人类代谢组数据库

版本

5.0

创建日期

2005-11-16 15:48:42 UTC

更新日期

2021-09-14 15:40:00 UTC

登录号

HMDB0000038

地位

quantified

二级访问

HMDB00038

名称

Dihydrobiopterin

描述

Dihydrobiopterin, also known as BH2, 7,8-dihydrobiopterin, L-erythro-7,8-dihydrobiopterin, quinonoid dihydrobiopterin or q-BH2, belongs to the class of organic compounds known as biopterins and derivatives. These are coenzymes containing a 2-amino-pteridine-4-one derivative. Dihydrobiopterin is also classified as a pteridine. Pteridines are aromatic compounds composed of fused pyrimidine and pyrazine rings. Dihydrobiopterin is produced during the synthesis of neurotransmitters L-DOPA, dopamine, norepinephrine and epinephrine. It is restored to the required cofactor tetrahydrobiopterin via the NADPH-dependant reduction of dihydrobiopterin reductase. Dihydrobiopterin can also be converted to tetrahydrobiopterin by nitric oxide synthase (NOS) which is catalyzed by the flavoprotein "diaphorase" activity of NOS. This activity is located on the reductase (C-terminal) domain of NOS, whereas the high affinity tetrahydrobiopterin site involved in NOS activation is located on the oxygenase (N-terminal) domain (PMID: 8626754). Sepiapterin reductase (SPR) is another enzyme that plays a role in the production of dihydrobiopterin. SPR catalyzes the reduction of sepiapterin to dihydrobiopterin (BH2), the precursor for tetrahydrobiopterin (BH4). BH4 is a cofactor critical for nitric oxide biosynthesis and alkylglycerol and aromatic amino acid metabolism (PMID: 25550200). Dihydrobiopterin is known to be synthesized in several parts of the body, including the pineal gland. Dihydrobiopterin exists in all eukaryotes, ranging from yeast to humans. In humans, dihydrobiopterin is involved in several metabolic disorders including dihydropteridine reductase (DHPR) deficiency. DHPR deficiency is a severe form of hyperphenylalaninemia (HPA) due to impaired regeneration of tetrahydrobiopterin (BH4) leading to decreased levels of neurotransmitters (dopamine, serotonin) and folate in cerebrospinal fluid, and causing neurological symptoms such as psychomotor delay, hypotonia, seizures, abnormal movements, hypersalivation, and swallowing difficulties. Dihydrobiopterin is also associated with another metabolic disorder known as sepiapterin reductase deficiency (SRD). Sepiapterin reductase catalyzes the (NADP-dependent) reduction of carbonyl derivatives, including pteridines, and plays an important role in tetrahydrobiopterin biosynthesis. Low dihydrofolate reductase activity in the brain leads to the accumulation of dihydrobiopterin, which in turn, inhibits tyrosine and tryptophan hydroxylases. This uncouples neuronal nitric oxide synthase, leading to neurotransmitter deficiencies and neuronal cell death. SRD is characterized by low cerebrospinal fluid neurotransmitter levels and the presence of elevated cerebrospinal fluid dihydrobiopterin. SRD is characterized by motor delay, axial hypotonia, language delay, diurnal fluctuation of symptoms, dystonia, weakness, oculogyric crises, dysarthria, parkinsonian signs and hyperreflexia.

同义词

1:7,8-DIHYDROBIOPTERIN

2:L-Erythro-Q-dihydrobiopterin

3:Q-BH2

4:Quinonoid dihydrobiopterin

5:L-Erythro-7,8-dihydrobiopterin

6:L-Erythro-dihydrobiopterin

7:7,8-Dihydro-L-biopterin

8:BH2

9:Dihydrobiopterin

化学式

C9H13N5O3

平均分子量

239.2312

等位分子量

239.101839307

化学名称

2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-1,4,7,8-tetrahydropteridin-4-one

传统名称

dihydrobiopterin

CAS 登记号

6779-87-9

代谢物结构字符串

C[C@H](O)[C@H](O)C1=NC2=C(NC1)NC(N)=NC2=O

inchi标识符

InChI=1S/C9H13N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h3,6,15-16H,2H2,1H3,(H4,10,11,13,14,17)/t3-,6-/m0/s1

