ALS-associated mutations in TDP43, SOD1, FUS, and C9ORF72 (C9 open reading frame 72) (Watabe and Nakaki, 2004; Andersen and Al-Chalabi, 2011; Fecto and Siddique, 2011; Farg et al., 2014) result in protein misfolding, aggregate accumulation and is associated with ER stress (Dafinca et al., 2016). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.