SOD1 and amyotrophic lateral sclerosis: Whilst SOD1 mutations represent only 2% of all ALS, and may not accurately represent pathology in the more common forms of disease, similar findings were obtained in post-mortem human spinal cord tissues of sporadic ALS patients (Ilieva et al., 2007; Atkin et al., 2008; Ito et al., 2009), thus placing ER stress on a more generic pathophysiology for ALS.