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MGP000790

UniProt Annotations

Entry Information
Gene Namedopamine beta-hydroxylase (dopamine beta-monooxygenase)
Protein EntryDOPO_HUMAN
UniProt IDP09172
SpeciesHuman
Comments
Comment typeDescription
Catalytic Activity3,4-dihydroxyphenethylamine + ascorbate + O(2) = noradrenaline + dehydroascorbate + H(2)O.
CofactorName=Cu(2+); Xref=ChEBI:CHEBI:29036; Note=Binds 2 copper ions per subunit.;
CofactorName=pyrroloquinoline quinone; Xref=ChEBI:CHEBI:58442; Note=Binds 1 PQQ per subunit.;
DiseaseDopamine beta-hydroxylase deficiency (DBH deficiency) [MIM:223360]: Characterized by profound deficits in autonomic and cardiovascular function, but apparently only subtle signs, if any, of central nervous system dysfunction. {ECO:0000269|PubMed:11857564}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionConversion of dopamine to noradrenaline.
InductionActivity is enhanced by nerve growth factor (in superior cervical ganglia and adrenal medulla). Trans-synaptic stimulation with reserpine, acetylcholine and glucocorticoids.
PathwayCatecholamine biosynthesis; (R)-noradrenaline biosynthesis; (R)-noradrenaline from dopamine: step 1/1.
PolymorphismThere are two main alleles of DBH: DBH-A with Ala- 318 and DBH-B with Ser-318.
Sequence CautionSequence=AAH17174.1; Type=Erroneous initiation; Evidence={ECO:0000305}; Sequence=CAA31631.1; Type=Erroneous initiation; Evidence={ECO:0000305}; Sequence=CAA31632.1; Type=Erroneous initiation; Evidence={ECO:0000305}; Sequence=CAA68285.1; Type=Erroneous initiation; Evidence={ECO:0000305};
SimilarityBelongs to the copper type II ascorbate-dependent monooxygenase family. {ECO:0000305}.
SimilarityContains 1 DOMON domain. {ECO:0000255|PROSITE- ProRule:PRU00246}.
Subcellular LocationCytoplasmic vesicle, secretory vesicle membrane; Single-pass type II membrane protein. Cytoplasmic vesicle, secretory vesicle, chromaffin granule membrane {ECO:0000305}; Single-pass type II membrane protein {ECO:0000305}.
Subcellular LocationSoluble dopamine beta-hydroxylase: Cytoplasmic vesicle, secretory vesicle lumen. Cytoplasmic vesicle, secretory vesicle, chromaffin granule lumen.
SubunitHomotetramer composed of two non-covalently bound disulfide-linked dimers.
Web ResourceName=Wikipedia; Note=Dopamine beta hydroxylase entry; URL="http://en.wikipedia.org/wiki/Dopamine_beta_hydroxylase";
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