Dysregulation of PGC-1α has been connected to many neurodegenerative and metabolic disorders; Parkinson’s, Alzheimer’s and Huntington’s disease, Amyotrophic Lateral Sclerosis (ALS)9, 10, type 2 diabetes11, obesity12 and heart failure13, 14 are just some examples. This evidence concerns the gene PPARGC1A and amyotrophic lateral sclerosis.