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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...
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 |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
WILEY-VCH Verlag
2011
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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 |
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