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Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2006-05-22 14:17:46 UTC
Update Date2019-07-23 05:45:26 UTC
HMDB IDHMDB0002281
Secondary Accession Numbers
  • HMDB0012482
  • HMDB02281
  • HMDB12482
Metabolite Identification
Common Name4a-Hydroxytetrahydrobiopterin
DescriptionTetrahydrobiopterin (BH4) is essential for catalyzing the conversion of phenylalanine into tyrosine by phenylalanine hydroxylase. During this physiological reaction, the oxidation of BH4 creates 4a-hydroxytetrahydropterin (CAS: 70110-58-6) intermediates and hydrogen peroxide is formed. The hydrogen peroxide and the hydroxytetrahydropterin can both be derived from alternate breakdown routes of a common precursor, the corresponding 4a-hydroperoxytetrahydropterin (PMID: 8323303 ).
Structure
Data?1563860726
Synonyms
ValueSource
(4AS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinChEBI
(6R)-6-(L-Erythro-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxypterinChEBI
4a-Hydroxy-5,6,4,8-tetrahydrobiopterinKegg
4a-HTHBHMDB
4a-Hydroxy-5,6,7,8-tetrahydrobiopterinHMDB
4a-HydroxytetrahydrobiopterinHMDB
4a-Hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinHMDB
Chemical FormulaC9H15N5O4
Average Molecular Weight257.25
Monoisotopic Molecular Weight257.112403983
IUPAC Name(4aS,6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-4a-hydroxy-4,4a,5,6,7,8-hexahydropteridin-4-one
Traditional Name(4aS,6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-4a-hydroxy-5,6,7,8-tetrahydropteridin-4-one
CAS Registry Number1379003-93-6
SMILES
[H][C@@]1(CNC2=NC(N)=NC(=O)[C@]2(O)N1)[C@@H](O)[C@H](C)O
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)/t3-,4+,5-,9-/m0/s1
InChI KeyKJKIEFUPAPPGBC-XXKOCQOQSA-N
Chemical Taxonomy
Description 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
  • Alpha-amino acid or derivatives
  • Pyrimidone
  • Imidolactam
  • Hydropyrimidine
  • 1,4,5,6-tetrahydropyrimidine
  • Pyrimidine
  • 1,2-diol
  • Amino acid or derivatives
  • Guanidine
  • N-acylimine
  • Secondary alcohol
  • Alkanolamine
  • Amidine
  • Carboxylic acid amidine
  • Carboxylic acid derivative
  • Azacycle
  • Organic 1,3-dipolar compound
  • Propargyl-type 1,3-dipolar organic compound
  • Carboximidamide
  • Secondary amine
  • Carbonyl group
  • Organic nitrogen compound
  • Organic oxygen compound
  • Alcohol
  • Amine
  • Organonitrogen compound
  • Organooxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Aliphatic heteropolycyclic compound
Molecular FrameworkAliphatic heteropolycyclic compounds
External DescriptorsNot Available
Ontology
Disposition

Biological location:

Source:

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 g/LALOGPS
logP-2.3ALOGPS
logP-3.1ChemAxon
logS-1.7ALOGPS
pKa (Strongest Acidic)9.81ChemAxon
pKa (Strongest Basic)3.1ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count9ChemAxon
Hydrogen Donor Count6ChemAxon
Polar Surface Area152.56 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity58.6 m³·mol⁻¹ChemAxon
Polarizability23.97 ųChemAxon
Number of Rings2ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0a4l-0090000000-364ec2c50acd77d83ef0JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00gl-0690000000-f15f00b5ba6b8f2959b9JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0005-9720000000-9884aa4c6eb7899b87e9JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0bt9-2290000000-43723819afb4c98b5cebJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0006-9210000000-fa8eb33fdfed731f1c82JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0006-9100000000-c550affb7745a16d59b4JSpectraViewer | MoNA
Biological Properties
Cellular LocationsNot Available
Biospecimen LocationsNot Available
Tissue LocationsNot Available
Pathways
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022944
KNApSAcK IDNot Available
Chemspider ID30792101
KEGG Compound IDC15522
BioCyc IDCPD-5881
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID6591
PubChem Compound135476770
PDB IDNot Available
ChEBI ID15642
Food Biomarker OntologyNot Available
VMH IDNot Available
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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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 + Sapropterin + 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 + Sapropterin + 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 + Sapropterin + Oxygen → 5-Hydroxy-L-tryptophan + 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 + Sapropterin + Oxygen → 5-Hydroxy-L-tryptophan + 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