Possibly representing a cellular response to this kind of aggregation and as a reaction against ER stress and unfolded protein response (UPR), protein disulfide isomerase (PDI) expression levels are upregulated in spinal cord tissue of ALS patients where PDI co-localizes with SOD1, TDP-43, and FUS (Atkin et al., 2008; Honjo et al., 2011; Farg et al., 2012; Walker et al., 2013). Here, TARDBP is linked to amyotrophic lateral sclerosis.