Tetrahydrobiopterin (BioCAD00000018384)
Metabolite Card
Chinese Name: 四氢生物喋呤
Formula: C9H15N5O3 (241.1175)
SMILES:
[H][C@@]1(CNC2=C(N1)C(=O)N=C(N)N2)[C@@H](O)[C@H](C)O
Synonyms [en]
tetrahydrobiopterin; sapropterin; L-erythro-tetrahydrobiopterin; 5,6,7,8-tetrahydrobiopterin; Sapropterina; 2-Amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydoro-4(1H)-pteridinone
Last reviewed on 2026-04-16.
Cite this Page
Tetrahydrobiopterin. 数据之源,洞见之始. SMRUCC genomics institute, a synthetic life researcher from China.
https://biocad_registry.innovation.ac.cn/s/Tetrahydrobiopterin
(retrieved
2026-08-20) (CAD Registry RN: BioCAD00000018384). Licensed
under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).
Note
Tetrahydrobiopterin (CAS: 17528-72-2), also known as BH4, is an essential cofactor in the synthesis of neurotransmitters and nitric oxide (PMID: 16946131). In fact, it is used by all three human nitric-oxide synthases (NOS) eNOS, nNOS, and iNOS as well as the enzyme glyceryl-ether monooxygenase. It is also essential in the conversion of phenylalanine into tyrosine by the enzyme phenylalanine-4-hydroxylase; the conversion of tyrosine into L-dopa by the enzyme tyrosine hydroxylase; and the conversion of tryptophan into 5-hydroxytryptophan via tryptophan hydroxylase. Specifically, tetrahydrobiopterin is a cofactor for tryptophan 5-hydroxylase 1, tyrosine 3-monooxygenase, and phenylalanine hydroxylase, all of which are essential for the formation of the neurotransmitters dopamine, noradrenaline, and adrenaline. Tetrahydrobiopterin has been proposed to be involved in the promotion of neurotransmitter release in the brain and the regulation of human melanogenesis. A defect in BH4 production and/or a defect in the enzyme dihydropteridine reductase (DHPR) causes phenylketonuria type IV, as well as dopa-responsive dystonias. BH4 is also implicated in Parkinson's disease, Alzheimer's disease, and depression. Tetrahydrobiopterin is present in probably every cell or tissue of higher animals. On the other hand, most bacteria, fungi and plants do not synthesize tetrahydrobiopterin (Wikipedia).
DBLinks
- CAS Registry Number: 17528-72-2
- PubChem CID: 44257
- ChEBI: 15372
- HMDB: HMDB0000027
- LipidMaps:
- KEGG: C00272
- BioCyc: CPD-14053
- NCBI MeSH: sapropterin
- Wikipedia: Sapropterin
Other DBLinks
- CAS Registry Number: 17528-72-2
- CAS Registry Number: 27070-47-9
- CAS Registry Number: 62989-33-7
- CAS Registry Number: 69056-38-8
- PubChem: 135398654
- PubChem: 135402045
- PubChem: 135409383
- PubChem: 135409384
- PubChem: 135433600
- PubChem: 136153088
- PubChem: 44257
- ChEBI: ChEBI:136570
- ChEBI: ChEBI:15372
- ChEBI: ChEBI:52447
- ChEBI: ChEBI:59560
- ChEBI: ChEBI:64491
- HMDB: HMDB0000027
- HMDB: HMDB0000787
- HMDB: HMDB00787
- KEGG: C00272
- BioCyc: CPD-14053
- NCBI MeSH: sapropterin
- Wikipedia: Sapropterin
- Wikipedia: Tetrahydrobiopterin
- DrugBank: DB00360
- RefMet: RM0018304
- MoNA: HMDB0059658_c_ms_1999
- MoNA: TOF_alkaloids_pos000314
- Metlin: METLIN_5753
- Metlin: METLIN_64878
- Coconut NaturalProduct: CNP0163424.2
- Coconut NaturalProduct: CNP0163424.3
- Coconut NaturalProduct: CNP0593584.1
- PMHub: MS000000219
- metaboanalyst: 214bdcdd4ac7783b597e20c1401c29e2
- metaboanalyst: a009c66a4c7ae15b0f28ae3f03b7b038
Class / Ontology
- WishartLab ClassyFire: [Pterins and derivatives] Pterins and derivatives
- RefMet: [Biopterins] Biopterins
- ChEBI: [CHEBI:15372] 5,6,7,8-tetrahydrobiopterin
- ChEBI: [CHEBI:59560] sapropterin
- Coconut NaturalProduct: [pteridine alkaloids] pteridine alkaloids
| ID | EC Number | Name |
|---|---|---|
