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Peripheral oxidative stress biomarkers in spinocerebellar ataxia type 3/Machado–Joseph disease
dc.contributor.author | Assis, Adriano Martimbianco de | pt_BR |
dc.contributor.author | Saute, Jonas Alex Morales | pt_BR |
dc.contributor.author | Santos, Aline Longoni dos | pt_BR |
dc.contributor.author | Haas, Clarissa Branco | pt_BR |
dc.contributor.author | Torrez, Vitor Rocco | pt_BR |
dc.contributor.author | Brochier, Andressa Wigner | pt_BR |
dc.contributor.author | Souza, Gabriele Nunes | pt_BR |
dc.contributor.author | Furtado, Gabriel Vasata | pt_BR |
dc.contributor.author | Gheno, Tailise Conte | pt_BR |
dc.contributor.author | Russo, Aline | pt_BR |
dc.contributor.author | Monte, Thais Lampert | pt_BR |
dc.contributor.author | Castilhos, Raphael Machado de | pt_BR |
dc.contributor.author | Schuh, Artur Francisco Schumacher | pt_BR |
dc.contributor.author | Davila, Rui | pt_BR |
dc.contributor.author | Donis, Karina Carvalho | pt_BR |
dc.contributor.author | Rieder, Carlos Roberto de Mello | pt_BR |
dc.contributor.author | Souza, Diogo Onofre Gomes de | pt_BR |
dc.contributor.author | Camey, Suzi Alves | pt_BR |
dc.contributor.author | Leotti, Vanessa Bielefeldt | pt_BR |
dc.contributor.author | Jardim, Laura Bannach | pt_BR |
dc.contributor.author | Portela, Luis Valmor Cruz | pt_BR |
dc.date.accessioned | 2018-09-25T02:34:13Z | pt_BR |
dc.date.issued | 2017 | pt_BR |
dc.identifier.issn | 1664-2295 | pt_BR |
dc.identifier.uri | http://hdl.handle.net/10183/182527 | pt_BR |
dc.description.abstract | Objectives: Spinocerebellar ataxia type 3/Machado–Joseph disease (SCA3/MJD) is a polyglutamine disorder with no current disease-modifying treatment. Conformational changes in mutant ataxin-3 trigger different pathogenic cascades, including reactive oxygen species (ROS) generation; however, the clinical relevance of oxidative stress elements as peripheral biomarkers of SCA3/MJD remains unknown. We aimed to evaluate ROS production and antioxidant defense capacity in symptomatic and presymptomatic SCA3/MJD individuals and correlate these markers with clinical and molecular data with the goal of assessing their properties as disease biomarkers. Methods: Molecularly confirmed SCA3/MJD carriers and controls were included in an exploratory case–control study. Serum ROS, measured by 2′,7′-dichlorofluorescein diacetate (DCFH-DA) as well as superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) antioxidant enzyme activities, levels were assessed. Results: Fifty-eight early/moderate stage symptomatic SCA3/MJD, 12 presymptomatic SCA3/MJD, and 47 control individuals were assessed. The DCFH-DA levels in the symptomatic group were 152.82 nmol/mg of protein [95% confidence interval (CI), 82.57–223.08, p < 0.001] higher than in the control and 243.80 nmol/mg of protein (95% CI, 130.64–356.96, p < 0.001) higher than in the presymptomatic group. The SOD activity in the symptomatic group was 3 U/mg of protein (95% CI, 0.015–6.00, p = 0.048) lower than in the presymptomatic group. The GSH-Px activity in the symptomatic group was 13.96 U/mg of protein (95% CI, 5.90–22.03, p < 0.001) lower than in the control group and 20.52 U/mg of protein (95% CI, 6.79–34.24, p < 0.001) lower than in the presymptomatic group and was inversely correlated with the neurological examination score for spinocerebellar ataxias (R = −0.309, p = 0.049). Conclusion: Early/moderate stage SCA3/MJD patients presented a decreased antioxidant capacity and increased ROS generation. GSH-Px activity was the most promising oxidative stress disease biomarker in SCA3/MJD. Further longitudinal studies are necessary to identify both the roles of redox parameters in SCA3/MJD pathophysiology and as surrogate outcomes for clinical trials. | en |
dc.format.mimetype | application/pdf | pt_BR |
dc.language.iso | eng | pt_BR |
dc.relation.ispartof | Frontiers in neurology. Lausanne. Vol. 8, article 485 (Sept. 2017), p. 1-8 | pt_BR |
dc.rights | Open Access | en |
dc.subject | Doença de Machado-Joseph | pt_BR |
dc.subject | Spinocerebellar ataxia type 3 | en |
dc.subject | Machado–Joseph disease | en |
dc.subject | Estresse oxidativo | pt_BR |
dc.subject | Oxidative stress | en |
dc.subject | Espécies reativas de oxigênio | pt_BR |
dc.subject | Reactive oxygen species | en |
dc.subject | Estatística médica | pt_BR |
dc.subject | Polyglutamine disorders | en |
dc.title | Peripheral oxidative stress biomarkers in spinocerebellar ataxia type 3/Machado–Joseph disease | pt_BR |
dc.type | Artigo de periódico | pt_BR |
dc.identifier.nrb | 001049856 | pt_BR |
dc.type.origin | Estrangeiro | pt_BR |
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