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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-04-16 17:03:57 UTC
HMDB IDHMDB0000208
Secondary Accession Numbers
  • HMDB0002812
  • HMDB00208
  • HMDB02812
Metabolite Identification
Common NameOxoglutaric acid
DescriptionOxoglutaric 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 ).
Structure
Thumb
Synonyms
ValueSource
2-Ketoglutaric acidChEBI
alpha-Ketoglutaric acidChEBI
2-KetoglutarateGenerator
OxoglutarateGenerator
a-KetoglutarateGenerator
a-Ketoglutaric acidGenerator
alpha-KetoglutarateGenerator
α-ketoglutarateGenerator
α-ketoglutaric acidGenerator
2-oxo-1,5-PentanedioateHMDB
2-oxo-1,5-Pentanedioic acidHMDB
2-OxoglutarateHMDB
2-Oxoglutaric acidHMDB
2-OxopentanedioateHMDB
2-Oxopentanedioic acidHMDB
alpha-Ketoglutaric acid, calcium salt (2:1)MeSH
alpha-Ketoglutaric acid, sodium saltMeSH
Calcium alpha-ketoglutarateMeSH
alpha-Ketoglutaric acid, disodium saltMeSH
alpha-Ketoglutaric acid, monosodium saltMeSH
Calcium ketoglutarateMeSH
alpha-Ketoglutaric acid, diammonium saltMeSH
alpha-Ketoglutaric acid, dipotassium saltMeSH
alpha-Ketoglutaric acid, monopotassium saltMeSH
alpha-Ketoglutaric acid, potassium saltMeSH
alpha-OxoglutarateMeSH
Chemical FormulaC5H6O5
Average Molecular Weight146.0981
Monoisotopic Molecular Weight146.021523302
IUPAC Name2-oxopentanedioic acid
Traditional Nameoxoglutarate
CAS Registry Number328-50-7
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 KeyKPGXRSRHYNQIFN-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound 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.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassKeto acids and derivatives
Sub ClassGamma-keto acids and derivatives
Direct ParentGamma-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 FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

Industrial application:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point115.5 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility541.5 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility53.1 g/LALOGPS
logP-0.6ALOGPS
logP-0.11ChemAxon
logS-0.44ALOGPS
pKa (Strongest Acidic)2.66ChemAxon
pKa (Strongest Basic)-9.7ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area91.67 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity28.88 m³·mol⁻¹ChemAxon
Polarizability12.17 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-000b-3910000000-4097b7eb52c2910a4277View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0002-1910000000-3277227b9c4da4baa1f7View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS; 1 MEOX)splash10-00di-9300000000-974bce546caa4fbe6b0fView in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 2 TMS)splash10-000i-9710000000-d0aea11e947b042571f9View in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 2 TMS)splash10-052s-4920000000-bf7ed92c68fd4c8dbb6bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000b-3910000000-4097b7eb52c2910a4277View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-1910000000-3277227b9c4da4baa1f7View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-9300000000-974bce546caa4fbe6b0fView in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-000i-9710000000-d0aea11e947b042571f9View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-052s-4920000000-bf7ed92c68fd4c8dbb6bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000b-2900000000-e514f7afea2490bbe9edView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0kfx-9200000000-0ee77a39e9862659f8a7View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00di-9730000000-5e17bd8cfd944963c79fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)splash10-0zfr-6900000000-7fc343a243ce5d2a4f39View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)splash10-0a4i-9200000000-1a7af9946604baa8b3d2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)splash10-0a4l-9800000000-30495b2afdd3526854e7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-0002-0925200000-af33449c9638276466c2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-0a4i-9000000000-6e43f767847790112b65View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-03di-0900000000-4cacc41705b7b385e91eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-0udi-0900000000-1367eeeb6ad64c67a0aaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-000t-0915051100-91cda631ee54800322b8View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-0a4i-9000000000-4f842c3673925d29c402View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-0udi-0900000000-5e751351656f6eb62de3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-001i-0910000000-7729faa24733fbad77b7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-0002-0900000000-c3c62f83bff99f44f7abView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-0zfr-6900000000-b6d8379db3e3f1480b14View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0a4i-9300000000-4fde39bb93cde5a27f23