SOD1 and amyotrophic lateral sclerosis: Aberrant mitochondrial structures have been reported in both familial and sporadic ALS patients (Hirano et al., 1984a, b; Sasaki and Iwata, 1996, 2007), as well as in mutant SOD1 mouse models (Dal Canto and Gurney, 1994; Wong et al., 1995; Kong and Xu, 1998; Higgins et al., 2003).