PDI associates with misfolded protein inclusions in patients with ALS (Honjo et al., 2011; Parakh et al., 2018a) cellular models (Farg et al., 2012; Jeon et al., 2014), and canine degenerative myelopathy (DM; Chang et al., 2019), and both PDI and ERp57 inhibit the formation of mutant SOD1 inclusions in neuronal cells (Walker et al., 2010; Parakh et al., 2018a). Here, SOD1 is linked to amyotrophic lateral sclerosis.