版本
5.0
创建日期
2005-11-16 15:48:42 UTC
更新日期
2021-09-14 15:44:51 UTC
登录号
HMDB0000008
地位
quantified
二级访问
HMDB00008

名称
2-Hydroxybutyric acid
描述
2-Hydroxybutyric acid (CAS: 600-15-7), also known as alpha-hydroxybutyrate, is an organic acid derived from alpha-ketobutyrate. alpha-Ketobutyrate is produced by amino acid catabolism (threonine and methionine) and glutathione anabolism (cysteine formation pathway) and is metabolized into propionyl-CoA and carbon dioxide (PMID: 20526369). 2-Hydroxybutyric acid is formed as a byproduct from the formation of alpha-ketobutyrate via a reaction catalyzed by lactate dehydrogenase (LDH) or alpha-hydroxybutyrate dehydrogenase (alphaHBDH). alpha-Hydroxybutyric acid is primarily produced in mammalian hepatic tissues that catabolize L-threonine or synthesize glutathione. Oxidative stress or detoxification of xenobiotics in the liver can dramatically increase the rate of hepatic glutathione synthesis. Under such metabolic stress conditions, supplies of L-cysteine for glutathione synthesis become limiting, so homocysteine is diverted from the transmethylation pathway (which forms methionine) into the transsulfuration pathway (which forms cystathionine). alpha-Ketobutyrate is released as a byproduct when cystathionine is cleaved into cysteine that is incorporated into glutathione. Chronic shifts in the rate of glutathione synthesis may be reflected by urinary excretion of 2-hydroxybutyrate. 2-Hydroxybutyrate is an early marker for both insulin resistance and impaired glucose regulation that appears to arise due to increased lipid oxidation and oxidative stress (PMID: 20526369). 2-Hydroxybutyric acid is often found in the urine of patients suffering from lactic acidosis and ketoacidosis. 2-Hydroxybutyric acid generally appears at high concentrations in situations related to deficient energy metabolism (e.g. birth asphyxia) and also in inherited metabolic diseases affecting the central nervous system during neonatal development, such as "cerebral" lactic acidosis, glutaric aciduria type II, dihydrolipoyl dehydrogenase (E3) deficiency, and propionic acidemia. More recently it has been noted that elevated levels of alpha-hydroxybutyrate in the plasma is a good marker for early-stage type II diabetes (PMID: 19166731). It was concluded from studies done in the mid-1970s that an increased NADH2/NAD ratio was the most important factor for the production of 2-hydroxybutyric acid (PMID: 168632).
同义词
1:(S)-2-Hydroxybutanoic acid

