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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2021-09-14 15:20:01 UTC
HMDB IDHMDB0000238
Secondary Accession Numbers
  • HMDB00238
Metabolite Identification
Common NameSepiapterin
DescriptionSepiapterin, also known as 2-amino-6-lactoyl-7,8-dihydropteridin-4(3H)-one, 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. Sepiapterin is also classified as a member of the pteridine class of organic chemicals. It is a yellow fluorescing pigment. Sepiapterin is an intermediate in the salvage pathway of tetrahydrobiopterin (BH(4)). More specifically, sepiapterin can be metabolized into tetrahydrobiopterin via the BH(4) salvage pathway. Tetrahydrobiopterin is an essential cofactor in humans for breakdown of phenylalanine and a catalyst of the metabolism of phenylalanine, tyrosine, and tryptophan to the neurotransmitters dopamine and serotonin. A deficiency of tetrahydrobiopterin can cause toxic buildup of phenylalanine (phenylketonuria) as well as deficiencies of dopamine, norepinephrine, and epinephrine, leading to dystonia and other neurological illnesses. Sepiapterin accumulates in the brain of patients with sepiapterin reductase (SR) deficiency, an inborn error of metabolism. Sepiapterin reductase deficiency is a condition characterized by movement problems, most often a pattern of involuntary, sustained muscle contractions known as dystonia. Other movement problems can include muscle stiffness (spasticity), tremors, problems with coordination and balance (ataxia), and involuntary jerking movements (chorea). People with sepiapterin reductase deficiency can experience episodes called oculogyric crises. These episodes involve abnormal rotation of the eyeballs; extreme irritability and agitation; and pain, muscle spasms, and uncontrolled movements, especially of the head and neck. Movement abnormalities are often worse late in the day. Most affected individuals have delayed development of motor skills such as sitting and crawling, and they typically are not able to walk unassisted. The problems with movement tend to worsen over time. Within humans, sepiapterin participates in a number of enzymatic reactions. In particular, sepiapterin can be converted into 7,8-dihydroneopterin; which is mediated by the enzyme sepiapterin reductase. In addition, sepiapterin can be converted into 7,8-dihydroneopterin through its interaction with the enzyme carbonyl reductase [NADPH] 1.
Structure
Data?1582752118
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

Source:

Biological location:

Process

Naturally occurring process:

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Molecular 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
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Spectral Properties

Collision Cross Sections

NameAdductTypeData SourceValueReference
DeepCCS[M+H]+ExperimentalMetCCS_train_pos152.79930932474
AllCCS[M-H]-ExperimentalNot Available148.587http://allccs.zhulab.cn/database/detail?ID=AllCCS00000315
AllCCS[M+H]+ExperimentalNot Available153.025http://allccs.zhulab.cn/database/detail?ID=AllCCS00000315
DarkChem[M+H]+PredictedNot Available155.74231661259
DarkChem[M-H]-PredictedNot Available153.85231661259
AllCCS[M+H]+PredictedNot Available152.17732859911
AllCCS[M-H]-PredictedNot Available152.3132859911

Retention Indices

Underivatized

Not Available

Derivatized

DerivativeValueReference
Sepiapterin,1TMS,#12418.8164https://arxiv.org/abs/1905.12712
Sepiapterin,1TMS,#22469.765https://arxiv.org/abs/1905.12712
Sepiapterin,1TMS,#32439.3237https://arxiv.org/abs/1905.12712
Sepiapterin,1TMS,#42287.7498https://arxiv.org/abs/1905.12712
Sepiapterin,1TMS,#52423.3142https://arxiv.org/abs/1905.12712
Sepiapterin,1TBDMS,#12635.5972https://arxiv.org/abs/1905.12712
Sepiapterin,1TBDMS,#22692.674https://arxiv.org/abs/1905.12712
Sepiapterin,1TBDMS,#32643.8757https://arxiv.org/abs/1905.12712
Sepiapterin,1TBDMS,#42523.7634https://arxiv.org/abs/1905.12712
Sepiapterin,1TBDMS,#52623.1343https://arxiv.org/abs/1905.12712
Spectra

GC-MS

Spectrum TypeDescriptionSplash KeyDeposition DateView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-03di-5910000000-428f8e46f66f7bfd31192017-09-01View Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-000j-6590000000-f5711e35dabd2148a9902017-10-06View Spectrum

LC-MS/MS

Spectrum TypeDescriptionSplash KeyDeposition DateView
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-00ko-0950000000-2cf5caf622d639c893592012-07-24View Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0297-2900000000-f9fe1627a36e71b61db62012-07-24View Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-00di-3900000000-82cabe6461c502831c782012-07-24View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0079-0090000000-1e9c5af672b8662896af2017-09-01View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-03di-0940000000-1e5701cfd0eed57e783e2017-09-01View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-03k9-2910000000-3e376e485f9ccca285f42017-09-01View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-000i-0290000000-9eff7a7965f178fa7e5e2017-09-01View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0006-1920000000-a634b611cb12a5f44b6b2017-09-01View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-006x-9200000000-ddd484b0e0f2ed7d6cd72017-09-01View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-000l-0970000000-ade13f4ae6be4ca0759d2021-09-07View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-01ox-0900000000-1fc5e869af92f5737ea42021-09-07View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0006-9500000000-d928f0c83deb69c4394c2021-09-07View Spectrum

NMR

Spectrum TypeDescriptionDeposition DateView
1D NMR1H NMR Spectrum (1D, 600 MHz, 100%_DMSO, experimental)2012-12-04View Spectrum
1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16View Spectrum
2D NMR[1H, 13C] NMR Spectrum (2D, 600 MHz, 100%_DMSO, predicted)2012-12-05View Spectrum
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
FooDB 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
MarkerDB IDMDB00000116
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