Other studies with both ALS patients and transgenic G93A*SOD1 mice support the role of ER stress-related cellular dysfunction in ALS pathophysiology (Atkin et al., 2006, 2008; Kikuchi et al., 2006; Ilieva et al., 2007; Nishitoh et al., 2008; Saxena et al., 2009; Wang et al., 2011). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.