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Record Information
Version1.0
Creation Date2016-09-30 22:26:43 UTC
Update Date2020-05-21 16:28:27 UTC
BMDB IDBMDB0000208
Secondary Accession Numbers
  • BMDB00208
Metabolite Identification
Common NameOxoglutaric acid
DescriptionOxoglutaric acid, also known as oxoglutarate or alpha-ketoglutarate, 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. Oxoglutaric acid exists as a solid, possibly soluble (in water), and an extremely weak basic (essentially neutral) compound (based on its pKa) molecule. Oxoglutaric acid exists in all living species, ranging from bacteria to humans. Oxoglutaric acid is a potentially toxic compound. Oxoglutaric acid has been found to be associated with several diseases known as schizophrenia, eosinophilic esophagitis, amish lethal microcephaly, and anoxia; also oxoglutaric acid has been linked to the inborn metabolic disorders including d-2-hydroxyglutaric aciduria.
Structure
Thumb
Synonyms
ValueSource
2-Ketoglutaric acidChEBI
alpha-Ketoglutaric acidChEBI
2-KetoglutarateGenerator
a-KetoglutarateGenerator
a-Ketoglutaric acidGenerator
alpha-KetoglutarateGenerator
Α-ketoglutarateGenerator
Α-ketoglutaric acidGenerator
OxoglutarateGenerator
2-oxo-1,5-PentanedioateHMDB
2-oxo-1,5-Pentanedioic acidHMDB
2-OxoglutarateHMDB
2-Oxoglutaric acidHMDB
2-OxopentanedioateHMDB
2-Oxopentanedioic acidHMDB
alpha-OxoglutarateHMDB
Oxogluric acidHMDB
2 Oxoglutaric acidHMDB
Ketoglutaric acidHMDB
alpha Ketoglutaric acidHMDB
2 Ketoglutaric acidHMDB
alpha KetoglutarateHMDB
alpha OxoglutarateHMDB
2 KetoglutarateHMDB
2 OxoglutarateHMDB
alpha-Oxoglutaric acidHMDB
alpha-Oxopentanedioic acidHMDB
alpha-Keto-glutaric acidHMDB
Α-oxoglutaric acidHMDB
Α-oxopentanedioic acidHMDB
Α-keto-glutaric acidHMDB
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
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.
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
StatusDetected and Quantified
Origin
  • Endogenous
  • Exogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locations
  • Cytoplasm
  • Endoplasmic reticulum
  • Mitochondria
  • Peroxisome
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
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
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
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
  • Cytoplasm
  • Endoplasmic reticulum
  • Mitochondria
  • Peroxisome
Biospecimen Locations
  • Blood
  • Mammary Gland
  • Milk
  • Placenta
  • Ruminal Fluid
  • Semen
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
Mammary GlandDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
Mammary GlandDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
MilkDetected and Quantified117.93 +/- 17.22 uMNot SpecifiedNot Specified
Normal
details
MilkDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
MilkDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
MilkDetected and Quantified104 +/- 7 uMNot SpecifiedNot Specified
Normal
details
MilkDetected and Quantified277.1 uMNot SpecifiedNot Specified
Normal
details
MilkDetected and Quantified112 +/- 7 uMNot SpecifiedNot Specified
Normal
details
MilkDetected and Quantified103 +/- 3 uMNot SpecifiedNot Specified
Normal
details
MilkDetected and Quantified109 +/- 6 uMNot SpecifiedNot Specified
Normal
details
MilkDetected and Quantified37 - 156 uMNot SpecifiedNot SpecifiedNormal details
MilkDetected and Quantified70 - 156 uMNot SpecifiedNot SpecifiedNormal details
MilkDetected and Quantified240 uMNot SpecifiedNot Specified
Normal
details
MilkDetected and Quantified90 uMNot SpecifiedNot Specified
Normal
details
MilkDetected and Quantified102 uMNot SpecifiedNot Specified
Normal
details
MilkDetected and Quantified116.48 +/- 18.59 uMNot SpecifiedNot Specified
Normal
details
MilkDetected and Quantified111.39 +/- 15.62 uMNot SpecifiedNot Specified
Normal
details
PlacentaExpected but not QuantifiedNot ApplicableNot SpecifiedNot SpecifiedNormal
  • Not Applicable
details
Ruminal FluidDetected and Quantified40+/-24 uMNot SpecifiedNot Specified
Normal
    • The Bovine Metabo...
