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The effects of idebenone on mitochondrial bioenergetics

We have studied the effects of idebenone on mitochondrial function in cybrids derived from one normal donor (HQB17) and one patient harboring the G3460A/MT-ND1 mutation of Leber's Hereditary Optic Neuropathy (RJ206); and in XTC.UC1 cells bearing a premature stop codon at aminoacid 101 of MT-ND1...

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Detalles Bibliográficos
Autores principales: Giorgio, Valentina, Petronilli, Valeria, Ghelli, Anna, Carelli, Valerio, Rugolo, Michela, Lenaz, Giorgio, Bernardi, Paolo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier Pub. Co 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3265671/
https://www.ncbi.nlm.nih.gov/pubmed/22086148
http://dx.doi.org/10.1016/j.bbabio.2011.10.012