MiMeDB: Showing metabocard for Oxoglutaric acid (MMDBc0000067)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-10-27 23:29:11 UTC

Update Date

2024-10-09 16:31:37 UTC

Metabolite ID

MMDBc0000067

Metabolite Identification

Common Name

Oxoglutaric acid

Description

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).

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

Synonyms

Value	Source
2-Ketoglutaric acid	ChEBI
alpha-Ketoglutaric acid	ChEBI
2-Ketoglutarate	Generator
a-Ketoglutarate	Generator
a-Ketoglutaric acid	Generator
alpha-Ketoglutarate	Generator
Α-ketoglutarate	Generator
Α-ketoglutaric acid	Generator
Oxoglutarate	Generator
2-oxo-1,5-Pentanedioate	HMDB
2-oxo-1,5-Pentanedioic acid	HMDB
2-Oxoglutarate	HMDB
2-Oxoglutaric acid	HMDB
2-Oxopentanedioate	HMDB
2-Oxopentanedioic acid	HMDB
alpha-Oxoglutarate	HMDB
Oxogluric acid	HMDB
2 Oxoglutaric acid	HMDB
Ketoglutaric acid	HMDB
alpha Ketoglutaric acid	HMDB
2 Ketoglutaric acid	HMDB
alpha Ketoglutarate	HMDB
alpha Oxoglutarate	HMDB
2 Ketoglutarate	HMDB
2 Oxoglutarate	HMDB
alpha-Oxoglutaric acid	HMDB
alpha-Oxopentanedioic acid	HMDB
alpha-Keto-glutaric acid	HMDB
Α-oxoglutaric acid	HMDB
Α-oxopentanedioic acid	HMDB
Α-keto-glutaric acid	HMDB

Molecular Formula

C₅H₆O₅

Average Mass

146.0981

Monoisotopic Mass

146.021523302

IUPAC Name

2-oxopentanedioic acid

Traditional Name

oxoglutarate

CAS Registry Number

328-50-7

SMILES

OC(=O)CCC(=O)C(O)=O

InChI Identifier

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

InChI Key

KPGXRSRHYNQIFN-UHFFFAOYSA-N

Chemical Taxonomy

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.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Keto acids and derivatives

Sub Class

Gamma-keto acids and derivatives

Direct Parent

Gamma-keto acids and derivatives

Alternative Parents

Substituents

Gamma-keto acid
Short-chain keto acid
Dicarboxylic acid or derivatives
Alpha-keto acid
Alpha-hydroxy ketone
Ketone
Carboxylic acid
Carboxylic acid derivative
Organic oxygen compound
Organic oxide
Hydrocarbon derivative
Organooxygen compound
Carbonyl group
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

oxo dicarboxylic acid (CHEBI:30915 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Property	Value	Source
Water Solubility	53.1 g/L	ALOGPS
logP	-0.6	ALOGPS
logP	-0.11	ChemAxon
logS	-0.44	ALOGPS
pKa (Strongest Acidic)	2.66	ChemAxon
pKa (Strongest Basic)	-9.7	ChemAxon
Physiological Charge	-2	ChemAxon
Hydrogen Acceptor Count	5	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	91.67 Å²	ChemAxon
Rotatable Bond Count	4	ChemAxon
Refractivity	28.88 m³·mol⁻¹	ChemAxon
Polarizability	12.17 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-000b-3910000000-4097b7eb52c2910a4277	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-0002-1910000000-3277227b9c4da4baa1f7	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS; 1 MEOX)	splash10-00di-9300000000-974bce546caa4fbe6b0f	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (1 MEOX; 2 TMS)	splash10-000i-9710000000-d0aea11e947b042571f9	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (1 MEOX; 2 TMS)	splash10-052s-4920000000-bf7ed92c68fd4c8dbb6b	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-000b-3910000000-4097b7eb52c2910a4277	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0002-1910000000-3277227b9c4da4baa1f7	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00di-9300000000-974bce546caa4fbe6b0f	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-000i-9710000000-d0aea11e947b042571f9	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-052s-4920000000-bf7ed92c68fd4c8dbb6b	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-000b-2900000000-e514f7afea2490bbe9ed	2017-09-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-0kfx-9200000000-0ee77a39e9862659f8a7	2016-09-22	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positive	splash10-00di-9730000000-5e17bd8cfd944963c79f	2017-10-06	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)	splash10-0zfr-6900000000-7fc343a243ce5d2a4f39	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)	splash10-0a4i-9200000000-1a7af9946604baa8b3d2	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)	splash10-0a4l-9800000000-30495b2afdd3526854e7	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-0002-0925200000-af33449c9638276466c2	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-0a4i-9000000000-6e43f767847790112b65	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-03di-0900000000-4cacc41705b7b385e91e	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-0udi-0900000000-1367eeeb6ad64c67a0aa	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-000t-0915051100-91cda631ee54800322b8	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-0a4i-9000000000-4f842c3673925d29c402	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-0udi-0900000000-5e751351656f6eb62de3	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-001i-0910000000-7729faa24733fbad77b7	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative	splash10-0002-0900000000-c3c62f83bff99f44f7ab	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative	splash10-0zfr-6900000000-b6d8379db3e3f1480b14	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative	splash10-0a4i-9300000000-4fde39bb93cde5a27f23	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative	splash10-00i0-9000000000-4343832f3a55842423a9	2012-08-31	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-004i-2900000000-5aa3d6d620db1d733a65	2015-05-26	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-0m30-7900000000-fc77f69ad43f915bf23e	2015-05-26	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0a6r-9000000000-c597f1f5e416037aa82d	2015-05-26	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-004i-2900000000-5aa3d6d620db1d733a65	2015-05-26	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-0m30-7900000000-fc77f69ad43f915bf23e	2015-05-26	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0a6r-9000000000-c597f1f5e416037aa82d	2015-05-26	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-0002-1900000000-f2b463076a150a514f0c	2015-05-27	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-0f9t-8900000000-b2d3fa8d7ab01a220d9a	2015-05-27	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-053r-9100000000-554bfbd6839bc376cba0	2015-05-27	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-0002-1900000000-f2b463076a150a514f0c	2015-05-27	View Spectrum
MS	Mass Spectrum (Electron Ionization)	splash10-0fba-9200000000-4e367c66e94c51eb1a1b	2014-09-20	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, experimental)		2012-12-04	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 125 MHz, H₂O, experimental)		2012-12-04	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, DMSO-d6, experimental)		2014-09-20	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100.54 MHz, DMSO-d6, experimental)		2014-09-23	View Spectrum
1D NMR	¹H NMR Spectrum (1D, D₂O, experimental)		2016-10-22	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-29	View Spectrum
2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)		2012-12-05	View Spectrum

