Function: Conversion of dopamine to noradrenaline.
Catalytic activity: 3,4-dihydroxyphenethylamine + ascorbate + O2 = noradrenaline + dehydroascorbate + H2O.
Cofactor: Binds 1 PQQ per subunit.Binds 2 copper ions per subunit.
Pathway: Catecholamine biosynthesis; (R)-noradrenaline biosynthesis; (R)-noradrenaline from dopamine: step 1/1.
Subunit structure: Homotetramer composed of two non-covalently bound disulfide-linked dimers.
Subcellular location: Soluble dopamine beta-hydroxylase: Cytoplasmic vesicle › secretory vesicle lumen. Cytoplasmic vesicle › secretory vesicle › chromaffin granule lumen. Cytoplasmic vesicle › secretory vesicle membrane; Single-pass type II membrane protein. Cytoplasmic vesicle › secretory vesicle › chromaffin granule membrane; Single-pass type II membrane protein
Potential.
Induction: Activity is enhanced by nerve growth factor (in superior cervical ganglia and adrenal medulla). Trans-synaptic stimulation with reserpine, acetylcholine and glucocorticoids.
Polymorphism: There are two main alleles of DBH: DBH-A with Ala-318 and DBH-B with Ser-318.
Involvement in disease: Dopamine 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.Note: The disease is caused by mutations affecting the gene represented in this entry.
Sequence similarities: Belongs to the copper type II ascorbate-dependent monooxygenase family.Contains 1 DOMON domain.
Sequence caution: The sequence AAH17174.1 differs from that shown. Reason: Erroneous initiation. The sequence CAA31631.1 differs from that shown. Reason: Erroneous initiation. The sequence CAA31632.1 differs from that shown. Reason: Erroneous initiation. The sequence CAA68285.1 differs from that shown. Reason: Erroneous initiation.
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