版本
5.0
创建日期
2005-11-16 15:48:42 UTC
更新日期
2021-09-14 15:00:08 UTC
登录号
HMDB0000011
地位
quantified
二级访问
HMDB0000357

HMDB00011

HMDB00357

名称
3-Hydroxybutyric acid
描述
3-Hydroxybutyric acid (CAS: 300-85-6), also known as beta-hydroxybutanoic acid, is a typical partial-degradation product of branched-chain amino acids (primarily valine) released from muscle for hepatic and renal gluconeogenesis. This acid is metabolized by 3-hydroxybutyrate dehydrogenase (catalyzes the oxidation of 3-hydroxybutyrate to form acetoacetate, using NAD+ as an electron acceptor). The enzyme functions in nervous tissues and muscles, enabling the use of circulating hydroxybutyrate as a fuel. In the liver mitochondrial matrix, the enzyme can also catalyze the reverse reaction, a step in ketogenesis. 3-Hydroxybutyric acid is a chiral compound having two enantiomers, D-3-hydroxybutyric acid and L-3-hydroxybutyric acid, and is a ketone body. Like the other ketone bodies (acetoacetate and acetone), levels of 3-hydroxybutyrate in blood and urine are raised in ketosis. In humans, 3-hydroxybutyrate is synthesized in the liver from acetyl-CoA and can be used as an energy source by the brain when blood glucose is low. Blood levels of 3-hydroxybutyric acid levels may be monitored in diabetic patients to look for diabetic ketoacidosis. Persistent mild hyperketonemia is a common finding in newborns. Ketone bodies serve as an indispensable source of energy for extrahepatic tissues, especially the brain and lung of developing mammals. Another important function of ketone bodies is to provide acetoacetyl-CoA and acetyl-CoA for the synthesis of cholesterol, fatty acids, and complex lipids. During the early postnatal period, acetoacetate (AcAc) and beta-hydroxybutyrate are preferred over glucose as substrates for the synthesis of phospholipids and sphingolipids in accord with requirements for brain growth and myelination. Thus, during the first two weeks of postnatal development, when the accumulation of cholesterol and phospholipids accelerates, the proportion of ketone bodies incorporated into these lipids increases. On the other hand, an increased proportion of ketone bodies is utilized for cerebroside synthesis during the period of active myelination. In the lung, AcAc serves better than glucose as a precursor for the synthesis of lung phospholipids. The synthesized lipids, particularly dipalmitoylphosphatidylcholine, are incorporated into surfactant, and thus have a potential role in supplying adequate surfactant lipids to maintain lung function during the early days of life (PMID: 3884391). 3-Hydroxybutyric acid is found to be associated with fumarase deficiency and medium-chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism. 3-Hydroxybutyric acid is a metabolite of Alcaligenes and can be produced from plastic metabolization or incorporated into polymers, depending on the species (PMID: 7646009, 18615882).
同义词
1:(R)-(-)-beta-Hydroxybutyric acid