details
SemenDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Treated with growth-promoting hormones
details
BloodDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Sub/clinical ketosis
details
HMDB IDHMDB0000208
DrugBank IDDB08845
Phenol Explorer Compound IDNot Available
FooDB IDFDB003361
KNApSAcK IDC00000769
Chemspider ID50
KEGG Compound IDC00026
BioCyc ID2-KETOGLUTARATE
BiGG ID33565
Wikipedia LinkAlpha-Ketoglutaric_acid
METLIN ID5218
PubChem Compound51
PDB IDNot Available
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)Not Available
General References
  1. Faulkner A, Pollock HT: Changes in the concentration of metabolites in milk from cows fed on diets supplemented with soyabean oil or fatty acids. J Dairy Res. 1989 May;56(2):179-83. [PubMed:2760296 ]
  2. Hurtaud C, Rulquin H, Verite R: Effects of graded duodenal infusions of glucose on yield and composition of milk from dairy cows. 1. Diets based on corn silage. J Dairy Sci. 1998 Dec;81(12):3239-47. doi: 10.3168/jds.S0022-0302(98)75888-6. [PubMed:9891269 ]
  3. Hurtaud C, Lemosquet S, Rulquin H: Effect of graded duodenal infusions of glucose on yield and composition of milk from dairy cows. 2. Diets based on grass silage. J Dairy Sci. 2000 Dec;83(12):2952-62. doi: 10.3168/jds.S0022-0302(00)75195-2. [PubMed:11132867 ]
  4. Klein MS, Buttchereit N, Miemczyk SP, Immervoll AK, Louis C, Wiedemann S, Junge W, Thaller G, Oefner PJ, Gronwald W: NMR metabolomic analysis of dairy cows reveals milk glycerophosphocholine to phosphocholine ratio as prognostic biomarker for risk of ketosis. J Proteome Res. 2012 Feb 3;11(2):1373-81. doi: 10.1021/pr201017n. Epub 2011 Dec 9. [PubMed:22098372 ]
  5. Melzer N, Wittenburg D, Hartwig S, Jakubowski S, Kesting U, Willmitzer L, Lisec J, Reinsch N, Repsilber D: Investigating associations between milk metabolite profiles and milk traits of Holstein cows. J Dairy Sci. 2013 Mar;96(3):1521-34. doi: 10.3168/jds.2012-5743. [PubMed:23438684 ]
  6. Buitenhuis AJ, Sundekilde UK, Poulsen NA, Bertram HC, Larsen LB, Sorensen P: Estimation of genetic parameters and detection of quantitative trait loci for metabolites in Danish Holstein milk. J Dairy Sci. 2013 May;96(5):3285-95. doi: 10.3168/jds.2012-5914. Epub 2013 Mar 15. [PubMed:23497994 ]
  7. O'Callaghan TF, Vazquez-Fresno R, Serra-Cayuela A, Dong E, Mandal R, Hennessy D, McAuliffe S, Dillon P, Wishart DS, Stanton C, Ross RP: Pasture Feeding Changes the Bovine Rumen and Milk Metabolome. Metabolites. 2018 Apr 6;8(2). pii: metabo8020027. doi: 10.3390/metabo8020027. [PubMed:29642378 ]
  8. Antunes-Fernandes EC, van Gastelen S, Dijkstra J, Hettinga KA, Vervoort J: Milk metabolome relates enteric methane emission to milk synthesis and energy metabolism pathways. J Dairy Sci. 2016 Aug;99(8):6251-6262. doi: 10.3168/jds.2015-10248. Epub 2016 May 26. [PubMed:27236769 ]
  9. Kurt J. Boudonck, Matthew W. Mitchell, Jacob Wulff and John A. Ryals (2009). Kurt J. Boudonck, Matthew W. Mitchell, Jacob Wulff and John A. Ryals. Characterization of the biochemical variability of bovine milk using metabolomics. Metabolomics (2009) 5:375?386. Metabolomics.
  10. A. Foroutan et al. (2019). A. Foroutan et al. The Chemical Composition of Commercial Cow's Milk (in preparation). Journal of Agricultural and Food Chemistry.

Enzymes

General function:
Amino acid transport and metabolism
Specific function:
Not Available
Gene Name:
BCAT1
Uniprot ID:
A4IFQ7
Molecular weight:
43185.0
General function:
Amino acid transport and metabolism
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids (By similarity).