| KEGG:R01793 | 1.5.1.34 | 5,6,7,8-tetrahydrobiopterin:NAD+ oxidoreductase |
| KEGG:R01794 | 1.5.1.34 | 5,6,7,8-tetrahydrobiopterin:NADP+ oxidoreductase |
| KEGG:R01795 | 1.14.16.1 | L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase (4-hydroxylating) |
| KEGG:R01812 | 1.5.1.33 | 5,6,7,8-tetrahydrobiopterin:NADP+ oxidoreductase |
| KEGG:R01813 | 1.1.1.153 | tetrahydrobiopterin:NADP+ oxidoreductase |
| KEGG:R01814 | 1.14.16.4 | L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase (5-hydroxylating) |
| KEGG:R01815 | 1.14.16.2 | L-tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating) |
| KEGG:R07211 | 1.14.16.1 | L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating) |
| KEGG:R07212 | 1.14.16.2 | L-tyrosine,tetrahydrobiopterin:oxygen oxidoreductase(3-hydroxylating) |
| KEGG:R07213 | 1.14.16.4 | L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating) |
| KEGG:R08208 | 1.1.1.153 | tetrahydrobiopterin:NADP+ oxidoreductase (6-pyruvoyltetrahydropterin-forming) |
| KEGG:R10729 | 1.14.16.7 | L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating) |
| KEGG:R11764 | 1.1.1.21 | tetrahydrobiopterin:NADP+ oxidoreductase [6-(1'-hydroxy-2'-oxopropyl)-tetrahydropterin-forming] |
| KEGG:R11765 | 1.5.1.3 | tetrahydrobiopterin:NADP+ oxidoreductase (7,8-dihydrobiopterin-forming) |
| Rhea:RHEA:16710 | 1.14.16.4 | (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 => 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin |
| Rhea:RHEA:16711 | 1.14.16.4 | 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin => (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 |
| Rhea:RHEA:16712 | 1.14.16.4 | (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 <=> 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin |
| Rhea:RHEA:18202 | 1.14.16.2 | (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine + O2 => (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-dopa |
| Rhea:RHEA:18203 | 1.14.16.2 | (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-dopa => (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine + O2 |
| Rhea:RHEA:18204 | 1.14.16.2 | (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine + O2 <=> (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-dopa |
Taxonomy Source
Pathway Synthetic
| pathway id | name |
|---|---|
| BioCyc:HUMAN_PWY66-401 | superpathway of tryptophan utilization |
| BioCyc:META_PWY-6134 | L-tyrosine biosynthesis IV |
| BioCyc:HUMAN_PHENYLALANINE-DEG1-PWY | phenylalanine degradation/tyrosine biosynthesis |
| BioCyc:META_PWY66-301 | catecholamine biosynthesis |
| BioCyc:META_PHENYLALANINE-DEG1-PWY | L-phenylalanine degradation I (aerobic) |
| BioCyc:META_PWY-5394 | betalamic acid biosynthesis |
| BioCyc:META_PWY-5664 | erythro-tetrahydrobiopterin biosynthesis II |
| BioCyc:VCHO_PHENYLALANINE-DEG1-PWY | L-phenylalanine degradation I (aerobic) |
| BioCyc:MTBH37RV_PHENYLALANINE-DEG1-PWY | L-phenylalanine degradation I (aerobic) |
| BioCyc:HUMAN_PWY-5663 | tetrahydrobiopterin de novo biosynthesis |
| BioCyc:HUMAN_PWY66-301 | catecholamine biosynthesis |
| BioCyc:META_PWY-5071 | superpathway of rosmarinic acid biosynthesis |
| BioCyc:FLY_PWY-5664 | tetrahydrobiopterin biosynthesis II |
| BioCyc:HUMAN_PWY-6030 | serotonin and melatonin biosynthesis |
| BioCyc:META_PWY-5049 | rosmarinic acid biosynthesis II |
| BioCyc:META_PWY-6030 | serotonin and melatonin biosynthesis |
| BioCyc:META_PWY-5405 | superpathway of betalain biosynthesis |
| BioCyc:META_PWY-5663 | erythro-tetrahydrobiopterin biosynthesis I |
| BioCyc:FLY_PWY-5663 | tetrahydrobiopterin biosynthesis I |
| BioCyc:SYNEL_PWY-5663 | tetrahydrobiopterin biosynthesis I |