2:(R)-3-Hydroxybutanoic acid

3:3-D-Hydroxybutyric acid

4:D-3-Hydroxybutyric acid

5:D-beta-Hydroxybutyric acid

6:(R)-(-)-b-Hydroxybutyrate

7:(R)-(-)-b-Hydroxybutyric acid

8:(R)-(-)-beta-Hydroxybutyrate

9:(R)-(-)-Β-hydroxybutyrate

10:(R)-(-)-Β-hydroxybutyric acid

11:(R)-3-Hydroxybutanoate

12:3-D-Hydroxybutyrate

13:D-3-Hydroxybutyrate

14:D-b-Hydroxybutyrate

15:D-b-Hydroxybutyric acid

16:D-beta-Hydroxybutyrate

17:D-Β-hydroxybutyrate

18:D-Β-hydroxybutyric acid

19:(R)-3-Hydroxybutyrate

20:3-delta-Hydroxybutyrate

21:3-delta-Hydroxybutyric acid

22:BHIB

23:D-(-)-3-Hydroxybutyrate

24:delta-(-)-3-Hydroxybutyrate

25:delta-3-Hydroxybutyrate

26:delta-3-Hydroxybutyric acid

27:delta-beta-Hydroxybutyrate

28:3R-Hydroxy-butanoate

29:(R)-3-Hydroxybutyric acid

30:(-)-3-Hydroxy-n-butyric acid

31:(-)-3-Hydroxybutyric acid

32:(3R)-3-Hydroxybutanoic acid

33:(3R)-3-Hydroxybutyric acid

34:(3R)-Hydroxybutyrate

35:(R)-(-)-3-Hydroxybutyric acid

36:(R)-beta-Hydroxybutanoic acid

37:(R)-beta-Hydroxybutyric acid

38:(R)-β-Hydroxybutanoic acid

39:(R)-β-Hydroxybutyric acid

40:3-Hydroxy-n-butyric acid

41:3-Hydroxybutanoic acid

42:3-Hydroxybutyric acid

43:3R-Hydroxybutanoic acid

44:D-(-)-3-Hydroxybutanoic acid

45:D-(-)-3-Hydroxybutyric acid

46:D-(-)-beta-Hydroxybutyric acid

47:D-(-)-β-Hydroxybutyric acid

48:beta-Hydroxy-n-butyric acid

49:beta-Hydroxybutanoic acid

50:beta-Hydroxybutyric acid

51:β-Hydroxy-n-butyric acid

52:β-Hydroxybutanoic acid

53:β-Hydroxybutyric acid

化学式
C4H8O3
平均分子量
104.1045
等位分子量
104.047344122
化学名称
(3R)-3-hydroxybutanoic acid
传统名称
(-)-3-hydroxybutyric acid
CAS 登记号
625-72-9
代谢物结构字符串
C[C@@H](O)CC(O)=O
inchi标识符
InChI=1S/C4H8O3/c1-3(5)2-4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m1/s1
印记键
WHBMMWSBFZVSSR-GSVOUGTGSA-N
分类学
description: belongs to the class of organic compounds known as beta hydroxy acids and derivatives. Beta hydroxy acids and derivatives are compounds containing a carboxylic acid substituted with a hydroxyl group on the C3 carbon atom.

description:Beta hydroxy acids and derivatives

kingdom:Organic compounds

super_class:Organic acids and derivatives

class:Hydroxy acids and derivatives

sub_class:Beta 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:Short-chain hydroxy acids and derivatives

9:Alcohol

10:Aliphatic acyclic compound

11:Beta-hydroxy acid

12:Carbonyl group

13:Carboxylic acid

14:Carboxylic acid derivative

15:Fatty acid

16:Hydrocarbon derivative

17:Monocarboxylic acid or derivatives

18:Organic oxide

19:Organic oxygen compound

20:Organooxygen compound

21:Secondary alcohol

22:Short-chain hydroxy acid

23:3-hydroxybutyric acid

24:Hydroxy fatty acids

25:ketone body

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:Diabetes mellitus type 2

definition:

parent_id:7695

***level:4

type:child

synonym:Non-insulin-dependent diabetes mellitus

synonym:Niddm

synonym:Adult-onset diabetes

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:Anoxia

definition:

parent_id:7695

***level:4

type:child

term:Metabolism and nutrition disorders

definition:A group of disorders, inherited or not, that result in a metabolic malfunction.

parent_id:7695

***level:4

type:parent

term:Infections and infestations

definition:Any disorder resulting from the presence and activity of a microbial, viral, or parasitic agent. it can be transmitted by direct or indirect contact. (nci).

parent_id:7695

***level:4

type:parent

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

definition:A living organism (species or a higher taxonomy rank), in which a chemical can be found.

parent_id:7735

**level:3

type:parent

term:Microbe

definition:A living organism that is of a microscopic scale, either single-celled or a cluster of cells.

parent_id:7736

***level:4

type:parent

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:Brain

definition:An organ composed of grey and white matter that is the center for intelligence and reasoning. it is protected by the bony cranium. (nci).

parent_id:7727

***level:4

type:child

term:Liver

definition:An abdominal organ that has variable lobation which are composed mainly of hepatic lobules. (nci).

parent_id:7727

***level:4

type:child

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: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: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: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: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:Cell

definition:

parent_id:7732

***level:4

type:parent

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
1:

kind:melting_point

value:49 - 50 °C

source:

预测性质
1:

kind:logp

value:-0.50

source:ALOGPS

2:

kind:logs

value:0.71

source:ALOGPS

3:

kind:solubility

value:539 g/L

source:ALOGPS

4:

kind:logp

value:-0.39

source:ChemAxon

5:

kind:pka_strongest_acidic

value:4.41

source:ChemAxon

6:

kind:pka_strongest_basic

value:-2.6

source:ChemAxon

7:

kind:iupac

value:(3R)-3-hydroxybutanoic acid

source:ChemAxon

8:

kind:average_mass

value:104.1045

source:ChemAxon

9:

kind:mono_mass

value:104.047344122

source:ChemAxon

10:

kind:smiles

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

source:ChemAxon

11:

kind:formula

value:C4H8O3

source:ChemAxon

12:

kind:inchi

value:InChI=1S/C4H8O3/c1-3(5)2-4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m1/s1

source:ChemAxon

13:

kind:inchikey

value:WHBMMWSBFZVSSR-GSVOUGTGSA-N

source:ChemAxon

14:

kind:polar_surface_area

value:57.53

source:ChemAxon

15:

kind:refractivity

value:23.46

source:ChemAxon

16:

kind:polarizability

value:9.96

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::CMs

spectrum_id:26212

2:

type:Specdb::CMs

spectrum_id:30136

3:

type:Specdb::CMs

spectrum_id:37241

4:

type:Specdb::CMs

spectrum_id:99520

5:

type:Specdb::CMs

spectrum_id:133294

6:

type:Specdb::CMs

spectrum_id:141028

7:

type:Specdb::EiMs

spectrum_id:2010

8:

type:Specdb::NmrTwoD

spectrum_id:920

9:

type:Specdb::NmrOneD

spectrum_id:1027

10:

type:Specdb::NmrOneD

spectrum_id:5332

11:

type:Specdb::NmrOneD

spectrum_id:5333

12:

type:Specdb::NmrOneD

spectrum_id:5334

13:

type:Specdb::NmrOneD

spectrum_id:5335

14:

type:Specdb::NmrOneD

spectrum_id:5336

15:

type:Specdb::NmrOneD

spectrum_id:5337

16:

type:Specdb::NmrOneD

spectrum_id:5338

17:

type:Specdb::NmrOneD

spectrum_id:5339

18:

type:Specdb::NmrOneD

spectrum_id:5340

19:

type:Specdb::NmrOneD

spectrum_id:5341

20:

type:Specdb::NmrOneD

spectrum_id:5342

21:

type:Specdb::NmrOneD

spectrum_id:5343

22:

type:Specdb::NmrOneD

spectrum_id:5344

23:

type:Specdb::NmrOneD

spectrum_id:5345

24:

type:Specdb::NmrOneD

spectrum_id:5346

25:

type:Specdb::NmrOneD

spectrum_id:5347

26:

type:Specdb::NmrOneD

spectrum_id:5348

27:

type:Specdb::NmrOneD

spectrum_id:5349

28:

type:Specdb::NmrOneD

spectrum_id:5350

29:

type:Specdb::NmrOneD

spectrum_id:5351

30:

type:Specdb::MsMs

spectrum_id:16

31:

type:Specdb::MsMs

spectrum_id:17

32:

type:Specdb::MsMs

spectrum_id:18

33:

type:Specdb::MsMs

spectrum_id:2526

34:

type:Specdb::MsMs

spectrum_id:2527

35:

type:Specdb::MsMs

spectrum_id:2528

36:

type:Specdb::MsMs

spectrum_id:2529

37:

type:Specdb::MsMs

spectrum_id:2530

38:

type:Specdb::MsMs

spectrum_id:324085

39:

type:Specdb::MsMs

spectrum_id:324086

40:

type:Specdb::MsMs

spectrum_id:324087

41:

type:Specdb::MsMs

spectrum_id:372409

42:

type:Specdb::MsMs

spectrum_id:372410

43:

type:Specdb::MsMs

spectrum_id:372411

44:

type:Specdb::MsMs

spectrum_id:437816

45:

type:Specdb::MsMs

spectrum_id:437817

46:

type:Specdb::MsMs

spectrum_id:437818

47:

type:Specdb::MsMs

spectrum_id:437819

48:

type:Specdb::MsMs

spectrum_id:437820

49:

type:Specdb::MsMs

spectrum_id:440114

50:

type:Specdb::MsMs

spectrum_id:2233646

51:

type:Specdb::MsMs

spectrum_id:2252490

52:

type:Specdb::MsMs

spectrum_id:2253978

53:

type:Specdb::MsMs

spectrum_id:2254601

54:

type:Specdb::MsMs

spectrum_id:2255993

光谱
生物学性质
1:

cellular:(1):Cytoplasm

cellular:(2):Extracellular

cellular:(3):Mitochondria

2:

biospecimen:(1):Blood

biospecimen:(2):Cellular Cytoplasm

biospecimen:(3):Cerebrospinal Fluid (CSF)

biospecimen:(4):Feces

biospecimen:(5):Saliva

biospecimen:(6):Urine

3:

4:

[1]:

name:Fatty Acid Biosynthesis

smpdb_id:SMP00456

kegg_map_id:

[2]:

name:Ketone Body Metabolism

smpdb_id:SMP00071

kegg_map_id:map00072

[3]:

name:Succinyl CoA: 3-ketoacid CoA transferase deficiency

smpdb_id:SMP00569

kegg_map_id:

正常浓度
1:

biospecimen:Blood

concentration_value:60.0 +/- 20.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

2:

biospecimen:Blood

concentration_value:40.0 +/- 10.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

3:

biospecimen:Blood

concentration_value:323.0 +/- 29.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