2:2-Hydroxybutyrate

3:2-Hydroxybutyric acid

4:L-2-Hydroxybutanoic acid

5:L-2-Hydroxybutyric acid

6:L-alpha-Hydroxybutanoic acid

7:L-alpha-Hydroxybutyric acid

8:(S)-2-Hydroxybutanoate

9:L-2-Hydroxybutanoate

10:L-2-Hydroxybutyrate

11:L-a-Hydroxybutanoate

12:L-a-Hydroxybutanoic acid

13:L-alpha-Hydroxybutanoate

14:L-Α-hydroxybutanoate

15:L-Α-hydroxybutanoic acid

16:L-a-Hydroxybutyrate

17:L-a-Hydroxybutyric acid

18:L-alpha-Hydroxybutyrate

19:L-Α-hydroxybutyrate

20:L-Α-hydroxybutyric acid

21:(S)-2-Hydroxybutyrate

22:2-Hydroxybutanoate

23:(+)-2-Hydroxy-n-butyric acid

24:(+)-2-Hydroxybutanoic acid

25:(+)-2-Hydroxybutyric acid

26:(+)-alpha-Hydroxybutyric acid

27:(+)-α-Hydroxybutyric acid

28:(2S)-2-Hydroxybutanoic acid

29:(S)-(+)-2-Hydroxybutanoic acid

30:(S)-2-Hydroxybutyric acid

31:2-Hydroxybutanoic acid

32:alpha-Hydroxy-n-butyric acid

33:alpha-Hydroxybutanoic acid

34:alpha-Hydroxybutyric acid

35:α-Hydroxy-n-butyric acid

36:α-Hydroxybutanoic acid

37:α-Hydroxybutyric acid

化学式
C4H8O3
平均分子量
104.105
等位分子量
104.047344118
化学名称
(2S)-2-hydroxybutanoic acid
传统名称
(S)-2-hydroxybutyric acid
CAS 登记号
3347-90-8
代谢物结构字符串
CC[C@H](O)C(O)=O
inchi标识符
InChI=1S/C4H8O3/c1-2-3(5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1
印记键
AFENDNXGAFYKQO-VKHMYHEASA-N
分类学
description: belongs to the class of organic compounds known as alpha hydroxy acids and derivatives. These are organic compounds containing a carboxylic acid substituted with a hydroxyl group on the adjacent carbon.

description:Alpha hydroxy acids and derivatives

kingdom:Organic compounds

super_class:Organic acids and derivatives

class:Hydroxy acids and derivatives

sub_class:Alpha hydroxy acids and derivatives

molecular_framework:Aliphatic acyclic compounds

1:Carbonyl compounds

2:Carboxylic acids

3:Fatty acids and conjugates

4:Hydrocarbon derivatives

5:Monocarboxylic acids and derivatives

6:Organic oxides

7:Secondary alcohols

8:Alcohol

9:Aliphatic acyclic compound

10:Alpha-hydroxy acid

11:Carbonyl group

12:Carboxylic acid

13:Carboxylic acid derivative

14:Fatty acid

15:Hydrocarbon derivative

16:Monocarboxylic acid or derivatives

17:Organic oxide

18:Organic oxygen compound

19:Organooxygen compound

20:Secondary alcohol

21:2-hydroxybutyric acid

22:Hydroxy fatty acids

23:Hydroxy fatty acids

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:Schizophrenia

definition:A psychotic disorder that is characterized by a disintegration of thought processes and of emotional responsiveness. (do).

parent_id:7695

***level:4

type:child

term:Pyruvate dehydrogenase deficiency

definition:

parent_id:7695

***level:4

type:child

synonym:Pdh

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:Tissue and substructures

definition:An anatomical organizational level including multiple cells yet not comprising a complete organ .

parent_id:7725

**level:3

type:parent

term:Placenta

definition:An organ present in some vertebrates during embryonic gestation that surrounds the fetus and provides it with nutrients and oxygen, facilitates gas and waste exchange between the fetus and mother, and provides parasitic cloaking from the mother's immune system by excretion of neurokinin b. (nci).

parent_id:7729

***level:4

type:child

term:Organ and components

definition:An anatomical organizational level including multiple tissues or substructures, which enables a common biological function.

parent_id:7725

**level:3

type:parent

term:Prostate

definition:The male reproductive accessory gland that produces prostatic fluid and is located adjacent to or around the urethra distal to the urinary bladder in mammals. (nci).

parent_id:7727

***level:4

type:child

synonym:Prostate gland

term:Biofluid and excreta

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

parent_id:7725

**level:3

type:parent

term:Urine

definition:Excretion in liquid state processed by the kidney.

parent_id:7731

***level:4

type:child

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: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: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:Sweat

definition:Secretion produced by a sweat gland.

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:Cell and elements

definition:An anatomical organizational level comprising of a single cell (or a fragment in some exceptions). a cell is defined by its type or function. it is the smallest unit of living structure capable of independent existence, composed of a membrane-enclosed mass of protoplasm and containing a nucleus or nucleoid.

parent_id:7725

**level:3

type:parent

term:Extracellular

definition:The space external to the outermost structure of a cell.

parent_id:7732

***level:4

type:child

synonym:Extracellular region

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

term:Role

definition:The purpose or function assumed by a chemical, either naturally or as intended by humans .

