Association between Machado-Joseph disease and oxidative stress biomarkers.

Autor: Pacheco LS; Center for Health Sciences, Federal University of Santa Maria, UFSM, Brazil., da Silveira AF, Trott A, Houenou LJ, Algarve TD, Belló C, Lenz AF, Mânica-Cattani MF, da Cruz IB
Jazyk: angličtina
Zdroj: Mutation research. Genetic toxicology and environmental mutagenesis [Mutat Res Genet Toxicol Environ Mutagen] 2013 Oct 09; Vol. 757 (2), pp. 99-103. Date of Electronic Publication: 2013 Aug 30.
DOI: 10.1016/j.mrgentox.2013.06.023
Abstrakt: 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.
(Copyright © 2013 Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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