4:

biospecimen:Blood

concentration_value:36 +/- 20

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

5:

biospecimen:Blood

concentration_value:86 +/- 53

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

6:

biospecimen:Blood

concentration_value:38 +/- 31

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

7:

biospecimen:Blood

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

8:

biospecimen:Blood

concentration_value:<500

concentration_units:uM

subject_age:Infant (0-1 year old)

subject_sex:Not Specified

subject_condition:Normal

9:

biospecimen:Blood

concentration_value:260-5040

concentration_units:uM

subject_age:Infant (0-1 year old)

subject_sex:Not Specified

subject_condition:Normal

10:

biospecimen:Blood

concentration_value:50.0 (0.0-100.0)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

11:

biospecimen:Blood

concentration_value:46.5 +/- 10.1

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

12:

biospecimen:Blood

concentration_value:<600

concentration_units:uM

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

13:

biospecimen:Blood

concentration_value:<150

concentration_units:uM

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

14:

biospecimen:Blood

concentration_value:100.0 +/- 90.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

15:

biospecimen:Blood

concentration_value:565.0 +/- 255.0

concentration_units:uM

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

16:

biospecimen:Blood

concentration_value:36.0 (13.0-95.0)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

17:

biospecimen:Blood

concentration_value:80-3600

concentration_units:uM

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

18:

biospecimen:Blood

concentration_value:64.88(60.4)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

19:

biospecimen:Blood

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

20:

biospecimen:Blood

concentration_value:200 +/- 120

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

21:

biospecimen:Blood

concentration_value:170 +/- 110

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

22:

biospecimen:Blood

concentration_value:10.60-143.2

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

23:

biospecimen:Blood

concentration_value:500-3000

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Male

subject_condition:Normal

24:

biospecimen:Blood

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

25:

biospecimen:Blood

concentration_value:76.9 +/- 66.3

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

26:

biospecimen:Blood

concentration_value:180.0 (22.0-700.0)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

27:

biospecimen:Cellular Cytoplasm

concentration_value:1300 (1200-1400)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

28:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:34 +/- 31

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

29:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:50.0 (4.0 - 96.0)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

30:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:286 (207-365)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

31:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:46.4 +/- 23.9

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

32:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:48.0 +/- 48.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

33:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

34:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

35:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

36:

biospecimen:Feces

concentration_value:22.0932 +/- 9.606

concentration_units:nmol/g wet feces

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

37:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

38:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

39:

biospecimen:Saliva

concentration_value:5.00 +/- 4.25

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

40:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

41:

biospecimen:Saliva

concentration_value:2.64 +/- 1.83

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

42:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

43:

biospecimen:Saliva

concentration_value:3.22 +/- 2.77

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

44:

biospecimen:Saliva

concentration_value:3.88 +/- 4.26

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

45:

biospecimen:Saliva

concentration_value:16.44 +/- 19.52

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

46:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

47:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

48:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

49:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

50:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

51:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

52:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

53:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

54:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

55:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

56:

biospecimen:Saliva

concentration_value:4.71 +/- 7.21

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

57:

biospecimen:Urine

concentration_value:0.5-9.8

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

58:

biospecimen:Urine

concentration_value:<99

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

59:

biospecimen:Urine

concentration_value:1.4-2.7

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

60:

biospecimen:Urine

concentration_value:<54.301

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Male

subject_condition:Normal

61:

biospecimen:Urine

concentration_value:180.280 +/- 115.119

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

62:

biospecimen:Urine

concentration_value:27.151 +/- 14.118

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

63:

biospecimen:Urine

concentration_value:272.592 +/- 419.205

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

64:

biospecimen:Urine

concentration_value:3.4

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

65:

biospecimen:Urine

concentration_value:3397.0835 +/- 5584.336

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

66:

biospecimen:Urine

concentration_value:616.862 +/- 982.852

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

67:

biospecimen:Urine

concentration_value:7-120

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Both

subject_condition:Normal

68:

biospecimen:Urine

concentration_value:7569.588 +/- 3599.0840

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

69:

biospecimen:Urine

concentration_value:0.72-23.3

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

70:

biospecimen:Urine

concentration_value:4.02(0.78-12.39)

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Female

subject_condition:Normal

71:

biospecimen:Urine

concentration_value:3.14(0.71-23.84)

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Male

subject_condition:Normal

72:

biospecimen:Urine

concentration_value:2.60 +/- 3.50

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Both

subject_condition:Normal

73:

biospecimen:Urine

concentration_value:27.151 +/- 14.118

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

74:

biospecimen:Urine

concentration_value:272.592 +/- 419.205

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

75:

biospecimen:Urine

concentration_value:636.410 +/- 982.852

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

76:

biospecimen:Urine

concentration_value:6.651 +/- 7.809

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

77:

biospecimen:Urine

concentration_value:180.4 +/- 115.00

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

78:

biospecimen:Urine

concentration_value:3397.0835 +/- 5584.336

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

79:

biospecimen:Urine

concentration_value:7569.588 +/- 3599.0840

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

80:

biospecimen:Urine

concentration_value:13.0 (1.0-27.0)