parent_id:

level:1

type:parent

term:Biological role

definition:The biological function of a chemical. the biological role answers the question how a chemical is involved in molecular processes in an organism. this can include biochemical effects of non-endogenous chemicals, which are also assigned an industrial application such as pharmaceuticals. the biological role is limited to cellular levls, and will not include role at system process level, such as a chemical which has a role in a disease.

parent_id:7671

*level:2

type:parent

term:Metabolite

definition:A chemical substance produced during metabolism process, as an intermediate or end-product.

parent_id:7672

**level:3

type:child

状态
Solid
实验性质
1:

kind:melting_point

value:44.2 °C

source:

预测性质
1:

kind:logp

value:-0.16

source:ALOGPS

2:

kind:logs

value:0.67

source:ALOGPS

3:

kind:solubility

value:484 g/L

source:ALOGPS

4:

kind:logp

value:0.051

source:ChemAxon

5:

kind:pka_strongest_acidic

value:3.99

source:ChemAxon

6:

kind:pka_strongest_basic

value:-3.8

source:ChemAxon

7:

kind:iupac

value:(2S)-2-hydroxybutanoic acid

source:ChemAxon

8:

kind:average_mass

value:104.105

source:ChemAxon

9:

kind:mono_mass

value:104.047344118

source:ChemAxon

10:

kind:smiles

value:CC[C@H](O)C(O)=O

source:ChemAxon

11:

kind:formula

value:C4H8O3

source:ChemAxon

12:

kind:inchi

value:InChI=1S/C4H8O3/c1-2-3(5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1

source:ChemAxon

13:

kind:inchikey

value:AFENDNXGAFYKQO-VKHMYHEASA-N

source:ChemAxon

14:

kind:polar_surface_area

value:57.53

source:ChemAxon

15:

kind:refractivity

value:23.36

source:ChemAxon

16:

kind:polarizability

value:9.98

source:ChemAxon

17:

kind:rotatable_bond_count

value:2

source:ChemAxon

18:

kind:acceptor_count

value:3

source:ChemAxon

19:

kind:donor_count

value:2

source:ChemAxon

20:

kind:physiological_charge

value:-1

source:ChemAxon

21:

kind:formal_charge

value:0

source:ChemAxon

22:

kind:number_of_rings

value:0

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

1:

type:Specdb::NmrTwoD

spectrum_id:2063

2:

type:Specdb::CMs

spectrum_id:102797

3:

type:Specdb::CMs

spectrum_id:102798

4:

type:Specdb::CMs

spectrum_id:102799

5:

type:Specdb::CMs

spectrum_id:102800

6:

type:Specdb::CMs

spectrum_id:102801

7:

type:Specdb::CMs

spectrum_id:102802

8:

type:Specdb::CMs

spectrum_id:102803

9:

type:Specdb::CMs

spectrum_id:159343

10:

type:Specdb::NmrOneD

spectrum_id:5245

11:

type:Specdb::NmrOneD

spectrum_id:5246

12:

type:Specdb::NmrOneD

spectrum_id:5312

13:

type:Specdb::NmrOneD

spectrum_id:5313

14:

type:Specdb::NmrOneD

spectrum_id:5314

15:

type:Specdb::NmrOneD

spectrum_id:5315

16:

type:Specdb::NmrOneD

spectrum_id:5316

17:

type:Specdb::NmrOneD

spectrum_id:5317

18:

type:Specdb::NmrOneD

spectrum_id:5318

19:

type:Specdb::NmrOneD

spectrum_id:5319

20:

type:Specdb::NmrOneD

spectrum_id:5320

21:

type:Specdb::NmrOneD

spectrum_id:5321

22:

type:Specdb::NmrOneD

spectrum_id:5322

23:

type:Specdb::NmrOneD

spectrum_id:5323

24:

type:Specdb::NmrOneD

spectrum_id:5324

25:

type:Specdb::NmrOneD

spectrum_id:5325

26:

type:Specdb::NmrOneD

spectrum_id:5326

27:

type:Specdb::NmrOneD

spectrum_id:5327

28:

type:Specdb::NmrOneD

spectrum_id:5328

29:

type:Specdb::NmrOneD

spectrum_id:5329

30:

type:Specdb::NmrOneD

spectrum_id:5330

31:

type:Specdb::NmrOneD

spectrum_id:5331

32:

type:Specdb::MsMs

spectrum_id:1250182

33:

type:Specdb::MsMs

spectrum_id:1250183

34:

type:Specdb::MsMs

spectrum_id:1250184

35:

type:Specdb::MsMs

spectrum_id:1365478

36:

type:Specdb::MsMs

spectrum_id:1365479

37:

type:Specdb::MsMs

spectrum_id:1365480

38:

type:Specdb::MsMs

spectrum_id:1448541

39:

type:Specdb::MsMs

spectrum_id:1448542

40:

type:Specdb::MsMs

spectrum_id:1448543

41:

type:Specdb::MsMs

spectrum_id:1448544

42:

type:Specdb::MsMs

spectrum_id:1448545

43:

type:Specdb::MsMs

spectrum_id:1448546

44:

type:Specdb::MsMs

spectrum_id:2253310

45:

type:Specdb::MsMs

spectrum_id:2257260

46:

type:Specdb::MsMs

spectrum_id:2257338

47:

type:Specdb::MsMs

spectrum_id:2259269

48:

type:Specdb::MsMs

spectrum_id:2259286

49:

type:Specdb::MsMs

spectrum_id:2734025

50:

type:Specdb::MsMs

spectrum_id:2734026

51:

type:Specdb::MsMs

spectrum_id:2734027

52:

type:Specdb::MsMs

spectrum_id:2971863

53:

type:Specdb::MsMs

spectrum_id:2971864

54:

type:Specdb::MsMs

spectrum_id:2971865

光谱
生物学性质
1:

cellular:(1):Cytoplasm

cellular:(2):Extracellular

2:

biospecimen:(1):Blood

biospecimen:(2):Cerebrospinal Fluid (CSF)

biospecimen:(3):Feces

biospecimen:(4):Saliva

biospecimen:(5):Sweat

biospecimen:(6):Urine

3:

tissue:(1):Placenta

tissue:(2):Prostate

4:

[1]:

name:Malonic Aciduria

smpdb_id:SMP00198

kegg_map_id:

[2]:

name:Malonyl-coa decarboxylase deficiency

smpdb_id:SMP00502

kegg_map_id:

[3]:

name:Methylmalonic Aciduria Due to Cobalamin-Related Disorders

smpdb_id:SMP00201

kegg_map_id:

[4]:

name:Propanoate metabolism

smpdb_id:

kegg_map_id:map00640

正常浓度
1:

biospecimen:Blood

concentration_value:8.00-80.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

2:

biospecimen:Blood

concentration_value:26.9(32.92)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

3:

biospecimen:Blood

concentration_value:8.00-80.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

4:

biospecimen:Blood

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

5:

biospecimen:Blood

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

6:

biospecimen:Blood

concentration_value:54.0 (8.0-80.0)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

7:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:40.0 +/- 24.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

8:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:85.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

9:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:37 +/- 21 (10 - 76)

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

10:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:35.0 +/- 24.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

11:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

12:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

13:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

14:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

15:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

16:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

17:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

18:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

19:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

20:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

21:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

22:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

23:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

24:

biospecimen:Saliva

concentration_value:10.42 +/- 9.20

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

25:

biospecimen:Sweat

concentration_value:

concentration_units:

subject_age:Adult

subject_sex:Both

subject_condition:Normal

26:

biospecimen:Urine

concentration_value:0.31-0.69

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

27:

biospecimen:Urine

concentration_value:2.445 (0-4.89)

concentration_units:umol/mmol creatinine

subject_age:Not Specified

subject_sex:Both

subject_condition:Normal

28:

biospecimen:Urine

concentration_value:0-3

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Male

subject_condition:Normal

29:

biospecimen:Urine

concentration_value:0-6.0

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Male

subject_condition:Normal

30:

biospecimen:Urine

concentration_value:0-6.1

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Female

subject_condition:Normal

31:

biospecimen:Urine

concentration_value:0-9.3

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Female

subject_condition:Normal

32:

biospecimen:Urine

concentration_value:3.29 +/- 3.529

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

33:

biospecimen:Urine

concentration_value:2.8 (1.2-6.9)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

