title: Association between Machado-Joseph disease and oxidative stress biomarkers.
abstract: Spinocerebellar ataxia type 3, also called Machado-Joseph disease (MJD), is an hereditary autosomal dominant neurodegenerative disease that affects the cerebellum and its afferent and efferent connections. Since the mechanism by which mutant ataxin-3 eventually leads to neuronal death is poorly understood, additional investigations to clarify the biological alterations related to Machado-Joseph disease are necessary. Recent investigations suggest that oxidative stress may contribute significantly to Machado-Joseph disease. We compared markers of oxidative stress between Machado-Joseph disease and healthy control subjects. The results showed that Machado-Joseph patients have higher catalase levels and lower thiol protein levels compared to control subjects. The peripheral blood lymphocyes of MJD patients also showed higher levels of DNA damage by the comet assay than control subjects. Our results corroborate the hypothesis that the oxidative stress is associated with MJD patients. However, whether strategies to increase cellular antioxidative capacity may be effective therapies for the treatment of Machado-Joseph disease is an open question.
authors.affiliation: Center for Health Sciences, Federal University of Santa Maria, UFSM, Brazil.
authors.forename: Laura Segabinazzi
authors.initials: LS
authors.lastname: Pacheco
authors.forename: Aron Ferreira
authors.initials: AF
authors.lastname: da Silveira
authors.forename: Alexis
authors.initials: A
authors.lastname: Trott
authors.forename: Lucien J
authors.initials: LJ
authors.lastname: Houenou
authors.forename: Thaís Doeler
authors.initials: TD
authors.lastname: Algarve
authors.forename: Caroline
authors.initials: C
authors.lastname: Belló
authors.forename: Adriano Flesch
authors.initials: AF
authors.lastname: Lenz
authors.forename: Maria Fernanda
authors.initials: MF
authors.lastname: Mânica-Cattani
authors.forename: Ivana Beatrice Mânica
authors.initials: IB
authors.lastname: da Cruz
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D002374:Catalase / Q000097:blood*; D005260:Female; D006801:Humans; D008214:Lymphocytes / Q000201:enzymology* / Q000473:pathology; D017827:Machado-Joseph Disease / Q000097:blood* / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged; D018384:Oxidative Stress
chemical_list: D015415:Biomarkers; D002374:Catalase
keywords: Antioxidant enzymes; Genotoxicity; Machado–Joseph disease; Oxidative stress; Spinocerebellar ataxia