Structure via PubChem · Public domain (PubChem)
sapropterin
Sign in to saveAlso known as 5,6,7,8-Tetrahydrobiopterin, 5,6,7,8 Tetrahydrobiopterin, BH4, (6R)-2-Amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydropteridin-4(1H)-one, 2-Amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydoro-4(1H)-pteridinone, 2-amino-6-(1,2-dihydroxypropyl)-3,4,5,6,7,8-hexahydropteridin-4-one, L-Erythro-2-Amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4(3H)-pteridinon, 5,6,7,8-Erythro-tetrahydrobiopterin
Tetrahydrobiopterin (BH4, THB), also known as sapropterin (INN), is a cofactor of the three aromatic amino acid hydroxylase enzymes, used in the metabolism 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 (dihydropteridine reductase) reduced pteridine derivative (quinonoid dihydrobiopterin).
Key facts
- Drug.ChemSpiderID
- 40270
- Drug.ChemSpiderID2
- 552166
- Drug.Verifiedfields
- changed
- Drug.verifiedrevid
- 470604083
- Drug.INN
- Sapropterin
- Drug.image
- (6R)-Tetrahydrobiopterin structure.svg
- Drug.image_class
- skin-invert-image
- Drug.JAN
- Sapropterin hydrochloride
- Drug.USAN
- Sapropterin dihydrochloride
- Drug.tradename
- Kuvan, Biopten
- Drug.MedlinePlus
- a608020
- Drug.licence_EU
- yes
- Drug.DailyMedID
- Sapropterin
- Drug.licence_US
- Sapropterin
- Drug.pregnancy_AU
- B1
- Drug.routes_of_administration
- By mouth
- Drug.ATC_prefix
- A16
- Drug.ATC_suffix
- AX07
via Wikipedia infobox
Chemical data
- Formula
- C9H15N5O3
- Molecular weight
- 241.25 g/mol
- IUPAC name
- (6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydro-1H-pteridin-4-one
- SMILES
- C[C@@H]([C@@H]([C@H]1CNC2=C(N1)C(=O)N=C(N2)N)O)O
- InChIKey
- FNKQXYHWGSIFBK-RPDRRWSUSA-N
- XLogP
- -1.9
- Polar surface area
- 132 Ų
- H-bond donors
- 6
- H-bond acceptors
- 5
- Formal charge
- 0
via PubChem
Wikidata facts
- Mass
- 241.117489
Show 4 more facts
- chemical formula
- C₉H₁₅N₅O₃
- isomeric SMILES
- C[C@H](O)[C@H](O)[C@H]1CNc2[nH]c(=N)nc(O)c2N1
- canonical SMILES
- N=C1N=C(O)C=2NC(CNC2N1)C(O)C(O)C
- Commons category
- Tetrahydrobiopterin
via Wikidata · CC0
~9 min read
Article
20 sectionsContents
- Medical uses
- Adverse effects
- Interactions
- Functions
- Cofactor for tryptophan hydroxylases
- Cofactor for phenylalanine hydroxylase
- Cofactor for tyrosine hydroxylase
- Cofactor for nitric oxide synthase
- Cofactor for ether lipid oxidase
- History
- Biosynthesis and recycling
- Research
- Depression
- Autism
- Cardiovascular disease
- Neuroprotection in prenatal hypoxia
- Programmed cell death
- References
- Further reading
- External links
Tetrahydrobiopterin (BH4, THB), also known as sapropterin (INN), is a cofactor of the three aromatic amino acid hydroxylase enzymes, used in the metabolism 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 (dihydropteridine reductase) reduced pteridine derivative (quinonoid dihydrobiopterin).
Tetrahydrobiopterin is available as a tablet for oral administration in the form of sapropterin dihydrochloride (BH4*2HCL). It was approved for use in the United States as a tablet in December 2007 and as a powder in December 2013. It was approved for use in the European Union in December 2008, Canada in April 2010, and Japan in July 2008. It is sold under the brand names Kuvan and Biopten. The typical cost of treating a patient with Kuvan is per year. BioMarin holds the patent for Kuvan until at least 2024, but Par Pharmaceutical has a right to produce a generic version by 2020.