ALS-associated mutations lead to increased localization of misfolded SOD1 protein to the mitochondrial inter-membrane space (Liu et al., 2004; Deng et al., 2006), and mutant SOD1-mitochondrial interactions lead to the alteration of mitochondrial redox potential (Ferri et al., 2006). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.