Although we do not observe nuclear CHMP4B and CHMP2B pathology in C9orf72 ALS/FTD or sALS human neurons (Figs. 1, 2), we cannot rule out the possibility that the cytoplasmic functions of these ESCRT-III proteins are impaired in ALS pathogenesis. This evidence concerns the gene CHMP4B and amyotrophic lateral sclerosis.