A mutation in CHMP2B, a subunit of the endosomal sorting complex required for transport-III (ESCRT-III), causes an autosomal dominant form of frontotemporal dementia (FTD) in a Danish cohort (Skibinski et al., 2005; Lindquist et al., 2008). The gene discussed is CHMP2B; the disease is frontotemporal dementia.