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Probucol ameliorates renal and metabolic sequelae of primary CoQ deficiency in Pdss2 mutant mice

Therapy of mitochondrial respiratory chain diseases is complicated by limited understanding of cellular mechanisms that cause the widely variable clinical findings. Here, we show that focal segmental glomerulopathy-like kidney disease in Pdss2 mutant animals with primary coenzyme Q (CoQ) deficiency...

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Detalles Bibliográficos
Autores principales: Falk, Marni J, Polyak, Erzsebet, Zhang, Zhe, Peng, Min, King, Rhonda, Maltzman, Jonathan S, Okwuego, Ezinne, Horyn, Oksana, Nakamaru-Ogiso, Eiko, Ostrovsky, Julian, Xie, Letian X, Chen, Jia Yan, Marbois, Beth, Nissim, Itzhak, Clarke, Catherine F, Gasser, David L
Formato: Online Artículo Texto
Lenguaje:English
Publicado: WILEY-VCH Verlag 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3394513/
https://www.ncbi.nlm.nih.gov/pubmed/21567994
http://dx.doi.org/10.1002/emmm.201100149