KCNV1 and amyotrophic lateral sclerosis: To independently confirm this result, we also knocked down KCNV1 expression in control 39b-cor SOD1+/+ MNs to mimic the KCNV1 downregulation in amyotrophic lateral sclerosis, using two different small hairpin RNAs (shRNAs) targeting KCNV1/Kv8.1, and found that the knockdown of KCNV1 in these healthy control MNs increased MG132-induced cell death significantly, while not affecting basal survival (Fig. 2C–E; Supplementary Fig. 4).