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Synonyms: Kuvan® | sapropterin dihydrochloride | SUN-0588 | T-1401 | tetrahydrobiopterin (THB)
sapropterin is an approved drug (FDA (2007), EMA (2008))
Compound class: Metabolite or derivative
Comment: Sapropterin (also known as tetrahydrobiopterin or BH4) is an essential enzymatic cofactor for aromatic amino acid hydroxylase enzymes; phenylalanine hydroxylase (PAH), tryptophan hydroxylase, tyrosine hydroxylase and for nitric oxide synthase. It is used by PAH to degrade phenylalanine, and is required for the biosynthesis of the neurotransmitters serotonin, melatonin, dopamine, norepinephrine (noradrenaline) and epinephrine (adrenaline).
Sapropterin is produced via a pathway of reactions catalysed by the enzymes GTP cyclohydrolase I (GCH1; the primary and rate-limiting reaction), 6-pyruvoyltetrahydropterin synthase (PTPS) and sepiapterin reductase (SR; the terminal reaction).
Ligand Activity Visualisation Charts
These are box plot that provide a unique visualisation, summarising all the activity data for a ligand taken from ChEMBL and GtoPdb across multiple targets and species. Click on a plot to see the median, interquartile range, low and high data points. A value of zero indicates that no data are available. A separate chart is created for each target, and where possible the algorithm tries to merge ChEMBL and GtoPdb targets by matching them on name and UniProt accession, for each available species. However, please note that inconsistency in naming of targets may lead to data for the same target being reported across multiple charts.✖
1. Bélanger-Quintana A, Burlina A, Harding CO, Muntau AC. (2011)
Up to date knowledge on different treatment strategies for phenylketonuria.
Mol Genet Metab, 104 Suppl: S19-25. [PMID:21967857]
2. Cronin SJF, Seehus C, Weidinger A, Talbot S, Reissig S, Seifert M, Pierson Y, McNeill E, Longhi MS, Turnes BL et al.. (2018)
The metabolite BH4 controls T cell proliferation in autoimmunity and cancer.
Nature, 563 (7732): 564-568. [PMID:30405245]
3. Teigen K, Dao KK, McKinney JA, Gorren AC, Mayer B, Frøystein NA, Haavik J, Martínez A. (2004)
Tetrahydrobiopterin binding to aromatic amino acid hydroxylases. Ligand recognition and specificity.
J Med Chem, 47 (24): 5962-71. [PMID:15537351]