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Record Information
Created at2005-11-16 15:48:42 UTC
Updated at2021-07-01 14:27:31 UTC
NP-MRD IDNP0000204
Secondary Accession NumbersNone
Natural Product 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.
2-Amino-6-(S)-lactoyl-7,8-dihydro-4(3H)- pteridinoneHMDB
2-Amino-7,8-dihydro-6-((2S)-2-hydroxy-1-oxopropyl)-4(3H)-pteridinone acidHMDB
Chemical FormulaC9H11N5O3
Average Mass237.2153 Da
Monoisotopic Mass237.08619 Da
IUPAC Name2-amino-6-[(2S)-2-hydroxypropanoyl]-1,4,7,8-tetrahydropteridin-4-one
Traditional Namesepiapterin
CAS Registry Number17094-01-8
InChI Identifier
Experimental Spectra
Spectrum TypeDescriptionDepositor EmailDepositor OrganizationDepositorDeposition DateView
1D NMR1H NMR Spectrum (1D, 600 MHz, DMSO, simulated)V.dorna832021-09-07View Spectrum
1D NMR1H NMR Spectrum (1D, 600 MHz, DMSO, experimental)V.dorna832021-09-07View Spectrum
2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, 100%_DMSO, experimental)Wishart LabWishart LabDavid Wishart2021-06-20View Spectrum
Predicted Spectra
Not Available
Chemical Shift Submissions
Spectrum TypeDescriptionDepositor EmailDepositor OrganizationDepositorDeposition DateView
1D NMR1H NMR Spectrum (1D, 600 MHz, DMSO, simulated)v.dorna83@yahoo.comNot AvailableNot Available2021-08-12View Spectrum
Species of Origin
Species NameSourceReference
Anas platyrhynchosFooDB
Anser anserFooDB
Bison bisonFooDB
Bombyx moriLOTUS Database
Bos taurusFooDB
Bos taurus X Bison bisonFooDB
Bubalus bubalisFooDB
Capra aegagrus hircusFooDB
Cervus canadensisFooDB
Dromaius novaehollandiaeFooDB
Equus caballusFooDB
Gallus gallusFooDB
Lagopus mutaFooDB
Lepus timidusFooDB
Melanitta fuscaFooDB
Meleagris gallopavoFooDB
Numida meleagrisFooDB
Ovis ariesFooDB
Phasianus colchicusFooDB
Struthio camelusFooDB
Sus scrofaFooDB
Sus scrofa domesticaFooDB
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
  • 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
Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility1.18 g/LALOGPS
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
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
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
PubChem Compound65253
PDB IDNot Available
ChEBI ID804632
Good Scents IDNot Available
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 ]
  2. Keller M, Brennenstuhl H, Kuseyri Hubschmann O, Manti F, Julia Palacios NA, Friedman J, Yildiz Y, Koht JA, Wong SN, Zafeiriou DI, Lopez-Laso E, Pons R, Kulhanek J, Jeltsch K, Serrano-Lomelin J, Garbade SF, Opladen T, Goez H, Burlina A, Cortes-Saladelafont E, Fernandez Ramos JA, Garcia-Cazorla A, Hoffmann GF, Kiat Hong ST, Honzik T, Kavecan I, Kurian MA, Leuzzi V, Lucke T, Manzoni F, Mastrangelo M, Mercimek-Andrews S, Mir P, Oppeboen M, Pearson TS, Sivri HS, Steel D, Stevanovic G, Fung CW: Assessment of intellectual impairment, health-related quality of life, and behavioral phenotype in patients with neurotransmitter related disorders: Data from the iNTD registry. J Inherit Metab Dis. 2021 Jul 9. doi: 10.1002/jimd.12416. [PubMed:34245036 ]
  3. Abell TL, Garcia LM, Wiener GJ, Wo JM, Bulat RS, Smith N: Effect of Oral CNSA-001 (sepiapterin, PTC923) on gastric accommodation in women with diabetic gastroparesis: A randomized, placebo-controlled, Phase 2 trial. J Diabetes Complications. 2021 Sep;35(9):107961. doi: 10.1016/j.jdiacomp.2021.107961. Epub 2021 Jun 17. [PubMed:34176722 ]
  4. Bozaci AE, Er E, Yazici H, Canda E, Kalkan Ucar S, Guvenc Saka M, Eraslan C, Onay H, Habif S, Thony B, Coker M: Tetrahydrobiopterin deficiencies: Lesson from clinical experience. JIMD Rep. 2021 Feb 1;59(1):42-51. doi: 10.1002/jmd2.12199. eCollection 2021 May. [PubMed:33977029 ]
