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Impaired Nuclear Nrf2 Translocation Undermines the Oxidative Stress Response in Friedreich Ataxia
BACKGROUND: Friedreich ataxia originates from a decrease in mitochondrial frataxin, which causes the death of a subset of neurons. The biochemical hallmarks of the disease include low activity of the iron sulfur cluster-containing proteins (ISP) and impairment of antioxidant defense mechanisms that...
Autores principales: | Paupe, Vincent, Dassa, Emmanuel P., Goncalves, Sergio, Auchère, Françoise, Lönn, Maria, Holmgren, Arne, Rustin, Pierre |
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Formato: | Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2009
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2617762/ https://www.ncbi.nlm.nih.gov/pubmed/19158945 http://dx.doi.org/10.1371/journal.pone.0004253 |
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