Dopamine Beta Hydroxylase

From Anvita Health Wiki

Contents 1 Function 2 Structure 3 Compartment 4 Expression 5 Pathology 6 Polymorphism 7 More General Terms 8 Internet Database 9 References

Function

• conversion of dopamine to noradrenaline
• catecholamine biosynthesis

* 3,4-dihydroxyphenethylamine + ascorbate + O2 
*               <-->  
* noradrenaline + dehydroascorbate + H2O

• Cofactor:

• binds 1 PQQ per subunit
• binds 2 copper ions per subunit

Structure

• homotetramer composed of two non- covalently bound disulfide-linked dimers
• belongs to the copper type 2 ascorbate-dependent monooxygenase family
• contains 1 DOMON domain

Compartment

• soluble dopamine beta- hydroxylase: cytoplasmic vesicle, secretory vesicle lumen, chromaffin granule lumen
• secretory vesicle membrane
• chromaffin granule membrane

Expression

• activity is enhanced by nerve growth factor (in superior cervical ganglia & adrenal medulla)
• trans- synaptic stimulation with reserpine, acetylcholine & glucocorticoids

Pathology

• defects in DBH are the cause of dopamine-beta hydroxylase deficiency

Polymorphism

• 2 main alleles of DBH:

• DBH-A with Ala- 318
• DBH-B with Ser-318

More General Terms

• hydroxylase; monooxygenase
• glycoprotein
• membrane protein

Internet Database

UniProt: [1]
Kegg: [2]
OMIM: 223360
Prosite: [3]
Prosite: [4]

References

1. UniProt [5]
2. GeneReviews [6]
3. Wikipedia, dopamine beta hydroxylase entry [7]

dopamine beta-hydroxylase (DBH)