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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-07-23 05:43:59 UTC
HMDB IDHMDB0000238
Secondary Accession Numbers
  • HMDB00238
Metabolite Identification
Common NameSepiapterin
DescriptionSepiapterin is an intermediate in the salvage pathway of tetrahydrobiopterin (BH(4)). It is a yellow fluorescing pigment. Sepiapterin accumulates in the brain of patients with sepiapterin reductase (SR) deficiency, which is an inborn error of metabolism.
Structure
Data?1563860639
Synonyms
ValueSource
(S)-2-Amino-7,8-dihydro-6-(2-hydroxy-1-oxopropyl)-4(1H)-pteridinoneHMDB
1-(2-Amino-7,8-dihydro-4-hydroxy-6-pteridinyl)-2-hydroxy-1-propanoneHMDB
2-Amino-6-(S)-lactoyl-7,8-dihydro-4(3H)-pteridinoneHMDB
2-Amino-7,8-dihydro-6-[(2S)-2-hydroxy-1-oxopropyl]-4(1H)pteridinoneHMDB
L-SepiapterinHMDB
Lopac-S-154HMDB
SepiapterineHMDB
Sepiapterin-CHMDB
2-Amino-6-(S)-lactoyl-7,8-dihydro-4(3H)- pteridinoneHMDB
Sepia-pterinHMDB
2-Amino-7,8-dihydro-6-((2S)-2-hydroxy-1-oxopropyl)-4(3H)-pteridinone acidHMDB
Chemical FormulaC9H11N5O3
Average Molecular Weight237.2153
Monoisotopic Molecular Weight237.086189243
IUPAC Name2-amino-6-[(2S)-2-hydroxypropanoyl]-1,4,7,8-tetrahydropteridin-4-one
Traditional Namesepiapterin
CAS Registry Number17094-01-8
SMILES
C[C@H](O)C(=O)C1=NC2=C(NC1)NC(N)=NC2=O
InChI Identifier
InChI=1S/C9H11N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h3,15H,2H2,1H3,(H4,10,11,13,14,17)/t3-/m0/s1
InChI KeyVPVOXUSPXFPWBN-VKHMYHEASA-N
Chemical Taxonomy
Description belongs to the class of organic compounds known as pterins and derivatives. These are polycyclic aromatic compounds containing a pterin moiety, which consist of a pteridine ring bearing a ketone and an amine group to form 2-aminopteridin-4(3H)-one.
KingdomOrganic compounds
Super ClassOrganoheterocyclic compounds
ClassPteridines and derivatives
Sub ClassPterins and derivatives
Direct ParentPterins and derivatives
Alternative Parents
Substituents
  • Pterin
  • Aminopyrimidine
  • Pyrimidone
  • Secondary aliphatic/aromatic amine
  • Acyloin
  • Pyrimidine
  • Alpha-hydroxy ketone
  • Heteroaromatic compound
  • Vinylogous amide
  • Secondary alcohol
  • Ketimine
  • Ketone
  • Propargyl-type 1,3-dipolar organic compound
  • Secondary amine
  • Azacycle
  • Organic 1,3-dipolar compound
  • Alcohol
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Organopnictogen compound
  • Organic oxide
  • Imine
  • Carbonyl group
  • Organic oxygen compound
  • Amine
  • Hydrocarbon derivative
  • Organic nitrogen compound
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic 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 Solubility1.18 g/LALOGPS
logP-1.1ALOGPS
logP-1.4ChemAxon
logS-2.3ALOGPS
pKa (Strongest Acidic)9.18ChemAxon
pKa (Strongest Basic)-1.3ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count8ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area129.17 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity67.5 m³·mol⁻¹ChemAxon
Polarizability22.44 ųChemAxon
Number of Rings2ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-03di-5910000000-428f8e46f66f7bfd3119JSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-000j-6590000000-f5711e35dabd2148a990JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-00ko-0950000000-2cf5caf622d639c89359JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0297-2900000000-f9fe1627a36e71b61db6JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-00di-3900000000-82cabe6461c502831c78JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0079-0090000000-1e9c5af672b8662896afJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-03di-0940000000-1e5701cfd0eed57e783eJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-03k9-2910000000-3e376e485f9ccca285f4JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-000i-0290000000-9eff7a7965f178fa7e5eJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0006-1920000000-a634b611cb12a5f44b6bJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-006x-9200000000-ddd484b0e0f2ed7d6cd7JSpectraViewer | MoNA
