For example, misfolded and aggregated SOD1 mutants localized within the mitochondrial membrane of spinal cord MN cause dysfunction in oxidative phosphorylation and bind aberrantly to Bcl-2, generating toxicity (Jung et al., 2002; Mattiazzi et al., 2002; Liu et al., 2004; Vande Velde et al., 2008; Pedrini et al., 2010); also, the ALS linked P56S mutation in VAPB, or th A4V, G85R and G93A SOD1 mutations leads to toxic protein aggregation and ER stress (Prosser et al., 2008; Kim et al., 2010; Atkin et al., 2014). Here, SOD1 is linked to amyotrophic lateral sclerosis.