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Dihydroorotate dehydrogenase is an enzyme that in humans is encoded by the DHODH gene on chromosome 16. The protein encoded by this gene catalyzes the fourth enzymatic step, the ubiquinone-mediated oxidation of dihydroorotate to orotate, in de novo pyrimidine biosynthesis. This protein is a mitochondrial protein located on the outer surface of the inner mitochondrial membrane . Inhibitors of this enzyme are used to treat autoimmune diseases such as rheumatoid arthritis.

Dihydroorotate oxidase
Identifiers
EC number 1.3.3.1
CAS number 9029-03-2
Databases
IntEnz IntEnz view
BRENDA BRENDA entry
ExPASy NiceZyme view
KEGG KEGG entry
MetaCyc metabolic pathway
PRIAM profile
PDB structures
Gene Ontology
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Dihydroorotate dehydrogenase from E. coli
Identifiers
Symbol DHO_dh
Pfam PF01180
InterPro IPR001295
PROSITE PDOC00708
SCOP 1dor
SUPERFAMILY 1dor
OPM superfamily 59
OPM protein 1uum
CDD cd02810
Human dihydroorotate dehydrogenase
Identifiers
Symbol DHODH
Entrez 1723
HUGO 2867
OMIM 126064
PDB 1D3G
RefSeq NM_001361
UniProt Q02127
Other data
EC number 1.3.3.1
Locus Chr. 16 q22

Dihydroorotate dehydrogenase (DHODH) is an enzyme that in humans is encoded by the DHODH gene on chromosome 16. The protein encoded by this gene catalyzes the fourth enzymatic step, the ubiquinone-mediated oxidation of dihydroorotate to orotate, in de novo pyrimidine biosynthesis. This protein is a mitochondrial protein located on the outer surface of the inner mitochondrial membrane (IMM). Inhibitors of this enzyme are used to treat autoimmune diseases such as rheumatoid arthritis.

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