In particular, mutations in the ESCRT-III protein charged multivesicular body protein 2b (CHMP2b), responsible for ILV scission through its recruitment of the membrane-severing AAA-ATPase Vps4 (Teis et al, 2008), result in frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (Skibinski et al, 2005; Parkinson et al, 2006; Cox et al, 2010; Ghanim et al, 2010; Gao et al, 2017; Waegaert et al, 2022). Here, CHMP2B is linked to amyotrophic lateral sclerosis.