| Record Information |
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| Version | 1.0 |
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| Created at | 2005-11-16 15:48:42 UTC |
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| Updated at | 2021-08-09 22:33:05 UTC |
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| NP-MRD ID | NP0001044 |
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| Secondary Accession Numbers | None |
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| Natural Product Identification |
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| Common Name | Biopterin |
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| Description | Biopterin, also known as tetrahydrobiopterin or BH4, belongs to the class of organic compounds known as biopterins and derivatives. These are coenzymes containing a 2-amino-pteridine-4-one derivative. Biopterin or tetrahydrobiopterin is also classified as a pterin derivative that consists of pterin group bearing an amino, an oxo and a 1,2-dihydroxypropyl substituent at positions 2, 4 and 6, respectively. Biopterin compounds found within the animals include BH4 (tetrahydrobiopterin), the free radical BH3, and BH2 (also a free radical, called Dihydrobiopterin). BH2 is produced in the synthesis of L-DOPA, dopamine, norepinephrine and epinephrine. It is restored to the required cofactor tetrahydrobiopterin by the enzyme dihydrobiopterin reductase. Tetrahydrobiopterin (BH4) is a cofactor of the three aromatic amino acid hydroxylase enzymes, used in the degradation of amino acid phenylalanine and in the biosynthesis of the neurotransmitters serotonin (5-hydroxytryptamine, 5-HT), melatonin, dopamine, norepinephrine (noradrenaline), epinephrine (adrenaline). It is also a cofactor for the production of nitric oxide (NO) by the nitric oxide syntheses. Tetrahydrobiopterin is biosynthesized from guanosine triphosphate (GTP) by three chemical reactions mediated by the enzymes GTP cyclohydrolase I (GTPCH), 6-pyruvoyltetrahydropterin synthase (PTPS), and sepiapterin reductase (SR). Biopterin synthesis disorders are a cause of hyperphenylalaninemia. There are 3 distinct forms of phenylketonuria or hyperphenylalaninemia, each caused by lack of aromatic amino acid hydroxylase enzymes. The variant forms of hyperphenylalaninemia that are caused by the lack of dihydropteridine reductase or tetrahydrobiopterin are characterized by severe neurological deterioration, impaired functioning of tyrosine and tryptophan hydroxylases, and the resultant deficiency of tyrosine- and tryptophan-derived monoamine neurotransmitters in brain. (PMID 3930837 ). |
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| Structure | C[C@H](O)[C@H](O)C1=CNC2=NC(N)=NC(=O)C2=N1 InChI=1S/C9H11N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h2-3,6,15-16H,1H3,(H3,10,11,13,14,17)/t3-,6-/m0/s1 |
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| Synonyms | | Value | Source |
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| (-)-Biopterin | ChEBI | | (1'r,1's) Biopterin | HMDB | | 2-Amino-6-(L-erythro-1,2-dihydroxypropyl)-4(3H)-pteridinone | HMDB | | 6-Biopterin | HMDB | | L-Biopterin | HMDB | | L-Erythro-biopterin | HMDB | | Pterin H b2 | HMDB | | [S-(R*,s*)]-2-amino-6-(1,2-dihydroxypropyl)-4(1H)-pteridinone | HMDB | | Orinapterin | HMDB | | Dictyopterin | HMDB | | 2-Amino-6-(1,2-dihydroxypropyl)-4(1H)-pteridinone | HMDB | | Biopterin | MeSH |
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| Chemical Formula | C9H11N5O3 |
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| Average Mass | 237.2153 Da |
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| Monoisotopic Mass | 237.08619 Da |
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| IUPAC Name | 2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-4,8-dihydropteridin-4-one |
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| Traditional Name | 2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-8H-pteridin-4-one |
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| CAS Registry Number | 22150-76-1 |
