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Record Information
Version3.6
Creation Date2006-05-22 14:17:46 UTC
Update Date2016-02-11 01:05:32 UTC
HMDB IDHMDB02281
Secondary Accession NumbersNone
Metabolite Identification
Common Name4a-Hydroxytetrahydrobiopterin
DescriptionTetrahydrobiopterin (BH4) is essential to catalyze the conversion of phenylalanine to tyrosine by phenylalanine hydroxylase. During this physiological reaction, the oxidation of BH4 creates 4a-hydroxytetrahydropterin intermediates and hydrogen peroxide is formed. The hydrogen peroxide and the hydroxytetrahydropterin can both derive from alternate routes of breakdown of a common precursor, the corresponding 4a-hydroperoxytetrahydropterin. (PMID 8323303 ).
Structure
Thumb
Synonyms
ValueSource
(6R)-6-(L-erythro-1,2-Dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxypterinChEBI
4a-Hydroxy-5,6,4,8-tetrahydrobiopterinChEBI
2-amino-6-(1,2-Dihydroxypropyl)-4a-hydroxy-5,6,7,8-tetrahydropteridin-4(4ah)-oneHMDB
2-amino-6-(1,2-Dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxy-4(4ah)-pteridinoneHMDB
2-amino-6-[(1R,2S)-1,2-Dihydroxypropyl]-4a-hydroxy-1,5,6,7-tetrahydropteridin-4-oneHMDB
4a-Hydroxy-5,6,7,8-tetrahydrobiopterinHMDB
4alpha-HydroxytetrahydrobiopterinHMDB
Chemical FormulaC9H15N5O4
Average Molecular Weight257.2465
Monoisotopic Molecular Weight257.112403993
IUPAC Name2-amino-6-(1,2-dihydroxypropyl)-4a-hydroxy-4,4a,5,6,7,8-hexahydropteridin-4-one
Traditional Name4a-hydroxytetrahydrobiopterin
CAS Registry Number70110-58-6
SMILES
CC(O)C(O)C1CNC2=NC(N)=NC(=O)C2(O)N1
InChI Identifier
InChI=1S/C9H15N5O4/c1-3(15)5(16)4-2-11-6-9(18,14-4)7(17)13-8(10)12-6/h3-5,14-16,18H,2H2,1H3,(H3,10,11,12,13,17)
InChI KeyInChIKey=KJKIEFUPAPPGBC-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as biopterins and derivatives. These are coenzymes containing a 2-amino-pteridine-4-one derivative. They are mainly synthesized in several parts of the body, including the pineal gland.
KingdomOrganic compounds
Super ClassOrganoheterocyclic compounds
ClassPteridines and derivatives
Sub ClassPterins and derivatives
Direct ParentBiopterins and derivatives
Alternative Parents
Substituents
  • Biopterin
  • Pyrimidone
  • Aminopyrimidine
  • Imidolactam
  • Pyrimidine
  • Piperazine
  • 5,6-dihydropyrimidine
  • Hydropyrimidine
  • 1,4-diazinane
  • N-acylimine
  • Secondary alcohol
  • Guanidine
  • Carboxamide group
  • 1,2-diol
  • Azacycle
  • Organic 1,3-dipolar compound
  • Propargyl-type 1,3-dipolar organic compound
  • Carboximidamide
  • Secondary amine
  • Carboxylic acid derivative
  • Carboxylic acid amidine
  • Amidine
  • Alkanolamine
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Alcohol
  • Aliphatic heteropolycyclic compound
Molecular FrameworkAliphatic heteropolycyclic compounds
External Descriptors
Ontology
StatusExpected but not Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locationsNot Available
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility4.81 mg/mLALOGPS
logP-2.3ALOGPS
logP-3.1ChemAxon
logS-1.7ALOGPS
pKa (Strongest Acidic)9.81ChemAxon
pKa (Strongest Basic)3.85ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count9ChemAxon
Hydrogen Donor Count6ChemAxon
Polar Surface Area152.56 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity58.6 m3·mol-1ChemAxon
Polarizability24.2 Å3ChemAxon
Number of Rings2ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular LocationsNot Available
Biofluid LocationsNot Available
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
AlkaptonuriaSMP00169Not Available
Disulfiram PathwaySMP00429Not Available
Dopamine beta-hydroxylase deficiencySMP00498Not Available
HawkinsinuriaSMP00190Not Available
