Interestingly, mutations in CHMP2B, which is a major component of ESCRT-III, have been identified in chromosome 3-linked FTD (FTD-3), and the disease-associated CHMP2BIntron5 causes defects in protein trafficking in the endolysosomal pathway and in the accumulation of autophagosomes due to an impairment in the fusion of autophagosomes with lysosomes [18, 19]. Here, CHMP2B is linked to frontotemporal dementia.