To the possibilities that ER stressor/ALS drug kinetics were not optimized or that the ALS drugs work in non-ER related assays or specific ALS patient cohorts, corroboratory data in a large (∼50) ALS iPSC-derived cohort using extended maturation times (>45 days in culture) to reveal ALS MN neurite regression, cytotoxicity, and FUS + or phosphorylated-TDP-43 + protein aggregates, has also demonstrated that riluzole and edaravone fail to protect against these degenerative phenotypes in ALS MNs (Fujimori et al., 2018). The gene discussed is FUS; the disease is amyotrophic lateral sclerosis.