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Spastin recovery in hereditary spastic paraplegia by preventing neddylation-dependent degradation
Life Science Alliance
Hereditary Spastic Paraplegia (HSP) is a neurodegenerative disease most commonly caused by autosomal dominant mutations in the SPG4 gene encoding the microtubule-severing protein spastin. We hypothesise that SPG4-HSP is attributable to reduced spastin function because of haploinsufficiency; thus, therapeutic approaches which elevate levels of the wild-type spastin allele may be an effective therapy. However, until now, how spastin levels are regulated is largely unknown. Here, we show that thedoi:10.26508/lsa.202000799 pmid:33106322 fatcat:7nl4bpwq3ra3bl6rlvro3n32xm