In agreement with the cytoprotective role of canonical Nrf2 signaling, pharmacologic activation of the Nrf2 pathway is myoprotective in a mouse model of dystrophinopathy [38], while Nrf2 deletion enhances muscle pathology in a mouse model of dysferlinopathy [39], possibly because intact Nrf2 signaling is required for muscle regeneration [40]. Here, NFE2L2 is linked to neuromuscular disease caused by qualitative or quantitative defects of dysferlin.