Gene Name:
BCAT2
Uniprot ID:
Q5EA40
Molecular weight:
44648.0
Reactions
L-Valine + Oxoglutaric acid → Alpha-ketoisovaleric acid + L-Glutamic aciddetails
L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic aciddetails
L-Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + L-Glutamic aciddetails
General function:
Amino acid transport and metabolism
Specific function:
Not Available
Gene Name:
BCAT2
Uniprot ID:
Q0V8J6
Molecular weight:
44274.0
General function:
Amino acid transport and metabolism
Specific function:
Not Available
Gene Name:
BCAT2
Uniprot ID:
Q5E9U7
Molecular weight:
40447.0
General function:
Transcription
Specific function:
Transaminase with broad substrate specificity. Has transaminase activity towards aminoadipate, kynurenine, methionine and glutamate. Shows activity also towards tryptophan, aspartate and hydroxykynurenine. Accepts a variety of oxo-acids as amino-group acceptors, with a preference for 2-oxoglutarate, 2-oxocaproic acid, phenylpyruvate and alpha-oxo-gamma-methiol butyric acid. Can also use glyoxylate as amino-group acceptor (in vitro) (By similarity).
Gene Name:
AADAT
Uniprot ID:
Q5E9N4
Molecular weight:
47901.0
Reactions
Aminoadipic acid + Oxoglutaric acid → Oxoadipic acid + L-Glutamic aciddetails
L-Kynurenine + Oxoglutaric acid → 4-(2-Aminophenyl)-2,4-dioxobutanoic acid + L-Glutamic aciddetails
L-3-Hydroxykynurenine + Oxoglutaric acid → 4-(2-Amino-3-hydroxyphenyl)-2,4-dioxobutanoic acid + L-Glutamic aciddetails
General function:
Involved in 2-hydroxyglutarate dehydrogenase activity
Specific function:
Not Available
Gene Name:
L2HGDH
Uniprot ID:
A7MBI3
Molecular weight:
51022.0
General function:
Energy production and conversion
Specific function:
Plays a role in intermediary metabolism and energy production. It may tightly associate or interact with the pyruvate dehydrogenase complex.
Gene Name:
IDH2
Uniprot ID:
Q04467
Molecular weight:
50739.0
General function:
Energy production and conversion
Specific function:
Regulatory subunit which plays a role in the allosteric regulation of the enzyme catalyzing the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers.
Gene Name:
IDH3G
Uniprot ID:
Q58CP0
Molecular weight:
42863.0
General function:
Energy production and conversion
Specific function:
Plays a structural role to facilitate the assembly and ensure the full activity of the enzyme catalyzing the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers.
Gene Name:
IDH3B
Uniprot ID:
O77784
Molecular weight:
42497.0
General function:
Energy production and conversion
Specific function:
May act as a corneal epithelial crystallin and may be involved in maintaining corneal epithelial transparency.
Gene Name:
IDH1
Uniprot ID:
Q9XSG3
Molecular weight:
46785.0
Reactions
Isocitric acid + NADP → Oxoglutaric acid + NADPH + Carbon dioxidedetails
General function:
Energy production and conversion
Specific function:
Catalytic subunit of the enzyme which catalyzes the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers.
Gene Name:
IDH3A
Uniprot ID:
P41563
Molecular weight:
39668.0
General function:
Amino acid transport and metabolism
Specific function:
Biosynthesis of L-glutamate from L-aspartate or L-cysteine. Important regulator of levels of glutamate, the major excitatory neurotransmitter of the vertebrate central nervous system. Acts as a scavenger of glutamate in brain neuroprotection. The aspartate aminotransferase activity is involved in hepatic glucose synthesis during development and in adipocyte glyceroneogenesis. Using L-cysteine as substrate, regulates levels of mercaptopyruvate, an important source of hydrogen sulfide. Mercaptopyruvate is converted into H(2)S via the action of 3-mercaptopyruvate sulfurtransferase (3MST). Hydrogen sulfide is an important synaptic modulator and neuroprotectant in the brain (By similarity).