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

81:

biospecimen:Urine

concentration_value:1.4 +/- 1.3

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

82:

biospecimen:Urine

concentration_value:0.7 (0-2.0)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

83:

biospecimen:Urine

concentration_value:<21.17

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 18 years old)

subject_sex:Both

subject_condition:Normal

84:

biospecimen:Urine

concentration_value:38.9 +/- 13.9

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

85:

biospecimen:Urine

concentration_value:32.3 +/- 8.7

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

86:

biospecimen:Urine

concentration_value:48.0 (0.00-200.0)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

87:

biospecimen:Urine

concentration_value:1.4-2.2

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

88:

biospecimen:Urine

concentration_value:3.6 (1.3-6.4)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

89:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

90:

biospecimen:Urine

concentration_value:2.257 +/- 1.254

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

91:

biospecimen:Urine

concentration_value:2.0 (0.5-9.8)

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

92:

biospecimen:Urine

concentration_value:1.4 (0.1-2.7)

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

93:

biospecimen:Urine

concentration_value:1.3 (0.1-5.8)

concentration_units:umol/mmol creatinine

subject_age:Adolescent (13-18 years old)

subject_sex:Both

subject_condition:Normal

94:

biospecimen:Urine

concentration_value:29.712 +/- 14.532

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

95:

biospecimen:Urine

concentration_value:0.62 (0.0-12.09)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

96:

biospecimen:Urine

concentration_value:2.24 (0.0-17.6)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

97:

biospecimen:Urine

concentration_value:3.6 (1.3-6.4)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

98:

biospecimen:Urine

concentration_value:2.0 (0.6-5.6)

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Both

subject_condition:Normal

异常浓度
1:

biospecimen:Blood

concentration_value:235.0 +/- 17.0

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Obesity

2:

biospecimen:Blood

concentration_value:35.1 +/- 33.9

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Heart Transplant

3:

biospecimen:Blood

concentration_value:62.7 (85.0)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Down syndrome pregnancy

4:

biospecimen:Blood

concentration_value:21.0 (19.4)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

5:

biospecimen:Blood

concentration_value:500

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)

6:

biospecimen:Blood

concentration_value:660

concentration_units:uM

patient_age:Adolescent (13-18 years old)

patient_sex:Female

patient_information:3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)

7:

biospecimen:Blood

concentration_value:200-2800

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Female

patient_information:3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)

8:

biospecimen:Blood

concentration_value:770-2900

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)

9:

biospecimen:Blood

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Oesophageal cancer

10:

biospecimen:Blood

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Schizophrenia

11:

biospecimen:Blood

concentration_value:220

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Not Specified

patient_information:Very long-chain acyl-CoA dehydrogenase deficiency (vLCAD)

12:

biospecimen:Blood

concentration_value:80-200

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Not Specified

patient_information:Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

13:

biospecimen:Blood

concentration_value:700-1340

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Not Specified

patient_information:Medium chain acyl-CoA dehydrogenase deficiency (MCAD)

14:

biospecimen:Blood

concentration_value:1800

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Male

patient_information:Pyruvate dehydrogenase phosphatase deficiency

15:

biospecimen:Blood

concentration_value:<100

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Male

patient_information:Carnitine palmitoyltransferase deficiency I

16:

biospecimen:Blood

concentration_value:1500.0 +/- 100.0

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:Diabetes

17:

biospecimen:Blood

concentration_value:0-380

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Not Specified

patient_information:Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency

18:

biospecimen:Blood

concentration_value:1490-5040

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Not Specified

patient_information:Ketotic hypoglycemia

19:

biospecimen:Blood

concentration_value:10-1380

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Female

patient_information:3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)

20:

biospecimen:Blood

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal cancer

21:

biospecimen:Blood

concentration_value:35.5 +/- 92.2

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Diabetes

22:

biospecimen:Blood

concentration_value:17.6 +/- 3.84

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Schizophrenia

23:

biospecimen:Blood

concentration_value:7700.0 +/- 300.0

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:Diabetes

24:

biospecimen:Blood

concentration_value:54.57 +/- 64.53

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy with fetuses with trisomy 18

25:

biospecimen:Blood

concentration_value:32.9 +/- 53.42

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

26:

biospecimen:Blood

concentration_value:0-200

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Male

patient_information:3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)

27:

biospecimen:Blood

concentration_value:31.6 (19.1)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Early preeclampsia

28:

biospecimen:Blood

concentration_value:27.0 (16.2)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

