Both upregulation of Ppargc1 gene in skeletal muscle and increased lipid metabolism in fast-twitch type IIb fibers of SOD1 mice [62, 84] support the notion that the energy derived from muscle glycogen might gradually decline favoring an increase of plasma fatty acid oxidation and possibly hypermetabolism in ALS. The gene discussed is PPARGC1A; the disease is amyotrophic lateral sclerosis.