CHMP2B and frontotemporal dementia: The gene encoding CHMP2B the ESCRT-III subunit was found to be mutated in a form of frontotemporal dementia (Skibinski et al., 2005) and amyotrophic lateral sclerosis (Cox et al., 2010) suggesting that functional MVBs are required to prevent accumulation of abnormal proteins that can disrupt neural function and ultimately lead to neurodegeneration (Filimonenko et al., 2007).