Mutations in ALS disease causative genes, such as TARDBP/TDP-43, FUS/TLS, and vesicle-associated membrane protein-associated protein B (VAP-B), for instance, trigger aggregation of the mutant proteins leading to a gain of neurotoxic activity and provoking ER stress (Andersen and Al-Chalabi, 2011; Turner et al., 2013). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.