dihydroorotate dehydrogenase, mitochondrial; DHOdehase; dihydroorotate oxidase (DHODH)

Function

• catalyzes the 4th step in the de novo biosynthesis of pyrimidine, the conversion of dihydroorotate into orotate

Cofactor: binds 1 FMN per subunit

Structure

• belongs to the dihydroorotate dehydrogenase family

Compartment

• mitochondrial inner membrane

Pathology

• defects in DHODH are the cause of postaxial acrofacial dysostosis

Pharmacology

• an inhibitor of dihydro-orotate dehydrogenase, leflunomide (also inhibits tyrosine kinases) is used in the treatment of rheumatoid arthritis

More general terms

• dehydrogenase

Additional terms

• leflunomide; teriflunomide (Arava)

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=1723
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:1723
• OMIM: https://mirror.omim.org/entry/126064
• OMIM: https://mirror.omim.org/entry/263750
• UniProt: http://www.uniprot.org/uniprot/Q02127.html