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NameDihydroorotate dehydrogenase (quinone), mitochondrial
Synonyms
  • 1.3.5.2
  • DHOdehase
  • Dihydroorotate oxidase
Gene NameDHODH
OrganismHuman
Amino acid sequence
>lcl|BSEQ0010124|Dihydroorotate dehydrogenase (quinone), mitochondrial
MAWRHLKKRAQDAVIILGGGGLLFASYLMATGDERFYAEHLMPTLQGLLDPESAHRLAVR
FTSLGLLPRARFQDSDMLEVRVLGHKFRNPVGIAAGFDKHGEAVDGLYKMGFGFVEIGSV
TPKPQEGNPRPRVFRLPEDQAVINRYGFNSHGLSVVEHRLRARQQKQAKLTEDGLPLGVN
LGKNKTSVDAAEDYAEGVRVLGPLADYLVVNVSSPNTAGLRSLQGKAELRRLLTKVLQER
DGLRRVHRPAVLVKIAPDLTSQDKEDIASVVKELGIDGLIVTNTTVSRPAGLQGALRSET
GGLSGKPLRDLSTQTIREMYALTQGRVPIIGVGGVSSGQDALEKIRAGASLVQLYTALTF
WGPPVVGKVKRELEALLKEQGFGGVTDAIGADHRR
Number of residues395
Molecular Weight42866.93
Theoretical pI10.23
GO Classification
Functions
  • ubiquinone binding
  • drug binding
  • FMN binding
  • dihydroorotate dehydrogenase activity
Processes
  • nucleobase-containing small molecule metabolic process
  • female pregnancy
  • pyrimidine nucleobase metabolic process
  • 'de novo' pyrimidine nucleobase biosynthetic process
  • 'de novo' UMP biosynthetic process
  • pyrimidine nucleoside biosynthetic process
  • regulation of mitochondrial fission
  • response to caffeine
  • response to drug
  • response to starvation
  • lactation
  • small molecule metabolic process
  • positive regulation of apoptotic process
Components
  • neuronal cell body
  • cytoplasm
  • mitochondrion
  • mitochondrial inner membrane
  • integral component of membrane
  • nucleoplasm
General FunctionUbiquinone binding
Specific FunctionCatalyzes the conversion of dihydroorotate to orotate with quinone as electron acceptor.
Pfam Domain Function
Transmembrane Regions11-30
GenBank Protein ID555594
UniProtKB IDQ02127
UniProtKB Entry NamePYRD_HUMAN
Cellular LocationMitochondrion inner membrane
Gene sequence
>lcl|BSEQ0010125|Dihydroorotate dehydrogenase (quinone), mitochondrial (DHODH)
ATGGCGTGGAGACACCTGAAAAAGCGGGCCCAGGATGCTGTGATCATCCTGGGGGGAGGA
GGACTTCTCTTCGCCTCCTACCTGATGGCCACGGGAGATGAGCGTTTCTATGCTGAACAC
CTGATGCCGACTCTGCAGGGGCTGCTGGACCCGGAGTCAGCCCACAGACTGGCTGTTCGC
TTCACCTCCCTGGGGCTCCTTCCACGGGCCAGATTTCAAGACTCTGACATGCTGGAAGTG
AGAGTTCTGGGCCATAAATTCCGAAATCCAGTAGGAATTGCTGCAGGATTTGACAAGCAT
GGGGAAGCCGTGGACGGACTTTATAAGATGGGCTTTGGTTTTGTTGAGATAGGAAGTGTG
ACTCCAAAACCTCAGGAAGGAAACCCTAGACCCAGAGTCTTCCGCCTCCCTGAGGACCAA
GCTGTCATTAACAGGTATGGATTTAACAGTCACGGGCTTTCAGTGGTGGAACACAGGTTA
CGGGCCAGACAGCAGAAGCAGGCCAAGCTCACAGAAGATGGACTGCCTCTGGGGGTCAAC
TTGGGGAAGAACAAGACCTCAGTGGACGCCGCGGAGGACTACGCAGAAGGGGTGCGCGTA
CTGGGCCCCCTGGCCGACTACCTGGTGGTGAATGTGTCCAGCCCCAACACTGCCGGGCTG
CGGAGCCTTCAGGGAAAGGCCGAGCTGCGCCGCCTGCTGACCAAGGTGCTGCAGGAGAGG