29:

biospecimen:Blood

concentration_value:64

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Female

patient_information:3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency

30:

biospecimen:Blood

concentration_value:100

concentration_units:uM

patient_age:Newborn (0-30 days old)

patient_sex:Male

patient_information:2-Ketoglutarate dehydrogenase complex deficiency

31:

biospecimen:Blood

concentration_value:120-2000

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Male

patient_information:2-Ketoglutarate dehydrogenase complex deficiency

32:

biospecimen:Blood

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Pancreatic cancer

33:

biospecimen:Blood

concentration_value:33.63 (42.57)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy with fetus having congenital heart defect

34:

biospecimen:Blood

concentration_value:30.15 (37.69)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

35:

biospecimen:Blood

concentration_value:29.61(44.27)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Heart failure with reduced ejection fraction

36:

biospecimen:Blood

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Schizophrenia

37:

biospecimen:Blood

concentration_value:49.9 (46.7)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Late-onset preeclampsia

38:

biospecimen:Blood

concentration_value:29.7 (19.1)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

39:

biospecimen:Blood

concentration_value:130 +/- 80

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency

40:

biospecimen:Blood

concentration_value:300 +/- 390

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency

41:

biospecimen:Blood

concentration_value:300 +/- 200

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency

42:

biospecimen:Blood

concentration_value:250 +/- 160

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency

43:

biospecimen:Blood

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:By NMR. Significantly decreased vs control.

44:

biospecimen:Blood

concentration_value:1400.0 +/- 100.0

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Diabetes

45:

biospecimen:Blood

concentration_value:7700.0 +/- 200.0

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Diabetes

46:

biospecimen:Blood

concentration_value:5700 (4300-6000)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Diabetic ketoacidosis

47:

biospecimen:Blood

concentration_value:20-50

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Male

patient_information:3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency

48:

biospecimen:Cellular Cytoplasm

concentration_value:2600 (2500-2700)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Anoxia

49:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:430 (359-501)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Meningitis

50:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:6360 (5350-7370)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Diabetes

51:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal Cancer

52:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Ulcerative colitis

53:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Crohn disease

54:

biospecimen:Saliva

concentration_value:5.04 +/- 3.02

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Tooth alignment disorders

55:

biospecimen:Saliva

concentration_value:6.62 +/- 0.72

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:Alzheimer's disease

56:

biospecimen:Saliva

concentration_value:6.37 +/- 1.75

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:Frontotemporal lobe dementia

57:

biospecimen:Saliva

concentration_value:24.25 +/- 32.08

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Lewy body disease

58:

biospecimen:Urine

concentration_value:11.946

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Medium Chain Acyl-CoA Dehydrogenase Deficiency

59:

biospecimen:Urine

concentration_value:16.29

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Medium Chain Acyl-CoA Dehydrogenase Deficiency

60:

biospecimen:Urine

concentration_value:27.151

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Medium Chain Acyl-CoA Dehydrogenase Deficiency

61:

biospecimen:Urine

concentration_value:31.495

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Medium Chain Acyl-CoA Dehydrogenase Deficiency

62:

biospecimen:Urine

concentration_value:56.473

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Medium Chain Acyl-CoA Dehydrogenase Deficiency

63:

biospecimen:Urine

concentration_value:351.872

concentration_units:umol/mmol creatinine

patient_age:Infant (0-1 year old)

patient_sex:Male

patient_information:Fumarase deficiency

64:

biospecimen:Urine

concentration_value:45000

concentration_units:umol/mmol creatinine

patient_age:Children (1-13 years old)

patient_sex:

patient_information:Pyruvate carboxylase deficiency

65:

biospecimen:Urine

concentration_value:8359

concentration_units:umol/mmol creatinine

patient_age:Infant (0-1 year old)

patient_sex:

patient_information:Pyruvate carboxylase deficiency

66:

biospecimen:Urine

concentration_value:3-1050

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Both

patient_information:Medium Chain Acyl-CoA Dehydrogenase Deficiency

67:

biospecimen:Urine

concentration_value:55.399-1634.282

concentration_units:umol/mmol creatinine

patient_age:Children (1-13 years old)

patient_sex:Not Specified

patient_information:Medium Chain Acyl-CoA Dehydrogenase Deficiency

68:

biospecimen:Urine

concentration_value:170-390

concentration_units:umol/mmol creatinine

patient_age:Children (1-13 years old)

patient_sex:Male

patient_information:3-Hydroxyisobutyric acid dehydrogenase deficiency

69:

biospecimen:Urine

concentration_value:35.352 +/- 40.756

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Eosinophilic esophagitis

70:

biospecimen:Urine

concentration_value:51.0431-56.473

concentration_units:umol/mmol creatinine

patient_age:Infant (0-1 year old)

patient_sex:Not Specified

patient_information:3-Hydroxydicarboxylic aciduria

71:

biospecimen:Urine

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Bladder cancer

72:

biospecimen:Urine

concentration_value:224

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Diabetes

73:

biospecimen:Urine

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Schizophrenia

74:

biospecimen:Urine

concentration_value:30.883 +/- 22.266

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Eosinophilic esophagitis

75:

biospecimen:Urine

concentration_value:74.311 +/- 155.898

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Gastroesophageal reflux disease

