Sapropterin [Tetrahydrobiopterin (BH4, THB, trade name: Kuvan) or sapropterin] is a synthetic preparation of the dihydrochloride salt of naturally occurring tetrahydrobiopterin (BH4) that is approved for the treatment of BH4 responsive PKU. It is a phenylalanine hydroxylase activator. Sapropterin is a naturally occurring essential cofactor of the three aromatic amino acid hydroxylase enzymes, 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 production of nitric oxide (NO) by the nitric oxide synthases. Chemically, its structure is that of a reduced pteridine derivative.

纯度：≥98%

CAS：62989-33-7