Names & Taxonomy

Uniprot ID:
Q02127
Entry Name:
PYRD_HUMAN
Status:
reviewed
Protein Names:
Dihydroorotate dehydrogenase (quinone), mitochondrial (DHOdehase) (EC 1.3.5.2) (Dihydroorotate oxidase)
Gene Names:
DHODH
Gene Names Primary:
DHODH
Organism:
Homo sapiens (Human)

Structure

Length:
395
Sequence:
MAWRHLKKRAQDAVIILGGGGLLFASYLMATGDERFYAEHLMPTLQGLLDPESAHRLAVRFTSLGLLPRARFQDSDMLEVRVLGHKFRNPVGIAAGFDKHGEAVDGLYKMGFGFVEIGSVTPKPQEGNPRPRVFRLPEDQAVINRYGFNSHGLSVVEHRLRARQQKQAKLTEDGLPLGVNLGKNKTSVDAAEDYAEGVRVLGPLADYLVVNVSSPNTAGLRSLQGKAELRRLLTKVLQERDGLRRVHRPAVLVKIAPDLTSQDKEDIASVVKELGIDGLIVTNTTVSRPAGLQGALRSETGGLSGKPLRDLSTQTIREMYALTQGRVPIIGVGGVSSGQDALEKIRAGASLVQLYTALTFWGPPVVGKVKRELEALLKEQGFGGVTDAIGADHRR
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion inner membrane

Function

Function:
Catalyzes the conversion of dihydroorotate to orotate with quinone as electron acceptor.
Pathway:
Pyrimidine metabolism; UMP biosynthesis via de novo pathway; orotate from (S)-dihydroorotate (quinone route): step 1/1.
Catalytic Activity:
(S)-dihydroorotate + a quinone = orotate + a quinol.
Cofactor:
COFACTOR: Name=FMN; Xref=ChEBI:CHEBI:58210;
Active Site:
ACT_SITE 214 214 Nucleophile.
Cross Reference Drug Bank:
DB01117 DB01097 DB08880
Gene Ontology Go:
integral component of membrane
mitochondrial inner membrane
mitochondrion
neuronal cell body
nucleoplasm
dihydroorotate dehydrogenase activity
drug binding
FMN binding
ubiquinone binding
'de novo' pyrimidine nucleobase biosynthetic process
'de novo' UMP biosynthetic process
female pregnancy
lactation
nucleobase-containing small molecule metabolic process
positive regulation of apoptotic process
pyrimidine nucleobase metabolic process
pyrimidine nucleoside biosynthetic process
regulation of mitochondrial fission
response to caffeine
response to drug
response to starvation
small molecule metabolic process
Gene Ontology Biological Process:
'de novo' pyrimidine nucleobase biosynthetic process
'de novo' UMP biosynthetic process
female pregnancy
lactation
nucleobase-containing small molecule metabolic process
positive regulation of apoptotic process
pyrimidine nucleobase metabolic process
pyrimidine nucleoside biosynthetic process
regulation of mitochondrial fission
response to caffeine
response to drug
response to starvation
small molecule metabolic process
Gene Ontology Molecular Function:
dihydroorotate dehydrogenase activity
drug binding
FMN binding
ubiquinone binding
Gene Ontology Cellular Component:
integral component of membrane
mitochondrial inner membrane
mitochondrion
neuronal cell body
nucleoplasm
Keywords:
3D-structure
Complete proteome
Disease mutation
FMN
Flavoprotein
Membrane
Mitochondrion
Mitochondrion inner membrane
Oxidoreductase
Polymorphism
Pyrimidine biosynthesis
Reference proteome
Transit peptide
Transmembrane
Transmembrane helix

Publication

PubMed ID:
1446837 7487077 14702039 15489334 8925840 10727948 21269460 24275569 25944712 10673429 16480261 19915526