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Details on Person UniProt:Q02127 DHODH

Class:IdReferenceGeneProduct:62547
_chainChangeLogchain:1-395 added on Fri February 6 2015;transit peptide:1-10 added on Fri February 6 2015
_displayNameUniProt:Q02127 DHODH
_timestamp2025-02-21 19:42:11
chainchain:1-395
transit peptide:1-10
checksum072C3169E78C6440
commentFUNCTION Catalyzes the conversion of dihydroorotate to orotate with quinone as electron acceptor. Required for UMP biosynthesis via de novo pathway.CATALYTIC ACTIVITY (S)-dihydroorotate + a quinone = orotate + a quinolCOFACTOR Binds 1 FMN per subunit.PATHWAY Pyrimidine metabolism; UMP biosynthesis via de novo pathway; orotate from (S)-dihydroorotate (quinone route): step 1/1.SUBUNIT Monomer.INTERACTION The uncleaved transit peptide is required for mitochondrial targeting and proper membrane integration.DISEASE The disease is caused by variants affecting the gene represented in this entry.MISCELLANEOUS The identification of DHODH defects as the cause of postaxial acrofacial dysostosis (POADS) was obtained via exome sequencing (PubMed:19915526), demonstrating that this method is a powerful tool for identifying genes underlying rare Mendelian disorders. Exome sequencing consists of targeted resequencing of all protein-coding subsequences, which requires around 5% as much sequencing as a whole human genome.SIMILARITY Belongs to the dihydroorotate dehydrogenase family. Type 2 subfamily.
descriptionrecommendedName: Dihydroorotate dehydrogenase (quinone), mitochondrial shortName: DHOdehase ecNumber evidence="8"1.3.5.2 alternativeName: Dihydroorotate oxidase
geneNameDHODH
identifierQ02127
isSequenceChangedFALSE
keyword3D-structure
Disease variant
Flavoprotein
FMN
Membrane
Mitochondrion
Mitochondrion inner membrane
Oxidoreductase
Proteomics identification
Pyrimidine biosynthesis
Reference proteome
Transit peptide
Transmembrane
Transmembrane helix
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameDHODH
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8995073] ENSEMBL:ENSG00000102967 DHODH [Homo sapiens]
secondaryIdentifierPYRD_HUMAN
A8K8C8
Q6P176
sequenceLength395
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:73523] DHODH [mitochondrial inner membrane] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:Q02127 DHODH (62547)