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Tetrahydrobiopterin information


Tetrahydrobiopterin
INN: sapropterin
Clinical data
Trade namesKuvan, Biopten
Other namesSapropterin hydrochloride (JAN JP), Sapropterin dihydrochloride (USAN US)
AHFS/Drugs.comMonograph
MedlinePlusa608020
License data
  • EU EMA: by INN
  • US DailyMed: Sapropterin
  • US FDA: Sapropterin
Pregnancy
category
  • AU: B1[1]
Routes of
administration
By mouth
ATC code
  • A16AX07 (WHO)
Legal status
Legal status
  • AU: S4 (Prescription only)
  • CA: ℞-only[2]
  • US: ℞-only[3]
  • EU: Rx-only[4]
  • In general: ℞ (Prescription only)
Pharmacokinetic data
Elimination half-life4 hours (healthy adults)
6–7 hours (PKU patients)
Identifiers
IUPAC name
  • (6R)-2-Amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydropteridin-4(1H)-one
CAS Number
  • 62989-33-7 checkY
  • as salt: 69056-38-8
PubChem CID
  • 135398654
IUPHAR/BPS
  • 5276
DrugBank
  • DB00360 checkY
  • as salt: DBSALT001133
ChemSpider
  • 40270 checkY
  • as salt: 552166
UNII
  • EGX657432I
KEGG
  • D08505
  • as salt: D01798
ChEBI
  • CHEBI:59560 checkY
  • as salt: CHEBI:32120
ChEMBL
  • ChEMBL1201774 ☒N
  • as salt: ChEMBL1201775
PDB ligand
  • H4B (PDBe, RCSB PDB)
CompTox Dashboard (EPA)
  • DTXSID1041138 Edit this at Wikidata
ECHA InfoCard100.164.121 Edit this at Wikidata
Chemical and physical data
FormulaC9H15N5O3
Molar mass241.251 g·mol−1
3D model (JSmol)
  • Interactive image
SMILES
  • CC(C(C1CNC2=C(N1)C(=O)N=C(N2)N)O)O
InChI
  • InChI=1S/C9H15N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h3-4,6,12,15-16H,2H2,1H3,(H4,10,11,13,14,17)/t3-,4+,6-/m0/s1 checkY
  • Key:FNKQXYHWGSIFBK-RPDRRWSUSA-N checkY
 ☒NcheckY (what is this?)  (verify)

Tetrahydrobiopterin (BH4, 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 production of nitric oxide (NO) by the nitric oxide synthases.[8][9] Chemically, its structure is that of a (dihydropteridine reductase) reduced pteridine derivative (quinonoid dihydrobiopterin).[10][citation needed]

  1. ^ "Sapropterin (Kuvan) Use During Pregnancy". Drugs.com. 17 May 2019. Retrieved 4 March 2020.
  2. ^ Cite error: The named reference Kuvan CA Product information was invoked but never defined (see the help page).
  3. ^ Cite error: The named reference Kuvan FDA label was invoked but never defined (see the help page).
  4. ^ Cite error: The named reference Kuvan EPAR was invoked but never defined (see the help page).
  5. ^ "Sapropterin". Drugs.com. 28 February 2020. Retrieved 4 March 2020.
  6. ^ "International Non-proprietary Names for Pharmaceutical Substances (INN)". Fimea. Retrieved 4 March 2020.
  7. ^ Kappock TJ, Caradonna JP (November 1996). "Pterin-Dependent Amino Acid Hydroxylases". Chemical Reviews. 96 (7): 2659–2756. doi:10.1021/CR9402034. PMID 11848840.
  8. ^ Cavaleri et al. Blood concentrations of neopterin and biopterin in subjects with depression: A systematic review and meta-analysis Progress in Neuro-Psychopharmacology and Biological Psychiatry 2023. 120:110633. http://dx.doi.org/10.1016/j.pnpbp.2022.110633
  9. ^ Całka J (2006). "The role of nitric oxide in the hypothalamic control of LHRH and oxytocin release, sexual behavior and aging of the LHRH and oxytocin neurons". Folia Histochemica et Cytobiologica. 44 (1): 3–12. PMID 16584085.
  10. ^ Bhagavan NV (2015). Essentials of Medical Biochemistry With Clinical Cases, 2nd Edition. USA: Elsevier. p. 256. ISBN 978-0-12-416687-5.

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Tetrahydrobiopterin

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Tetrahydrobiopterin (BH4, THB), also known as sapropterin (INN), is a cofactor of the three aromatic amino acid hydroxylase enzymes, used in the degradation...