GATGGCTTGCGGAGAGTGCACAGGCCGGCAGTCCTGGTGAAGATCGCTCCTGACCTCACC
AGCCAGGATAAGGAGGACATTGCCAGTGTGGTCAAAGAGTTGGGCATCGATGGGCTGATT
GTTACGAACACCACCGTGAGTCGCCCTGCGGGCCTCCAGGGTGCCCTGCGCTCTGAAACA
GGAGGGCTGAGTGGGAAGCCCCTCCGGGATTTATCAACTCAAACCATTCGGGAGATGTAT
GCACTCACCCAAGGCCGAGTTCCCATAATTGGGGTTGGTGGTGTGAGCAGCGGGCAGGAC
GCGCTGGAGAAGATCCGGGCAGGGGCCTCCCTGGTGCAGCTGTACACGGCCCTCACCTTC
TGGGGGCCACCCGTTGTGGGCAAAGTCAAGCGGGAACTGGAGGCCCTTCTGAAAGAGCAG
GGCTTTGGCGGAGTCACAGATGCCATTGGAGCAGATCATCGGAGGTGA
GenBank Gene IDM94065
GeneCard IDNot Available
GenAtlas IDDHODH
HGNC IDHGNC:2867
Chromosome Location16
Locus16q22
References
  1. Minet M, Dufour ME, Lacroute F: Cloning and sequencing of a human cDNA coding for dihydroorotate dehydrogenase by complementation of the corresponding yeast mutant. Gene. 1992 Nov 16;121(2):393-6. 1446837
  2. Copeland RA, Davis JP, Dowling RL, Lombardo D, Murphy KB, Patterson TA: Recombinant human dihydroorotate dehydrogenase: expression, purification, and characterization of a catalytically functional truncated enzyme. Arch Biochem Biophys. 1995 Oct 20;323(1):79-86. 7487077
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  5. Knecht W, Bergjohann U, Gonski S, Kirschbaum B, Loffler M: Functional expression of a fragment of human dihydroorotate dehydrogenase by means of the baculovirus expression vector system, and kinetic investigation of the purified recombinant enzyme. Eur J Biochem. 1996 Aug 15;240(1):292-301. 8925840
  6. Rawls J, Knecht W, Diekert K, Lill R, Loffler M: Requirements for the mitochondrial import and localization of dihydroorotate dehydrogenase. Eur J Biochem. 2000 Apr;267(7):2079-87. 10727948
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  8. Bian Y, Song C, Cheng K, Dong M, Wang F, Huang J, Sun D, Wang L, Ye M, Zou H: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J Proteomics. 2014 Jan 16;96:253-62. doi: 10.1016/j.jprot.2013.11.014. Epub 2013 Nov 22. 24275569
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  10. Liu S, Neidhardt EA, Grossman TH, Ocain T, Clardy J: Structures of human dihydroorotate dehydrogenase in complex with antiproliferative agents. Structure. 2000 Jan 15;8(1):25-33. 10673429
  11. Baumgartner R, Walloschek M, Kralik M, Gotschlich A, Tasler S, Mies J, Leban J: Dual binding mode of a novel series of DHODH inhibitors. J Med Chem. 2006 Feb 23;49(4):1239-47. 16480261
  12. Ng SB, Buckingham KJ, Lee C, Bigham AW, Tabor HK, Dent KM, Huff CD, Shannon PT, Jabs EW, Nickerson DA, Shendure J, Bamshad MJ: Exome sequencing identifies the cause of a mendelian disorder. Nat Genet. 2010 Jan;42(1):30-5. doi: 10.1038/ng.499. Epub 2009 Nov 13. 19915526