版本
5.0
创建日期
2005-11-16 15:48:42 UTC
更新日期
2020-11-09 23:12:46 UTC
登录号
HMDB0000208
地位
quantified
二级访问
HMDB0002812

HMDB00208

HMDB0062781

HMDB02812

HMDB62781

名称
Oxoglutaric acid
描述
Oxoglutaric acid, also known as alpha-ketoglutarate, alpha-ketoglutaric acid, AKG, or 2-oxoglutaric acid, is classified as a gamma-keto acid or a gamma-keto acid derivative. gamma-Keto acids are organic compounds containing an aldehyde substituted with a keto group on the C4 carbon atom. alpha-Ketoglutarate is considered to be soluble (in water) and acidic. alpha-Ketoglutarate is a key molecule in the TCA cycle, playing a fundamental role in determining the overall rate of this important metabolic process (PMID: 26759695). In the TCA cycle, AKG is decarboxylated to succinyl-CoA and carbon dioxide by AKG dehydrogenase, which functions as a key control point of the TCA cycle. Additionally, AKG can be generated from isocitrate by oxidative decarboxylation catalyzed by the enzyme known as isocitrate dehydrogenase (IDH). In addition to these routes of production, AKG can be produced from glutamate by oxidative deamination via glutamate dehydrogenase, and as a product of pyridoxal phosphate-dependent transamination reactions (mediated by branched-chain amino acid transaminases) in which glutamate is a common amino donor. AKG is a nitrogen scavenger and a source of glutamate and glutamine that stimulates protein synthesis and inhibits protein degradation in muscles. In particular, AKG can decrease protein catabolism and increase protein synthesis to enhance bone tissue formation in skeletal muscles (PMID: 26759695). Interestingly, enteric feeding of AKG supplements can significantly increase circulating plasma levels of hormones such as insulin, growth hormone, and insulin-like growth factor-1 (PMID: 26759695). It has recently been shown that AKG can extend the lifespan of adult C. elegans by inhibiting ATP synthase and TOR (PMID: 24828042). In combination with molecular oxygen, alpha-ketoglutarate is required for the hydroxylation of proline to hydroxyproline in the production of type I collagen. A recent study has shown that alpha-ketoglutarate promotes TH1 differentiation along with the depletion of glutamine thereby favouring Treg (regulatory T-cell) differentiation (PMID: 26420908). alpha-Ketoglutarate has been found to be associated with fumarase deficiency, 2-ketoglutarate dehydrogenase complex deficiency, and D-2-hydroxyglutaric aciduria, which are all inborn errors of metabolism (PMID: 8338207). Oxoglutaric acid has been found to be a metabolite produced by Corynebacterium and yeast (PMID: 27872963) (YMDB).
同义词
1:2-Ketoglutaric acid

2:alpha-Ketoglutaric acid

3:2-Ketoglutarate

4:a-Ketoglutarate

5:a-Ketoglutaric acid

6:alpha-Ketoglutarate

7:Α-ketoglutarate

8:Α-ketoglutaric acid

9:Oxoglutarate

10:2-oxo-1,5-Pentanedioate

11:2-oxo-1,5-Pentanedioic acid

12:2-Oxoglutarate

13:2-Oxoglutaric acid

14:2-Oxopentanedioate

15:2-Oxopentanedioic acid

16:alpha-Oxoglutarate

17:Oxogluric acid

18:2 Oxoglutaric acid

19:Ketoglutaric acid

20:alpha Ketoglutaric acid

21:2 Ketoglutaric acid

22:alpha Ketoglutarate

23:alpha Oxoglutarate

24:2 Ketoglutarate

25:2 Oxoglutarate

26:alpha-Oxoglutaric acid

27:alpha-Oxopentanedioic acid

28:alpha-Keto-glutaric acid

29:Α-oxoglutaric acid

30:Α-oxopentanedioic acid

31:Α-keto-glutaric acid

化学式
C5H6O5
平均分子量
146.0981
等位分子量
146.021523302
化学名称
2-oxopentanedioic acid
传统名称
oxoglutarate
CAS 登记号
328-50-7
代谢物结构字符串
OC(=O)CCC(=O)C(O)=O
inchi标识符
InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)
印记键
KPGXRSRHYNQIFN-UHFFFAOYSA-N
分类学
description: belongs to the class of organic compounds known as gamma-keto acids and derivatives. These are organic compounds containing an aldehyde substituted with a keto group on the C4 carbon atom.

description:Gamma-keto acids and derivatives

kingdom:Organic compounds

super_class:Organic acids and derivatives

class:Keto acids and derivatives

sub_class:Gamma-keto acids and derivatives

molecular_framework:Aliphatic acyclic compounds

1:Alpha-hydroxy ketones

2:Alpha-keto acids and derivatives

3:Carboxylic acids

4:Dicarboxylic acids and derivatives

5:Hydrocarbon derivatives

6:Organic oxides

7:Short-chain keto acids and derivatives

8:Aliphatic acyclic compound

9:Alpha-hydroxy ketone

10:Alpha-keto acid

11:Carbonyl group

12:Carboxylic acid

13:Carboxylic acid derivative

14:Dicarboxylic acid or derivatives

15:Gamma-keto acid

16:Hydrocarbon derivative

17:Ketone

18:Organic oxide

19:Organic oxygen compound

20:Organooxygen compound

21:Short-chain keto acid

22:oxo dicarboxylic acid

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:Anoxia

definition:

parent_id:7695

***level:4

type:child

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:Route of exposure

definition:A mean by which a chemical agent comes in contact with an organism, either under intended or unintended circumstances.

parent_id:7724

*level:2

type:parent

term:Enteral

definition:Chemical exposure via the alimentary canal (mouth to anus).

parent_id:7743

**level:3

type:parent

term:Ingestion

definition:Chemical exposure facilitated by entry through the mouth.

parent_id:7744

***level:4

type:child

synonym:Digestion

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:Food

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:Animal

definition:A living organism belonging to the kingdom animalia. it feeds on organic matter, typically having specialized sense organs and nervous system and able to respond rapidly to stimuli.

parent_id:7736

***level:4

type:child

synonym:Fauna

term:Fungi

definition:Living eukaryotic organisms belonging to the kingdom fungi. they include mushrooms as well as microbes such as yeast and mold.

parent_id:7736

***level:4

type:parent

synonym:Fungus

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:Plant

definition:A living organism belonging to the kingdom plantea. typically, it grows in a permanent site, absorbs water and inorganic substances through its roots, and synthesizes nutrients in its leaves by photosynthesis using the green pigment chlorophyll. examples incude trees, shrubs, herbs, grasses, ferns, and mosses.

parent_id:7736

***level:4

type:parent

synonym:Flora

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: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:Breast milk

definition:Portion of secreted substance produced by the mammary 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: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:Subcellular

