Catecholamine biosynthesis; (R)-noradrenaline biosynthesis; (R)-noradrenaline from dopamine: step 1/1.
Defects in DBH are the cause of dopamine beta-hydroxylase deficiency (DBH deficiency) [MIM:223360]; also known as norepinephrine deficiency or noradrenaline deficiency. This disorder is characterized by profound deficits in autonomic and cardiovascular function, but apparently only subtle signs, if any, of central nervous system dysfunction.
Belongs to the copper type II ascorbate-dependent monooxygenase family. Contains 1 DOMON domain.