Tissue-specific oxidative stress and loss of mitochondria in CoQ-deficient Pdss2 mutant mice
Tissue-specific oxidative stress and loss of mitochondria in CoQ-deficient Pdss2 mutant mice
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DOI:
10.1096/fj.12-209361
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发表时间:
2013-02-01
期刊:
影响因子:
4.8
通讯作者:
Hirano, Michio
中科院分区:
文献类型:
--
作者:
Quinzii, Catarina M.;Garone, Caterina;Hirano, Michio
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