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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...

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Detalles Bibliográficos
Autores principales: Paupe, Vincent, Dassa, Emmanuel P., Goncalves, Sergio, Auchère, Françoise, Lönn, Maria, Holmgren, Arne, Rustin, Pierre
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2009
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