34:

biospecimen:Urine

concentration_value:5.0 (0.0-10.0)

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Both

subject_condition:Normal

35:

biospecimen:Urine

concentration_value:0.26-0.53

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

36:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

异常浓度
1:

biospecimen:Blood

concentration_value:26.96 +/- 13.11

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy with fetuses with trisomy 18

2:

biospecimen:Blood

concentration_value:16.93 +/- 8.17

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

3:

biospecimen:Blood

concentration_value:21.4 (5.6)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Early preeclampsia

4:

biospecimen:Blood

concentration_value:19.5 (7.4)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

5:

biospecimen:Blood

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal cancer

6:

biospecimen:Blood

concentration_value:17.13 (7.48)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy with fetus having congenital heart defect

7:

biospecimen:Blood

concentration_value:16.69 (6.25)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

8:

biospecimen:Blood

concentration_value:28.0 (14.4)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Late-onset preeclampsia

9:

biospecimen:Blood

concentration_value:4.14(10.18)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Heart failure with reduced ejection fraction

10:

biospecimen:Blood

concentration_value:21.2 (7.5)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

11:

biospecimen:Blood

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Schizophrenia

12:

biospecimen:Blood

concentration_value:23.1 (14.1)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Down syndrome pregnancy

13:

biospecimen:Blood

concentration_value:15.0 (7.4)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

14:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal cancer

15:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal Cancer

16:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal cancer

17:

biospecimen:Saliva

concentration_value:7.26 +/- 4.47

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:Alzheimer's disease

18:

biospecimen:Saliva

concentration_value:7.30 +/- 1.32

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:Frontotemporal lobe dementia

19:

biospecimen:Saliva

concentration_value:8.12 +/- 5.32

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Lewy body disease

20:

biospecimen:Urine

concentration_value:>25.0

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Pyruvate dehydrogenase deficiency

21:

biospecimen:Urine

concentration_value:8.699 +/- 10.366

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Eosinophilic esophagitis

22:

biospecimen:Urine

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Schizophrenia

23:

biospecimen:Urine

concentration_value:248.732

concentration_units:umol/mmol creatinine

patient_age:Infant (0-1 year old)

patient_sex:Male

patient_information:Dihydrolipoamide Dehydrogenase Deficiency

24:

biospecimen:Urine

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal adenoma

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reference_text:Selley ML, Close DR, Stern SE: The effect of increased concentrations of homocysteine on the concentration of (E)-4-hydroxy-2-nonenal in the plasma and cerebrospinal fluid of patients with Alzheimer's disease. Neurobiol Aging. 2002 May-Jun;23(3):383-8.

pubmed_id:11959400

[3]:

reference_text:Shetty HU, Holloway HW, Schapiro MB: Cerebrospinal fluid and plasma distribution of myo-inositol and other polyols in Alzheimer disease. Clin Chem. 1996 Feb;42(2):298-302.

pubmed_id:8595727

[4]:

reference_text:Jia JP, Jia JM, Zhou WD, Xu M, Chu CB, Yan X, Sun YX: Differential acetylcholine and choline concentrations in the cerebrospinal fluid of patients with Alzheimer's disease and vascular dementia. Chin Med J (Engl). 2004 Aug;117(8):1161-4.

pubmed_id:15361288

[5]:

reference_text:Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4.

pubmed_id:9693263

[6]:

reference_text:Walter A, Korth U, Hilgert M, Hartmann J, Weichel O, Hilgert M, Fassbender K, Schmitt A, Klein J: Glycerophosphocholine is elevated in cerebrospinal fluid of Alzheimer patients. Neurobiol Aging. 2004 Nov-Dec;25(10):1299-303.