Biological Properties

Cellular Locations

Endoplasmic reticulum
Extracellular
Mitochondria
Peroxisome

Biospecimen Locations

Blood
Breast Milk
Cellular Cytoplasm
Cerebrospinal Fluid (CSF)
Feces
Placenta
Saliva
Urine

Tissue Locations

Placenta

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species
MMDBp0191728	Dihydrolipoyl dehydrogenase, mitochondrial	Unknown	P09622	Homo sapiens
MMDBp0191918	4-aminobutyrate aminotransferase, mitochondrial	Enzyme	P80404	Homo sapiens
MMDBp0191940	Phytanoyl-CoA dioxygenase, peroxisomal	Unknown	O14832	Homo sapiens
MMDBp0191947	Tyrosine aminotransferase	Enzyme	P17735	Homo sapiens
MMDBp0191953	Aspartate aminotransferase, cytoplasmic	Enzyme	P17174	Homo sapiens
MMDBp0191954	Aspartate aminotransferase, mitochondrial	Enzyme	P00505	Homo sapiens
MMDBp0191965	Aldehyde dehydrogenase, mitochondrial	Unknown	P05091	Homo sapiens
MMDBp0191966	Fatty aldehyde dehydrogenase	Unknown	P51648	Homo sapiens
MMDBp0191967	Aldehyde dehydrogenase X, mitochondrial	Unknown	P30837	Homo sapiens
MMDBp0191971	Branched-chain-amino-acid aminotransferase, cytosolic	Enzyme	P54687	Homo sapiens
MMDBp0191972	Prolyl 4-hydroxylase subunit alpha-2	Enzyme	O15460	Homo sapiens
MMDBp0191974	Prolyl 4-hydroxylase subunit alpha-1	Enzyme	P13674	Homo sapiens
MMDBp0192045	Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial	Enzyme	Q8N5Z0	Homo sapiens
MMDBp0192049	Gamma-butyrobetaine dioxygenase	Unknown	O75936	Homo sapiens
MMDBp0192063	Alanine--glyoxylate aminotransferase 2, mitochondrial	Enzyme	Q9BYV1	Homo sapiens
MMDBp0192067	Ornithine aminotransferase, mitochondrial	Enzyme	P04181	Homo sapiens
MMDBp0192105	2-oxoglutarate dehydrogenase, mitochondrial	Enzyme	Q02218	Homo sapiens
MMDBp0192119	Procollagen-lysine,2-oxoglutarate 5-dioxygenase 1	Unknown	Q02809	Homo sapiens
MMDBp0192120	Procollagen-lysine,2-oxoglutarate 5-dioxygenase 2	Unknown	O00469	Homo sapiens
MMDBp0192121	Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3	Unknown	O60568	Homo sapiens
MMDBp0192123	Kynurenine--oxoglutarate transaminase 1	Enzyme	Q16773	Homo sapiens
MMDBp0192169	Branched-chain-amino-acid aminotransferase, mitochondrial	Enzyme	O15382	Homo sapiens
MMDBp0192306	Glutamate dehydrogenase 2, mitochondrial	Unknown	P49448	Homo sapiens
MMDBp0192333	Glutamate dehydrogenase 1, mitochondrial	Unknown	P00367	Homo sapiens
MMDBp0192409	Serine--pyruvate aminotransferase	Unknown	P21549	Homo sapiens
MMDBp0192470	Alanine aminotransferase 1	Enzyme	P24298	Homo sapiens
MMDBp0192471	Phosphoserine aminotransferase	Enzyme	Q9Y617	Homo sapiens
MMDBp0192472	Alpha-aminoadipic semialdehyde synthase, mitochondrial	Unknown	Q9UDR5	Homo sapiens
MMDBp0192514	Isocitrate dehydrogenase [NADP], mitochondrial	Unknown	P48735	Homo sapiens
MMDBp0192515	Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial	Unknown	O43837	Homo sapiens
MMDBp0192516	Isocitrate dehydrogenase [NADP] cytoplasmic	Unknown	O75874	Homo sapiens
MMDBp0192517	Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial	Unknown	P50213	Homo sapiens
MMDBp0192518	Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial	Unknown	P51553	Homo sapiens
MMDBp0192540	D-3-phosphoglycerate dehydrogenase	Unknown	O43175	Homo sapiens
MMDBp0192633	Aspartyl/asparaginyl beta-hydroxylase	Unknown	Q12797	Homo sapiens
MMDBp0192634	Hypoxia-inducible factor 1-alpha inhibitor	Unknown	Q9NWT6	Homo sapiens
MMDBp0192721	Mitochondrial 2-oxoglutarate/malate carrier protein	Enzyme	Q02978	Homo sapiens
MMDBp0191674	D-2-hydroxyglutarate dehydrogenase, mitochondrial	Enzyme	Q8N465	Saccharomyces cerevisiae
MMDBp0191676	L-2-hydroxyglutarate dehydrogenase, mitochondrial	Enzyme	Q9H9P8	Saccharomyces cerevisiae
MMDBp0193784	Aminoadipate aminotransferase, isoform CRA_b	Enzyme	Q4W5N8	Homo sapiens
MMDBp0193925	Trimethyllysine dioxygenase, mitochondrial	Unknown	Q9NVH6	Homo sapiens
MMDBp0193926	Alanine aminotransferase 2	Enzyme	Q8TD30	Homo sapiens
MMDBp0193939	Hydroxyacid-oxoacid transhydrogenase, mitochondrial	Unknown	Q8IWW8	Homo sapiens
MMDBp0194178	Aminoadipate-semialdehyde synthase	Enzyme	A4D0W4	Homo sapiens
MMDBp0195435	Alpha-ketoglutarate-dependent dioxygenase alkB homolog 2	Unknown	Q6NS38	Homo sapiens
MMDBp0195448	Prolyl 3-hydroxylase 1	Unknown	Q32P28	Homo sapiens
MMDBp0195449	Prolyl 3-hydroxylase 2	Unknown	Q8IVL5	Homo sapiens
MMDBp0195450	Prolyl 3-hydroxylase 3	Unknown	Q8IVL6	Homo sapiens
MMDBp0195723	Putative aspartate aminotransferase, cytoplasmic 2	Enzyme	Q8NHS2	Homo sapiens
MMDBp0195726	Kynurenine--oxoglutarate transaminase 3	Enzyme	Q6YP21	Homo sapiens
MMDBp0195851	Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial	Enzyme	Q96HY7	Homo sapiens
MMDBp0195852	2-oxoglutarate dehydrogenase-like, mitochondrial	Enzyme	Q9ULD0	Homo sapiens
MMDBp0195859	Prolyl 4-hydroxylase subunit alpha-3	Enzyme	Q7Z4N8	Homo sapiens
MMDBp0195895	Lysine-specific demethylase 2A	Enzyme	Q9Y2K7	Homo sapiens
MMDBp0195896	Lysine-specific demethylase 2B	Enzyme	Q8NHM5	Homo sapiens
MMDBp0195916	Egl nine homolog 1	Enzyme	Q9GZT9	Homo sapiens
MMDBp0195917	Egl nine homolog 2	Enzyme	Q96KS0	Homo sapiens
MMDBp0195918	Egl nine homolog 3	Enzyme	Q9H6Z9	Homo sapiens
MMDBp0195919	Transmembrane prolyl 4-hydroxylase	Enzyme	Q9NXG6	Homo sapiens
MMDBp0196686	Alkylated DNA repair protein alkB homolog 1	Unknown	Q13686
MMDBp0196862	Lysine-specific demethylase 8	Unknown	Q8N371
MMDBp0196886	Bifunctional lysine-specific demethylase and histidyl-hydroxylase MINA	Unknown	Q8IUF8
MMDBp0196912	Omega-amidase NIT2	Unknown	Q9NQR4
MMDBp0196914	Bifunctional lysine-specific demethylase and histidyl-hydroxylase NO66	Unknown	Q9H6W3
MMDBp0196925	Histone lysine demethylase PHF8	Unknown	Q9UPP1
MMDBp0196987	Methylcytosine dioxygenase TET1	Unknown	Q8NFU7
MMDBp0196988	Methylcytosine dioxygenase TET2	Unknown	Q6N021
MMDBp0196989	Methylcytosine dioxygenase TET3	Unknown	O43151

