Background
Dopamine β-Hydroxylase (DBH) is an enzyme of the copper type II ascorbate-dependent mono-oxygenase family. This enzyme forms homotetramers composed of two noncovalently bound disulfide-linked dimers and is found as both membrane-associated and soluble forms. The soluble form is present in the lumen of secretory granules and is released from cells by exocytosis. DBH converts dopamine to noradrenaline. Deficiency in this enzyme causes a rare disease characterized by a complete absence of noradrenaline and adrenaline in plasma together with increased plasma dopamine levels. Orthostatic hypotension, the main symptom of DBH deficiency, can be alleviated by administration of dihydroxyphenylserine, a synthetic precursor of noradrenaline.
Cellular location
Cytoplasmic vesicle, Secreted
