Association between Machado–Joseph disease and oxidative stress biomarkers
| Autor: | Caroline Belló, Ivana Beatrice Mânica da Cruz, Laura Segabinazzi Pacheco, Adriano Flesch Lenz, Aron Ferreira da Silveira, Maria Fernanda Mânica-Cattani, Thaís Doeler Algarve, Lucien J. Houenou, Alexis Trott |
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| Rok vydání: | 2013 |
| Předmět: |
Adult
Male congenital hereditary and neonatal diseases and abnormalities Cerebellum DNA damage Health Toxicology and Mutagenesis Disease Biology medicine.disease_cause Genetics medicine Humans Lymphocytes Machado-Joseph Disease Middle Aged Catalase medicine.disease body regions Comet assay Oxidative Stress medicine.anatomical_structure Case-Control Studies Immunology biology.protein Spinocerebellar ataxia Female Machado–Joseph disease Biomarkers Oxidative stress |
| Zdroj: | Mutation Research/Genetic Toxicology and Environmental Mutagenesis. 757:99-103 |
| ISSN: | 1383-5718 |
| DOI: | 10.1016/j.mrgentox.2013.06.023 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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