CHMP2B and amyotrophic lateral sclerosis: The carriers of CHMP2B mutations are characterized by ubiquitinated inclusions negative for TDP-43.[49] Dysfunction of the ESCRT results in the inability of multi-vesicular bodies to internalize membrane bound cargoes, leading to distorted endosomes and reduced protein turnover.[50] Besides FTD, CHMP2B mutations can also cause additional phenotypes including ALS and FTD-ALS.[51]