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| SMILES | C[C@H](O)[C@H](O)C1=CNC2=NC(N)=NC(=O)C2=N1 |
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| InChI Identifier | InChI=1S/C9H11N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h2-3,6,15-16H,1H3,(H3,10,11,13,14,17)/t3-,6-/m0/s1 |
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| InChI Key | LHQIJBMDNUYRAM-DZSWIPIPSA-N |
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| Experimental Spectra |
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| | Spectrum Type | Description | Depositor Email | Depositor Organization | Depositor | Deposition Date | View |
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| 1D NMR | 1H NMR Spectrum (1D, 600 MHz, DMSO, simulated) | V.dorna83 | | | 2021-08-22 | View Spectrum | | 1D NMR | 1H NMR Spectrum (1D, 600 MHz, DMSO, experimental) | V.dorna83 | | | 2021-08-22 | View Spectrum | | 2D NMR | [1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, 100%_DMSO, experimental) | Wishart Lab | Wishart Lab | David Wishart | 2021-06-20 | View Spectrum |
| | Predicted Spectra |
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| Not Available | | Chemical Shift Submissions |
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| | Spectrum Type | Description | Depositor ID | Depositor Organization | Depositor | Deposition Date | View |
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| 1D NMR | 1H NMR Spectrum (1D, 600 MHz, DMSO, simulated) | V.dorna83 | | | 2021-08-16 | View Spectrum |
| | Species |
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| Species of Origin | |
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| Species Where Detected | |
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| Chemical Taxonomy |
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| Description | Belongs to the class of organic compounds known as biopterins and derivatives. These are coenzymes containing a 2-amino-pteridine-4-one derivative. They are mainly synthesized in several parts of the body, including the pineal gland. |
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| Kingdom | Organic compounds |
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| Super Class | Organoheterocyclic compounds |
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| Class | Pteridines and derivatives |
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| Sub Class | Pterins and derivatives |
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| Direct Parent | Biopterins and derivatives |
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| Alternative Parents | |
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| Substituents | - Biopterin
- Aminopyrimidine
- Pyrimidone
- Pyrazine
- Pyrimidine
- Heteroaromatic compound
- Vinylogous amide
- 1,2-diol
- Secondary alcohol
- Azacycle
- Organic oxygen compound
- Alcohol
- Aromatic alcohol
- Hydrocarbon derivative
- Primary amine
- Organooxygen compound
- Organonitrogen compound
- Organic nitrogen compound
- Organic oxide
- Amine
- Organopnictogen compound
- Aromatic heteropolycyclic compound
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| Molecular Framework | Aromatic heteropolycyclic compounds |
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| External Descriptors | |
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| Physical Properties |
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| State | Solid |
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| Experimental Properties | | Property | Value | Reference |
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| Melting Point | Not Available | Not Available | | Boiling Point | Not Available | Not Available | | Water Solubility | 0.7 mg/mL | Not Available | | LogP | Not Available | Not Available |
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| Predicted Properties | |
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| General References | - Thony B, Leimbacher W, Blau N, Harvie A, Heizmann CW: Hyperphenylalaninemia due to defects in tetrahydrobiopterin metabolism: molecular characterization of mutations in 6-pyruvoyl-tetrahydropterin synthase. Am J Hum Genet. 1994 May;54(5):782-92. [PubMed:8178819 ]
