SOD1 and amyotrophic lateral sclerosis: In mutant SOD1 [90, 129, 188] and FUS [183] ALS mouse models, mitochondrial dysfunction is an early event and overexpressing peroxisome proliferator activated receptor-gamma coactivator 1 alpha (PGC1α), a major regulator of mitochondrial biogenesis, improved survival, motor function, and motor neuron survival in mutant SOD1G93A mice [221].