View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-00i0-9000000000-4343832f3a55842423a9View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-004i-2900000000-5aa3d6d620db1d733a65View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0m30-7900000000-fc77f69ad43f915bf23eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a6r-9000000000-c597f1f5e416037aa82dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-004i-2900000000-5aa3d6d620db1d733a65View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0m30-7900000000-fc77f69ad43f915bf23eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a6r-9000000000-c597f1f5e416037aa82dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0002-1900000000-f2b463076a150a514f0cView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0f9t-8900000000-b2d3fa8d7ab01a220d9aView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-053r-9100000000-554bfbd6839bc376cba0View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0002-1900000000-f2b463076a150a514f0cView in MoNA
MSMass Spectrum (Electron Ionization)splash10-0fba-9200000000-4e367c66e94c51eb1a1bView in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
  • Endoplasmic reticulum
  • Peroxisome
Biospecimen Locations
  • Blood
  • Breast Milk
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue LocationNot Available
Pathways
NameSMPDB/PathwhizKEGG
2-aminoadipic 2-oxoadipic aciduriaThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
2-Hydroxyglutric Aciduria (D And L Form)ThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
2-ketoglutarate dehydrogenase complex deficiencyThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase DeficiencyThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencyThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not Quantified Adult (>18 years old)BothNormal details
BloodDetected and Quantified7.0 (0.0-23.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified8.6 +/- 2.6 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified9.3 +/- 2.3 uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified8.9 +/- 2.7 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified13-Apr uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified20.7 +/- 2.5 uMNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
details
BloodDetected but not Quantified Adult (>18 years old)Both
Normal
details
Breast MilkDetected and Quantified41.0 +/- 16.1 uMAdult (>18 years old)Female
Normal
details
Cellular CytoplasmDetected and Quantified320 (290-350) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified5 +/- 4 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2.1 +/- 0.7 uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified8.9 +/- 3.4 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified16-Feb uMNot SpecifiedNot SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2.0 +/- 2.0 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.80 (3.40-6.20) uMChildren (1-13 years old)BothNormal details
FecesDetected but not Quantified Infant (0-1 year old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected and Quantified5.27 +/- 3.61 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected and Quantified1.53 +/- 1.18 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified1.64 +/- 3.47 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified2.65 +/- 1.07 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified2.65 +/- 1.07 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified3.78 +/- 2.33 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
UrineDetected and Quantified<110 umol/mmol creatinineNewborn (0-30 days old)FemaleNormal details
UrineDetected and Quantified32.164 +/- 9.964 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified12-20 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified<150 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified2.87 (0.18-14.3) umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified2.98 (0.42-18.3) umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified23.6 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected and Quantified29.893 +/- 15.594 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified<60 umol/mmol creatinineNot SpecifiedNot Specified
Normal
details
UrineDetected and Quantified<75 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<95 umol/mmol creatinineChildren (1-13 years old)Female
Normal
details
UrineDetected and Quantified<115 umol/mmol creatinineChildren (1-13 years old)Female
Normal
details
UrineDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified<350 umol/mmol creatinineInfant (0-1 year old)MaleNormal details