Human Pathways

Pathways

Name	SMPDB/PathBank
Succinate Signalling

Metabolic Reactions

Reactions

Reaction ID	Precursor	Product	Enzyme	Data Source	Reference
MMDBr0000039	Oxoglutaric acid	Oxoglutaric acid	solute carrier family 25 (mitochondrial oxoadipate carrier), member 21	VMH	11083877
MMDBr0000046	Oxoadipic acid	Oxoadipic acid	solute carrier family 25 (mitochondrial oxoadipate carrier), member 21	VMH	11083877
MMDBr0000058	2-Methyl-3-oxopropanoic acid	(S)-b-aminoisobutyric acid	4-aminobutyrate aminotransferase	VMH	30371894
MMDBr0000066	Oxoglutaric acid	4-(2-Amino-3-hydroxyphenyl)-2,4-dioxobutanoic acid	aminoadipate aminotransferase	VMH	13357462
MMDBr0000083	3-Sulfinoalanine	3-Sulfinylpyruvic acid	glutamic-oxaloacetic transaminase 1, soluble	VMH	3346220
MMDBr0000084	3-Sulfinoalanine	3-Sulfinylpyruvic acid	glutamic-oxaloacetic transaminase 2, mitochondrial	VMH	3346220
MMDBr0000143	Oxoglutaric acid	Oxoadipic acid	Not Available	VMH	12126930
MMDBr0000144	gamma-Aminobutyric acid	L-Glutamic acid	4-aminobutyrate aminotransferase	VMH; KEGG	10407778 7721088
MMDBr0000254	Oxoglutaric acid	Succinyl-CoA	dihydrolipoamide dehydrogenase	VMH; KEGG	1542694
MMDBr0000272	Oxoglutaric acid	L-Glutamic acid	Not Available	VMH; KEGG	11863375
MMDBr0000314	Oxoglutaric acid	L-Glutamic acid	4-aminobutyrate aminotransferase	VMH; KEGG	15304357
MMDBr0000354	Oxoglutaric acid	L-Glutamic acid	glutamic-oxaloacetic transaminase 2, mitochondrial	VMH; KEGG	6930651
MMDBr0000376	4-Trimethylammoniobutanoic acid	L-Carnitine	butyrobetaine (gamma), 2-oxoglutarate dioxygenase (gamma-butyrobetaine hydroxylase) 1	VMH; KEGG	9753662
MMDBr0000568	Oxoglutaric acid	L-Glutamic acid	Not Available	VMH; KEGG	15189131 7113743
MMDBr0000569	Oxoglutaric acid	L-Glutamic acid	glutamic-oxaloacetic transaminase 2, mitochondrial	VMH	15189131 754189
MMDBr0000855	Oxoglutaric acid	4-Hydroxy-2-oxoglutaric acid	glutamic-oxaloacetic transaminase 2, mitochondrial	VMH	15849464
MMDBr0001337	L-Glutamic acid	Oxoglutaric acid	glutamate dehydrogenase 1	VMH	12324473 3368458
MMDBr0001338	L-Glutamic acid	Oxoglutaric acid	glutamate dehydrogenase 1	VMH; KEGG	30371894
MMDBr0001492	Isocitric acid	Oxoglutaric acid	isocitrate dehydrogenase 3 (NAD+) alpha	VMH; KEGG	30371894
MMDBr0001493	Isocitric acid	Oxoglutaric acid	Not Available	VMH	10521434
MMDBr0001494	Isocitric acid	Oxoglutaric acid	isocitrate dehydrogenase 1 (NADP+), soluble	VMH; KEGG	30371894
MMDBr0001495	Isocitric acid	Oxoglutaric acid	isocitrate dehydrogenase 2 (NADP+), mitochondrial	VMH	12234803 8187868
MMDBr0001504	Oxoglutaric acid	3-Methyl-2-oxovaleric acid	branched chain amino-acid transaminase 2, mitochondrial	VMH; KEGG	30371894
MMDBr0001534	Oxoglutaric acid	4-(2-Aminophenyl)-2,4-dioxobutanoic acid	aminoadipate aminotransferase	VMH	15016471
MMDBr0001546	Oxoglutaric acid	3-Sulfopyruvic acid	glutamic-oxaloacetic transaminase 1, soluble	VMH; KEGG	30371894
MMDBr0001547	Oxoglutaric acid	3-Sulfopyruvic acid	glutamic-oxaloacetic transaminase 2, mitochondrial	VMH	3346220
MMDBr0001561	Oxoglutaric acid	Ketoleucine	branched chain amino-acid transaminase 2, mitochondrial	VMH; KEGG	30371894
MMDBr0001835	Oxoglutaric acid	L-Glutamic acid	ornithine aminotransferase	VMH; KEGG	30371894
MMDBr0001933	Oxoglutaric acid	L-Glutamic acid	glutamic-oxaloacetic transaminase 2, mitochondrial	VMH; KEGG	30371894
MMDBr0001937	Oxoglutaric acid	2-Hydroxyphytanoyl Coenzyme A	phytanoyl-CoA 2-hydroxylase	VMH	8954107 9326940
MMDBr0001998	Oxoglutaric acid	4-Hydroxyproline	prolyl 4-hydroxylase, alpha polypeptide I	VMH	12714038 2543975 3034602
MMDBr0002084	Oxoglutaric acid	Saccharopine	aminoadipate-semialdehyde synthase	VMH; KEGG	30371894
MMDBr0002259	Oxoglutaric acid	3-Hydroxy-N6,N6,N6-trimethyl-L-lysine	trimethyllysine hydroxylase, epsilon	VMH	11431483 11802770 627563
MMDBr0002303	Oxoglutaric acid	4-Hydroxyphenylpyruvic acid	glutamic-oxaloacetic transaminase 2, mitochondrial	VMH	1973834
MMDBr0002372	Oxoglutaric acid	alpha-Ketoisovaleric acid	branched chain amino-acid transaminase 2, mitochondrial	VMH	6933702 9165094
MMDBr0002450	Oxoglutaric acid	L-Glutamic acid	glutamic-pyruvate transaminase (alanine aminotransferase)	VMH	15663181 2088925 454616
MMDBr0002451	Oxalosuccinic acid	Oxoglutaric acid	isocitrate dehydrogenase 1 (NADP+), soluble	VMH; KEGG	30371894
MMDBr0002452	Oxalosuccinic acid	Oxoglutaric acid	isocitrate dehydrogenase 1 (NADP+), soluble	VMH; KEGG	30371894
MMDBr0002453	2-Keto-glutaramic acid	Oxoglutaric acid	Not Available	VMH; KEGG	30371894
MMDBr0002454	2-Keto-glutaramic acid	Oxoglutaric acid	nitrilase family, member 2	VMH; KEGG	30371894
MMDBr0002472	Oxoglutaric acid	3-Carboxy-1-hydroxypropylthiamine diphosphate	oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)	VMH	10676873 3207422
MMDBr0002528	Oxoglutaric acid	(S)-Succinyldihydrolipoamide	oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)	VMH	10676873
MMDBr0002563	Oxoglutaric acid	Oxoadipic acid	aminoadipate aminotransferase	VMH; KEGG	30371894