Gene Name:
GOT1
Uniprot ID:
P33097
Molecular weight:
46399.0
Reactions
3-Sulfinoalanine + Oxoglutaric acid → 3-Sulfinylpyruvic acid + L-Glutamic aciddetails
L-Cysteine + Oxoglutaric acid → 3-Mercaptopyruvic acid + L-Glutamic aciddetails
L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
Oxoglutaric acid + L-Aspartic acid → Oxalacetic acid + L-Glutamic aciddetails
General function:
Posttranslational modification, protein turnover, chaperones
Specific function:
Specifically hydroxylates an Asp or Asn residue in certain epidermal growth factor-like (EGF) domains of a number of proteins.
Gene Name:
ASPH
Uniprot ID:
Q28056
Molecular weight:
84999.0
General function:
Amino acid transport and metabolism
Specific function:
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids (By similarity).
Gene Name:
GOT2
Uniprot ID:
P12344
Molecular weight:
47514.0
Reactions
L-Aspartic acid + Oxoglutaric acid → Oxalacetic acid + L-Glutamic aciddetails
General function:
Amino acid transport and metabolism
Specific function:
Not Available
Gene Name:
GOT1L1
Uniprot ID:
Q2T9S8
Molecular weight:
45918.0
General function:
Energy production and conversion
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). L-3-hydroxybutyrate (L-3-OHB) is also a substrate for HOT when using 2-KG as hydrogen acceptor, resulting in the formation of D-2-HG (By similarity).
Gene Name:
ADHFE1
Uniprot ID:
A6QP15
Molecular weight:
50343.0
General function:
Amino acid transport and metabolism
Specific function:
Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine (By similarity).
Gene Name:
ABAT
Uniprot ID:
Q9BGI0
Molecular weight:
56731.0
Reactions
Beta-Alanine + Oxoglutaric acid → Malonic semialdehyde + L-Glutamic aciddetails
Gamma-Aminobutyric acid + Oxoglutaric acid → Succinic acid semialdehyde + L-Glutamic aciddetails
(S)-b-aminoisobutyric acid + Oxoglutaric acid → (S)-Methylmalonic acid semialdehyde + L-Glutamic aciddetails
General function:
Secondary metabolites biosynthesis, transport and catabolism
Specific function:
Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).
Gene Name:
TMLHE
Uniprot ID:
Q0VC74
Molecular weight:
49837.0
Reactions
N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen → 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine + Succinic acid + Carbon dioxidedetails
3-Dehydroxycarnitine + Oxoglutaric acid + Oxygen → L-Carnitine + Succinic acid + Carbon dioxidedetails
General function:
Amino acid transport and metabolism
Specific function:
Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-oxoglutarate reductase and saccharopine dehydrogenase activity, respectively (By similarity).
Gene Name:
AASS
Uniprot ID:
A8E657
Molecular weight:
102084.0
Reactions
L-Lysine + NADPH + Oxoglutaric acid → Saccharopine + NADP + Waterdetails
General function:
Involved in iron ion binding
Specific function:
Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Gene Name:
P4HA1
Uniprot ID:
Q1RMU3
Molecular weight:
61010.0
General function:
Involved in iron ion binding
Specific function:
Part of a complex composed of PLOD1, P3H3 and P3H4 that catalyzes hydroxylation of lysine residues in collagen alpha chains and is required for normal assembly and cross-linkling of collagen fibrils (By similarity). Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens (By similarity). These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links (By similarity).
Gene Name:
PLOD1
Uniprot ID:
O77588
Molecular weight:
83487.0
General function:
Involved in iron ion binding
Specific function:
Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
Gene Name:
PHYH
Uniprot ID:
O18778
Molecular weight:
38770.0
Reactions
Phytanoyl-CoA + Oxoglutaric acid + Oxygen → 2-Hydroxyphytanoyl-CoA + Succinic acid + Carbon dioxidedetails
General function:
Involved in iron ion binding
Specific function:
Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Gene Name:
P4HA3
Uniprot ID:
Q75UG4
Molecular weight:
61023.0
Reactions
L-Proline + Oxoglutaric acid + Oxygen → Hydroxyproline + Succinic acid + Carbon dioxidedetails
General function:
Energy production and conversion
Specific function:
Catalyzes the oxidation of D-2-hydroxyglutarate to alpha-ketoglutarate.
