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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2013-02-09 00:08:08 UTC
HMDB IDHMDB00238
Secondary Accession NumbersNone
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
  1. (S)-2-amino-7,8-dihydro-6-(2-hydroxy-1-oxopropyl)-4(1H)-Pteridinone
  2. 1-(2-Amino-7,8-dihydro-4-hydroxy-6-pteridinyl)-2-hydroxy-1-Propanone
  3. 2-Amino-6-(S)-lactoyl-7,8-dihydro-4(3H)-pteridinone
  4. 2-Amino-7,8-dihydro-6-[(2S)-2-hydroxy-1-oxopropyl]-4(1H)Pteridinone
  5. L-Sepiapterin
  6. Lopac-S-154
  7. Sepiapterin
  8. Sepiapterine
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
KingdomOrganic Compounds
Super ClassAromatic Heteropolycyclic Compounds
ClassPteridines and Derivatives
Sub ClassPterins and Derivatives
Other Descriptors
  • Pteridines and Derivatives
Substituents
  • Acyloin
  • Alpha Ketoaldehyde
  • Aminopyrimidine
  • Beta Aminoketone
  • Imine
  • Ketone
  • Pyrimidine
  • Pyrimidone
  • Secondary Alcohol
Direct ParentPterins and Derivatives
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Folate biosynthesis
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
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 Solubility1.18ALOGPS
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 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity67.5 m3·mol-1ChemAxon
Polarizability22.44 Å3ChemAxon
Spectra
SpectraMS/MS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Cerebrospinal Fluid (CSF)
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Pterine BiosynthesisSMP00005map00790
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 LinkNot Available
NuGOwiki LinkHMDB00238
Metagene LinkHMDB00238
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