62989-33-7
Chemical Structure
Sapropterin
Synonym(s): (6R)-BH4; (6R)-Tetrahydro-L-biopterin
- CAS No.: 62989-33-7
- Formula:C9H15N5O3
- Molecular Weight:241.25
IUPAC Name: (R)-2-amino-6-((1R,2S)-1,2-dihydroxypropyl)-5,6,7,8-tetrahydropteridin-4(1H)-one
InChIKey: FNKQXYHWGSIFBK-RPDRRWSUSA-N
SMILES: O=C1C2=C(NC(N)=N1)NC[C@](N2)([H])[C@@H](O)[C@@H](O)C
Biological Activity: Sapropterin ((6R)-BH4; (6R)-Tetrahydro-L-biopterin) is an orally active phenylalanine hydroxylase (PAH) cofactor, which is effective in reducing blood phenylalanine concentrations. Sapropterin can be used in study of phenylketonuria (PKU) phenylketonuria. Sapropterin can aggravate experimental autoimmune encephalomyelitis (EAE)[1][2].
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Sapropterin | Sapropterin ((6R)-BH4; (6R)-Tetrahydro-L-biopterin) is an orally active phenylalanine hydroxylase (PAH) cofactor, which is effective in reducing blood phenylalanine concentrations. Sapropterin can be used in study of phenylketonuria (PKU) phenylketonuria. Sapropterin can aggravate experimental autoimmune encephalomyelitis (EAE). | |||||||||||||||||||||
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Sapropterin-d3 | Sapropterin-d3 ((6R)-BH4-d3) is deuterium labeled Sapropterin. Sapropterin ((6R)-BH4) is an orally active phenylalanine hydroxylase (PAH) cofactor, which is effective in reducing blood phenylalanine concentrations. Sapropterin can be used in study of phenylketonuria (PKU). | |||||||||||||||||||||
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- [1]. Levy H L, et al. Efficacy of sapropterin dihydrochloride (tetrahydrobiopterin, 6R-BH4) for reduction of phenylalanine concentration in patients with phenylketonuria: a phase III randomised placebo-controlled study[J]. The Lancet, 2007, 370(9586): 504-510. [Content Brief]
- [2]. Schmitz K, et al. Sapropterin (BH4) Aggravates Autoimmune Encephalomyelitis in Mice. Neurotherapeutics. 2021;18(3):1862-1879. [Content Brief]
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