Studies in mice models of ALS (SOD1 G93A and SOD1 G37R mouse) and Duchenne muscular dystrophy (mdx mouse; Sheng et al., 2012) have shown that increased expression and activity of PGC-1α can improve the clinical manifestations (Handschin et al., 2007b; Liang et al., 2011) by increasing mitochondrial biogenesis and ROS detoxification (Austin and St-Pierre, 2012). Here, PPARGC1A is linked to amyotrophic lateral sclerosis.