Taken together, the changes in the proteome indicate a broad shift from glucose metabolism to mitochondrial respiration, a prominent feature of muscle metabolism in the PGC-1α transgenic animals (Lin et al, 2002; Wende et al, 2007), but unexpected in the dysferlinopathy mice. This evidence concerns the gene PPARGC1A and neuromuscular disease caused by qualitative or quantitative defects of dysferlin.