ER stress is a feature of sporadic human ALS [23], [27], in familial forms of ALS and FTLD, including those linked to FUS [26], vesicle-associated membrane protein-associated protein B (VAPB) [59] and valosin containing protein [60], and in mutant SOD1 animal and cell models [23], [24], [25], [61]. This evidence concerns the gene VCP and amyotrophic lateral sclerosis.