印记键

FEMXZDUTFRTWPE-DZSWIPIPSA-N

分类学

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.

description:Biopterins and derivatives

kingdom:Organic compounds

super_class:Organoheterocyclic compounds

class:Pteridines and derivatives

sub_class:Pterins and derivatives

molecular_framework:Aromatic heteropolycyclic compounds

1:1,2-diols

2:Azacyclic compounds

3:Heteroaromatic compounds

4:Hydrocarbon derivatives

5:Hydroxypyrimidines

6:Ketimines

7:Organopnictogen compounds

8:Propargyl-type 1,3-dipolar organic compounds

9:Secondary alcohols

10:Secondary alkylarylamines

11:1,2-diol

12:Alcohol

13:Amine

14:Aromatic heteropolycyclic compound

15:Azacycle

16:Biopterin

17:Heteroaromatic compound

18:Hydrocarbon derivative

19:Hydroxypyrimidine

20:Imine

21:Ketimine

22:Organic 1,3-dipolar compound

23:Organic nitrogen compound

24:Organic oxygen compound

25:Organonitrogen compound

26:Organooxygen compound

27:Organopnictogen compound

28:Propargyl-type 1,3-dipolar organic compound

29:Pyrimidine

30:Secondary alcohol

31:Secondary aliphatic/aromatic amine

32:Secondary amine

33:7,8-dihydrobiopterin

本体论

term:Physiological effect

definition:The effect on an organism physiology, resulting from its exposure to a chemical.

parent_id:

level:1

type:parent

term:Health effect

definition:A health condition or observation associated with a stimuli or with a biological activity of a chemical.

parent_id:7693

*level:2

type:parent

term:Health condition

definition:A health effect that consists on short or long-term disease, condition, disorder, syndrome or constant abnormality.

parent_id:7694

**level:3

type:parent

term:Kidney disease

definition:A urinary system disease that is located in the kidney. (do).

parent_id:7695

***level:4

type:child

synonym:Renal disease

synonym:Nephropathy

term:Disposition

definition:A concept that describes the origin of a chemical, its location within an organism, or its route of exposure.

parent_id:

level:1

type:parent

term:Source

definition:Natural or synthetic origin of a chemical.

parent_id:7724

*level:2

type:parent

term:Endogenous

definition:

parent_id:7735

**level:3

type:child

term:Biological location

definition:The physiological origin within an organism, including anatomical compnents, biofluids and excreta.

parent_id:7724

*level:2

type:parent

term:Biofluid and excreta

definition:A liquid, semi-solid or solid material originating in the body.

parent_id:7725

**level:3

type:parent

term:Feces

definition:The material discharged from the bowel during defecation. It consists of undigested food, intestinal mucus, epithelial cells, and bacteria.

parent_id:7731

***level:4

type:child

synonym:Fecal

synonym:Stool

synonym:Faecal

synonym:Faeces

term:Saliva

definition:The watery fluid in the mouth made by the salivary glands. saliva moistens food to help digestion and it helps protect the mouth against infections. (nci).

parent_id:7731

***level:4

type:child

term:Blood

definition:A liquid tissue with the primary function of transporting oxygen and carbon dioxide (nci). it supplies the tissues with nutrients, removes waste products, and contains various components of the immune system defending the body against infection.

parent_id:7731

***level:4

type:child

term:Cerebrospinal fluid

definition:The fluid that is contained within the brain ventricles, the subarachnoid space and the central canal of the spinal cord. (nci).

parent_id:7731

***level:4

type:child

synonym:Csf

term:Subcellular

definition:An anatomical organizational level including a component within a biological cell .

parent_id:7725

**level:3

type:parent

term:Cytoplasm

definition:The portion of the cell contained within the plasma membrane but excluding the nucleus.

parent_id:7730

***level:4

type:child

synonym:Cytoplasma

term:Mitochondria

definition:

parent_id:7730

***level:4

type:child

term:Process

definition:Biological or chemical events, or a series thereof, leading to a known function or end-product.

parent_id:

level:1

type:parent

term:Naturally occurring process

definition:Naturally-occurring molecular events or a series thereof, leading to a known function or end-product.

