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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: | , , , , , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
Federation of American Societies for Experimental Biology
2013
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| Assuntos: | |
| Acesso em linha: | 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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