  5. Obeng S, Hiranita T, Leon F, McMahon LR, McCurdy CR: Novel Approaches, Drug Candidates, and Targets in Pain Drug Discovery. J Med Chem. 2021 May 27;64(10):6523-6548. doi: 10.1021/acs.jmedchem.1c00028. Epub 2021 May 6. [PubMed:33956427 ]
  6. Furukawa Y, Tomizawa Y, Nakahara T: Neurometabolic causes of dystonia: Sepiapterin reductase-deficient dopamine- and serotonin-responsive dystonia-plus syndrome. J Neurol Sci. 2021 Jun 15;425:117468. doi: 10.1016/j.jns.2021.117468. Epub 2021 Apr 21. [PubMed:33926741 ]
  7. Himmelreich N, Blau N, Thony B: Molecular and metabolic bases of tetrahydrobiopterin (BH4) deficiencies. Mol Genet Metab. 2021 Jun;133(2):123-136. doi: 10.1016/j.ymgme.2021.04.003. Epub 2021 Apr 19. [PubMed:33903016 ]
  8. Phua CS, Kumar KR, Levy S: Reply to: Neurometabolic causes of dystonia: Sepiapterin reductase-deficient dopamine- and serotonin-responsive dystonia-plus syndrome. J Neurol Sci. 2021 Jun 15;425:117469. doi: 10.1016/j.jns.2021.117469. Epub 2021 Apr 21. [PubMed:33902913 ]
  9. Rabender CS, Mezzaroma E, Yakovlev VA, Mauro AG, Bonaventura A, Abbate A, Mikkelsen RB: Mitigation of Radiation-Induced Lung and Heart Injuries in Mice by Oral Sepiapterin after Irradiation. Radiat Res. 2021 May 1;195(5):463-473. doi: 10.1667/RADE-20-00249.1. [PubMed:33822229 ]
  10. Ranjpour M, Wajid S, Jain SK: Elevated expression of sepiapterin reductase, regulator of G protein signaling 1, hypothetical protein CXorf58 homolog, and zinc finger and BTB domain-containing protein 21 isoform X2 is associated with progression of hepatocellular carcinoma. Protoplasma. 2021 Mar 8. pii: 10.1007/s00709-021-01632-2. doi: 10.1007/s00709-021-01632-2. [PubMed:33683453 ]
  11. Lindsay A, Kemp B, Larson AA, Baumann CW, McCourt PM, Holm J, Karachunski P, Lowe DA, Ervasti JM: Tetrahydrobiopterin synthesis and metabolism is impaired in dystrophin-deficient mdx mice and humans. Acta Physiol (Oxf). 2021 Apr;231(4):e13627. doi: 10.1111/apha.13627. Epub 2021 Mar 8. [PubMed:33580591 ]
  12. Cherian A, Paramasivan NK, Divya KP: Dopa-responsive dystonia, DRD-plus and DRD look-alike: a pragmatic review. Acta Neurol Belg. 2021 Jun;121(3):613-623. doi: 10.1007/s13760-020-01574-1. Epub 2021 Jan 16. [PubMed:33453040 ]
  13. Zhang X, Chen Y, Wang K, Tang J, Chen Y, Jin G, Liu X: The knockdown of the sepiapterin reductase gene suppresses the proliferation of breast cancer by inducing ROS-mediated apoptosis. Int J Clin Exp Pathol. 2020 Sep 1;13(9):2228-2239. eCollection 2020. [PubMed:33042327 ]
  14. Wu Y, Chen P, Sun L, Yuan S, Cheng Z, Lu L, Du H, Zhan M: Sepiapterin reductase: Characteristics and role in diseases. J Cell Mol Med. 2020 Sep;24(17):9495-9506. doi: 10.1111/jcmm.15608. Epub 2020 Jul 30. [PubMed:32734666 ]
  15. Oates JC, Ramakrishnan V, Nietert PJ, Spence JD, Fleury TW, Markiewicz M, Russell DL, Lopes-Virella MF: ASSOCIATIONS BETWEEN ACCELERATED ATHEROSCLEROSIS, OXIDIZED LDL IMMUNE COMPLEXES, AND IN VITRO ENDOTHELIAL DYSFUNCTION IN SYSTEMIC LUPUS ERYTHEMATOSUS. Trans Am Clin Climatol Assoc. 2020;131:157-177. [PubMed:32675856 ]
  16. Soares AG, Muscara MN, Costa SKP: Molecular mechanism and health effects of 1,2-Naphtoquinone. EXCLI J. 2020 Jun 3;19:707-717. doi: 10.17179/excli2020-1210. eCollection 2020. [PubMed:32636724 ]
  17. Mainka T, Hoffmann J, Kuhn AA, Biskup S, Ganos C: Teaching Video NeuroImages: Paroxysmal hyperkinesia with diurnal fluctuations due to sepiapterin-reductase deficiency. Neurology. 2020 Jul 21;95(3):e332-e334. doi: 10.1212/WNL.0000000000009901. Epub 2020 Jun 26. [PubMed:32591469 ]