CHMP2B and amyotrophic lateral sclerosis: Dysfunction of ESCRT-III, either by depletion of its essential subunit mSnf7-2, or by expression of a mutant CHMP2B protein (another component of the complex) is associated with frontotemporal dementia linked to chromosome 3 (FTD3) and amyotrophic lateral sclerosis (ALS), both characterized by progressive neuronal accumulation of ubiquitin-positive protein aggregates (Parkinson et al., 2006; Skibinski et al., 2005).