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26:

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27:

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28:

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31:

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KEGG数据库编号
C01089
DrugBank数据库编号
foodb数据库编号
FDB021869
ChemSpider数据库编号
83181
公共化学化合物编号
92135
PDB数据库编号
生物利益的化学实体数据库编号
17066
knapsack数据库编号
维基百科编号
Beta-Hydroxybutyric_acid
代谢途径的数据库编号
CPD-335
苯酚资源管理器化合物数据库编号
比格数据库编号
36784
梅林编号
虚拟机身份证编号
BHB
fbonto数据库编号
综合参考
Le Sann, Christine; Munoz, Dulce M.; Saunders, Natalie; Simpson, Thomas J.; Smith, David I.; Soulas, Florilene; Watts, Paul; Willis, Christine L. Assembly intermediates in polyketide biosynthesis: enantioselective syntheses of b-hydroxycarbonyl compounds. Org Biomol Chem. 2005 May 7;3(9):1719-28. Epub 2005 Mar 31. Pubmed: 15858656
一般参考
1:

protein_accession:HMDBP00355

name:Sulfotransferase family cytosolic 2B member 1

uniprot_id:O00204

gene_name:SULT2B1

protein_type:Enzyme

2:

protein_accession:HMDBP00483

name:UDP-glucuronosyltransferase 1-1

uniprot_id:P22309

gene_name:UGT1A1

protein_type:Enzyme

3:

protein_accession:HMDBP00540

name:D-beta-hydroxybutyrate dehydrogenase, mitochondrial

uniprot_id:Q02338

gene_name:BDH1

protein_type:Unknown

4:

protein_accession:HMDBP07259

name:3-hydroxybutyrate dehydrogenase type 2

uniprot_id:Q9BUT1

gene_name:BDH2

protein_type:Unknown

5:

protein_accession:HMDBP09102

name:Novel protein similar to 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (Hydroxymethylglutaricaciduria)

uniprot_id:Q9NT06

gene_name:DKFZp434G1411

protein_type:Enzyme

蛋白质结合
1:

reference_text:Pan JW, Rothman TL, Behar KL, Stein DT, Hetherington HP: Human brain beta-hydroxybutyrate and lactate increase in fasting-induced ketosis. J Cereb Blood Flow Metab. 2000 Oct;20(10):1502-7.

pubmed_id:11043913

2:

reference_text:Pan JW, Telang FW, Lee JH, de Graaf RA, Rothman DL, Stein DT, Hetherington HP: Measurement of beta-hydroxybutyrate in acute hyperketonemia in human brain. J Neurochem. 2001 Nov;79(3):539-44.

pubmed_id:11701757

3:

reference_text:Plecko B, Stoeckler-Ipsiroglu S, Schober E, Harrer G, Mlynarik V, Gruber S, Moser E, Moeslinger D, Silgoner H, Ipsiroglu O: Oral beta-hydroxybutyrate supplementation in two patients with hyperinsulinemic hypoglycemia: monitoring of beta-hydroxybutyrate levels in blood and cerebrospinal fluid, and in the brain by in vivo magnetic resonance spectroscopy. Pediatr Res. 2002 Aug;52(2):301-6.

pubmed_id:12149510

4:

reference_text:Raunio RP, Leivo PV, Kuusinen AM: Bioluminescent assay of D-3-hydroxybutyrate in serum. J Biolumin Chemilumin. 1986 Jun;1(1):11-4.

pubmed_id:3503522

5:

reference_text:Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38.

pubmed_id:2026685

6:

reference_text:Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69.

pubmed_id:8412012

7:

reference_text:Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36.

pubmed_id:8579834

8:

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

9:

reference_text:Frenkel G, Peterson RN, Freund M: Oxidative and glycolytic metabolism of semen components by washed guinea pig spermatozoa. Fertil Steril. 1975 Feb;26(2):144-7.

pubmed_id:1126459

10:

reference_text:Abrahamsson K, Eriksson BO, Holme E, Jodal U, Jonsson A, Lindstedt S: Pivalic acid-induced carnitine deficiency and physical exercise in humans. Metabolism. 1996 Dec;45(12):1501-7.

pubmed_id:8969283

11:

reference_text:Teresinski G, Buszewicz G, Madro R: The influence of ethanol on the level of ketone bodies in hypothermia. Forensic Sci Int. 2002 Jun 25;127(1-2):88-96.