MMDBr0002571	(R)-b-aminoisobutyric acid	2-Methyl-3-oxopropanoic acid	4-aminobutyrate aminotransferase	VMH	5773299
MMDBr0003964	Oxoglutaric acid	L-Glutamic acid	cysteine conjugate-beta lyase, cytoplasmic	VMH	10756023
MMDBr0003965	Oxoglutaric acid	L-Glutamic acid	aminoadipate aminotransferase	VMH	10756023
MMDBr0004152	Oxoglutaric acid	2-Ketobutyric acid	Not Available	VMH; KEGG	30371894
MMDBr0004237	Oxoglutaric acid	Xanthurenic acid	cysteine conjugate-beta lyase, cytoplasmic	VMH	10559215 8908429
MMDBr0004238	Oxoglutaric acid	Xanthurenic acid	aminoadipate aminotransferase	VMH	10559215 8908429
MMDBr0004307	Oxoglutaric acid	Xanthurenate-8-O-Beta-D-Glucoside	Not Available	VMH	10409626
MMDBr0006785	2-Oxo-4-methylthiobutanoic acid	Oxoglutaric acid	Not Available	VMH	30371894
MMDBr0006942	L-Glutamic acid	Oxoglutaric acid	Not Available	VMH	30371894
MMDBr0006985	LL-2,6-Diaminoheptanedioate	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0007007	Oxoglutaric acid	2-Hydroxyglutarate	Not Available	VMH	30371894
MMDBr0007014	gamma-Aminobutyric acid	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0007027	1,5-Diaminopentane	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0007029	5-Aminopentanoic acid	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0007044	N2-Acetylornithine	N-Acetyl-L-glutamate 5-semialdehyde	Not Available	VMH	30371894
MMDBr0007068	L-Glutamic acid	Oxoglutaric acid	Not Available	VMH	30371894
MMDBr0007142	Oxoglutaric acid	2-Succinyl-5-enolpyruvyl-6-hydroxy-3-cyclohexene-1-carboxylate	Not Available	VMH	30371894
MMDBr0007274	Oxoglutaric acid	L-Glutamic acid	Not Available	VMH; KEGG	30371894
MMDBr0007328	Oxoglutaric acid	4-Hydroxy-2-oxoglutaric acid	Not Available	VMH	30371894
MMDBr0007431	L-Glutamic acid	Oxoglutaric acid	Not Available	VMH	30371894
MMDBr0007468	Oxoglutaric acid	3-Methyl-2-oxovaleric acid	Not Available	VMH; KEGG	30371894
MMDBr0007476	Oxoglutaric acid	Ketoleucine	Not Available	VMH; KEGG	30371894
MMDBr0007548	Phosphohydroxypyruvic acid	Oxoglutaric acid	Not Available	VMH; KEGG	30371894
MMDBr0007567	Oxoglutaric acid	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0007598	Oxoglutaric acid	alpha-Ketoisovaleric acid	Not Available	VMH; KEGG	30371894
MMDBr0007604	Oxalosuccinic acid	Oxoglutaric acid	Not Available	VMH; KEGG	30371894
MMDBr0007939	Oxoglutaric acid	L-Glutamic acid	Not Available	VMH; KEGG	30371894
MMDBr0007949	Oxoglutaric acid	4-Aminobutyraldehyde	Not Available	VMH	30371894
MMDBr0007964	Oxoglutaric acid	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0007987	Oxoglutaric acid	4-Hydroxyphenylpyruvic acid	Not Available	VMH; KEGG	30371894
MMDBr0007992	2-Oxo-4-methylthiobutanoic acid	Oxoglutaric acid	Not Available	VMH; KEGG	30371894
MMDBr0008013	Oxoglutaric acid	3-Carboxy-1-hydroxypropylthiamine diphosphate	Not Available	VMH	30371894
MMDBr0008084	Oxoglutaric acid	N_Acetyl_L_2_amino_6_oxopimelate	Not Available	VMH	30371894
MMDBr0008124	Oxoglutaric acid	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0008177	Oxoglutaric acid	Aminoacetaldehyde	Not Available	VMH	30371894
MMDBr0008191	Oxoglutaric acid	(2R)-Homocitric acid	Not Available	VMH	30371894
MMDBr0008193	Isocitric acid	Oxoglutaric acid	Not Available	VMH	30371894
MMDBr0008280	Oxoglutaric acid	D-Glutamate	Not Available	VMH	30371894
MMDBr0008281	L-Glutamic acid	Oxoglutaric acid	Not Available	VMH	30371894
MMDBr0008315	2,5-Dioxopentanoate	Oxoglutaric acid	Not Available	VMH	30371894
MMDBr0008333	Oxoglutaric acid	4-Hydroxyproline	Not Available	VMH	30371894
MMDBr0008438	Succinyl-CoA	Oxoglutaric acid	Not Available	VMH	30371894
MMDBr0008461	Oxoglutaric acid	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0008462	Oxoglutaric acid	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0008490	Oxoglutaric acid	Succinic acid semialdehyde	Not Available	VMH	30371894
MMDBr0008506	gamma-Aminobutyric acid	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0008516	L-Glutamic acid	LL-2,6-Diaminoheptanedioate	Not Available	VMH	30371894
MMDBr0008564	Oxoglutaric acid	L-Glutamic acid	glutamic-pyruvate transaminase (alanine aminotransferase)	VMH	30371894
MMDBr0009370	Oxoglutaric acid	2-Hydroxyglutarate	L-2-hydroxyglutarate dehydrogenase	VMH; KEGG	30371894
MMDBr0009371	Oxoglutaric acid	2-Hydroxyglutarate	phosphoglycerate dehydrogenase	VMH; KEGG	30371894
MMDBr0010306	Oxoglutaric acid	4-Hydroxyproline	prolyl 4-hydroxylase, alpha polypeptide I	VMH; KEGG	30371894
MMDBr0010307	Oxoglutaric acid	4-Hydroxyproline	prolyl 4-hydroxylase, alpha polypeptide I	VMH; KEGG	30371894
MMDBr0010992	Saccharopine	Oxoglutaric acid	Not Available	VMH	30371894
MMDBr0011025	Oxoglutaric acid	Succinic acid	Not Available	VMH	30371894
MMDBr0012498	Oxoglutaric acid	Oxoglutaric acid	solute carrier family 22 (organic anion transporter), member 6	VMH	30371894
MMDBr0012658	Oxoglutaric acid	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0012706	Isocitric acid	Oxoglutaric acid	Not Available	VMH	30371894

