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Article

Spastin recovery in hereditary spastic paraplegia by preventing neddylation-dependent degradation.

Life science alliance - 1 Dec 2020

Sardina Francesca, Pisciottani Alessandra, Ferrara Manuela, Valente Davide, Casella Marialuisa, Crescenzi Marco, Peschiaroli Angelo, Casali Carlo, Soddu Silvia, Grierson Andrew J, Rinaldo Cinzia

Abstract excerpt

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,...

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Identifiers

PMID: 33106322
DOI: 10.26508/lsa.202000799
PMCID: PMC7652396

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