SOD1 and amyotrophic lateral sclerosis: Both in mouse and rat models of ALS and post-mortem tissue samples from ALS patients, mutant SOD1 was found in fractions enriched for mitochondria derived only from affected but not unaffected tissues (Mattiazzi et al., 2002; Liu et al., 2004; Vijayvergiya et al., 2005; Bergemalm et al., 2006; Deng et al., 2006; Vande Velde et al., 2008).