pubmed_id:15465626

[7]:

reference_text:Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91.

pubmed_id:15061359

[8]:

reference_text:Raskind MA, Peskind ER, Holmes C, Goldstein DS: Patterns of cerebrospinal fluid catechols support increased central noradrenergic responsiveness in aging and Alzheimer's disease. Biol Psychiatry. 1999 Sep 15;46(6):756-65.

pubmed_id:10494443

[9]:

reference_text:Lovell MA, Markesbery WR: Ratio of 8-hydroxyguanine in intact DNA to free 8-hydroxyguanine is increased in Alzheimer disease ventricular cerebrospinal fluid. Arch Neurol. 2001 Mar;58(3):392-6.

pubmed_id:11255442

[10]:

reference_text:Bar KJ, Franke S, Wenda B, Muller S, Kientsch-Engel R, Stein G, Sauer H: Pentosidine and N(epsilon)-(carboxymethyl)-lysine in Alzheimer's disease and vascular dementia. Neurobiol Aging. 2003 Mar-Apr;24(2):333-8.

pubmed_id:12498967

[11]:

reference_text:Serot JM, Barbe F, Arning E, Bottiglieri T, Franck P, Montagne P, Nicolas JP: Homocysteine and methylmalonic acid concentrations in cerebrospinal fluid: relation with age and Alzheimer's disease. J Neurol Neurosurg Psychiatry. 2005 Nov;76(11):1585-7.

pubmed_id:16227558

[12]:

reference_text:Molina JA, Jimenez-Jimenez FJ, Aguilar MV, Meseguer I, Mateos-Vega CJ, Gonzalez-Munoz MJ, de Bustos F, Porta J, Orti-Pareja M, Zurdo M, Barrios E, Martinez-Para MC: Cerebrospinal fluid levels of transition metals in patients with Alzheimer's disease. J Neural Transm (Vienna). 1998;105(4-5):479-88.

pubmed_id:9720975

[13]:

reference_text:Molina JA, Jimenez-Jimenez FJ, Hernanz A, Fernandez-Vivancos E, Medina S, de Bustos F, Gomez-Escalonilla C, Sayed Y: Cerebrospinal fluid levels of thiamine in patients with Alzheimer's disease. J Neural Transm (Vienna). 2002 Jul;109(7-8):1035-44.

pubmed_id:12111441

[14]:

reference_text:Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203.

pubmed_id:16244393

[15]:

reference_text:Kristensen MO, Gulmann NC, Christensen JE, Ostergaard K, Rasmussen K: Serum cobalamin and methylmalonic acid in Alzheimer dementia. Acta Neurol Scand. 1993 Jun;87(6):475-81.

pubmed_id:8356878

[16]:

reference_text:Abe T, Tohgi H, Isobe C, Murata T, Sato C: Remarkable increase in the concentration of 8-hydroxyguanosine in cerebrospinal fluid from patients with Alzheimer's disease. J Neurosci Res. 2002 Nov 1;70(3):447-50.

pubmed_id:12391605

[17]:

reference_text:Reichman ME, Judd JT, Longcope C, Schatzkin A, Clevidence BA, Nair PP, Campbell WS, Taylor PR: Effects of alcohol consumption on plasma and urinary hormone concentrations in premenopausal women. J Natl Cancer Inst. 1993 May 5;85(9):722-7.

pubmed_id:8478958

[18]:

reference_text:Hozumi I, Hasegawa T, Honda A, Ozawa K, Hayashi Y, Hashimoto K, Yamada M, Koumura A, Sakurai T, Kimura A, Tanaka Y, Satoh M, Inuzuka T: Patterns of levels of biological metals in CSF differ among neurodegenerative diseases. J Neurol Sci. 2011 Apr 15;303(1-2):95-9. doi: 10.1016/j.jns.2011.01.003. Epub 2011 Feb 2.

