To assess whether elevated muscle PGC-1α ameliorates disease progression of dysferlin-deficient mice, analogous to previous findings in Duchenne muscular dystrophy and other muscle wasting models, we crossed skeletal muscle-specific PGC-1α transgenic mice (Lin et al, 2002) with a model for dysferlinopathy (Bansal et al, 2003; Wiktorowicz et al, 2015). Here, DYSF is linked to neuromuscular disease caused by qualitative or quantitative defects of dysferlin.