Activation of ER stress has been documented in tissue samples from ALS and FTLD patients as well as cellular and animal models carrying mutations in familiar ALS-associated genes such as SOD1, VAPB, or FUS (Kikuchi et al., 2006; Gitcho et al., 2009; Saxena et al., 2009; Chen et al., 2010; Langou et al., 2010; Farg et al., 2012). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.