UrineDetected and Quantified8.90-17.12 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified4.2 +/- 1.97 umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified24 (4-74) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified150.5 (10.0-836.3) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified236.9 (8.0-631.4) umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified21.4 (4.9-123.8) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified25.6 (10.2-146.0) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified15.477-154.773 umol/mmol creatinineInfant (0-1 year old)Both
Normal
details
UrineDetected and Quantified7-11 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified4.8 (2.0-17.0) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified2.2-8.2 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.548-39.467 umol/mmol creatinineChildren (1 - 13 years old)MaleNormal details
UrineDetected and Quantified43.46 +/- 53.46 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified<79 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified> 0.658 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified3.4 (1.4-12.5) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not Quantified Adult (>18 years old)Both
Schizophrenia
details
BloodDetected and Quantified17-37 uMChildren (1-13 years old)Both2-Ketoglutarate dehydrogenase complex deficiency details
BloodDetected but not Quantified Adult (>18 years old)Both
Schizophrenia
details
Cellular CytoplasmDetected and Quantified150 (144-156) uMAdult (>18 years old)BothAnoxia details
Cerebrospinal Fluid (CSF)Detected and Quantified3.600-12.800 uMChildren (1-13 years old)Both2-Ketoglutarate dehydrogenase complex deficiency details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
FecesDetected but not Quantified Adult (>18 years old)BothImmunoglobulin A nephropathy (IgAN) progressor details
UrineDetected and Quantified1374 umol/mmol creatinineNewborn (0-30 days old)FemaleDeafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome details
UrineDetected and Quantified28.849 +/- 8.834 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified39.88 +/- 30.024 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
UrineDetected and Quantified30-1600 umol/mmol creatinineInfant (0-1 year old)Male
2-Ketoglutarate dehydrogenase complex deficiency
details
UrineDetected and Quantified24.882 +/- 26.016 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected but not Quantified Adult (>18 years old)Both
Schizophrenia
details
UrineDetected and Quantified100-166 umol/mmol creatinineAdult (>18 years old)BothHyperinsulinism-hyperammonemia syndrome details
UrineDetected and Quantified149-247 umol/mmol creatinineChildren (1-13 years old)Female
Hyperinsulinism-hyperammonemia syndrome
details
UrineDetected and Quantified15-107 umol/mmol creatinineChildren (1-13 years old)Female
Hyperinsulinism-hyperammonemia syndrome
details
UrineDetected and Quantified1172.1 umol/mmol creatinineInfant (0-1 year old)MaleFumarase deficiency details
UrineDetected and Quantified385.200-1367.300 umol/mmol creatinineInfant (0-1 year old)MaleFumarase deficiency details
UrineDetected and Quantified673.261-2970.0885 umol/mmol creatinineInfant (0-1 year old)Both
Amish lethal microcephaly
details
UrineDetected and Quantified1119.294 umol/mmol creatinineInfant (0-1 year old)MaleDihydrolipoamide Dehydrogenase Deficiency details
UrineDetected and Quantified26.667 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified46.432 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified193.466 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified6224 umol/mmol creatinineChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
UrineDetected and Quantified101.0 (50.0-152.0) umol/mmol creatinineAdult (>18 years old)Both2-Ketoglutarate dehydrogenase complex deficiency
    • MetaGene: Metabol...
details
UrineDetected and Quantified875.0 (150.0-1600.0) umol/mmol creatinineChildren (1-13 years old)Both2-Ketoglutarate Dehydrogenase Complex Deficiency
    • MetaGene: Metabol...
details
Associated Disorders and Diseases
Disease References
2-Ketoglutarate dehydrogenase complex deficiency
  1. Bonnefont JP, Chretien D, Rustin P, Robinson B, Vassault A, Aupetit J, Charpentier C, Rabier D, Saudubray JM, Munnich A: Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis. J Pediatr. 1992 Aug;121(2):255-8. [PubMed:1640293 ]
  2. Kohlschutter A, Behbehani A, Langenbeck U, Albani M, Heidemann P, Hoffmann G, Kleineke J, Lehnert W, Wendel U: A familial progressive neurodegenerative disease with 2-oxoglutaric aciduria. Eur J Pediatr. 1982 Feb;138(1):32-7. [PubMed:7075624 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Anoxia
  1. 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:8579834 ]
D-2-hydroxyglutaric aciduria
  1. Chalmers RA, Lawson AM, Watts RW, Tavill AS, Kamerling JP, Hey E, Ogilvie D: D-2-hydroxyglutaric aciduria: case report and biochemical studies. J Inherit Metab Dis. 1980;3(1):11-5. [PubMed:6774165 ]
Schizophrenia
  1. Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]
