Among the non-histone protein substrates, the peroxisome proliferator-activated receptor gamma coactivator 1 alpha (PGC1-α; Cantó and Auwerx, 2009) and the forkhead box O3a (FoxO3a) transcription factor (Zhao et al., 2011b) are two of the most studied, and their relation to ALS will be discussed below. Here, PPARGC1A is linked to amyotrophic lateral sclerosis.