Dihydroorotate oxidase

Dihydroorotate oxidase
EC number CAS number IntEnz BRENDA ExPASy KEGG MetaCyc metabolic pathway
PRIAM PDB structures PDBsum
Gene Ontology EGO
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Dihydroorotate dehydrogenase from E. coli
Symbol DHO_dh
Pfam InterPro PROSITE PDOC00708
SCOP SUPERFAMILY OPM superfamily OPM protein 1uum
Human dihydroorotate dehydrogenase
Symbol DHODH
Entrez HUGO OMIM PDB 1D3G (RefSeq UniProt EC number Locus q22

Dihydroorotate dehydrogenase (orotate:

(S)-dihydroorotate + O2 \rightleftharpoons orotate + H2O2

Human dihydroorotate dehydrogenase is a ubiquitous FMN flavoprotein. In bacteria (gene pyrD), it is located on the inner side of the cytosolic membrane. In some yeasts, such as in Saccharomyces cerevisiae (gene URA1), it is a cytosolic protein, whereas, in other eukaryotes, it is found in the mitochondria.[1]

Human proteins containing this domain


Clinical significance

The anti-inflammatory drug leflunomide has been shown to inhibit DHODH. Human DHODH has two domains: an alpha/beta-barrel domain containing the active site and an alpha-helical domain that forms the opening of a tunnel leading to the active site. Leflunomide has been shown to bind in this tunnel.[2] Leflunomide is being used for treatment of rheumatoid and psoriatic arthritis.

Mutations in this gene have been shown to cause Miller syndrome [3] also known as Genee-Wiedemann syndrome, Wildervanck-Smith syndrome or post axial acrofacial dystosis (POADS).


Further reading

External links

  • Medical Subject Headings (MeSH)

This article incorporates text from the IPR001295

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