Morphological and functional abnormalities in mitochondria are a common feature of ALS, and are detected in biopsied and post-mortem tissue from symptomatic sALS and fALS patients, as well as in mutant SOD1 mouse models and the cell cultures derived from these mice (reviewed in Manfredi and Xu, 2005; Bento-Abreu et al., 2010; Grosskreutz et al., 2010; Tan et al., 2014). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.