Enhanced phosphorylation of eIF2α increased levels of ER foldase PDIA1, and upregulation of CHOP has been found in postmortem brain sections of ALS patients (Atkin et al., 2008; Ilieva et al., 2007; Ito et al., 2009; Matus et al., 2013), all indicating ER stress. This evidence concerns the gene P4HB and amyotrophic lateral sclerosis.