- Bonafe L, Thony B, Penzien JM, Czarnecki B, Blau N: Mutations in the sepiapterin reductase gene cause a novel tetrahydrobiopterin-dependent monoamine-neurotransmitter deficiency without hyperphenylalaninemia. Am J Hum Genet. 2001 Aug;69(2):269-77. Epub 2001 Jul 6. [PubMed:11443547 ]
- Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
- Fiege B, Ballhausen D, Kierat L, Leimbacher W, Goriounov D, Schircks B, Thony B, Blau N: Plasma tetrahydrobiopterin and its pharmacokinetic following oral administration. Mol Genet Metab. 2004 Jan;81(1):45-51. [PubMed:14728990 ]
- Friedland RP, Koss E, Haxby JV, Grady CL, Luxenberg J, Schapiro MB, Kaye J: NIH conference. Alzheimer disease: clinical and biological heterogeneity. Ann Intern Med. 1988 Aug 15;109(4):298-311. [PubMed:2969203 ]
- Snyderman SE, Sansaricq C, Pulmones MT: Successful long term therapy of biopterin deficiency. J Inherit Metab Dis. 1987;10(3):260-6. [PubMed:3123784 ]
- Nagatsu T, Yamaguchi T, Kato T, Sugimoto T, Matsuura S, Akino M, Nagatsu I, Iizuka R, Narabayashi H: Biopterin in human brain and urine from controls and parkinsonian patients: application of a new radioimmunoassay. Clin Chim Acta. 1981 Feb 5;109(3):305-11. [PubMed:6112078 ]
- Katoh S, Sueoka T, Matsuura S, Sugimoto T: Biopterin and neopterin in human saliva. Life Sci. 1989;45(26):2561-8. [PubMed:2615555 ]
- Ichinose H, Ohye T, Shinotoh H, Arai K, Yamazaki S, Mizuta E, Kuno S, Nagatsu T: Biopterin metabolism in patients with malignant syndrome. Parkinsonism Relat Disord. 2003 Apr;9 Suppl 1:S11-4. [PubMed:12735910 ]
- Zurfluh MR, Giovannini M, Fiori L, Fiege B, Gokdemir Y, Baykal T, Kierat L, Gartner KH, Thony B, Blau N: Screening for tetrahydrobiopterin deficiencies using dried blood spots on filter paper. Mol Genet Metab. 2005 Dec;86 Suppl 1:S96-103. Epub 2005 Nov 7. [PubMed:16275037 ]
- Ogiwara S, Kiuchi K, Nagatsu T, Teradaira R, Nagatsu I, Fujita K, Sugimoto T: Highly sensitive, specific enzyme-linked immunosorbent assay of neopterin and biopterin in biological samples. Clin Chem. 1992 Oct;38(10):1954-8. [PubMed:1394977 ]
- Hirata Y, Sawada M, Minami M, Arai H, Iizuka R, Nagatsu T: Tyrosine hydroxylase, tryptophan hydroxylase, biopterin, and neopterin in the brain of anorexia nervosa. J Neural Transm Gen Sect. 1990;80(2):145-50. [PubMed:1969283 ]
- Dhondt JL, Tilmont P, Ringel J, Farriaux JP: Pterins analysis in amniotic fluid for the prenatal diagnosis of GTP cyclohydrolase deficiency. J Inherit Metab Dis. 1990;13(6):879-82. [PubMed:2079836 ]
- Furukawa Y, Kapatos G, Haycock JW, Worsley J, Wong H, Kish SJ, Nygaard TG: Brain biopterin and tyrosine hydroxylase in asymptomatic dopa-responsive dystonia. Ann Neurol. 2002 May;51(5):637-41. [PubMed:12112113 ]
- Bonafe L, Thony B, Leimbacher W, Kierat L, Blau N: Diagnosis of dopa-responsive dystonia and other tetrahydrobiopterin disorders by the study of biopterin metabolism in fibroblasts. Clin Chem. 2001 Mar;47(3):477-85. [PubMed:11238300 ]
- Slazyk WE, Spierto FW: Liquid-chromatographic measurement of biopterin and neopterin in serum and urine. Clin Chem. 1990 Jul;36(7):1364-8. [PubMed:2372953 ]
- Dhondt JL, Hayte JM, Forzy G, Delcroix M, Farriaux JP: Unconjugated pteridines in amniotic fluid during gestation. Clin Chim Acta. 1986 Dec 30;161(3):269-73. [PubMed:3802534 ]
- Iizuka T, Sasaki M, Oishi K, Uemura S, Koike M, Minatogawa Y: Nitric oxide may trigger lactation in humans. J Pediatr. 1997 Dec;131(6):839-43. [PubMed:9427887 ]
- Zoghbi HY, Milstien S, Butler IJ, Smith EO, Kaufman S, Glaze DG, Percy AK: Cerebrospinal fluid biogenic amines and biopterin in Rett syndrome. Ann Neurol. 1989 Jan;25(1):56-60. [PubMed:2913929 ]
- Kaufman S: Hyperphenylalaninaemia caused by defects in biopterin metabolism. J Inherit Metab Dis. 1985;8 Suppl 1:20-7. [PubMed:3930837 ]
- (). Nagatsu T, Ichinose H, Mogi M, Togari A (1999) Neopterin and cytokines in hereditary dystonia and Parkinson’s disease. Pteridines 10: 5–13.. .
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