A reduction in mitochondrial repair enzymes in patients and SOD1 mice (Kikuchi et al., 2002; Murakami et al., 2007), and markers of oxidative stress such as lipid peroxidation, protein glycoxidation and altered protein carbonyl levels in patient samples (Shibata et al., 2002) emphasize an exacerbating mitochondrial dysfunction in ALS (Mahoney et al., 2006). Here, SOD1 is linked to amyotrophic lateral sclerosis.