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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-10-23 17:15:38 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.
Structure
Thumb
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-CMeSH
2-amino-6-(S)-Lactoyl-7,8-dihydro-4(3H)- pteridinoneMeSH
Sepia-pterinMeSH
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
DescriptionThis compound belongs to the class of chemical entities 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.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganoheterocyclic compounds
Sub ClassPteridines and derivatives
Direct ParentPterins and derivatives
Alternative Parents
Substituents
  • Pterin
  • Aminopyrimidine
  • Pyrimidone
  • Secondary aliphatic/aromatic amine
  • Acyloin
  • Primary aromatic amine
  • Pyrimidine
  • Alpha-hydroxy ketone
  • Heteroaromatic compound
  • Vinylogous amide
  • Secondary alcohol
  • Ketimine
  • Ketone
  • Azacycle
  • Organic 1,3-dipolar compound
  • Propargyl-type 1,3-dipolar organic compound
  • Organic nitrogen compound
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Organopnictogen compound
  • Organic oxide
  • Imine
  • Carbonyl group
  • Organic oxygen compound
  • Amine
  • Hydrocarbon derivative
  • Alcohol
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External DescriptorsNot Available
Ontology
Physiological effect

Health effect:

  Health condition:

Disposition

Biological Location:

  Subcellular:

Source:

Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

Role

Indirect biological role:

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)7.93ChemAxon
pKa (Strongest Basic)3.16ChemAxon
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
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-03di-5910000000-428f8e46f66f7bfd3119View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-000j-6590000000-f5711e35dabd2148a990View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-00ko-0950000000-2cf5caf622d639c89359View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0297-2900000000-f9fe1627a36e71b61db6View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-00di-3900000000-82cabe6461c502831c78View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0079-0090000000-1e9c5af672b8662896afView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-03di-0940000000-1e5701cfd0eed57e783eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-03k9-2910000000-3e376e485f9ccca285f4View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-000i-0290000000-9eff7a7965f178fa7e5eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0006-1920000000-a634b611cb12a5f44b6bView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-006x-9200000000-ddd484b0e0f2ed7d6cd7View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Cerebrospinal Fluid (CSF)
Tissue LocationNot Available
Pathways
NameSMPDB/PathwhizKEGG
Dopa-responsive dystoniaPw000462Pw000462 greyscalePw000462 simpleNot Available
Hyperphenylalaniemia due to guanosine triphosphate cyclohydrolase deficiencyPw000463Pw000463 greyscalePw000463 simpleNot Available
Hyperphenylalaninemia due to 6-pyruvoyltetrahydropterin synthase deficiency (ptps)Pw000464Pw000464 greyscalePw000464 simpleNot Available
Hyperphenylalaninemia due to dhpr-deficiencyPw000465Pw000465 greyscalePw000465 simpleNot Available
Pterine BiosynthesisPw000140Pw000140 greyscalePw000140 simpleMap00790
Displaying entries 1 - 5 of 7 in total
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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
BiofluidStatusValueAgeSexConditionReferenceDetails
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 ]
Associated OMIM IDs
  • 182125 (Sepiapterin reductase deficiency)
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB021914
KNApSAcK IDNot Available
Chemspider ID58746
KEGG Compound IDC00835
BioCyc IDCPD-374
BiGG IDNot Available
Wikipedia LinkSepiapterin
METLIN ID5244
PubChem Compound65253
PDB IDNot Available
ChEBI ID804632
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