Monoamine oxidase-a deficiency (MAO-A)SMP00533Not Available
Phenylalanine and Tyrosine MetabolismSMP00008map00360
PhenylketonuriaSMP00206Not Available
Tryptophan MetabolismSMP00063map00380
Tyrosine MetabolismSMP00006map00350
Tyrosinemia Type 2 (or Richner-Hanhart syndrome)SMP00369Not Available
Tyrosinemia Type 3 (TYRO3)SMP00370Not Available
Tyrosinemia Type ISMP00218Not Available
Tyrosinemia, transient, of the newbornSMP00494Not Available
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022944
KNApSAcK IDNot Available
Chemspider ID114908
KEGG Compound IDC15522
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB02281
Metagene LinkHMDB02281
METLIN ID6591
PubChem Compound129803
PDB IDNot Available
ChEBI ID15374
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Rebrin I, Thony B, Bailey SW, Ayling JE: Stereospecificity and catalytic function of histidine residues in 4a-hydroxy-tetrahydropterin dehydratase/DCoH. Biochemistry. 1998 Aug 11;37(32):11246-54. [9698371 ]
  2. Almas B, Haavik J, Flatmark T: Characterization of a novel pterin intermediate formed in the catalytic cycle of tyrosine hydroxylase. Biochem J. 1996 Nov 1;319 ( Pt 3):947-51. [8921004 ]
  3. Davis MD, Kaufman S: Products of the tyrosine-dependent oxidation of tetrahydrobiopterin by rat liver phenylalanine hydroxylase. Arch Biochem Biophys. 1993 Jul;304(1):9-16. [8323303 ]
  4. Rebrin I, Bailey SW, Boerth SR, Ardell MD, Ayling JE: Catalytic characterization of 4a-hydroxytetrahydropterin dehydratase. Biochemistry. 1995 May 2;34(17):5801-10. [7727440 ]

Enzymes

General function:
Involved in monooxygenase activity
Specific function:
Plays an important role in the physiology of adrenergic neurons.
Gene Name:
TH
Uniprot ID:
P07101
Molecular weight:
55611.26
Reactions
L-Tyrosine + L-erythro-tetrahydrobiopterin + Oxygen → L-Dopa + 4a-Hydroxytetrahydrobiopterindetails
General function:
Involved in amino acid binding
Specific function:
Not Available
Gene Name:
PAH
Uniprot ID:
P00439
Molecular weight:
51861.565
Reactions
L-Phenylalanine + L-erythro-tetrahydrobiopterin + Oxygen → L-Tyrosine + 4a-Hydroxytetrahydrobiopterindetails
General function:
Involved in amino acid binding
Specific function:
Not Available
Gene Name:
TPH1
Uniprot ID:
P17752
Molecular weight:
50984.725
Reactions
L-Tryptophan + L-erythro-tetrahydrobiopterin + Oxygen → Oxitriptan + 4a-Hydroxytetrahydrobiopterindetails
General function:
Involved in amino acid binding
Specific function:
Not Available
Gene Name:
TPH2
Uniprot ID:
Q8IWU9
Molecular weight:
56056.295
Reactions
L-Tryptophan + L-erythro-tetrahydrobiopterin + Oxygen → Oxitriptan + 4a-Hydroxytetrahydrobiopterindetails
General function:
Involved in 4-alpha-hydroxytetrahydrobiopterin dehydratase activity
Specific function:
Involved in tetrahydrobiopterin biosynthesis. Seems to both prevent the formation of 7-pterins and accelerate the formation of quinonoid-BH2. Coactivator for HNF1A-dependent transcription. Regulates the dimerization of homeodomain protein HNF1A and enhances its transcriptional activity.
Gene Name:
PCBD1
Uniprot ID:
P61457
Molecular weight:
11999.515
Reactions
4a-Hydroxytetrahydrobiopterin → 4a-Carbinolamine tetrahydrobiopterin + Waterdetails
General function:
Involved in 4-alpha-hydroxytetrahydrobiopterin dehydratase activity
Specific function:
Involved in tetrahydrobiopterin biosynthesis. Seems to both prevent the formation of 7-pterins and accelerate the formation of quinonoid-BH2 (By similarity). Regulates the dimerization of homeodomain protein HNF-1-alpha and enhances its transcriptional activity.
Gene Name:
PCBD2
Uniprot ID:
Q9H0N5
Molecular weight:
14365.325
Reactions
4a-Hydroxytetrahydrobiopterin → 4a-Carbinolamine tetrahydrobiopterin + Waterdetails