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Tetrahydrobiopterin (BH 4, THB), also known as sapropterin (INN), [5] [6] is a cofactor of the three aromatic amino acid hydroxylase enzymes, [7] used in the degradation of amino acid phenylalanine and in the biosynthesis of the neurotransmitters serotonin (5-hydroxytryptamine, 5-HT), melatonin, dopamine, norepinephrine (noradrenaline), epinephrine (adrenaline), and is a cofactor for the ...
BH4 is the principal active cofactor. BH4 synthesis occurs through two principal pathways; the de novo pathway involves three enzymatic steps and proceeds from GTP, while the salvage pathway converts sepiapterin to BH4 using dihydrofolate reductase. [4] In addition, BH2 is recycled to BH4 by dihydrobiopterin reductase.
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Ball-and-stick model of the tetrahydroborate anion, [BH 4] −. Borohydride refers to the anion [B H 4] −, which is also called tetrahydridoborate, and its salts. [1] Borohydride or hydroborate is also the term used for compounds containing [BH 4−n X n] −, where n is an integer from 0 to 3, for example cyanoborohydride or cyanotrihydroborate [BH 3 (CN)] − and triethylborohydride or ...
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This process uses BH4 as a cofactor. During a sleep study ( polysomnography ), decreased twitching may be noticed during REM sleep . An MRI scan of the brain can be used to look for conditions that can mimic dopamine-responsive dystonia (for example, metal deposition in the basal ganglia can indicate Wilson's disease or pantothenate kinase ...
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