Gene Name:
D2HGDH
Uniprot ID:
Q1JPD3
Molecular weight:
59056.0
General function:
Coenzyme transport and metabolism
Specific function:
Not Available
Gene Name:
PSAT1
Uniprot ID:
A6QR28
Molecular weight:
40529.0
Reactions
Phosphohydroxypyruvic acid + L-Glutamic acid → Phosphoserine + Oxoglutaric aciddetails
General function:
Replication, recombination and repair
Specific function:
Mitochondrial glutamate dehydrogenase that converts L-glutamate into alpha-ketoglutarate. Plays a key role in glutamine anaplerosis by producing alpha-ketoglutarate, an important intermediate in the tricarboxylic acid cycle. May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate.
Gene Name:
GLUD1
Uniprot ID:
P00366
Molecular weight:
61512.0
Reactions
L-Glutamic acid + Water + NAD → Oxoglutaric acid + Ammonia + NADHdetails
General function:
Amino acid transport and metabolism
Specific function:
Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate. Participates in cellular nitrogen metabolism and also in liver gluconeogenesis starting with precursors transported from skeletal muscles (By similarity).
Gene Name:
GPT
Uniprot ID:
A4IFH5
Molecular weight:
55275.0
General function:
Amino acid transport and metabolism
Specific function:
Transaminase involved in tyrosine breakdown. Converts tyrosine to p-hydroxyphenylpyruvate. Can catalyze the reverse reaction, using glutamic acid, with 2-oxoglutarate as cosubstrate (in vitro). Has much lower affinity and transaminase activity for phenylalanine (By similarity).
Gene Name:
TAT
Uniprot ID:
Q58CZ9
Molecular weight:
49691.0
Reactions
L-Tyrosine + Oxoglutaric acid → 4-Hydroxyphenylpyruvic acid + L-Glutamic aciddetails
L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
General function:
Amino acid transport and metabolism
Specific function:
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). May catalyze the beta-elimination of S-conjugates and Se-conjugates of L-(seleno)cysteine, resulting in the cleavage of the C-S or C-Se bond (By similarity). Has transaminase activity towards L-kynurenine, tryptophan, phenylalanine, serine, cysteine, methionine, histidine, glutamine and asparagine with glyoxylate as an amino group acceptor (in vitro). Has lower activity with 2-oxoglutarate as amino group acceptor (in vitro) (By similarity).
Gene Name:
KYAT3
Uniprot ID:
Q0P5G4
Molecular weight:
51472.0
General function:
Coenzyme transport and metabolism
Specific function:
Catalyzes the reversible oxidation of 3-phospho-D-glycerate to 3-phosphonooxypyruvate, the first step of the phosphorylated L-serine biosynthesis pathway. Also catalyzes the reversible oxidation of 2-hydroxyglutarate to 2-oxoglutarate and the reversible oxidation of (S)-malate to oxaloacetate.
Gene Name:
PHGDH
Uniprot ID:
Q5EAD2
Molecular weight:
56452.0
General function:
Energy production and conversion
Specific function:
2-oxoglutarate dehydrogenase (E1) component of the 2-oxoglutarate dehydrogenase complex, which mediates the decarboxylation of alpha-ketoglutarate. The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). The 2-oxoglutarate dehydrogenase complex is mainly active in the mitochondrion. A fraction of the 2-oxoglutarate dehydrogenase complex also localizes in the nucleus and is required for lysine succinylation of histones: associates with KAT2A on chromatin and provides succinyl-CoA to histone succinyltransferase KAT2A.
Gene Name:
OGDH
Uniprot ID:
Q148N0
Molecular weight:
115808.0
General function:
Amino acid transport and metabolism
Specific function:
Not Available
Gene Name:
OAT
Uniprot ID:
Q3ZCF5
Molecular weight:
48075.0
Reactions
Ornithine + Oxoglutaric acid → L-Glutamic-gamma-semialdehyde + L-Glutamic aciddetails
General function:
Involved in binding
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. Maintains mitochondrial fusion and fission events, and the organization and morphology of cristae. Involved in the regulation of apoptosis.
Gene Name:
SLC25A11
Uniprot ID:
P22292
Molecular weight:
34172.0
General function:
Involved in binding
Specific function:
Not Available
Gene Name:
SLC25A11
Uniprot ID:
A5D954
Molecular weight:
33899.0
General function:
Not Available
Specific function:
Not Available
Gene Name:
EARS2
Uniprot ID:
G3N260
Molecular weight:
58494.0
Reactions
Oxoglutaric acid + Adenosine triphosphate + tRNA(Glu) → L-Glutamyl-tRNA(Glu) + Adenosine monophosphate + Pyrophosphatedetails