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableJSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Cerebrospinal Fluid (CSF)
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified0.0005 (0.0-0.001) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified<0.0005 uMChildren (1-13 years old)MaleNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified0.0083 (0.0056-0.011) uMAdult (>18 years old)BothSepiapterin Reductase Deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified0.01375 (0.0075-0.02) uMChildren (1-13 years old)Malesepiapterin reductase deficiency details
Associated Disorders and Diseases
Disease References
Sepiapterin reductase deficiency
  1. Zorzi G, Redweik U, Trippe H, Penzien JM, Thony B, Blau N: Detection of sepiapterin in CSF of patients with sepiapterin reductase deficiency. Mol Genet Metab. 2002 Feb;75(2):174-7. [PubMed:11855937 ]
  2. Verbeek MM, Willemsen MA, Wevers RA, Lagerwerf AJ, Abeling NG, Blau N, Thony B, Vargiami E, Zafeiriou DI: Two Greek siblings with sepiapterin reductase deficiency. Mol Genet Metab. 2008 Aug;94(4):403-9. doi: 10.1016/j.ymgme.2008.04.003. Epub 2008 May 27. [PubMed:18502672 ]
Associated OMIM IDs
  • 182125 (Sepiapterin reductase deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB021914
KNApSAcK IDNot Available
Chemspider ID58746
KEGG Compound IDC00835
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkSepiapterin
METLIN ID5244
PubChem Compound65253
PDB IDNot Available
ChEBI ID804632
Food Biomarker OntologyNot Available
VMH IDNot Available
References
Synthesis ReferencePfleiderer, Wolfgang. Synthesis and absolute configuration of sepiapterin. Chem. Biol. Pteridines, Proc. Int. Symp., 5th (1975), 941-9.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Zorzi G, Redweik U, Trippe H, Penzien JM, Thony B, Blau N: Detection of sepiapterin in CSF of patients with sepiapterin reductase deficiency. Mol Genet Metab. 2002 Feb;75(2):174-7. [PubMed:11855937 ]

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the final one or two reductions in tetra-hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin.
Gene Name:
SPR
Uniprot ID:
P35270
Molecular weight:
28048.13
Reactions
Dihydrobiopterin + NADP → Sepiapterin + NADPHdetails
General function:
Involved in protein domain specific binding
Specific function:
Adapter protein implicated in the regulation of a large spectrum of both general and specialized signaling pathway. Binds to a large number of partners, usually by recognition of a phosphoserine or phosphothreonine motif. Binding generally results in the modulation of the activity of the binding partner
Gene Name:
YWHAZ
Uniprot ID:
P63104
Molecular weight:
27744.8
General function:
Involved in protein domain specific binding
Specific function:
Adapter protein implicated in the regulation of a large spectrum of both general and specialized signaling pathway. Binds to a large number of partners, usually by recognition of a phosphoserine or phosphothreonine motif. Binding generally results in the modulation of the activity of the binding partner. Negative regulator of osteogenesis
Gene Name:
YWHAB
Uniprot ID:
P31946
Molecular weight:
28082.2
General function:
Involved in protein domain specific binding
Specific function:
Adapter protein implicated in the regulation of a large spectrum of both general and specialized signaling pathway. Binds to a large number of partners, usually by recognition of a phosphoserine or phosphothreonine motif. Binding generally results in the modulation of the activity of the binding partner
Gene Name:
YWHAG
Uniprot ID:
P61981
Molecular weight:
28302.3