parent_id:7659

*level:2

type:parent

term:Biological process

definition:Biological or chemical events or a series thereof, leading to a known function or end-product within an organism.

parent_id:7660

**level:3

type:parent

term:Biochemical pathway

definition:A linked series of chemical reactions that occur in a defined order within or between organism cells, and lead to a known function or end product.

parent_id:7661

***level:4

type:parent

状态

Solid

实验性质

预测性质

kind:logp

value:-1.43

source:ALOGPS

kind:logs

value:-2.17

source:ALOGPS

kind:solubility

value:1.63 g/L

source:ALOGPS

kind:logp

value:-2.3

source:ChemAxon

kind:pka_strongest_acidic

value:9.91

source:ChemAxon

kind:pka_strongest_basic

value:0.4

source:ChemAxon

kind:iupac

value:2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-1,4,7,8-tetrahydropteridin-4-one

source:ChemAxon

kind:average_mass

value:239.2312

source:ChemAxon

kind:mono_mass

value:239.101839307

source:ChemAxon

10:

kind:smiles

value:C[C@H](O)[C@H](O)C1=NC2=C(NC1)NC(N)=NC2=O

source:ChemAxon

11:

kind:formula

value:C9H13N5O3

source:ChemAxon

12:

kind:inchi

value:InChI=1S/C9H13N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h3,6,15-16H,2H2,1H3,(H4,10,11,13,14,17)/t3-,6-/m0/s1

source:ChemAxon

13:

kind:inchikey

value:FEMXZDUTFRTWPE-DZSWIPIPSA-N

source:ChemAxon

14:

kind:polar_surface_area

value:132.33

source:ChemAxon

15:

kind:refractivity

value:68.11

source:ChemAxon

16:

kind:polarizability

value:23.11

source:ChemAxon

17:

kind:rotatable_bond_count

value:2

source:ChemAxon

18:

kind:acceptor_count

value:8

source:ChemAxon

19:

kind:donor_count

value:5

source:ChemAxon

20:

kind:physiological_charge

value:0

source:ChemAxon

21:

kind:formal_charge

value:0

source:ChemAxon

22:

kind:number_of_rings

value:2

source:ChemAxon

23:

kind:bioavailability

value:Yes

source:ChemAxon

24:

kind:rule_of_five

value:Yes

source:ChemAxon

25:

kind:ghose_filter

value:No

source:ChemAxon

26:

kind:veber_rule

value:No

source:ChemAxon

27:

kind:mddr_like_rule

value:No

source:ChemAxon

光谱

生物学性质

cellular:(1):Cytoplasm

biospecimen:(1):Blood

biospecimen:(2):Cerebrospinal Fluid (CSF)

biospecimen:(3):Feces

biospecimen:(4):Saliva

[1]:

name:Aromatic L-Aminoacid Decarboxylase Deficiency

smpdb_id:SMP00170

kegg_map_id:

[2]:

name:Dopa-responsive dystonia

smpdb_id:SMP00486

kegg_map_id:

[3]:

name:Hyperphenylalaniemia due to guanosine triphosphate cyclohydrolase deficiency

smpdb_id:SMP00487

kegg_map_id:

[4]:

name:Hyperphenylalaninemia due to 6-pyruvoyltetrahydropterin synthase deficiency (ptps)

smpdb_id:SMP00488

kegg_map_id:

[5]:

name:Hyperphenylalaninemia due to dhpr-deficiency

smpdb_id:SMP00489

kegg_map_id:

[6]:

name:Pterine Biosynthesis

smpdb_id:SMP00005

kegg_map_id:map00790

[7]:

name:Segawa syndrome

smpdb_id:SMP00490

kegg_map_id:

[8]:

name:Sepiapterin reductase deficiency

smpdb_id:SMP00491

kegg_map_id:

[9]:

name:Tyrosine hydroxylase deficiency

smpdb_id:SMP00497

kegg_map_id:

[10]:

name:Tyrosine metabolism

smpdb_id:

kegg_map_id:map00350

正常浓度

biospecimen:Blood

concentration_value:0.006 +/- 0.0003

concentration_units:uM