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
2-ketoglutarate dehydrogenase complex deficiency	Increased	Association	Human Blood	HMDB	7075624
2-ketoglutarate dehydrogenase complex deficiency	Increased	Association	Human Cerebrospinal Fluid (CSF)	HMDB	7075624
2-ketoglutarate dehydrogenase complex deficiency	Increased	Association	Human Urine	HMDB	1640293
Anoxia	Decreased	Association	Human Cellular Cytoplasm	HMDB	8579834
D-2-hydroxyglutaric aciduria		Association	Human Urine	HMDB	6774165
Fumarase deficiency	Increased	Association	Human Urine	HMDB	24182348
Eosinophilic esophagitis		Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Eosinophilic esophagitis	Slight Decrease	Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
2-ketoglutarate dehydrogenase complex deficiency	Increased	Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
2-ketoglutarate dehydrogenase complex deficiency	Increased	Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Actinobacteria	150	Human feces; Human sputum; Human bronchial brush; Human nasopharyngeal swab; Human pleural fluid plaque; Human dental plaque; Human ; Human mucosa; Human supragingival plaque; Human subgingival plaque; Human saliva	Microbial culture; Metabolic reconstruction
Bacteria	Verrucomicrobia	2	Human feces; Human stool; Human ; Human urine	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria	Firmicutes	474	Human feces; Human pleural fluid plaque; Pig ; Human sputum; Human ; Human subgingival plaque; Human saliva; Human urine; Human mucosa; Human supragingival plaque; Cattle ; Human stool	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria	Proteobacteria	312	Human feces; Human sputum; Human ; Human unknown; Pig ; Human pleural fluid plaque; Human dental plaque; Human nasal swab; Human stool; Human feces; pleural fluid plaque; bal; Human subgingival plaque; Human supragingival plaque	Metabolic reconstruction
Bacteria	Bacteroidetes	130	Human feces; Human appendix biopsy; Human ; Human stool; Human saliva; Human gastric fluid; Human urine; Human supragingival plaque; Human subgingival plaque	Metabolic reconstruction
Bacteria	Synergistetes	5	Human feces	Metabolic reconstruction
Bacteria	Spirochaetes	1	Human feces	Metabolic reconstruction
Archaea	Thaumarchaeota	1		Metabolic reconstruction
Bacteria	Fusobacteria	20	Human feces; Human	Metabolic reconstruction
Archaea	Euryarchaeota	5		Metabolic reconstruction
Bacteria	Candidatus melainabacteria	1	Human feces	Metabolic reconstruction
Bacteria	Tenericutes	3	Human feces	Metabolic reconstruction
Bacteria	Planctomycetes	1	Human feces	Metabolic reconstruction
Archaea	Crenarchaeota	1		Metabolic reconstruction
Eukaryota/Fungi	Ascomycota	5	Human feces; Human saliva	Unknown

Exposure Sources

Source Type	Source Sub-type	Source Name	Data Source	Reference
Food	Not Available	Alcoholic beverage	FooDB	Manually Annotated
Animal	Milk	Cow	FooDB	11132867
Plant	Fruit	Star fruit	FooDB	DUKE

Source Type	Activity	Data Source	Reference
Drugs	Antimicrobial	U.S. Environmental Protection Agency	CompTox
Cosmetics	Not Available	U.S. Environmental Protection Agency	CompTox
Domestics	Not Available	U.S. Environmental Protection Agency	CompTox
Domestics	Not Available	U.S. Environmental Protection Agency	CompTox
Cosmetics	Not Available	U.S. Environmental Protection Agency	CompTox

