Dihydro-orotate dehydrogenase is physically associated with the respiratory complex and its loss leads to mitochondrial dysfunction

Dihydro-orotate dehydrogenase is physically associated with the respiratory complex and its loss leads to mitochondrial dysfunction

Some mutations of the DHODH (dihydro-orotate dehydrogenase) gene lead to postaxial acrofacial dysostosis or Miller syndrome. Only DHODH is localized at mitochondria among enzymes of the de novo pyrimidine biosynthesis pathway. Since the pyrimidine biosynthesis pathway is coupled to the mitochondrial...

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Bibliographic Details
Main Authors: JingXian Fang, Takeshi Uchiumi, Mikako Yagi, Shinya Matsumoto, Rie Amamoto, Shinya Takazaki, Haruyoshi Yamaza, Kazuaki Nonaka, Dongchon Kang
Format: Article
Language:English
Published: Portland Press, Biochemical Society 2013-02-01
Series:Bioscience Reports
Subjects:
dihydro-orotate dehydrogenase (DHODH)
Miller syndrome
mitochondrial dysfunction
pyrimidine pathway
ubiquinone
Online Access:http://www.bioscirep.org/bsr/033/e021/bsr033e021.htm

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http://www.bioscirep.org/bsr/033/e021/bsr033e021.htm

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