pubmed_id:12098531

12:

reference_text:Nalecz KA, Miecz D, Berezowski V, Cecchelli R: Carnitine: transport and physiological functions in the brain. Mol Aspects Med. 2004 Oct-Dec;25(5-6):551-67.

pubmed_id:15363641

13:

reference_text:Blomqvist G, Alvarsson M, Grill V, Von Heijne G, Ingvar M, Thorell JO, Stone-Elander S, Widen L, Ekberg K: Effect of acute hyperketonemia on the cerebral uptake of ketone bodies in nondiabetic subjects and IDDM patients. Am J Physiol Endocrinol Metab. 2002 Jul;283(1):E20-8.

pubmed_id:12067838

14:

reference_text:Krotkiewski M: Value of VLCD supplementation with medium chain triglycerides. Int J Obes Relat Metab Disord. 2001 Sep;25(9):1393-400.

pubmed_id:11571605

15:

reference_text:Kamijo T, Indo Y, Souri M, Aoyama T, Hara T, Yamamoto S, Ushikubo S, Rinaldo P, Matsuda I, Komiyama A, Hashimoto T: Medium chain 3-ketoacyl-coenzyme A thiolase deficiency: a new disorder of mitochondrial fatty acid beta-oxidation. Pediatr Res. 1997 Nov;42(5):569-76.

pubmed_id:9357925

16:

reference_text:Geary N, Grotschel H, Scharrer E: Blood metabolites and feeding during postinsulin hypophagia. Am J Physiol. 1982 Sep;243(3):R304-11.

pubmed_id:7051864

17:

reference_text:Yeh YY, Sheehan PM: Preferential utilization of ketone bodies in the brain and lung of newborn rats. Fed Proc. 1985 Apr;44(7):2352-8.

pubmed_id:3884391

18:

reference_text:Kita K, Ishimaru K, Teraoka M, Yanase H, Kato N: Properties of poly(3-hydroxybutyrate) depolymerase from a marine bacterium, Alcaligenes faecalis AE122. Appl Environ Microbiol. 1995 May;61(5):1727-30.

pubmed_id:7646009

19:

reference_text:Kim BS, Lee SC, Lee SY, Chang HN, Chang YK, Woo SI: Production of poly(3-hydroxybutyric acid) by fed-batch culture of Alcaligenes eutrophus with glucose concentration control. Biotechnol Bioeng. 1994 Apr 15;43(9):892-8. doi: 10.1002/bit.260430908.

pubmed_id:18615882

20:

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

21:

reference_text:Bernardi, R. et al., Chem. Commun., 1984, 460, (resoln)

22:

reference_text:Encyclopedia of Food and Color Additives, (ed. Burdock, G.A.), CRC Press, 1997, 994-995, (Et ester)

23:

reference_text:Fenaroli's Handbook of Flavor Ingredients, 3rd edn., (ed. Burdock, G.A.), CRC Press, 1995, 1, 238, (Et ester)

24:

reference_text:Forsyth, W.G.C. et al., Nature (London), 1958, 182, 800, (isol)

25:

reference_text:Gottschalk, G. et al., Nature (London), 1965, 205, 308

26:

reference_text:Krajewski, D. et al., Phytochemistry, 1997, 45, 1627, (ester glucosides)

27:

reference_text:Levene, P.A. et al., J. Biol. Chem., 1926, 68, 418, (abs config)

28:

reference_text:Lewis, R.J., Sax's Dangerous Properties of Industrial Materials, 8th edn., Van Nostrand Reinhold, 1992, MEF500 MKP250

29:

reference_text:Marchessault, R.H. et al., Makromol. Chem., Macromol. Symp., 1988, 19, 235, (rev, polymers)

30:

reference_text:Muller, H.-M. et al., Angew. Chem., Int. Ed., 1993, 32, 477, (rev, polymer)

31:

reference_text:Org. Synth., 1993, 71, 39, (synth, acid, Meester, pmr, ir)

32:

reference_text:Quang DN, Hashimoto T, Toyota M, Asakawa Y. Occurrence of a high concentration of spider pheromones in the ascomycete fungus Hypoxylon truncatum. J Nat Prod. 2003 Dec;66(12):1613-4.

33:

reference_text:Schulz S, Toft S. Identification of a sex pheromone from a spider. Science. 1993 Jun 11;260(5114):1635-7.

34:

reference_text:Seebach, D. et al., Helv. Chim. Acta, 1982, 65, 495-503, (esters, synth)

35:

reference_text:Seebach, D. et al., Helv. Chim. Acta, 1988, 71, 155, (synth, dimer)

36:

reference_text:Wipf, B. et al., Helv. Chim. Acta, 1983, 66, 485

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