  2. Xuan J, Pan G, Qiu Y, Yang L, Su M, Liu Y, Chen J, Feng G, Fang Y, Jia W, Xing Q, He L: Metabolomic profiling to identify potential serum biomarkers for schizophrenia and risperidone action. J Proteome Res. 2011 Dec 2;10(12):5433-43. doi: 10.1021/pr2006796. Epub 2011 Nov 8. [PubMed:22007635 ]
  3. Cai HL, Li HD, Yan XZ, Sun B, Zhang Q, Yan M, Zhang WY, Jiang P, Zhu RH, Liu YP, Fang PF, Xu P, Yuan HY, Zhang XH, Hu L, Yang W, Ye HS: Metabolomic analysis of biochemical changes in the plasma and urine of first-episode neuroleptic-naive schizophrenia patients after treatment with risperidone. J Proteome Res. 2012 Aug 3;11(8):4338-50. doi: 10.1021/pr300459d. Epub 2012 Jul 26. [PubMed:22800120 ]
Fumarase deficiency
  1. Tregoning S, Salter W, Thorburn DR, Durkie M, Panayi M, Wu JY, Easterbrook A, Coman DJ: Fumarase deficiency in dichorionic diamniotic twins. Twin Res Hum Genet. 2013 Dec;16(6):1117-20. doi: 10.1017/thg.2013.72. Epub 2013 Nov 4. [PubMed:24182348 ]
Hyperinsulinism-hyperammonemia syndrome
  1. Meissner T, Mayatepek E, Kinner M, Santer R: Urinary alpha-ketoglutarate is elevated in patients with hyperinsulinism-hyperammonemia syndrome. Clin Chim Acta. 2004 Mar;341(1-2):23-6. doi: 10.1016/j.cccn.2003.10.023. [PubMed:14967154 ]
Amish lethal microcephaly
  1. 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:12376931 ]
Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome
  1. 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:17994565 ]
Dihydrolipoamide Dehydrogenase Deficiency
  1. 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:6688766 ]
Lipoyltransferase 1 Deficiency
  1. 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:24341803 ]
Associated OMIM IDs
  • 181500 (Schizophrenia)
  • 203740 (2-Ketoglutarate dehydrogenase complex deficiency)
  • 600721 (D-2-hydroxyglutaric aciduria)
  • 606762 (Hyperinsulinism-hyperammonemia syndrome)
  • 606812 (Fumarase deficiency)
  • 607196 (Amish lethal microcephaly)
  • 220500 (Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome)
  • 246900 (Dihydrolipoamide Dehydrogenase Deficiency)
  • 616299 (Lipoyltransferase 1 Deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB003361
KNApSAcK IDC00000769
Chemspider ID50
KEGG Compound IDC00026
BioCyc ID2-KETOGLUTARATE
BiGG ID33565
Wikipedia LinkAlpha-Ketoglutaric_acid
METLIN ID5218
PubChem Compound51
PDB IDAKG
ChEBI ID30915
References
Synthesis ReferenceTanaka, Katsunobu; kimura, Kazu; Yamaguchi, Ken. Fermentative production of a-oxoglutaric acid. U.S. (1973), 4 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. 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:7762816 ]
  2. 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:8257730 ]
  3. 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:9869358 ]
  4. 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:8087979 ]
  5. 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:12829005 ]
  6. 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:8579834 ]
  7. 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:6321058 ]
  8. 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:813176 ]
  9. 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:6704500 ]
  10. 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:12383482 ]
  11. 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:10333126 ]
  12. Kelley RI: Octenylsuccinic aciduria in children fed protein-hydrolysate formulas containing modified cornstarch. Pediatr Res. 1991 Dec;30(6):564-9. [PubMed:1805153 ]
  13. 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:9732975 ]
  14. 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:26759695 ]
  15. 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:24828042 ]
  16. 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:26420908 ]
  17. De Vivo DC: The expanding clinical spectrum of mitochondrial diseases. Brain Dev. 1993 Jan-Feb;15(1):1-22. [PubMed:8338207 ]

Only showing the first 10 proteins. There are 68 proteins in total.

Enzymes

General function:
Involved in magnesium ion binding
Specific function:
Not Available
Gene Name:
IDH1
Uniprot ID:
O75874
Molecular weight:
46659.005
Reactions
Isocitric acid + NADP → Oxoglutaric acid + CO(2) + NADPHdetails
Isocitric acid + NADP → Oxoglutaric acid + Carbon dioxide + NADPH + Hydrogen Iondetails
Oxalosuccinic acid → Oxoglutaric acid + Carbon dioxidedetails
General function:
Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
Specific function:
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
OGDH
Uniprot ID:
Q02218
Molecular weight:
48179.59
Reactions
Oxoglutaric acid + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine → [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO(2)details
Oxoglutaric acid + Thiamine pyrophosphate → 3-Carboxy-1-hydroxypropylthiamine diphosphate + Carbon dioxidedetails
General function:
Involved in oxidoreductase activity
Specific function:
May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate (By similarity).
