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Most notably, the ESCRT-III subunit charged multivesicular body protein 2B (CHMP2B) is mutated in amyotrophic lateral sclerosis and frontotemporal degeneration 11,30,31 , whereas mutations in the ESCRT-I subunit ubiquitin associated protein 1 (UBAP1) underlie hereditary spastic paraplegia (HSP) 32