Intriguingly, low PGC-1α expression is related to lower mitochondrial activity and to a worsened regulation of reactive oxygen species [54, 55], and fewer PGC-1α seems to be associated with a decrease in mitochondrial activity [140] and an increase in muscle degeneration in a different animal model of ALS [141, 142]. The gene discussed is PPARGC1A; the disease is amyotrophic lateral sclerosis.