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

parent_id:7725

**level:3

type:parent

term:Mitochondria

definition:

parent_id:7730

***level:4

type:child

term:Endoplasmic reticulum

definition:A network of tubular membranes within the cytoplasm of the cell, occurring either with a smooth surface (smooth endoplasmic reticulum) or studded with ribosomes (rough endoplasmic reticulum), involved in the transport of materials. (Infoplease Dictionary via NCI)

parent_id:7730

***level:4

type:child

synonym:Er

term:Peroxisome

definition:A small organelle enclosed by a single membrane, and found in most eukaryotic cells. Contains peroxidases and other enzymes involved in a variety of metabolic processes including free radical detoxification, lipid catabolism and biosynthesis, and hydrogen peroxide metabolism. (Gene Ontology).

parent_id:7730

***level:4

type:child

synonym:Peroxisome vesicle

synonym:Peroxisomal

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: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

term:Industrial application

definition:The assumed function of a chemical utilized by human.

parent_id:7671

*level:2

type:parent

term:Food and nutrition

definition:A chemical purpose related to manufacturing of food and nutrition products.

parent_id:7678

**level:3

type:child

term:Pharmaceutical industry

definition:A pharmacologic activity for which a chemical substance is utilized owing to its biological role.

parent_id:7678

**level:3

type:parent

term:Pharmaceutical

definition:

parent_id:7679

***level:4

type:child

状态
Solid
实验性质
1:

kind:water_solubility

value:541.5 mg/mL

source:

2:

kind:melting_point

value:115.5 °C

source:

预测性质
1:

kind:logp

value:-0.60

source:ALOGPS

2:

kind:logs

value:-0.44

source:ALOGPS

3:

kind:solubility

value:53.1 g/L

source:ALOGPS

4:

kind:logp

value:-0.11

source:ChemAxon

5:

kind:pka_strongest_acidic

value:2.66

source:ChemAxon

6:

kind:pka_strongest_basic

value:-9.7

source:ChemAxon

7:

kind:iupac

value:2-oxopentanedioic acid

source:ChemAxon

8:

kind:average_mass

value:146.0981

source:ChemAxon

9:

kind:mono_mass

value:146.021523302

source:ChemAxon

10:

kind:smiles

value:OC(=O)CCC(=O)C(O)=O

source:ChemAxon

11:

kind:formula

value:C5H6O5

source:ChemAxon

12:

kind:inchi

value:InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)

source:ChemAxon

13:

kind:inchikey

value:KPGXRSRHYNQIFN-UHFFFAOYSA-N

source:ChemAxon

14:

kind:polar_surface_area

value:91.67

source:ChemAxon

15:

kind:refractivity

value:28.88

source:ChemAxon

16:

kind:polarizability

value:12.17

source:ChemAxon

17:

kind:rotatable_bond_count

value:4

source:ChemAxon

18:

kind:acceptor_count

value:5

source:ChemAxon

19:

kind:donor_count

value:2

source:ChemAxon

20:

kind:physiological_charge

value:-2

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:1207

2:

type:Specdb::CMs

spectrum_id:464

3:

type:Specdb::CMs

spectrum_id:465

4:

type:Specdb::CMs

spectrum_id:466

5:

type:Specdb::CMs

spectrum_id:1231

6:

type:Specdb::CMs

spectrum_id:1263

7:

type:Specdb::CMs

spectrum_id:2723

8:

type:Specdb::CMs

spectrum_id:30238

9:

type:Specdb::CMs

spectrum_id:30479

10:

type:Specdb::CMs

spectrum_id:30912

11:

type:Specdb::CMs

spectrum_id:31073

12:

type:Specdb::CMs

spectrum_id:31074

13:

type:Specdb::CMs

spectrum_id:31910

14:

type:Specdb::CMs

spectrum_id:37357

15:

type:Specdb::CMs

spectrum_id:146405

16:

type:Specdb::NmrOneD

spectrum_id:1192

17:

type:Specdb::NmrOneD

spectrum_id:1202

18:

type:Specdb::NmrOneD

spectrum_id:2337

19:

type:Specdb::NmrOneD

spectrum_id:3037

20:

type:Specdb::NmrOneD

spectrum_id:4779

21:

type:Specdb::NmrOneD

spectrum_id:142870

22:

type:Specdb::NmrOneD

spectrum_id:142871

23:

type:Specdb::NmrOneD

spectrum_id:142872

24:

type:Specdb::NmrOneD

spectrum_id:142873

25:

type:Specdb::NmrOneD

spectrum_id:142874

26:

type:Specdb::NmrOneD

spectrum_id:142875

27:

type:Specdb::NmrOneD

spectrum_id:142876

28:

type:Specdb::NmrOneD

spectrum_id:142877

29:

type:Specdb::NmrOneD

spectrum_id:142878

30:

type:Specdb::NmrOneD

spectrum_id:142879

31:

type:Specdb::NmrOneD

spectrum_id:142880

32:

type:Specdb::NmrOneD

spectrum_id:142881

33:

type:Specdb::NmrOneD

spectrum_id:142882

34:

type:Specdb::NmrOneD

spectrum_id:142883

35:

type:Specdb::NmrOneD

spectrum_id:142884

36:

type:Specdb::NmrOneD

spectrum_id:142885

37:

type:Specdb::NmrOneD

spectrum_id:142886

38:

type:Specdb::NmrOneD

spectrum_id:142887

39:

type:Specdb::NmrOneD

spectrum_id:142888

40:

type:Specdb::NmrOneD

spectrum_id:142889

41:

type:Specdb::EiMs

spectrum_id:998

42:

type:Specdb::MsMs

spectrum_id:337

43:

type:Specdb::MsMs

spectrum_id:338

44:

type:Specdb::MsMs

spectrum_id:339

45:

type:Specdb::MsMs

spectrum_id:3615

46:

type:Specdb::MsMs

spectrum_id:3616

47:

type:Specdb::MsMs

spectrum_id:3617

48:

type:Specdb::MsMs

spectrum_id:3618

49:

type:Specdb::MsMs

spectrum_id:3619

50:

type:Specdb::MsMs

spectrum_id:3620

51:

type:Specdb::MsMs

spectrum_id:3621

52:

type:Specdb::MsMs

spectrum_id:3622

53:

type:Specdb::MsMs

spectrum_id:3623

54:

type:Specdb::MsMs

spectrum_id:3624

55:

type:Specdb::MsMs

spectrum_id:3625

56:

type:Specdb::MsMs

spectrum_id:3626

57:

type:Specdb::MsMs

spectrum_id:20237

58:

type:Specdb::MsMs

spectrum_id:20238

59:

type:Specdb::MsMs

spectrum_id:20239

60:

type:Specdb::MsMs

spectrum_id:20354

61:

type:Specdb::MsMs

spectrum_id:20355

62:

type:Specdb::MsMs

spectrum_id:20356

63:

type:Specdb::MsMs

spectrum_id:21788

64:

type:Specdb::MsMs

spectrum_id:21789

65:

type:Specdb::MsMs

spectrum_id:21790

66:

type:Specdb::MsMs

spectrum_id:21905

光谱
生物学性质
1:

cellular:(1):Endoplasmic reticulum

cellular:(2):Extracellular

cellular:(3):Mitochondria

cellular:(4):Peroxisome

2:

biospecimen:(1):Blood

biospecimen:(2):Breast Milk

biospecimen:(3):Cellular Cytoplasm

biospecimen:(4):Cerebrospinal Fluid (CSF)

biospecimen:(5):Feces

biospecimen:(6):Saliva

biospecimen:(7):Urine

3:

tissue:(1):Placenta

4:

[1]:

name:2-aminoadipic 2-oxoadipic aciduria

smpdb_id:SMP00719

kegg_map_id:

[2]:

name:2-Hydroxyglutric Aciduria (D And L Form)

smpdb_id:SMP00136

kegg_map_id:

[3]:

name:2-ketoglutarate dehydrogenase complex deficiency

smpdb_id:SMP00549

kegg_map_id:

[4]:

name:2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency

smpdb_id:SMP00137

kegg_map_id:

[5]:

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

smpdb_id:SMP00138

kegg_map_id:

[6]:

name:3-hydroxyisobutyric acid dehydrogenase deficiency

smpdb_id:SMP00521

kegg_map_id:

[7]:

name:3-hydroxyisobutyric aciduria

smpdb_id:SMP00522

kegg_map_id:

[8]:

name:3-Methylcrotonyl Coa Carboxylase Deficiency Type I

smpdb_id:SMP00237

kegg_map_id:

[9]:

name:3-Methylglutaconic Aciduria Type I

smpdb_id:SMP00139

kegg_map_id:

[10]:

name:3-Methylglutaconic Aciduria Type III

smpdb_id:SMP00140

kegg_map_id:

[11]:

name:3-Methylglutaconic Aciduria Type IV

smpdb_id:SMP00141

kegg_map_id:

[12]:

name:3-Phosphoglycerate dehydrogenase deficiency

smpdb_id:SMP00721

kegg_map_id:

[13]:

name:4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency

smpdb_id:SMP00243

kegg_map_id:

[14]:

name:Alanine, aspartate and glutamate metabolism

smpdb_id:

kegg_map_id:map00250

[15]:

name:Alkaptonuria

smpdb_id:SMP00169

kegg_map_id:

[16]:

name:Arginine and proline metabolism

smpdb_id:

kegg_map_id:map00330

[17]:

name:Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)

smpdb_id:SMP00362

kegg_map_id:

[18]:

name:Argininemia

smpdb_id:SMP00357

kegg_map_id:

[19]:

name:Argininosuccinic Aciduria

smpdb_id:SMP00003

kegg_map_id:

[20]:

name:Aspartate Metabolism

smpdb_id:SMP00067

kegg_map_id:map00250

[21]:

name:beta-Alanine metabolism

smpdb_id:

kegg_map_id:map00410

[22]:

name:Beta-Ketothiolase Deficiency

smpdb_id:SMP00173

kegg_map_id:

[23]:

name:Beta-mercaptolactate-cysteine disulfiduria

smpdb_id:SMP00499

kegg_map_id:

[24]:

name:Canavan Disease

smpdb_id:SMP00175

kegg_map_id:

[25]:

name:Carbamoyl Phosphate Synthetase Deficiency

smpdb_id:SMP00002

kegg_map_id:

[26]:

name:Carnitine Synthesis

smpdb_id:SMP00465

kegg_map_id:

[27]:

name:Carnosinuria, carnosinemia

smpdb_id:SMP00493

kegg_map_id:

[28]:

name:Citric Acid Cycle

smpdb_id:SMP00057

kegg_map_id:map00020

[29]:

name:Citrullinemia Type I

smpdb_id:SMP00001

kegg_map_id:

[30]:

name:Congenital lactic acidosis

smpdb_id:SMP00546

kegg_map_id:

[31]:

name:Creatine deficiency, guanidinoacetate methyltransferase deficiency

smpdb_id:SMP00504

kegg_map_id:

[32]:

name:Cysteine Metabolism

smpdb_id:SMP00013

kegg_map_id:map00270

[33]:

name:Cystinosis, ocular nonnephropathic

smpdb_id:SMP00722

kegg_map_id:

[34]:

name:Dihydropyrimidine Dehydrogenase Deficiency (DHPD)

smpdb_id:SMP00179

kegg_map_id:

[35]:

name:Dimethylglycine Dehydrogenase Deficiency

smpdb_id:SMP00242

kegg_map_id:

[36]:

name:Dimethylglycine Dehydrogenase Deficiency

smpdb_id:SMP00484

kegg_map_id:

[37]:

name:Disulfiram Action Pathway

smpdb_id:SMP00429

kegg_map_id:

[38]:

name:Dopamine beta-hydroxylase deficiency

smpdb_id:SMP00498

kegg_map_id:

[39]:

name:Fructose-1,6-diphosphatase deficiency

smpdb_id:SMP00562

kegg_map_id:

[40]:

name:Fumarase deficiency

smpdb_id:SMP00547

kegg_map_id:

[41]:

name:GABA-Transaminase Deficiency

smpdb_id:SMP00351

kegg_map_id:

[42]:

name:Glucose-Alanine Cycle

smpdb_id:SMP00127

kegg_map_id:

[43]:

name:Glutamate Metabolism

smpdb_id:SMP00072

kegg_map_id:map00250

[44]:

name:Glutaminolysis and Cancer

smpdb_id:SMP02298

kegg_map_id:

[45]:

name:Glutaric Aciduria Type I

smpdb_id:SMP00186

kegg_map_id:

[46]:

name:Glycine, serine and threonine metabolism

smpdb_id:

kegg_map_id:map00260

[47]:

name:Glycogen Storage Disease Type 1A (GSD1A) or Von Gierke Disease

smpdb_id:SMP00374

kegg_map_id:

[48]:

name:Glycogenosis, Type IA. Von gierke disease

smpdb_id:SMP00581

kegg_map_id:

[49]:

name:Glycogenosis, Type IB

smpdb_id:SMP00573

kegg_map_id:

[50]:

name:Glycogenosis, Type IC

smpdb_id:SMP00574

kegg_map_id:

[51]:

name:Glycolysis / Gluconeogenesis

smpdb_id:

kegg_map_id:map00010

[52]:

name:Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)

smpdb_id:SMP00188

kegg_map_id:

[53]:

name:Hawkinsinuria

smpdb_id:SMP00190

kegg_map_id:

[54]:

name:Homocarnosinosis

smpdb_id:SMP00385

kegg_map_id:

[55]:

name:Hyperglycinemia, non-ketotic

smpdb_id:SMP00485

kegg_map_id:

[56]:

name:Hyperinsulinism-Hyperammonemia Syndrome

smpdb_id:SMP00339

kegg_map_id:

[57]:

name:Hyperlysinemia I, Familial

smpdb_id:SMP00527

kegg_map_id:

[58]:

name:Hyperlysinemia II or Saccharopinuria

smpdb_id:SMP00528

kegg_map_id:

[59]:

name:Hyperornithinemia with gyrate atrophy (HOGA)

smpdb_id:SMP00505

kegg_map_id:

[60]:

name:Hyperornithinemia-hyperammonemia-homocitrullinuria [HHH-syndrome]

smpdb_id:SMP00506

kegg_map_id:

[61]:

name:Hyperprolinemia Type I

smpdb_id:SMP00361

kegg_map_id:

[62]:

name:Hyperprolinemia Type II

smpdb_id:SMP00360

kegg_map_id:

[63]:

name:Hypoacetylaspartia

smpdb_id:SMP00192

kegg_map_id:

[64]:

name:Isobutyryl-coa dehydrogenase deficiency

smpdb_id:SMP00523

kegg_map_id:

[65]:

name:Isovaleric acidemia

smpdb_id:SMP00524

kegg_map_id:

[66]:

name:Isovaleric Aciduria

smpdb_id:SMP00238

kegg_map_id:

[67]:

name:L-arginine:glycine amidinotransferase deficiency

smpdb_id:SMP00507

kegg_map_id:

[68]:

name:Lactic Acidemia

smpdb_id:SMP00313

kegg_map_id:

[69]:

name:Lysine degradation

smpdb_id:

kegg_map_id:map00310

[70]:

name:Malate-Aspartate Shuttle

smpdb_id:SMP00129

kegg_map_id:

[71]:

name:Malonic Aciduria

smpdb_id:SMP00198

kegg_map_id:

[72]:

name:Malonyl-coa decarboxylase deficiency

smpdb_id:SMP00502

kegg_map_id:

[73]:

name:Maple Syrup Urine Disease

smpdb_id:SMP00199

kegg_map_id:

[74]:

name:Methylmalonate Semialdehyde Dehydrogenase Deficiency

smpdb_id:SMP00384

kegg_map_id:

[75]:

name:Methylmalonic Aciduria

smpdb_id:SMP00200

kegg_map_id:

[76]:

name:Methylmalonic Aciduria Due to Cobalamin-Related Disorders

smpdb_id:SMP00201

kegg_map_id:

[77]:

name:Mitochondrial complex II deficiency

smpdb_id:SMP00548

kegg_map_id:

[78]:

name:Monoamine oxidase-a deficiency (MAO-A)

smpdb_id:SMP00533

kegg_map_id:

[79]:

name:Nitrogen metabolism

smpdb_id:

kegg_map_id:map00910

[80]:

name:Non Ketotic Hyperglycinemia

smpdb_id:SMP00223

kegg_map_id:

[81]:

name:Ornithine Aminotransferase Deficiency (OAT Deficiency)

smpdb_id:SMP00363

kegg_map_id:

[82]:

name:Ornithine Transcarbamylase Deficiency (OTC Deficiency)

smpdb_id:SMP00205

kegg_map_id:

[83]:

name:Oxidation of Branched Chain Fatty Acids

smpdb_id:SMP00030

kegg_map_id:

[84]:

name:Phenylalanine and Tyrosine Metabolism

smpdb_id:SMP00008

kegg_map_id:map00360

[85]:

name:Phenylketonuria

smpdb_id:SMP00206

kegg_map_id:

[86]:

name:Phosphoenolpyruvate carboxykinase deficiency 1 (PEPCK1)

smpdb_id:SMP00560

kegg_map_id:

[87]:

name:Phytanic Acid Peroxisomal Oxidation

smpdb_id:SMP00450

kegg_map_id:

[88]:

name:Primary Hyperoxaluria Type I

smpdb_id:SMP00352

kegg_map_id:

[89]:

name:Prolidase Deficiency (PD)

smpdb_id:SMP00207

kegg_map_id:

[90]:

name:Prolinemia Type II

smpdb_id:SMP00208

kegg_map_id:

[91]:

name:Propanoate metabolism

smpdb_id:

kegg_map_id:map00640

[92]:

name:Propionic Acidemia

smpdb_id:SMP00236

kegg_map_id:

[93]:

name:Pyridoxine dependency with seizures

smpdb_id:SMP00571

kegg_map_id:

[94]:

name:Pyruvate Carboxylase Deficiency

smpdb_id:SMP00350

kegg_map_id:

[95]:

name:Pyruvate dehydrogenase deficiency (E2)

smpdb_id:SMP00551

kegg_map_id:

[96]:

name:Pyruvate dehydrogenase deficiency (E3)

smpdb_id:SMP00550

kegg_map_id:

[97]:

name:Refsum Disease

smpdb_id:SMP00451

kegg_map_id:

[98]:

name:Saccharopinuria/Hyperlysinemia II

smpdb_id:SMP00239

kegg_map_id:

[99]:

name:Sarcosinemia

smpdb_id:SMP00244

kegg_map_id:

[100]:

name:Succinic semialdehyde dehydrogenase deficiency

smpdb_id:SMP00567

kegg_map_id:

[101]:

name:The oncogenic action of 2-hydroxyglutarate

smpdb_id:SMP02291

kegg_map_id:

[102]:

name:The oncogenic action of D-2-hydroxyglutarate in Hydroxygluaricaciduria

smpdb_id:SMP02359

kegg_map_id:

[103]:

name:The Oncogenic Action of Fumarate

smpdb_id:SMP02295

kegg_map_id:

[104]:

name:The oncogenic action of L-2-hydroxyglutarate in Hydroxygluaricaciduria

smpdb_id:SMP02358

kegg_map_id:

[105]:

name:The Oncogenic Action of Succinate

smpdb_id:SMP02292

kegg_map_id:

[106]:

name:Triosephosphate isomerase

smpdb_id:SMP00563

kegg_map_id:

[107]:

name:Tryptophan metabolism

smpdb_id:

kegg_map_id:map00380

[108]:

name:Tyrosine metabolism

smpdb_id:

kegg_map_id:map00350

[109]:

name:Tyrosinemia Type 2 (or Richner-Hanhart syndrome)

smpdb_id:SMP00369

kegg_map_id:

[110]:

name:Tyrosinemia Type 3 (TYRO3)

smpdb_id:SMP00370

kegg_map_id:

[111]:

name:Tyrosinemia Type I

smpdb_id:SMP00218

kegg_map_id:

[112]:

name:Tyrosinemia, transient, of the newborn

smpdb_id:SMP00494

kegg_map_id:

[113]:

name:Urea Cycle

smpdb_id:SMP00059

kegg_map_id:map00330

[114]:

name:Ureidopropionase Deficiency

smpdb_id:SMP00492

kegg_map_id:

[115]:

name:Valine, leucine and isoleucine degradation

smpdb_id:

kegg_map_id:map00280

[116]:

name:Warburg Effect

smpdb_id:SMP00654

kegg_map_id:

正常浓度
1:

biospecimen:Blood

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

2:

biospecimen:Blood

concentration_value:7.0 (0.0-23.0)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

3:

biospecimen:Blood

concentration_value:8.6 +/- 2.6

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

4:

biospecimen:Blood

concentration_value:9.3 +/- 2.3

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

5:

biospecimen:Blood

concentration_value:8.9 +/- 2.7

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

6:

biospecimen:Blood

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

7:

biospecimen:Blood

concentration_value:4-13

concentration_units:uM

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

8:

biospecimen:Blood

concentration_value:20.7 +/- 2.5

concentration_units:uM

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

9:

biospecimen:Blood

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

10:

biospecimen:Breast Milk

concentration_value:41.0 +/- 16.1

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

11:

biospecimen:Cellular Cytoplasm

concentration_value:320 (290-350)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

12:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:5 +/- 4

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

13:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:2.1 +/- 0.7

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

14:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:8.9 +/- 3.4

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

15:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:16-Feb

concentration_units:uM

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

16:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:2.0 +/- 2.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

17:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:4.80 (3.40-6.20)

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

18:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

19:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Infant (0-1 year old)

subject_sex:Both

subject_condition:Normal

20:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

21:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

22:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

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:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

25:

biospecimen:Saliva

concentration_value:5.27 +/- 3.61

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

26:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

27:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

28:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

29:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

30:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

31:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

32:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

33:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

34:

biospecimen:Saliva

concentration_value:1.53 +/- 1.18

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

35:

biospecimen:Saliva

concentration_value:1.64 +/- 3.47

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

36:

biospecimen:Saliva

concentration_value:2.65 +/- 1.07

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

37:

biospecimen:Saliva

concentration_value:2.65 +/- 1.07

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

38:

biospecimen:Saliva

concentration_value:3.78 +/- 2.33

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

39:

biospecimen:Urine

concentration_value:<110

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Female

subject_condition:Normal

40:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

41:

biospecimen:Urine

concentration_value:32.164 +/- 9.964

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

42:

biospecimen:Urine

concentration_value:30.79-398.2

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

43:

biospecimen:Urine

concentration_value:12-20

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

44:

biospecimen:Urine

concentration_value:90.83(45.64-331.1)

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Female

subject_condition:Normal

45:

biospecimen:Urine

concentration_value:<150

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

46:

biospecimen:Urine

concentration_value:104.8(30.21-413.8)

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Male

subject_condition:Normal

47:

biospecimen:Urine

concentration_value:2.87 (0.18-14.3)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

48:

biospecimen:Urine

concentration_value:2.98 (0.42-18.3)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

49:

biospecimen:Urine

concentration_value:23.6

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Not Specified

subject_condition:Normal

50:

biospecimen:Urine

concentration_value:29.893 +/- 15.594

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

51:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

52:

biospecimen:Urine

concentration_value:<60

concentration_units:umol/mmol creatinine

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

53:

biospecimen:Urine

concentration_value:<75

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

54:

biospecimen:Urine

concentration_value:<95

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Female

subject_condition:Normal

55:

biospecimen:Urine

concentration_value:<115

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Female

subject_condition:Normal

56:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

57:

biospecimen:Urine

concentration_value:<350

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Male

subject_condition:Normal

58:

biospecimen:Urine

concentration_value:0-25.3

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Male

subject_condition:Normal

59:

biospecimen:Urine

concentration_value:14.8-64.7

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Female

subject_condition:Normal

60:

biospecimen:Urine

concentration_value:4.3-36.3

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Male

subject_condition:Normal

61:

biospecimen:Urine

concentration_value:7.5-28.9

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Female

subject_condition:Normal

62:

biospecimen:Urine

concentration_value:8.90-17.12

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

63:

biospecimen:Urine

concentration_value:4.2 +/- 1.97

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

64:

biospecimen:Urine

concentration_value:24 (4-74)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

65:

biospecimen:Urine

concentration_value:150.5 (10.0-836.3)

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

66:

biospecimen:Urine

concentration_value:236.9 (8.0-631.4)

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Both

subject_condition:Normal

67:

biospecimen:Urine

concentration_value:21.4 (4.9-123.8)

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

68:

biospecimen:Urine

concentration_value:25.6 (10.2-146.0)

concentration_units:umol/mmol creatinine

subject_age:Adolescent (13-18 years old)

subject_sex:Both

subject_condition:Normal

69:

biospecimen:Urine

concentration_value:15.477-154.773

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Both

subject_condition:Normal

70:

biospecimen:Urine

concentration_value:7-11

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

71:

biospecimen:Urine

concentration_value:4.8 (2.0-17.0)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

72:

biospecimen:Urine

concentration_value:2.2-8.2

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

73:

biospecimen:Urine

concentration_value:0.083 +/- 0.002

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

74:

biospecimen:Urine

concentration_value:1.548-39.467

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Male

subject_condition:Normal

75:

biospecimen:Urine

concentration_value:43.46 +/- 53.46

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Both

subject_condition:Normal

76:

biospecimen:Urine

concentration_value:<79

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

77:

biospecimen:Urine

concentration_value:> 0.658

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

78:

biospecimen:Urine

concentration_value:160-590

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

79:

biospecimen:Urine

concentration_value:3.4 (1.4-12.5)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

异常浓度
1:

biospecimen:Blood

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Schizophrenia

2:

biospecimen:Blood

concentration_value:17-37

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:2-Ketoglutarate dehydrogenase complex deficiency

3:

biospecimen:Blood

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Schizophrenia

4:

biospecimen:Cellular Cytoplasm

concentration_value:150 (144-156)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Anoxia

5:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:3.600-12.800

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:2-Ketoglutarate dehydrogenase complex deficiency

6:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal Cancer

7:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Immunoglobulin A nephropathy (IgAN) progressor

8:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal cancer

9:

biospecimen:Urine

concentration_value:1374

concentration_units:umol/mmol creatinine

patient_age:Newborn (0-30 days old)

patient_sex:Female

patient_information:Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome

10:

biospecimen:Urine

concentration_value:28.849 +/- 8.834

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Eosinophilic esophagitis

11:

biospecimen:Urine

concentration_value:39.88 +/- 30.024

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Gastroesophageal reflux disease

12:

biospecimen:Urine

concentration_value:30-1600

concentration_units:umol/mmol creatinine

patient_age:Infant (0-1 year old)

patient_sex:Male

patient_information:2-Ketoglutarate dehydrogenase complex deficiency

13:

biospecimen:Urine

concentration_value:24.882 +/- 26.016

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Eosinophilic esophagitis

14:

biospecimen:Urine

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Schizophrenia

15:

biospecimen:Urine

concentration_value:100-166

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Hyperinsulinism-hyperammonemia syndrome

