In ALS, overexpression of PGC-1α in skeletal muscle of a mouse model increased mitochondrial biogenesis and muscle function without affecting disease progression, or extending survival,7 whereas general overexpression of PGC-1α seems to delay the progression of the disease in a mouse model of ALS.8 In Alzheimer’s disease (AD), overexpression of PGC-1α in a mouse model was detrimental for the disease through a mechanism involving impairing autophagy leading to the accumulation of toxic amyloid beta (AB).9 This evidence concerns the gene PPARGC1A and amyotrophic lateral sclerosis.