Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration

BACKGROUND: Leber's hereditary optic neuropathy (LHON) is caused by mutations in the complex I subunits of the respiratory chain. Although patients have been treated with idebenone since 1992, the efficacy of the drug is still a matter of debate. METHODS: We evaluated the effect of idebenone in...

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Detalles Bibliográficos
Autores principales: Angebault, Claire, Gueguen, Naïg, Desquiret-Dumas, Valérie, Chevrollier, Arnaud, Guillet, Virginie, Verny, Christophe, Cassereau, Julien, Ferre, Marc, Milea, Dan, Amati-Bonneau, Patrizia, Bonneau, Dominique, Procaccio, Vincent, Reynier, Pascal, Loiseau, Dominique
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2011
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3285568/
https://www.ncbi.nlm.nih.gov/pubmed/22192149
http://dx.doi.org/10.1186/1756-0500-4-557

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