SOD1 and amyotrophic lateral sclerosis: Mitochondrial dysfunction as a result of membrane integrity defects caused by misfolded SOD1 has been reported in vitro in neuronal cells (Carrì et al., 1997; Menzies et al., 2002) and in vivo in mutant SOD1 transgenic rodent models (Higgins et al., 2002; Mattiazzi et al., 2002), as well as in ALS patients (Swerdlow et al., 1998; Wiedemann et al., 2002).