onset and pathogenic progression of the disease. Here, DYSF is linked to neuromuscular disease caused by qualitative or quantitative defects of dysferlin.