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Urine	Detected and Quantified			30	1600	umol/mmol creatinine	Infant (0-1 year old)	Male	2-Ketoglutarate dehydrogenase complex deficiency	HMDB	1640293
Human Urine	Detected and Quantified	<60				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	1640293
Human Urine	Detected and Quantified	43.46	53.46			umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	2026685
Human Urine	Detected and Quantified	24		4	74	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	2702744
Human Urine	Detected and Quantified	> 0.658				umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	6321058
Human Urine	Detected and Quantified	1119.294				umol/mmol creatinine	Infant (0-1 year old)	Male	Dihydrolipoamide Dehydrogenase Deficiency	HMDB	6688766
Human Blood	Detected and Quantified	8.6	2.6			uM	Children (1-13 years old)	Both	Normal	HMDB	6704500
Human Cerebrospinal fluid (csf)	Detected and Quantified	4.8		3.4	6.2	uM	Children (1-13 years old)	Both	Normal	HMDB	6704500
Human Urine	Detected and Quantified	26.667				umol/mmol creatinine	Children (1 - 13 years old)	Male	D-2-Hydroxyglutaric aciduria	HMDB	6774165
Human Urine	Detected and Quantified	46.432				umol/mmol creatinine	Children (1 - 13 years old)	Male	D-2-Hydroxyglutaric aciduria	HMDB	6774165
Human Urine	Detected and Quantified	193.466				umol/mmol creatinine	Children (1 - 13 years old)	Male	D-2-Hydroxyglutaric aciduria	HMDB	6774165
Human Urine	Detected and Quantified			1.548	39.467	umol/mmol creatinine	Children (1 - 13 years old)	Male	Normal	HMDB	6774165
Human Blood	Detected and Quantified			17	37	uM	Children (1-13 years old)	Both	2-Ketoglutarate dehydrogenase complex deficiency	HMDB	7075624
Human Blood	Detected and Quantified			4	13	uM	Not Specified	Not Specified	Normal	HMDB	7075624
Human Cerebrospinal fluid (csf)	Detected and Quantified			3.6	12.8	uM	Children (1-13 years old)	Both	2-Ketoglutarate dehydrogenase complex deficiency	HMDB	7075624
Human Cerebrospinal fluid (csf)	Detected and Quantified			16	Feb	uM	Not Specified	Not Specified	Normal	HMDB	7075624
Human Urine	Detected and Quantified	25.6		10.2	146	umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	21.4		4.9	123.8	umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	236.9		8	631.4	umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	150.5		10	836.3	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	8087979
Human Blood	Detected and Quantified	7		0	23	uM	Adult (>18 years old)	Both	Normal	HMDB	8412012
Human Cerebrospinal fluid (csf)	Detected and Quantified	2	2			uM	Adult (>18 years old)	Both	Normal	HMDB	8412012
Human Cellular cytoplasm	Detected and Quantified	320		290	350	uM	Adult (>18 years old)	Both	Normal	HMDB	8579834
Human Cellular cytoplasm	Detected and Quantified	150		144	156	uM	Adult (>18 years old)	Both	Anoxia	HMDB	8579834
Human Urine	Detected and Quantified	<150				umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	HMDB	9732975
Human Urine	Detected and Quantified	2.98		0.42	18.3	umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	9869358
Human Urine	Detected and Quantified	2.87		0.18	14.3	umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	9869358
Human Urine	Detected and Quantified			15.477	154.773	umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	12376931
Human Urine	Detected and Quantified			673.261	2970.0885	umol/mmol creatinine	Infant (0-1 year old)	Both	Amish lethal microcephaly	HMDB	12376931
Human Urine	Detected and Quantified	23.6				umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	HMDB	12383482
Human Urine	Detected and Quantified			15	107	umol/mmol creatinine	Children (1-13 years old)	Female	Hyperinsulinism-hyperammonemia syndrome	HMDB	14967154
Human Urine	Detected and Quantified	<115				umol/mmol creatinine	Children (1-13 years old)	Female	Normal	HMDB	14967154
Human Urine	Detected and Quantified	<95				umol/mmol creatinine	Children (1-13 years old)	Female	Normal	HMDB	14967154
Human Urine	Detected and Quantified			149	247	umol/mmol creatinine	Children (1-13 years old)	Female	Hyperinsulinism-hyperammonemia syndrome	HMDB	14967154
Human Urine	Detected and Quantified	<75				umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	14967154
Human Urine	Detected and Quantified			100	166	umol/mmol creatinine	Adult (>18 years old)	Both	Hyperinsulinism-hyperammonemia syndrome	HMDB	14967154
Human Urine	Detected and Quantified			2.2	8.2	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	15992788
Human Urine	Detected and Quantified	0.083	0.002			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	16460720
Human Urine	Detected and Quantified	1374				umol/mmol creatinine	Newborn (0-30 days old)	Female	Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome	HMDB	17994565
Human Urine	Detected and Quantified	<110				umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	HMDB	17994565
Human Cerebrospinal fluid (csf)	Detected and Quantified	5	4			uM	Adult (>18 years old)	Both	Normal	HMDB	18502700
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Schizophrenia	HMDB	22007635
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	22007635
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Schizophrenia	HMDB	22024767
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	22024767
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Feces	Detected but not Quantified						Infant (0-1 year old)	Both	Normal	HMDB	22411374
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Schizophrenia	HMDB	22800120
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	22800120
Human Urine	Detected and Quantified	3.4		1.4	12.5	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified	4.8		2	17	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Breast milk	Detected and Quantified	41	16.1			uM	Adult (>18 years old)	Female	Normal	HMDB	24027187
Human Urine	Detected and Quantified	1172.1				umol/mmol creatinine	Infant (0-1 year old)	Male	Fumarase deficiency	HMDB	24182348
Human Urine	Detected and Quantified	<350				umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	24182348
Human Urine	Detected and Quantified			385.2	1367.3	umol/mmol creatinine	Infant (0-1 year old)	Male	Fumarase deficiency	HMDB	24182348
Human Urine	Detected and Quantified	6224				umol/mmol creatinine	Children (1-13 years old)	Male	Lipoyltransferase 1 Deficiency	HMDB	24341803
Human Urine	Detected and Quantified	<79				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	24341803
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	25037050
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Colorectal cancer	HMDB	25037050
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Immunoglobulin A nephropathy (IgAN) progressor	HMDB	26476344
Human Urine	Detected and Quantified			12	20	umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	27012787
Human Urine	Detected and Quantified			7	11	umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	27012787
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Colorectal Cancer	HMDB	27275383
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27543620
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Bladder cancer	HMDB	28157703
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030
Human Urine	Detected and Quantified			160	590	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	31506554
Human Urine	Detected and Quantified			4.3	36.3	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified			7.5	28.9	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Urine	Detected and Quantified			0	25.3	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified			14.8	64.7	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Urine	Detected and Quantified			30.79	398.2	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	32340350
Human Urine	Detected and Quantified	90.83		45.64	331.1	umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	HMDB	32340350
Human Urine	Detected and Quantified	104.8		30.21	413.8	umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	HMDB	32340350
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	32966057
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	32966057
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27065191
Human Blood	Detected and Quantified	9.3	2.3			uM	Children (1-13 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
Human Blood	Detected and Quantified	20.7	2.5			uM	Newborn (0-30 days old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
Human Blood	Detected and Quantified	8.9	2.7			uM	Adult (>18 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
Human Cerebrospinal fluid (csf)	Detected and Quantified	2.1	0.7			uM	Children (1-13 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
Human Cerebrospinal fluid (csf)	Detected and Quantified	8.9	3.4			uM	Adult (>18 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
Human Saliva	Detected and Quantified	2.65	1.07			uM	Adult (>18 years old)	Not Specified	Normal	HMDB	Sugimoto, M., Saruta, J., Matsuki, C. et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles. Metabolomics, 9, 454–463 (2013). https://doi.org/10.1007/s11306-012-0464-y
Human Saliva	Detected and Quantified	5.27	3.61			uM	Adult (>18 years old)	Not Specified	Normal	HMDB	Sugimoto, M., Saruta, J., Matsuki, C. et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles. Metabolomics, 9, 454–463 (2013). https://doi.org/10.1007/s11306-012-0464-y
Human Saliva	Detected and Quantified	1.53	1.18			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto, M., Saruta, J., Matsuki, C. et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles. Metabolomics, 9, 454–463 (2013). https://doi.org/10.1007/s11306-012-0464-y
Human Saliva	Detected and Quantified	3.78	2.33			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto, M., Saruta, J., Matsuki, C. et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles. Metabolomics, 9, 454–463 (2013). https://doi.org/10.1007/s11306-012-0464-y
Human Saliva	Detected and Quantified	1.64	3.47			uM	Adult (>18 years old)	Male	Normal	HMDB	Sugimoto, M., Saruta, J., Matsuki, C. et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles. Metabolomics, 9, 454–463 (2013). https://doi.org/10.1007/s11306-012-0464-y
Human Saliva	Detected and Quantified	2.65	1.07			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto, M., Saruta, J., Matsuki, C. et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles. Metabolomics, 9, 454–463 (2013). https://doi.org/10.1007/s11306-012-0464-y
