Tetrahydrobiopterin (BioCAD00000018384)

blood cerebrospinal fluid (csf) feces all tissues

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

Reviewed

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).

Entity Information

DBLinks

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

Metabolic Network
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
View More
Mass Spectrum
匿名访客 · 受限内容

内容已锁定

您当前以匿名访客身份浏览,此页面的内容由作者限定为已登录用户可见。
请使用您的邮箱登录后继续查看。

使用邮箱继续

首次使用将自动为您创建账户。
遇到问题?查看登录帮助



View All Mass Spectrum
Organism Source

Taxonomy Source

  1. Escherichia coli [ncbi taxid: 562]

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
View All Pathways