PGC1α and ERR-driven pathways are beneficial in the context of many skeletal muscle maladies including atrophy [69,70], mitochondrial dysfunction [71], ischemia [35,72], age-related deterioration [73], amyotrophic lateral sclerosis [74], and muscular dystrophy [75–77]. Here, PPARGC1A is linked to amyotrophic lateral sclerosis.