Mutations in the ESCRT-III subunit, CHMP2B (charged multivesicular body protein 2B or chromatin-modifying protein 2B)/Vps2B are reported in patients with neurodegenerative diseases, like FTD47 and ALS.48 Similar mutations result in accumulation of aggregates containing ubiquitinated proteins, p62 and Alfy (autophagy-linked FYVE protein)15 – key components of autophagy. This evidence concerns the gene SQSTM1 and amyotrophic lateral sclerosis.