Gene Name:
GLUD1
Uniprot ID:
P00367
Molecular weight:
61397.315
Reactions
L-Glutamic acid + Water + NAD(P)(+) → Oxoglutaric acid + Ammonia + NAD(P)Hdetails
L-Glutamic acid + NAD + Water → Oxoglutaric acid + Ammonia + NADH + Hydrogen Iondetails
L-Glutamic acid + NADP + Water → Oxoglutaric acid + Ammonia + NADPH + Hydrogen Iondetails
General function:
Involved in transferase activity, transferring nitrogenous groups
Specific function:
Plays a key role in amino acid metabolism (By similarity).
Gene Name:
GOT1
Uniprot ID:
P17174
Molecular weight:
46247.14
Reactions
L-Aspartic acid + Oxoglutaric acid → Oxalacetic acid + L-Glutamic aciddetails
L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
L-Tyrosine + Oxoglutaric acid → 4-Hydroxyphenylpyruvic acid + L-Glutamic aciddetails
L-Cysteine + Oxoglutaric acid → 3-Mercaptopyruvic acid + DL-Glutamatedetails
Cysteic acid + Oxoglutaric acid → 3-Sulfopyruvic acid + L-Glutamic aciddetails
3-Sulfinoalanine + Oxoglutaric acid → B-Sulfinyl pyruvate + L-Glutamic aciddetails
4-Hydroxy-L-glutamic acid + Oxoglutaric acid → D-4-Hydroxy-2-oxoglutarate + L-Glutamic aciddetails
General function:
Involved in transporter activity
Specific function:
Catalyzes the transport of 2-oxoglutarate across the inner mitochondrial membrane in an electroneutral exchange for malate or other dicarboxylic acids, and plays an important role in several metabolic processes, including the malate-aspartate shuttle, the oxoglutarate/isocitrate shuttle, in gluconeogenesis from lactate, and in nitrogen metabolism.
Gene Name:
SLC25A11
Uniprot ID:
Q02978
Molecular weight:
34061.405
General function:
Involved in magnesium ion binding
Specific function:
Not Available
Gene Name:
IDH3A
Uniprot ID:
P50213
Molecular weight:
39591.365
Reactions
Isocitric acid + NAD → Oxoglutaric acid + CO(2) + NADHdetails
Isocitric acid + NAD → Oxoglutaric acid + Carbon dioxide + NADH + Hydrogen Iondetails
General function:
Involved in magnesium ion binding
Specific function:
Not Available
Gene Name:
IDH3G
Uniprot ID:
P51553
Molecular weight:
42793.97
Reactions
Isocitric acid + NAD → Oxoglutaric acid + CO(2) + NADHdetails
Isocitric acid + NAD → Oxoglutaric acid + Carbon dioxide + NADH + Hydrogen Iondetails
General function:
Involved in 2-hydroxyglutarate dehydrogenase activity
Specific function:
Not Available
Gene Name:
L2HGDH
Uniprot ID:
Q9H9P8
Molecular weight:
50315.58
Reactions
L-2-Hydroxyglutaric acid + acceptor → Oxoglutaric acid + reduced acceptordetails
2-Hydroxyglutarate + FAD → Oxoglutaric acid + FADHdetails
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). D,L-3-hydroxyisobutyrate and L-3-hydroxybutyrate (L-3-OHB) are also substrates for HOT with 10-fold lower activities.
Gene Name:
ADHFE1
Uniprot ID:
Q8IWW8
Molecular weight:
50307.42
Reactions
(S)-3-Hydroxybutyric acid + Oxoglutaric acid → Acetoacetic acid + D-2-Hydroxyglutaric aciddetails
4-Hydroxybutyric acid + Oxoglutaric acid → Succinic acid semialdehyde + D-2-Hydroxyglutaric aciddetails
General function:
Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
Specific function:
Not Available
Gene Name:
OGDHL
Uniprot ID:
Q9ULD0
Molecular weight:
Not Available
Reactions
Oxoglutaric acid + Thiamine pyrophosphate → 3-Carboxy-1-hydroxypropylthiamine diphosphate + Carbon dioxidedetails

Only showing the first 10 proteins. There are 68 proteins in total.