Human Urine	Detected and Quantified	28.849	8.834			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	32.164	9.964			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	39.88	30.024			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	24.882	26.016			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	29.893	15.594			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	4.2	1.97			umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified			8.9	17.12	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	David F. Putnam Composition and Concentrative Properties of Human Urine. NASA Contractor Report. July 1971
Human Urine	Detected and Quantified	875		150	1600	umol/mmol creatinine	Children (1-13 years old)	Both	2-Ketoglutarate Dehydrogenase Complex Deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	101		50	152	umol/mmol creatinine	Adult (>18 years old)	Both	2-Ketoglutarate dehydrogenase complex deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Blood	Detected and Quantified			17	37	uM	Children (1-13 years old)	Both	2-Ketoglutarate dehydrogenase complex deficiency	HMDB	7075624
Human Cerebrospinal fluid (csf)	Detected and Quantified			3.600	12.800	uM	Children (1-13 years old)	Both	2-Ketoglutarate dehydrogenase complex deficiency	HMDB	7075624
Human Urine	Detected and Quantified			30	1600	umol/mmol creatinine	Infant (0-1 year old)	Male	2-Ketoglutarate dehydrogenase complex deficiency	HMDB	1640293
Human Urine	Detected and Quantified			673.261	2970.0885	umol/mmol creatinine	Infant (0-1 year old)	Both	Amish lethal microcephaly	HMDB	12376931
Human Cellular cytoplasm	Detected and Quantified	150		144	156	uM	Adult (>18 years old)	Both	Anoxia	HMDB	8579834
Human Urine	Detected and Quantified	26.667				umol/mmol creatinine	Children (1 - 13 years old)	Male	D-2-Hydroxyglutaric aciduria	HMDB	6774165
Human Urine	Detected and Quantified	46.432				umol/mmol creatinine	Children (1 - 13 years old)	Male	D-2-Hydroxyglutaric aciduria	HMDB	6774165
Human Urine	Detected and Quantified	193.466				umol/mmol creatinine	Children (1 - 13 years old)	Male	D-2-Hydroxyglutaric aciduria	HMDB	6774165
Human Urine	Detected and Quantified	1374				umol/mmol creatinine	Newborn (0-30 days old)	Female	Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome	HMDB	17994565
Human Urine	Detected and Quantified	1119.294				umol/mmol creatinine	Infant (0-1 year old)	Male	Dihydrolipoamide Dehydrogenase Deficiency	HMDB	6688766
Human Urine	Detected and Quantified	1172.1				umol/mmol creatinine	Infant (0-1 year old)	Male	Fumarase deficiency	HMDB	24182348
Human Urine	Detected and Quantified			385.200	1367.300	umol/mmol creatinine	Infant (0-1 year old)	Male	Fumarase deficiency	HMDB	24182348
Human Urine	Detected and Quantified			100	166	umol/mmol creatinine	Adult (>18 years old)	Both	Hyperinsulinism-hyperammonemia syndrome	HMDB	14967154
Human Urine	Detected and Quantified			149	247	umol/mmol creatinine	Children (1-13 years old)	Female	Hyperinsulinism-hyperammonemia syndrome	HMDB	14967154
Human Urine	Detected and Quantified			15	107	umol/mmol creatinine	Children (1-13 years old)	Female	Hyperinsulinism-hyperammonemia syndrome	HMDB	14967154
Human Urine	Detected and Quantified	6224				umol/mmol creatinine	Children (1-13 years old)	Male	Lipoyltransferase 1 Deficiency	HMDB	24341803
Human Urine	Detected and Quantified	23.6				umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	HMDB	12383482
Human Cerebrospinal fluid (csf)	Detected and Quantified			2	16	uM	Not Specified	Not Specified	Normal	HMDB	7075624
Human Blood	Detected and Quantified			4	13	uM	Not Specified	Not Specified	Normal	HMDB	7075624
Human Urine	Detected and Quantified			7	11	umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	27012787
Human Urine	Detected and Quantified			12	20	umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	27012787
Human Urine	Detected and Quantified	<110				umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	HMDB	17994565
Human Urine	Detected and Quantified	<115				umol/mmol creatinine	Children (1-13 years old)	Female	Normal	HMDB	14967154
Human Urine	Detected and Quantified	<150				umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	HMDB	9732975
Human Urine	Detected and Quantified	<350				umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	24182348
Human Urine	Detected and Quantified	<60				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	1640293
Human Urine	Detected and Quantified	<75				umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	14967154
Human Urine	Detected and Quantified	<79				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	24341803
Human Urine	Detected and Quantified	<95				umol/mmol creatinine	Children (1-13 years old)	Female	Normal	HMDB	14967154
Human Urine	Detected and Quantified	> 0.658				umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	6321058
Human Urine	Detected and Quantified			0	25.3	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified	0.083	0.002			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	16460720
Human Urine	Detected and Quantified			1.548	39.467	umol/mmol creatinine	Children (1 - 13 years old)	Male	Normal	HMDB	6774165
Human Urine	Detected and Quantified	104.8		30.21	413.8	umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	HMDB	32340350
Human Urine	Detected and Quantified			14.8	64.7	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Urine	Detected and Quantified			15.477	154.773	umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	12376931
Human Urine	Detected and Quantified	150.5		10.0	836.3	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified			160	590	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	31506554
Human Cerebrospinal fluid (csf)	Detected and Quantified	2.0	2.0			uM	Adult (>18 years old)	Both	Normal	HMDB	8412012
Human Urine	Detected and Quantified			2.2	8.2	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	15992788
Human Urine	Detected and Quantified	2.87		0.18	14.3	umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	9869358
Human Urine	Detected and Quantified	2.98		0.42	18.3	umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	9869358
Human Urine	Detected and Quantified	21.4		4.9	123.8	umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	236.9		8.0	631.4	umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	24		4	74	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	2702744
Human Urine	Detected and Quantified	25.6		10.2	146.0	umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	3.4		1.4	12.5	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified			30.79	398.2	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	32340350
Human Cellular cytoplasm	Detected and Quantified	320		290	350	uM	Adult (>18 years old)	Both	Normal	HMDB	8579834
Human Urine	Detected and Quantified			4.3	36.3	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified	4.8		2.0	17.0	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Cerebrospinal fluid (csf)	Detected and Quantified	4.80		3.40	6.20	uM	Children (1-13 years old)	Both	Normal	HMDB	6704500
Human Breast milk	Detected and Quantified	41.0	16.1			uM	Adult (>18 years old)	Female	Normal	HMDB	24027187
Human Urine	Detected and Quantified	43.46	53.46			umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	2026685
Human Cerebrospinal fluid (csf)	Detected and Quantified	5	4			uM	Adult (>18 years old)	Both	Normal	HMDB	18502700
Human Blood	Detected and Quantified	7.0		0.0	23.0	uM	Adult (>18 years old)	Both	Normal	HMDB	8412012
Human Urine	Detected and Quantified			7.5	28.9	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Blood	Detected and Quantified	8.6	2.6			uM	Children (1-13 years old)	Both	Normal	HMDB	6704500
Human Urine	Detected and Quantified	90.83		45.64	331.1	umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	HMDB	32340350
Human Urine	Detected and Quantified	24.882	26.016			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	28.849	8.834			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	101.0		50.0	152.0	umol/mmol creatinine	Adult (>18 years old)	Both	2-Ketoglutarate dehydrogenase complex deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	875.0		150.0	1600.0	umol/mmol creatinine	Children (1-13 years old)	Both	2-Ketoglutarate Dehydrogenase Complex Deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	29.893	15.594			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	32.164	9.964			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified			8.90	17.12	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	David F. Putnam Composition and Concentrative Properties of Human Urine. NASA Contractor Report. July 1971
Human Cerebrospinal fluid (csf)	Detected and Quantified	2.1	0.7			uM	Children (1-13 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Blood	Detected and Quantified	20.7	2.5			uM	Newborn (0-30 days old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	4.2	1.97			umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Blood	Detected and Quantified	8.9	2.7			uM	Adult (>18 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Cerebrospinal fluid (csf)	Detected and Quantified	8.9	3.4			uM	Adult (>18 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Blood	Detected and Quantified	9.3	2.3			uM	Children (1-13 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Saliva	Detected and Quantified	1.53	1.18			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	1.64	3.47			uM	Adult (>18 years old)	Male	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	2.65	1.07			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	2.65	1.07			uM	Adult (>18 years old)	Not Specified	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	3.78	2.33			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	5.27	3.61			uM	Adult (>18 years old)	Not Specified	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Placenta	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Feces	Detected but not Quantified						Infant (0-1 year old)	Both	Normal	HMDB	22411374
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	22024767
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	22007635
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	22800120
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27065191
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27543620
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	25037050
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	32966057
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	32966057