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Tetrahydrobiopterin deficiency

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Tetrahydrobiopterin deficiency (THBD, BH4D) is a rare metabolic disorder that increases the blood levels of phenylalanine. Phenylalanine is an amino acid...

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Phenylketonuria

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assemblies of phenylalanine. A rarer form of hyperphenylalaninemia is tetrahydrobiopterin deficiency, which occurs when the PAH enzyme is normal, and a defect...

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Tryptophan hydroxylase

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hydroxylases. TPH catalyzes the following chemical reaction L-tryptophan + tetrahydrobiopterin + O2 ⇌ {\displaystyle \rightleftharpoons } 5-Hydroxytryptophan +...

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Pterin

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insertion of molybdate into MPT to form Moco (molybdenum cofactor). Tetrahydrobiopterin, the major unconjugated pterin in vertebrates, is involved in three...

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Phenylalanine hydroxylase

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aromatic amino acid hydroxylases, a class of monooxygenase that uses tetrahydrobiopterin (BH4, a pteridine cofactor) and a non-heme iron for catalysis. During...

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Tyrosine hydroxylase

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does so using molecular oxygen (O2), as well as iron (Fe2+) and tetrahydrobiopterin as cofactors. L-DOPA is a precursor for dopamine, which, in turn...

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Phenylalanine

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(eventually, limited availability) of the associated cofactors, iron or tetrahydrobiopterin. [citation needed] The corresponding enzymes for those compounds...

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QDPR

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the pathway that recycles a substance called tetrahydrobiopterin, also known as BH4. Tetrahydrobiopterin works with an enzyme called phenylalanine hydroxylase...

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Catecholamine

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and tetrahydrobiopterin as cofactors. L-Tyrosine is converted into L-DOPA by another AAAH enzyme (tyrosine 3-hydroxylase) with tetrahydrobiopterin, O2...

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Dopamine

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(O2) and tetrahydrobiopterin as cofactors. L-Tyrosine is converted into L-DOPA by the enzyme tyrosine hydroxylase, with tetrahydrobiopterin, O2, and iron...

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Pteridine reductase

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1.33) is an enzyme that catalyzes the chemical reaction 5,6,7,8-tetrahydrobiopterin + 2 NADP+ ⇌ {\displaystyle \rightleftharpoons } biopterin + 2 NADPH...

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Enzyme

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throughout the structure cause phenylketonuria. Phenylalanine substrate and tetrahydrobiopterin coenzyme in black, and Fe2+ cofactor in yellow. (PDB: 1KW0​)...

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Nitric oxide synthase

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self-sufficient. The electron flow is: NADPH → FAD → FMN → heme → O2. Tetrahydrobiopterin provides an additional electron during the catalytic cycle which...

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Sepiapterin

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chemicals. Sepiapterin can be metabolized into tetrahydrobiopterin via a salvage pathway. Tetrahydrobiopterin is an essential cofactor in humans for breakdown...

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Norepinephrine

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oxygen (O2) and tetrahydrobiopterin as cofactors. Tyrosine is converted into L-DOPA by the enzyme tyrosine hydroxylase, with tetrahydrobiopterin, O2, and probably...

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Vitamin C

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explanation is that vitamin C increases intracellular concentrations of tetrahydrobiopterin, an endothelial nitric oxide synthase cofactor that promotes the...

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Alkylglycerol monooxygenase

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the cofactor tetrahydrobiopterin and iron. The reaction catalyzed by alkylglycerol monooxygenase: 1-alkyl-sn-glycerol + tetrahydrobiopterin + O2 ⇌ {\displaystyle...

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Minoxidil

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to chelate iron, thereby inhibiting PHD. Minoxidil is capable of tetrahydrobiopterin inhibition as a cofactor for nitric oxide synthase. Minoxidil stimulates...

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Sulfasalazine

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Latremoliere A, Woolf CJ (February 2012). "Analgesia by inhibiting tetrahydrobiopterin synthesis". Current Opinion in Pharmacology. 12 (1): 92–99. doi:10...

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Pteridine

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have diverse biological roles. Dihydrobiopterin Pyrazine Pyrimidine Tetrahydrobiopterin International Union of Pure and Applied Chemistry (2014). Nomenclature...

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Dihydrobiopterin

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norepinephrine and epinephrine. It is restored to the required cofactor tetrahydrobiopterin by dihydrobiopterin reductase. Pteridine Tetrahydrobiopterin v t e...

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GTP cyclohydrolase I

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[citation needed] GTPCH (GCH1) and tetrahydrobiopterin were found to protect against cell death by ferroptosis. Tetrahydrobiopterin (BH4) acts as a potent, diffusable...

Word Count : 630

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