16:

biospecimen:Urine

concentration_value:149-247

concentration_units:umol/mmol creatinine

patient_age:Children (1-13 years old)

patient_sex:Female

patient_information:Hyperinsulinism-hyperammonemia syndrome

17:

biospecimen:Urine

concentration_value:15-107

concentration_units:umol/mmol creatinine

patient_age:Children (1-13 years old)

patient_sex:Female

patient_information:Hyperinsulinism-hyperammonemia syndrome

18:

biospecimen:Urine

concentration_value:1172.1

concentration_units:umol/mmol creatinine

patient_age:Infant (0-1 year old)

patient_sex:Male

patient_information:Fumarase deficiency

19:

biospecimen:Urine

concentration_value:385.200-1367.300

concentration_units:umol/mmol creatinine

patient_age:Infant (0-1 year old)

patient_sex:Male

patient_information:Fumarase deficiency

20:

biospecimen:Urine

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Bladder cancer

21:

biospecimen:Urine

concentration_value:673.261-2970.0885

concentration_units:umol/mmol creatinine

patient_age:Infant (0-1 year old)

patient_sex:Both

patient_information:Amish lethal microcephaly

22:

biospecimen:Urine

concentration_value:1119.294

concentration_units:umol/mmol creatinine

patient_age:Infant (0-1 year old)

patient_sex:Male

patient_information:Dihydrolipoamide Dehydrogenase Deficiency

23:

biospecimen:Urine

concentration_value:26.667

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Male

patient_information:D-2-Hydroxyglutaric aciduria

24:

biospecimen:Urine

concentration_value:46.432

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Male

patient_information:D-2-Hydroxyglutaric aciduria

25:

biospecimen:Urine

concentration_value:193.466

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Male

patient_information:D-2-Hydroxyglutaric aciduria

26:

biospecimen:Urine

concentration_value:6224

concentration_units:umol/mmol creatinine

patient_age:Children (1-13 years old)

patient_sex:Male

patient_information:Lipoyltransferase 1 Deficiency

27:

biospecimen:Urine

concentration_value:101.0 (50.0-152.0)

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:2-Ketoglutarate dehydrogenase complex deficiency

28:

biospecimen:Urine

concentration_value:875.0 (150.0-1600.0)

concentration_units:umol/mmol creatinine

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:2-Ketoglutarate Dehydrogenase Complex Deficiency

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1:

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omim_id:181500

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pubmed_id:24182348

[4]:

reference_text:Whelan DT, Hill RE, McClorry S: Fumaric aciduria: a new organic aciduria, associated with mental retardation and speech impairment. Clin Chim Acta. 1983 Aug 31;132(3):301-8.

pubmed_id:6616883

[5]:

reference_text:Maradin M, Fumic K, Hansikova H, Tesarova M, Wenchich L, Dorner S, Sarnavka V, Zeman J, Baric I: Fumaric aciduria: mild phenotype in a 8-year-old girl with novel mutations. J Inherit Metab Dis. 2006 Oct;29(5):683. Epub 2006 Aug 5.

pubmed_id:16972175

[6]:

reference_text:MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)

pubmed_id:

9:

name:Amish lethal microcephaly

omim_id:607196

[1]:

reference_text:Kelley RI, Robinson D, Puffenberger EG, Strauss KA, Morton DH: Amish lethal microcephaly: a new metabolic disorder with severe congenital microcephaly and 2-ketoglutaric aciduria. Am J Med Genet. 2002 Nov 1;112(4):318-26. doi: 10.1002/ajmg.10529.

pubmed_id:12376931

10:

name:Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome

omim_id:220500

[1]:

reference_text:James AW, Miranda SG, Culver K, Hall BD, Golabi M: DOOR syndrome: clinical report, literature review and discussion of natural history. Am J Med Genet A. 2007 Dec 1;143A(23):2821-31. doi: 10.1002/ajmg.a.32054.

pubmed_id:17994565

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

12:

name:Lipoyltransferase 1 Deficiency

omim_id:616299

[1]:

reference_text:Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192.

pubmed_id:24341803

KEGG数据库编号
C00026
DrugBank数据库编号
DB08845
foodb数据库编号
FDB003361
ChemSpider数据库编号
50
公共化学化合物编号
51
PDB数据库编号
生物利益的化学实体数据库编号
30915
knapsack数据库编号
C00000769
维基百科编号
Alpha-Ketoglutaric_acid
代谢途径的数据库编号
2-KETOGLUTARATE
苯酚资源管理器化合物数据库编号
比格数据库编号
33565
梅林编号
5218
虚拟机身份证编号
AKG
fbonto数据库编号
综合参考
Tanaka, Katsunobu; kimura, Kazu; Yamaguchi, Ken. Fermentative production of a-oxoglutaric acid. U.S. (1973), 4 pp.
一般参考
1:

protein_accession:HMDBP00054

name:Dihydrolipoyl dehydrogenase, mitochondrial

uniprot_id:P09622

gene_name:DLD

protein_type:Unknown

2:

protein_accession:HMDBP00248

name:4-aminobutyrate aminotransferase, mitochondrial

uniprot_id:P80404

gene_name:ABAT

protein_type:Enzyme

3:

protein_accession:HMDBP00270

name:Phytanoyl-CoA dioxygenase, peroxisomal

uniprot_id:O14832

gene_name:PHYH

protein_type:Unknown

4:

protein_accession:HMDBP00277

name:Tyrosine aminotransferase

uniprot_id:P17735

gene_name:TAT

protein_type:Enzyme

5:

protein_accession:HMDBP00283

name:Aspartate aminotransferase, cytoplasmic

uniprot_id:P17174

gene_name:GOT1

protein_type:Enzyme

6:

protein_accession:HMDBP00284

name:Aspartate aminotransferase, mitochondrial

uniprot_id:P00505

gene_name:GOT2

protein_type:Enzyme

7:

protein_accession:HMDBP00295

name:Aldehyde dehydrogenase, mitochondrial

uniprot_id:P05091

gene_name:ALDH2

protein_type:Unknown

8:

protein_accession:HMDBP00296

name:Fatty aldehyde dehydrogenase

uniprot_id:P51648

gene_name:ALDH3A2

protein_type:Unknown

9:

protein_accession:HMDBP00297

name:Aldehyde dehydrogenase X, mitochondrial

uniprot_id:P30837

gene_name:ALDH1B1

protein_type:Unknown

10:

protein_accession:HMDBP00301

name:Branched-chain-amino-acid aminotransferase, cytosolic

uniprot_id:P54687

gene_name:BCAT1

protein_type:Enzyme

11:

protein_accession:HMDBP00302

name:Prolyl 4-hydroxylase subunit alpha-2

uniprot_id:O15460

gene_name:P4HA2

protein_type:Enzyme

12:

protein_accession:HMDBP00304

name:Prolyl 4-hydroxylase subunit alpha-1

uniprot_id:P13674

gene_name:P4HA1

protein_type:Enzyme

13:

protein_accession:HMDBP00378

name:Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial

uniprot_id:Q8N5Z0

gene_name:AADAT

protein_type:Enzyme

14:

protein_accession:HMDBP00382

name:Gamma-butyrobetaine dioxygenase

uniprot_id:O75936

gene_name:BBOX1

protein_type:Unknown

15:

protein_accession:HMDBP00396

name:Alanine--glyoxylate aminotransferase 2, mitochondrial

uniprot_id:Q9BYV1

gene_name:AGXT2

protein_type:Enzyme

16:

protein_accession:HMDBP00400

name:Ornithine aminotransferase, mitochondrial

uniprot_id:P04181

gene_name:OAT

protein_type:Enzyme

17:

protein_accession:HMDBP00439

name:2-oxoglutarate dehydrogenase, mitochondrial

uniprot_id:Q02218

gene_name:OGDH

protein_type:Enzyme

18:

protein_accession:HMDBP00460

name:Procollagen-lysine,2-oxoglutarate 5-dioxygenase 1

uniprot_id:Q02809

gene_name:PLOD1

protein_type:Unknown

19:

protein_accession:HMDBP00461

name:Procollagen-lysine,2-oxoglutarate 5-dioxygenase 2

uniprot_id:O00469

gene_name:PLOD2

protein_type:Unknown

20:

protein_accession:HMDBP00462

name:Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3

uniprot_id:O60568

gene_name:PLOD3

protein_type:Unknown

21:

protein_accession:HMDBP00464

name:Kynurenine--oxoglutarate transaminase 1

uniprot_id:Q16773

gene_name:CCBL1

protein_type:Enzyme

22:

protein_accession:HMDBP00516

name:Branched-chain-amino-acid aminotransferase, mitochondrial

uniprot_id:O15382

gene_name:BCAT2

protein_type:Enzyme

23:

protein_accession:HMDBP00666

name:Glutamate dehydrogenase 2, mitochondrial

uniprot_id:P49448

gene_name:GLUD2

protein_type:Unknown

24:

protein_accession:HMDBP00694

name:Glutamate dehydrogenase 1, mitochondrial

uniprot_id:P00367

gene_name:GLUD1

protein_type:Unknown

25:

protein_accession:HMDBP00789

name:Serine--pyruvate aminotransferase

uniprot_id:P21549

gene_name:AGXT

protein_type:Unknown

26:

protein_accession:HMDBP00850

name:Alanine aminotransferase 1

uniprot_id:P24298

gene_name:GPT

protein_type:Enzyme

27:

protein_accession:HMDBP00851

name:Phosphoserine aminotransferase

uniprot_id:Q9Y617

gene_name:PSAT1

protein_type:Enzyme

28:

protein_accession:HMDBP00852

name:Alpha-aminoadipic semialdehyde synthase, mitochondrial

uniprot_id:Q9UDR5

gene_name:AASS

protein_type:Unknown

29:

protein_accession:HMDBP00896

name:Isocitrate dehydrogenase [NADP], mitochondrial

uniprot_id:P48735

gene_name:IDH2

protein_type:Unknown

30:

protein_accession:HMDBP00897

name:Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial

uniprot_id:O43837

gene_name:IDH3B

protein_type:Unknown

31:

protein_accession:HMDBP00898

name:Isocitrate dehydrogenase [NADP] cytoplasmic

uniprot_id:O75874

gene_name:IDH1

protein_type:Unknown

32:

protein_accession:HMDBP00899

name:Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial

uniprot_id:P50213

gene_name:IDH3A

protein_type:Unknown

33:

protein_accession:HMDBP00900

name:Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial

uniprot_id:P51553

gene_name:IDH3G

protein_type:Unknown

34:

protein_accession:HMDBP00922

name:D-3-phosphoglycerate dehydrogenase

uniprot_id:O43175

gene_name:PHGDH

protein_type:Unknown

35:

protein_accession:HMDBP01022

name:Aspartyl/asparaginyl beta-hydroxylase

uniprot_id:Q12797

gene_name:ASPH

protein_type:Unknown

36:

protein_accession:HMDBP01023

name:Hypoxia-inducible factor 1-alpha inhibitor

uniprot_id:Q9NWT6

gene_name:HIF1AN

protein_type:Unknown

37:

protein_accession:HMDBP01116

name:Mitochondrial 2-oxoglutarate/malate carrier protein

uniprot_id:Q02978

gene_name:SLC25A11

protein_type:Enzyme

38:

protein_accession:HMDBP02431

name:D-2-hydroxyglutarate dehydrogenase, mitochondrial

uniprot_id:Q8N465

gene_name:D2HGDH

protein_type:Unknown

39:

protein_accession:HMDBP02492

name:L-2-hydroxyglutarate dehydrogenase, mitochondrial

uniprot_id:Q9H9P8

gene_name:L2HGDH

protein_type:Unknown

40:

protein_accession:HMDBP03053

name:Aminoadipate aminotransferase, isoform CRA_b

uniprot_id:Q4W5N8

gene_name:AADAT

protein_type:Enzyme

41:

protein_accession:HMDBP03654

name:Trimethyllysine dioxygenase, mitochondrial

uniprot_id:Q9NVH6

gene_name:TMLHE

protein_type:Unknown

42:

protein_accession:HMDBP03670

name:Alanine aminotransferase 2

uniprot_id:Q8TD30

gene_name:GPT2

protein_type:Enzyme

43:

protein_accession:HMDBP03831

name:Hydroxyacid-oxoacid transhydrogenase, mitochondrial

uniprot_id:Q8IWW8

gene_name:ADHFE1

protein_type:Unknown

44:

protein_accession:HMDBP07400

name:Aminoadipate-semialdehyde synthase

uniprot_id:A4D0W4

gene_name:AASS

protein_type:Enzyme

45:

protein_accession:HMDBP08668

name:Alpha-ketoglutarate-dependent dioxygenase alkB homolog 2

uniprot_id:Q6NS38

gene_name:ALKBH2

protein_type:Unknown

46:

protein_accession:HMDBP08681

name:Prolyl 3-hydroxylase 1

uniprot_id:Q32P28

gene_name:LEPRE1

protein_type:Unknown

47:

protein_accession:HMDBP08682

name:Prolyl 3-hydroxylase 2

uniprot_id:Q8IVL5

gene_name:LEPREL1

protein_type:Unknown

48:

protein_accession:HMDBP08683

name:Prolyl 3-hydroxylase 3

uniprot_id:Q8IVL6

gene_name:LEPREL2

protein_type:Unknown

49:

protein_accession:HMDBP08965

name:Putative aspartate aminotransferase, cytoplasmic 2

uniprot_id:Q8NHS2

gene_name:GOT1L1

protein_type:Enzyme

50:

protein_accession:HMDBP08968

name:Kynurenine--oxoglutarate transaminase 3

uniprot_id:Q6YP21

gene_name:CCBL2

protein_type:Enzyme

51:

protein_accession:HMDBP09099

name:Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial

uniprot_id:Q96HY7

gene_name:DHTKD1

protein_type:Enzyme

52:

protein_accession:HMDBP09100

name:2-oxoglutarate dehydrogenase-like, mitochondrial

uniprot_id:Q9ULD0

gene_name:OGDHL

protein_type:Enzyme

53:

protein_accession:HMDBP09111

name:Prolyl 4-hydroxylase subunit alpha-3

uniprot_id:Q7Z4N8

gene_name:P4HA3

protein_type:Enzyme

54:

protein_accession:HMDBP09184

name:Lysine-specific demethylase 2A

uniprot_id:Q9Y2K7

gene_name:KDM2A

protein_type:Enzyme

55:

protein_accession:HMDBP09185

name:Lysine-specific demethylase 2B

uniprot_id:Q8NHM5

gene_name:KDM2B

protein_type:Enzyme

56:

protein_accession:HMDBP09210

name:Egl nine homolog 1

uniprot_id:Q9GZT9

gene_name:EGLN1

protein_type:Enzyme

57:

protein_accession:HMDBP09211

name:Egl nine homolog 2

uniprot_id:Q96KS0

gene_name:EGLN2

protein_type:Enzyme

58:

protein_accession:HMDBP09212

name:Egl nine homolog 3

uniprot_id:Q9H6Z9

gene_name:EGLN3

protein_type:Enzyme

59:

protein_accession:HMDBP09213

name:Transmembrane prolyl 4-hydroxylase

uniprot_id:Q9NXG6

gene_name:P4HTM

protein_type:Enzyme

60:

protein_accession:HMDBP11611

name:Alkylated DNA repair protein alkB homolog 1

uniprot_id:Q13686

gene_name:ALKBH1

protein_type:Unknown

61:

protein_accession:HMDBP11803

name:Lysine-specific demethylase 8

uniprot_id:Q8N371

gene_name:KDM8

protein_type:Unknown

62:

protein_accession:HMDBP11829

name:Bifunctional lysine-specific demethylase and histidyl-hydroxylase MINA

uniprot_id:Q8IUF8

gene_name:MINA

protein_type:Unknown

63:

protein_accession:HMDBP11862

name:Omega-amidase NIT2

uniprot_id:Q9NQR4

gene_name:NIT2

protein_type:Unknown

64:

protein_accession:HMDBP11864

name:Bifunctional lysine-specific demethylase and histidyl-hydroxylase NO66

uniprot_id:Q9H6W3

gene_name:NO66

protein_type:Unknown

65:

protein_accession:HMDBP11880

name:Histone lysine demethylase PHF8

uniprot_id:Q9UPP1

gene_name:PHF8

protein_type:Unknown

66:

protein_accession:HMDBP11972

name:Methylcytosine dioxygenase TET1

uniprot_id:Q8NFU7

gene_name:TET1

protein_type:Unknown

67:

protein_accession:HMDBP11973

name:Methylcytosine dioxygenase TET2

uniprot_id:Q6N021

gene_name:TET2

protein_type:Unknown

68:

protein_accession:HMDBP11974

name:Methylcytosine dioxygenase TET3

uniprot_id:O43151

gene_name:TET3

protein_type:Unknown

蛋白质结合
1:

reference_text:Nicholson JK, Foxall PJ, Spraul M, Farrant RD, Lindon JC: 750 MHz 1H and 1H-13C NMR spectroscopy of human blood plasma. Anal Chem. 1995 Mar 1;67(5):793-811.

pubmed_id:7762816

2:

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

3:

reference_text:Lee SH, Kim SO, Chung BC: Gas chromatographic-mass spectrometric determination of urinary oxoacids using O-(2,3,4,5,6-pentafluorobenzyl)oxime-trimethylsilyl ester derivatization and cation-exchange chromatography. J Chromatogr B Biomed Sci Appl. 1998 Nov 20;719(1-2):1-7.

pubmed_id:9869358

4:

reference_text:Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6.

pubmed_id:8087979

5:

reference_text:Ohdoi C, Nyhan WL, Kuhara T: Chemical diagnosis of Lesch-Nyhan syndrome using gas chromatography-mass spectrometry detection. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 15;792(1):123-30.

pubmed_id:12829005

6:

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

7:

reference_text:Bales JR, Higham DP, Howe I, Nicholson JK, Sadler PJ: Use of high-resolution proton nuclear magnetic resonance spectroscopy for rapid multi-component analysis of urine. Clin Chem. 1984 Mar;30(3):426-32.

pubmed_id:6321058

8:

reference_text:Stromme JH, Borud O, Moe PJ: Fatal lactic acidosis in a newborn attributable to a congenital defect of pyruvate dehydrogenase. Pediatr Res. 1976 Jan;10(1):62-6.

pubmed_id:813176

9:

reference_text:Rocchiccioli F, Leroux JP, Cartier PH: Microdetermination of 2-ketoglutaric acid in plasma and cerebrospinal fluid by capillary gas chromatography mass spectrometry; application to pediatrics. Biomed Mass Spectrom. 1984 Jan;11(1):24-8.

pubmed_id:6704500

10:

reference_text:Barbas C, Garcia A, de Miguel L, Simo C: Evaluation of filter paper collection of urine samples for detection and measurement of organic acidurias by capillary electrophoresis. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Nov 15;780(1):73-82.

pubmed_id:12383482

11:

reference_text:Kitaura J, Miki Y, Kato H, Sakakihara Y, Yanagisawa M: Hyperinsulinaemic hypoglycaemia associated with persistent hyperammonaemia. Eur J Pediatr. 1999 May;158(5):410-3.

pubmed_id:10333126

12:

reference_text:Kelley RI: Octenylsuccinic aciduria in children fed protein-hydrolysate formulas containing modified cornstarch. Pediatr Res. 1991 Dec;30(6):564-9.

pubmed_id:1805153

13:

reference_text:Garcia A, Barbas C, Aguilar R, Castro M: Capillary electrophoresis for rapid profiling of organic acidurias. Clin Chem. 1998 Sep;44(9):1905-11.

pubmed_id:9732975

14:

reference_text:Wu N, Yang M, Gaur U, Xu H, Yao Y, Li D: Alpha-Ketoglutarate: Physiological Functions and Applications. Biomol Ther (Seoul). 2016 Jan;24(1):1-8. doi: 10.4062/biomolther.2015.078. Epub 2016 Jan 1.

pubmed_id:26759695

15:

reference_text:Chin RM, Fu X, Pai MY, Vergnes L, Hwang H, Deng G, Diep S, Lomenick B, Meli VS, Monsalve GC, Hu E, Whelan SA, Wang JX, Jung G, Solis GM, Fazlollahi F, Kaweeteerawat C, Quach A, Nili M, Krall AS, Godwin HA, Chang HR, Faull KF, Guo F, Jiang M, Trauger SA, Saghatelian A, Braas D, Christofk HR, Clarke CF, Teitell MA, Petrascheck M, Reue K, Jung ME, Frand AR, Huang J: The metabolite alpha-ketoglutarate extends lifespan by inhibiting ATP synthase and TOR. Nature. 2014 Jun 19;510(7505):397-401. doi: 10.1038/nature13264. Epub 2014 May 14.

pubmed_id:24828042

16:

reference_text:Klysz D, Tai X, Robert PA, Craveiro M, Cretenet G, Oburoglu L, Mongellaz C, Floess S, Fritz V, Matias MI, Yong C, Surh N, Marie JC, Huehn J, Zimmermann V, Kinet S, Dardalhon V, Taylor N: Glutamine-dependent alpha-ketoglutarate production regulates the balance between T helper 1 cell and regulatory T cell generation. Sci Signal. 2015 Sep 29;8(396):ra97. doi: 10.1126/scisignal.aab2610.

pubmed_id:26420908

17:

reference_text:De Vivo DC: The expanding clinical spectrum of mitochondrial diseases. Brain Dev. 1993 Jan-Feb;15(1):1-22.

pubmed_id:8338207

18:

reference_text:Wendisch VF: Microbial Production of Amino Acid-Related Compounds. Adv Biochem Eng Biotechnol. 2017;159:255-269. doi: 10.1007/10_2016_34.

pubmed_id:27872963

19:

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

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