pubmed_id:21292280

[19]:

reference_text:Motawaj M, Peoc'h K, Callebert J, Arrang JM: CSF levels of the histamine metabolite tele-methylhistamine are only slightly decreased in Alzheimer's disease. J Alzheimers Dis. 2010;22(3):861-71. doi: 10.3233/JAD-2010-100381.

pubmed_id:20858978

[20]:

reference_text:Smach MA, Jacob N, Golmard JL, Charfeddine B, Lammouchi T, Ben Othman L, Dridi H, Bennamou S, Limem K: Folate and homocysteine in the cerebrospinal fluid of patients with Alzheimer's disease or dementia: a case control study. Eur Neurol. 2011;65(5):270-8. doi: 10.1159/000326301. Epub 2011 Apr 8.

pubmed_id:21474939

[21]:

reference_text:Linnebank M, Popp J, Smulders Y, Smith D, Semmler A, Farkas M, Kulic L, Cvetanovska G, Blom H, Stoffel-Wagner B, Kolsch H, Weller M, Jessen F: S-adenosylmethionine is decreased in the cerebrospinal fluid of patients with Alzheimer's disease. Neurodegener Dis. 2010;7(6):373-8. doi: 10.1159/000309657. Epub 2010 Jun 3.

pubmed_id:20523031

[22]:

reference_text:Rosler N, Wichart I, Jellinger KA: Clinical significance of neurobiochemical profiles in the lumbar cerebrospinal fluid of Alzheimer's disease patients. J Neural Transm (Vienna). 2001;108(2):231-46.

pubmed_id:11314776

[23]:

reference_text:Sunderland T, Berrettini WH, Molchan SE, Lawlor BA, Martinez RA, Vitiello B, Tariot PN, Cohen RM: Reduced cerebrospinal fluid dynorphin A1-8 in Alzheimer's disease. Biol Psychiatry. 1991 Jul 1;30(1):81-7.

pubmed_id:1716470

[24]:

reference_text:Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6.

pubmed_id:23857558

7:

name:Frontotemporal dementia

omim_id:600274

[1]:

reference_text:Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6.

pubmed_id:23857558

8:

name:Lewy body disease

omim_id:

[1]:

reference_text:Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6.

pubmed_id:23857558

9:

name:Pyruvate dehydrogenase deficiency

omim_id:312170

[1]:

reference_text:Patel KP, O'Brien TW, Subramony SH, Shuster J, Stacpoole PW: The spectrum of pyruvate dehydrogenase complex deficiency: clinical, biochemical and genetic features in 371 patients. Mol Genet Metab. 2012 Jan;105(1):34-43. doi: 10.1016/j.ymgme.2011.09.032. Epub 2011 Oct 7.

pubmed_id:22079328

[2]:

reference_text:OMIM 238331

pubmed_id:

10:

name:Eosinophilic esophagitis

omim_id:610247

[1]:

reference_text:Mordechai, Hien, and David S. Wishart

pubmed_id:

11:

name:Dihydrolipoamide Dehydrogenase Deficiency

omim_id:246900

[1]:

reference_text:Kumps A, Duez P, Mardens Y: Metabolic, nutritional, iatrogenic, and artifactual sources of urinary organic acids: a comprehensive table. Clin Chem. 2002 May;48(5):708-17.

pubmed_id:11978597

[2]:

reference_text:Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40.

pubmed_id:6688766

KEGG数据库编号
C05984
DrugBank数据库编号
foodb数据库编号
FDB021867
ChemSpider数据库编号
389701
公共化学化合物编号
440864
PDB数据库编号
生物利益的化学实体数据库编号
50613
knapsack数据库编号
维基百科编号
2-Hydroxybutyric_acid
代谢途径的数据库编号
CPD-3564
苯酚资源管理器化合物数据库编号
比格数据库编号
梅林编号
虚拟机身份证编号
fbonto数据库编号
综合参考
Carlier, J. P.; Henry, C.; Lorin, V.; Rouffignat, K. Conversion of DL-threonine, D-threonine and 2-oxobutyrate into propionate and 2-hydroxybutyrate by Fusobacterium species. Letters in Applied Microbiology (1997), 25(5), 371-374.
一般参考
1:

protein_accession:HMDBP00054

name:Dihydrolipoyl dehydrogenase, mitochondrial

uniprot_id:P09622

gene_name:DLD

protein_type:Unknown

2:

protein_accession:HMDBP00355

name:Sulfotransferase family cytosolic 2B member 1

uniprot_id:O00204

gene_name:SULT2B1

protein_type:Enzyme

3:

protein_accession:HMDBP00483

name:UDP-glucuronosyltransferase 1-1

uniprot_id:P22309

gene_name:UGT1A1

protein_type:Enzyme

4:

protein_accession:HMDBP00893

name:L-lactate dehydrogenase A-like 6A

uniprot_id:Q6ZMR3

gene_name:LDHAL6A

protein_type:Unknown

5:

protein_accession:HMDBP03577

name:L-lactate dehydrogenase B chain

uniprot_id:P07195

gene_name:LDHB

protein_type:Unknown

6:

protein_accession:HMDBP03578

name:L-lactate dehydrogenase C chain

uniprot_id:P07864

gene_name:LDHC

protein_type:Unknown

7:

protein_accession:HMDBP03579

name:L-lactate dehydrogenase A chain

uniprot_id:P00338

gene_name:LDHA

protein_type:Unknown

8:

protein_accession:HMDBP03581

name:L-lactate dehydrogenase A-like 6B

uniprot_id:Q9BYZ2

gene_name:LDHAL6B

protein_type:Unknown

蛋白质结合
1:

reference_text:Sweatman BC, Farrant RD, Holmes E, Ghauri FY, Nicholson JK, Lindon JC: 600 MHz 1H-NMR spectroscopy of human cerebrospinal fluid: effects of sample manipulation and assignment of resonances. J Pharm Biomed Anal. 1993 Aug;11(8):651-64.

pubmed_id:8257730

2:

reference_text:Landaas S, Pettersen JE: Clinical conditions associated with urinary excretion of 2-hydroxybutyric acid. Scand J Clin Lab Invest. 1975 May;35(3):259-66.

pubmed_id:168632

3:

reference_text: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_id:19212411

4:

reference_text:Li X, Xu Z, Lu X, Yang X, Yin P, Kong H, Yu Y, Xu G: Comprehensive two-dimensional gas chromatography/time-of-flight mass spectrometry for metabonomics: Biomarker discovery for diabetes mellitus. Anal Chim Acta. 2009 Feb 9;633(2):257-62. doi: 10.1016/j.aca.2008.11.058. Epub 2008 Dec 3.

pubmed_id:19166731

5:

reference_text:Gall WE, Beebe K, Lawton KA, Adam KP, Mitchell MW, Nakhle PJ, Ryals JA, Milburn MV, Nannipieri M, Camastra S, Natali A, Ferrannini E: alpha-hydroxybutyrate is an early biomarker of insulin resistance and glucose intolerance in a nondiabetic population. PLoS One. 2010 May 28;5(5):e10883. doi: 10.1371/journal.pone.0010883.

pubmed_id:20526369

6:

reference_text:Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043.

pubmed_id:32033212

7:

reference_text:Aldrich Library of FT-IR Spectra, 1st edn., 1985, 1, 548D

8:

reference_text:Mori, K. et al., Tetrahedron, 1979, 35, 1601

9:

reference_text:Levene, P.A. et al., J. Biol. Chem., 1941, 141, 391

10:

reference_text:Duvillier, E., Ann. Chim. Phys., 1879, 17, 532

11:

reference_text:Chenault, H.K. et al., J.O.C., 1987, 52, 2608

12:

reference_text:Aldrich Library of 13C and 1H FT NMR Spectra, 1992, 1, 854B

13:

reference_text:Horn, D.H.S. et al., J.C.S., 1954, 1460

14:

reference_text:Wong, C.H. et al., J.O.C., 1985, 50, 1992

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