Previous studies support the hypothesis that dysfunction of the ESCRT complex may cause neurodegeneration presenting as HSP including variants in ubiquitin associated protein 1 (UBAP1), a component of the ESCRT-I complex, in which variants have recently been associated with autosomal dominant pure HSP (SPG80) with childhood onset [25]. This evidence concerns the gene UBAP1 and autosomal dominant pure spastic paraplegia.