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Tissue-specific oxidative stress and loss of mitochondria in CoQ-deficient Pdss2 mutant mice

Primary human CoQ(10) deficiencies are clinically heterogeneous diseases caused by mutations in PDSS2 and other genes required for CoQ(10) biosynthesis. Our in vitro studies of PDSS2 mutant fibroblasts, with <20% CoQ(10) of control cells, revealed reduced activity of CoQ(10)-dependent complex II+...

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Main Authors: Quinzii, Catarina M., Garone, Caterina, Emmanuele, Valentina, Tadesse, Saba, Krishna, Sindu, Dorado, Beatriz, Hirano, Michio
格式: Artigo
語言:Inglês
出版: Federation of American Societies for Experimental Biology 2013
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在線閱讀:https://ncbi.nlm.nih.gov/pmc/articles/PMC3545522/
https://ncbi.nlm.nih.gov/pubmed/23150520
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1096/fj.12-209361
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