External Links

HMDB ID

HMDB0000208

DrugBank ID

DB08845

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB003361

KNApSAcK ID

C00000769

Chemspider ID

KEGG Compound ID

C00026

BioCyc ID

2-KETOGLUTARATE

BiGG ID

33565

Wikipedia Link

Alpha-Ketoglutaric_acid

METLIN ID

5218

PubChem Compound

PDB ID

Not Available

ChEBI ID

30915

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

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. doi: 10.1002/bms.1200110105. [PubMed:6704500 ]
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. doi: 10.1203/00006450-197601000-00012. [PubMed:813176 ]
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Showing metabocard for Oxoglutaric acid (MMDBc0000067)

MOL for #<Metabolite:0x00007f7d1ceeb998>

3D MOL for #<Metabolite:0x00007f7d1ceeb998>

3D SDF for #<Metabolite:0x00007f7d1ceeb998>

3D-SDF for #<Metabolite:0x00007f7d1ceeb998>

PDB for #<Metabolite:0x00007f7d1ceeb998>

3D PDB for #<Metabolite:0x00007f7d1ceeb998>

SMILES for #<Metabolite:0x00007f7d1ceeb998>

INCHI for #<Metabolite:0x00007f7d1ceeb998>

Structure for #<Metabolite:0x00007f7d1ceeb998>

3D Structure for #<Metabolite:0x00007f7d1ceeb998>