Dihydroorotate Dehydrogenase (DHODH)

DHOdehase; Dihydroorotate oxidase; Dihydroorotate dehydrogenase (quinone), mitochondrial

SPN	O474
HGNC	2867
MGI	1928378
PubMed	21430780
RGD	68352
UniProt	Q02127
Wiki	DHODH

Dihydroorotate dehydrogenase (EC 1.3.3.1) catalyzes the fourth enzymatic step in de novo pyrimidine biosynthesis. Minet et al. (1992) isolated a truncated human cDNA encoding this enzyme from a HeLa cell cDNA library by functional complementation of a corresponding deletion mutant from the Saccharomyces cerevisiae. The gene in yeast is symbolized ura1. DHO dehydrogenase is a monofunctional protein which, in most eukaryotic organisms, is located on the outer surface of the inner mitochondrial membrane. In yeast, however, it has a cytosolic location.

Missense mutations of the DHODH gene cause Miller syndrome, an autosomal recessive disorder also known as postaxial acrofacial dysostosis.

Reference

"Cloning and sequencing of a human cDNA coding for dihydroorotate dehydrogenase by complementation of the corresponding yeast mutant."
Gene 121:393-396(1992) [PubMed] [Europe PMC] [Abstract]

"Recombinant human dihydroorotate dehydrogenase: expression, purification, and characterization of a catalytically functional truncated enzyme."
Arch. Biochem. Biophys. 323:79-86(1995) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"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. 240:292-301(1996) [PubMed] [Europe PMC] [Abstract]

"Requirements for the mitochondrial import and localization of dihydroorotate dehydrogenase."
Eur. J. Biochem. 267:2079-2087(2000) [PubMed] [Europe PMC] [Abstract]

"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"Structures of human dihydroorotate dehydrogenase in complex with antiproliferative agents."
Structure 8:25-33(2000) [PubMed] [Europe PMC] [Abstract]

"Dual binding mode of a novel series of DHODH inhibitors."
J. Med. Chem. 49:1239-1247(2006) [PubMed] [Europe PMC] [Abstract]

"Exome sequencing identifies the cause of a Mendelian disorder."
Nat. Genet. 42:30-35(2010) [PubMed] [Europe PMC] [Abstract]